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nt's exercise intolerance had disappeared, and she was now more physically active. Postoperative bar removal imaging showed an acceptable correction of the deformity (see Figs. 3 and 4 ). During follow-up, the patient is content with the aesthetic result of the procedure; also the patient can carry out normal exercise. Fig. 3 Postoperative results X-ray. ( A ) Anteroposterior (AP) chest X-ray after Nuss procedure. ( B ) Lateral chest X-ray after Nuss procedure. (c) AP chest X-ray after Nuss bar removal 2 years after placement. Fig. 4 Postoperative image of patient. ( A ) Postoperative oblique/lateral view of patient's chest. ( B ) Postoperative anteroposterior view of patient's chest. A complete correction of the anterior chest wall deformity can be observed. Table 1 Spirometry parameters: preoperative versus postoperative Observed/expected Preoperative Postoperative FEV1 74.4% 81.4% FVC 80.9% 84.6% FEV1/FVC ratio 5.8% 2.3% MMEF 75/25 48% 56.3% Abbreviations: FEV1, forced expiratory volume in 1 second; FVC, forced vital capacity; MMEF 75/25, maximal midexpiratory flow. Discussion

Fig. 4 Postoperative image of patient. ( A ) Postoperative oblique/lateral view of patient's chest. ( B ) Postoperative anteroposterior view of patient's chest. A complete correction of the anterior chest wall deformity can be observed. Table 1 Spirometry parameters: preoperative versus postoperative Observed/expected Preoperative Postoperative FEV1 74.4% 81.4% FVC 80.9% 84.6% FEV1/FVC ratio 5.8% 2.3% MMEF 75/25 48% 56.3% Abbreviations: FEV1, forced expiratory volume in 1 second; FVC, forced vital capacity; MMEF 75/25, maximal midexpiratory flow. Discussion Since 1998, when Nuss published the paper describing a minimally invasive repair, the open procedures have been replaced as the gold standard in the treatment of PE. 8 There have been modifications since its conception to improve reproducibility and prevent complications. The introduction of thoracoscopy, the development of bar stabilizers, as well as the addition of pericostal sutures have all contributed to minimize the number of complications associated with the substernal tunnel creation and displacement of the steel bars. 4 7

Introduction Cases of congenital accessory hepatic lobe torsion (AHL) are largely unheard of with only 22 reported cases in children, out of which there were only 7 reported in infants, in English literature as of 2017. 1 AHL may be sessile with a wide base of continuous hepatic parenchyma connected to the liver proper, appearing as small tongue-like projections from the surface of the liver (i.e., Riedel's lobe) or rarely exist as a pedunculated mass of hepatic parenchyma attached to a vascular pedicle. 2 Most of the cases are asymptomatic. When pedunculated, AHL can undergo torsion resulting in ischemia, which can then present as an acute abdomen. To help identify and properly treat such cases we aim to provide more insight on the subject. We present a case of torsion of the AHL with an embedded gallbladder, embedded in it, in an infant. Case Report An 11-month-old boy weighing 8 kg presented to the emergency department with severe pallor, cyanosis, and signs of respiratory distress. He was a known G6PD -deficient and had a history of omphalocele repair in the neonatal period. On examination, he was in distress, mildly cyanotic, afebrile, and not jaundiced. His vital signs were: heart rate (HR): 158 bpm, respiration rate: 40 bpm, blood pressure: 85/40 mm Hg, and temperature: 35.3°C. The examination of the respiratory system showed the presence of doubtful bilateral basal crepitations.

eriod. On examination, he was in distress, mildly cyanotic, afebrile, and not jaundiced. His vital signs were: heart rate (HR): 158 bpm, respiration rate: 40 bpm, blood pressure: 85/40 mm Hg, and temperature: 35.3°C. The examination of the respiratory system showed the presence of doubtful bilateral basal crepitations. On abdominal examination, there was soft distention with a palpable mass occupying the right half of the abdomen, extending to the umbilicus, which was tender and there was no blood or mucus on per rectal examination. Laboratory investigations revealed a white cell count of 36 × 10 3 /µL, hemoglobin: 9.7 g/dL, serum sodium, potassium, and creatinine levels were normal, the alanine aminotransferase (ALT) value was 48 U/L, serum bilirubin, and alkaline phosphatase levels were normal. The C-reactive protein was normal, and the venous blood gas analysis showed a pH of 7.1, P o 2 : 36%, P co 2 : 73%, HCO 3 : 22.7, and base excess of −7.5. Within 3 hours the patient became paler, and the hemoglobin dropped to 6 g/dL. He subsequently received an urgent blood transfusion and was started on antibiotics; chest X-ray was unremarkable. On abdominal ultrasound, a 10 × 8 cm mass was noted. Evidence of marked ascites was present, and the gallbladder was distended. A computed tomography scan of the abdomen with oral and intravenous contrast confirmed a large high density (100 HU) midline mass measuring 7.8 × 8.9 × 9.4 cm ( Fig. 1A–C ). Fig. 1 ( A – C ) White arrows show the accessory hepatic lobe and the black arrows show the embedded gallbladder.

On abdominal ultrasound, a 10 × 8 cm mass was noted. Evidence of marked ascites was present, and the gallbladder was distended. A computed tomography scan of the abdomen with oral and intravenous contrast confirmed a large high density (100 HU) midline mass measuring 7.8 × 8.9 × 9.4 cm ( Fig. 1A–C ). Fig. 1 ( A – C ) White arrows show the accessory hepatic lobe and the black arrows show the embedded gallbladder. This gave the initial impression of a volvulus involving a lobe of the liver and gallbladder or internal herniation. Moderate amount of free fluid was noted in the subhepatic, right paracolic gutter, and the pelvis. An urgent exploratory laparotomy was then performed through an upper transverse abdominal incision. We discovered a severely congested hepatic lobe with the gallbladder in situ. Due to the presence of the gallbladder, we presumed that this was the right lobe of the liver ( Fig. 2A ). Fig. 2 Operative pictures: ( A ) Dusky Congested accessory liver lobe with the congested gallbladder (white arrow) embedded in it. ( B ) The liver lobe regaining normal color after untwisting the pedicle (white arrow). This was found to be under torsion on an abnormally long pedicle. Also, a lobe of the normal liver was found in the location of the right lobe without the gallbladder attached to it. Following this observation, the lobe was untwisted, and signs of reperfusion were noted. The bowel was normal in color, the duodenojejunal flexure was located normally, but the cecum was mobile ( Fig. 2B ).

o, a lobe of the normal liver was found in the location of the right lobe without the gallbladder attached to it. Following this observation, the lobe was untwisted, and signs of reperfusion were noted. The bowel was normal in color, the duodenojejunal flexure was located normally, but the cecum was mobile ( Fig. 2B ). On the postoperative day 1, the patient showed signs of recovery inferred by his improving blood gases and vital signs, but 24 hours later he again suddenly became pale, tachycardiac with an HR of 185 bpm, and a concurrent drop in hemoglobin from 10 to 6 g/dL. The abdomen became tense again, and an urgent ultrasonography was done but was inconclusive regarding blood flow to the affected part of the liver. Liver function tests revealed marked elevation of ALT level up to 3,854 U/L. An urgent relaparotomy was performed, and the previously torted hepatic lobe was found to be under torsion again; with a dusky hue and patchy necrosis, therefore excision of the ischemic torted AHL along with the gallbladder was performed. The remaining lobes of the liver were noted to have an independent pedicle containing the portal triad. The patient was given blood products and was continued on antibiotics. On ultrasound 1 after relaparotomy, the liver had normal size and a uniform texture with normal hepatic and portal veins. There was no evidence of thrombus formation, and the common bile duct and intrahepatic biliary radicles were not dilated. The patient gradually recovered and was discharged in good condition 12 days after admission. He remained asymptomatic on follow-up after 14 months.

orm texture with normal hepatic and portal veins. There was no evidence of thrombus formation, and the common bile duct and intrahepatic biliary radicles were not dilated. The patient gradually recovered and was discharged in good condition 12 days after admission. He remained asymptomatic on follow-up after 14 months. Discussion The AHL by definition is a developmental congenital anomaly of the hepatic bud that arises from the endodermal caudal foregut. 3 There are differing ways to classify this entity, 4 5 largely it is classified based largely on the size (10–30 g) and location of the lobe (attached to the liver or ectopic) or the presence of a capsule and biliary drainage system. 3 The viability is assessed by the presence of the portal triad in the AHL. 5 Similar to previously reported cases, the diagnosis of the AHL torsion was only definitively made intraoperatively. As of 2017, only 22 cases have been reported in children regarding this anomaly with 7 cases reported in infancy. 1 There seems to be a male predominance of AHL in infancy whereas in females AHL was often an incidental finding. The reason for this phenomenon remains unknown. 1 4 Imaging techniques without a high index of suspicion make the preoperative diagnosis difficult. In this case, due to the history of an omphalocele and the presentation of an acute abdomen and a palpable mass, there was reasonable indication to suspect AHL pathology. 3

The reason for this phenomenon remains unknown. 1 4 Imaging techniques without a high index of suspicion make the preoperative diagnosis difficult. In this case, due to the history of an omphalocele and the presentation of an acute abdomen and a palpable mass, there was reasonable indication to suspect AHL pathology. 3 Our patient presented in a very sick state probably as the extent of torsion was severe, and the gallbladder was included in the torted lobe. Depending on the location and duration of torsion of the AHL, it can present with various symptoms. A pedunculated AHL carries a higher risk of torsion than the other types of AHLs. Moreover, severe congestion and insufficient blood supply may lead to rupture of larger vessels. 2 Strangulation of the vascular supply to the liver due to the twisting of the mesentery of a large AHL may lead to hepatic ischemia necessitating transplantation. 6 However, our patient had a successful outcome, like another case, 7 which is attributed to the prompt recognition and the surgical intervention performed. The torted AHL in our patient was not necrotic and had regained color upon untwisting the lobe, yet it underwent torsion again and had to be excised the following day. It can be postulated that an excision is a good option as the primary approach instead of trying to preserve any seemingly viable liver tissue after it has been untwisted. However, we cannot rule out that fixation of the untwisted lobe would have been successful too in our case.

and had to be excised the following day. It can be postulated that an excision is a good option as the primary approach instead of trying to preserve any seemingly viable liver tissue after it has been untwisted. However, we cannot rule out that fixation of the untwisted lobe would have been successful too in our case. Conclusion Without a high index of suspicion, the diagnosis of an AHL is difficult before surgical exploration. When discovered, the excision during initial laparotomy is a good option. New Insights and the Importance for the Pediatric Surgeon Torsion of the accessory hepatic lobe torsion is an extremely rare condition and has the potential to cause serious complications. Pediatric surgeons must be aware of this entity while managing cases of acute abdominal pain, particularly those with a history of omphalocele repair. Conflict of Interest None.

Introduction Almost 30% of pediatric primary care visits regard skin-related symptoms. 1 From the neonatal period throughout infancy, most of the skin lesions are benign, and self-limiting. 2 Differential diagnosis of local skin inflammation usually includes trauma, insect bites, atopic dermatitis, allergies, or inappropriate hygiene. 3 We report on an infant with a cellulitis of the thigh which turned out to be a subcutaneous T-cell lymphoma. Case Report An 8-month-old girl with a 2-month history of superficial cellulitis of the anterior-medial right thigh treated conservatively with ointments and local/oral antibiotics in basic and dermatological care with no clinical improvement. Clinically, a large (10 × 5 cm) single, rectangular, flat, stiff infiltration with redness and peeling of the skin was observed ( Fig. 1A ). The patient had a history of fever, sweating, and failure to thrive. Shortly before hospitalization at our surgical unit in the pediatric oncology department, laboratory tests showed elevated lactate dehydrogenase up to 869 U/L, aspartate transaminase up to 153 U/L, alanine transaminase up to 113 U/L, and low C-reactive protein (1.04 mg/L). However, a magnetic resonance imaging (MRI) of a thigh confirmed the diagnosis of a local inflammation with mildly enlarged lymph node of the groin. The intravenous antibiotic was introduced causing mild local improvement, but the episodes of high fever sustained. The patient was transferred to our pediatric surgery department for treatment and surgical biopsy of the lesion ( Fig. 1B ).

diagnosis of a local inflammation with mildly enlarged lymph node of the groin. The intravenous antibiotic was introduced causing mild local improvement, but the episodes of high fever sustained. The patient was transferred to our pediatric surgery department for treatment and surgical biopsy of the lesion ( Fig. 1B ). Fig. 1 ( A ) The clinical picture of the right thigh after 2-months of conservative treatment; ( B ) the lesion of the thigh after surgical biopsy (visible skin sutures) that confirmed the diagnosis; ( C ) the same skin surface after 4 months of chemotherapy. Histopathological examination showed a massive T-cell infiltration (cluster of differentiation [CD]) CD3+, CD8+, CD5+, CD7+, CD4+ with sporadic CD99+, CD34+, TdT-, CD10-, CD56- cells with numerous histiocytes, plasmatic cells and neutrophils. A very high proliferative index was noticed (Ki-67+ > 70% of cells). A subcutaneous panniculitis-like T-cell lymphoma (SPTCL) was diagnosed. Characteristic rimming of individual fat cells by tumor cells with immunohistochemical staining specific for SPTCL was visualized in our patient's specimen ( Fig. 2 ). Fig. 2 Hematoxylin and eosin staining (extension ×200). Skin biopsy showing subcutaneous tissue with neoplastic infiltrate. The neoplastic cells range in size and have irregular, hyperchromatic nuclei. The characteristic feature is the rimming of the neoplastic cells surrounding individual fat cells.

on to improve reproducibility and prevent complications. The introduction of thoracoscopy, the development of bar stabilizers, as well as the addition of pericostal sutures have all contributed to minimize the number of complications associated with the substernal tunnel creation and displacement of the steel bars. 4 7 The indication of MIRPE over open repair in cases of severe PE is still cause for discussion. This is mainly due to the difficulty of the substernal dissection and steel bar placement due to the higher risk of cardiac injury in those patients with pectus eccentricity or sternal rotation that causes cardiac displacement. 9 The open repair, however, offers a higher complication rate. 10 This patient had one of the most severe PE repaired with a Nuss procedure in our institution, and as expected several concerns were raised. One of the concerns was the patient's age; however, Kelly et al had shared their experience in over 21 years of surgical correction of PE, in which age at the time of repair ranged from 1 to 31 years of age, and indication in these young patients was based on symptoms. 6 Park et al (How early) also offered results of repair in children over 3 years, with +600 cases of children under the age of five. Although follow-up is currently around 10 years, the recurrence rate remains low (0.4%) without statistical differences in morbidity or recurrence rate when compared with those patients who underwent repair at a later age. 11

Histopathological examination showed a massive T-cell infiltration (cluster of differentiation [CD]) CD3+, CD8+, CD5+, CD7+, CD4+ with sporadic CD99+, CD34+, TdT-, CD10-, CD56- cells with numerous histiocytes, plasmatic cells and neutrophils. A very high proliferative index was noticed (Ki-67+ > 70% of cells). A subcutaneous panniculitis-like T-cell lymphoma (SPTCL) was diagnosed. Characteristic rimming of individual fat cells by tumor cells with immunohistochemical staining specific for SPTCL was visualized in our patient's specimen ( Fig. 2 ). Fig. 2 Hematoxylin and eosin staining (extension ×200). Skin biopsy showing subcutaneous tissue with neoplastic infiltrate. The neoplastic cells range in size and have irregular, hyperchromatic nuclei. The characteristic feature is the rimming of the neoplastic cells surrounding individual fat cells. The patient was transferred to the pediatric hematology department. Further staging included a whole-body computed tomography (CT) scan with contrast enhancement (as a whole-body MRI was unavailable and patient's general status was worsening). The patient was introduced to a special chemotherapy protocol EURO-LB 2002 (prednisone, 6-mercaptopurine, and methotrexate) mildly adjusted to young age, with a good response. During the first weeks of treatment, there was a Pseudomonas aeruginosa infection of the wound after the surgical biopsy which was treated with targeted antibiotic therapy and later underwent surgical debridement with good wound healing ( Fig. 1C ). A control MRI of the thigh showed a significant improvement with reduction of the lesion without enlargement of the surrounding lymph nodes.

a infection of the wound after the surgical biopsy which was treated with targeted antibiotic therapy and later underwent surgical debridement with good wound healing ( Fig. 1C ). A control MRI of the thigh showed a significant improvement with reduction of the lesion without enlargement of the surrounding lymph nodes. Discussion The SPTCL is a rare cytotoxic T-cell lymphoma representing less than 1% of non-Hodgkin lymphomas. 4 It predominantly affects young adults and is uncommon in children. 5 SPTCL preferentially infiltrates subcutaneous tissue. As observed in our case, the most common sites of localization are the extremities and trunk. Systemic symptoms are observed in 50% of patients. 5 Our patient experienced intensive sweating and fever episodes for a significant time period while treatment was only local without any further diagnostics. Definitive diagnosis is based on a complex histopathological examination. 6 Nevertheless, it is crucial to combine the biopsy with information about clinical symptoms to guide the pathologist in search of the accurate interpretation. In the presented diagnostic process, in the communication between surgeon and pathologist information about general symptoms as well as elevated parameters were very useful and led to the final diagnosis.

biopsy with information about clinical symptoms to guide the pathologist in search of the accurate interpretation. In the presented diagnostic process, in the communication between surgeon and pathologist information about general symptoms as well as elevated parameters were very useful and led to the final diagnosis. Based on published case series, the morbidity, and mortality related to the development of hemophagocytic syndrome that aggravates the prognosis, which optimistically was not present in our patient. 7 Further molecular diagnostic tests determine T-cell receptor phenotype. The World Health Organization (WHO) and European Organization for Research and Treatment of Cancer (EORTC) in 2008 distinguished SPTCL with alpha beta T cells from primary cutaneous gamma delta lymphoma (PCGD-TCL). 6 Immunophenotypic differences include CD56 positivity with lack of CD4 and CD8 in PCGD-TCL. Such a division correlates with differences in prognosis, with much better survival rates in SPTCL. 8 The rarity of SPTCL resulted in a wide range of treatment strategies: from no treatment, steroids, immunosuppressive therapy to multiagent chemotherapy. 8 In the presented case a chemotherapy protocol was introduced due to extensive general symptoms and progression of the local state. If available, a whole-body MRI should be considered in the staging process rather than a whole-body CT due to the high levels of radiation connected with the latter.

tiagent chemotherapy. 8 In the presented case a chemotherapy protocol was introduced due to extensive general symptoms and progression of the local state. If available, a whole-body MRI should be considered in the staging process rather than a whole-body CT due to the high levels of radiation connected with the latter. In a skin lesion that is associated with systemic symptoms and responding untypically to antibiotic treatment malignancy should be considered and biopsy not be postponed. New Insights and the Importance for the Pediatric Surgeon Our case of an extremely rare subcutaneous panniculitis-like T-cell lymphoma is a guideline for a pediatric surgeon that in a skin lesion associated with systemic symptoms and responding untypically to antibiotic treatment malignancy should be considered and biopsy not be postponed. Conflict of Interest None.

Introduction Wilms' tumor (WT) is the most common childhood malignant renal tumor, it represents almost 90% of pediatric renal tumors and 7% of all childhood malignancies, occurring mainly in patients younger than 5 years of age and it has a 5-year survival rate of 85%. 1 2 Surgical complications based on the National Wilms' Tumor Study (NWTS) protocol with an upfront primary nephrectomy has significantly decreased over the past decade with postoperative bowel obstruction of the small intestine being the most common. 3 Large bowel volvulus is a rare cause of bowel obstruction occurring mainly in pediatric patients, with the cecum being the most common location in some series and it represents less than 1% of all intestinal obstructions in children. 4 5 6 7 Although postoperative cecal volvulus following a nephrectomy in adult patients 5 8 9 10 have been described previously, to our knowledge, there are no reported cases of a cecal volvulus following a nephrectomy in children and more specifically after a WT resection. We present the case of a 15-month-old female patient with a cecal volvulus following a right nephrectomy of a WT. Case Report

Large bowel volvulus is a rare cause of bowel obstruction occurring mainly in pediatric patients, with the cecum being the most common location in some series and it represents less than 1% of all intestinal obstructions in children. 4 5 6 7 Although postoperative cecal volvulus following a nephrectomy in adult patients 5 8 9 10 have been described previously, to our knowledge, there are no reported cases of a cecal volvulus following a nephrectomy in children and more specifically after a WT resection. We present the case of a 15-month-old female patient with a cecal volvulus following a right nephrectomy of a WT. Case Report A 15-month-old female patient was brought to our teaching hospital and was admitted through the emergency room following a nonpainful abdominal distention and a right abdominal mass. An ultrasound confirmed the presence of a right renal mass, and a computed tomography (CT) scan showed an 8 cm × 7 cm right renal tumor; no thrombosis of the renal or inferior vena cava was observed ( Fig. 1 ). A chest CT scan showed no evidence of pulmonary metastasis. The patient was then scheduled for a right nephrectomy. Fig. 1 Abdominal computed tomography scan with intravenous contrast. (A, B) (Superior to inferior cuts) axial view; (C, D) (posterior to anterior cuts) coronal view; 8 × 7 cm right kidney tumor.

A 15-month-old female patient was brought to our teaching hospital and was admitted through the emergency room following a nonpainful abdominal distention and a right abdominal mass. An ultrasound confirmed the presence of a right renal mass, and a computed tomography (CT) scan showed an 8 cm × 7 cm right renal tumor; no thrombosis of the renal or inferior vena cava was observed ( Fig. 1 ). A chest CT scan showed no evidence of pulmonary metastasis. The patient was then scheduled for a right nephrectomy. Fig. 1 Abdominal computed tomography scan with intravenous contrast. (A, B) (Superior to inferior cuts) axial view; (C, D) (posterior to anterior cuts) coronal view; 8 × 7 cm right kidney tumor. For a better longitudinal exposure of the vena cava, we entered through an incision in the midline, immediately displacing the cecum and the right colon medially. The right lateral peritoneum was divided to allow access to the renal tumor and finally the right kidney was removed along with a para-aortic lymphadenectomy. The right colon and the small bowel were left in place inside the abdominal cavity during the entire procedure, and we did not close the lateral peritoneum before closing the abdominal wall. Forty-eight hours after surgery, the patient showed abdominal distention and one episode of bilious vomiting. An abdominal X-ray showed the presence of fixed right lower abdominal air fluid levels and showed small bowel dilatation ( Fig. 2 ). The patient was taken to the operating room for an exploratory laparotomy performed through the previous incision. The small bowel was intact, and a cecal volvulus was found ( Fig. 3 ). A detorsion was performed showing a viable bowel and the lateral peritoneum was closed. The patient had an uneventful recovery and was discharged on postoperative day 5. Pathology confirmed a multifocal right renal stage I WT.

the previous incision. The small bowel was intact, and a cecal volvulus was found ( Fig. 3 ). A detorsion was performed showing a viable bowel and the lateral peritoneum was closed. The patient had an uneventful recovery and was discharged on postoperative day 5. Pathology confirmed a multifocal right renal stage I WT. Fig. 2 Abdominal X-ray. Right lower quadrant fixed air fluid level and small bowel dilatation. Fig. 3 (A) Scheme demonstrating the cecal volvulus found during reintervention. (B) Solid arrow showing medial mobilization of right colon. Dashed lined showing the transition point of obstruction after detorsion of the cecum (“x” mark). Discussion Based on the NWTS protocol, nephrectomy is the primary upfront treatment for WT. Preoperative chemotherapy is reserved for patients who have a solitary kidney, a horseshoe kidney, thrombosis of the inferior vena cava above the level of the hepatic veins, or if the child presents with respiratory distress due to extensive pulmonary metastases. 11 12 13 Based on the CT scan, our patient did not meet the criteria for preoperative chemotherapy according to the NWTS protocols; therefore, we decided to perform a nephrectomy. Several postoperative complications of primary nephrectomy following the NWTS protocol approach have been described, with bowel obstruction being the most common appearing in 5.1 to 6.9% of the patients, compared with 2.5% of the patients following the Societe Internationale D'oncologie Pediatrique protocol; postoperative adhesions followed by intussusceptions and internal hernias have all been described. 3 14

ch have been described, with bowel obstruction being the most common appearing in 5.1 to 6.9% of the patients, compared with 2.5% of the patients following the Societe Internationale D'oncologie Pediatrique protocol; postoperative adhesions followed by intussusceptions and internal hernias have all been described. 3 14 Postoperative intussusception usually occurs within the first postoperative week with a mean of 5 days; 15 16 bowel adhesions in infants and children causing bowel obstruction usually have a late presentation with 66% of them presenting 1 year after the initial procedure in some series, and only 6.8% of them being secondary to the resection of WT. 17 Internal hernias following a nephrectomy with small bowel obstruction usually have an acute presentation and can be either transmesenteric or in the retroperitoneal fossa. 14 18 19 20 An important differential diagnosis of patients presenting with volvulus is malrotation. This entity should always be kept in mind when assessing any infant or child with symptoms of vomiting and pain, particularly when the vomiting is bile stained. Ultrasound examination may be helpful but is not secure enough to exclude the diagnosis. Laparotomy or laparoscopy is the only way to be sure. 21 From the several risk factors identified that can increase the rate of postoperative surgical complications following the resection of a WT (including higher local tumor stage, intravascular extension, and en bloc resection of other visceral organs 3 ), none of them has been reported as a specific risk factor for postoperative bowel obstruction. 22

identified that can increase the rate of postoperative surgical complications following the resection of a WT (including higher local tumor stage, intravascular extension, and en bloc resection of other visceral organs 3 ), none of them has been reported as a specific risk factor for postoperative bowel obstruction. 22 A cecal volvulus following a right nephrectomy in adults has been described previously after releasing the cecum from the peritoneum and positioning it medially, 5 9 10 although it has also been described following a left-sided nephrectomy. 8 The following have been described as options for cecal volvulus management: cecopexy, simple detorsion without fixation, detorsion with cecopexy, tube cecostomy, and ileocolectomy. 23 There is no consensus on which is the best option for the management of the cecal volvulus, but there is an agreement between series that the management should rely on the viability of the bowel. 24 25 26 Detorsion might be sufficient for patients with a viable bowel, without gangrene, and a cecostomy has had a higher rate of reported complications in almost half of the series, with recurrence in approximately 15% and mortality rates of 20%. 23 We decided to do a detorsion of the cecal volvulus because the bowel was viable; even though we do not usually close the lateral peritoneum following a nephrectomy for WT, we decided to close the lateral peritoneum on the second intervention as a safety measure to keep the colon in its place.

The following have been described as options for cecal volvulus management: cecopexy, simple detorsion without fixation, detorsion with cecopexy, tube cecostomy, and ileocolectomy. 23 There is no consensus on which is the best option for the management of the cecal volvulus, but there is an agreement between series that the management should rely on the viability of the bowel. 24 25 26 Detorsion might be sufficient for patients with a viable bowel, without gangrene, and a cecostomy has had a higher rate of reported complications in almost half of the series, with recurrence in approximately 15% and mortality rates of 20%. 23 We decided to do a detorsion of the cecal volvulus because the bowel was viable; even though we do not usually close the lateral peritoneum following a nephrectomy for WT, we decided to close the lateral peritoneum on the second intervention as a safety measure to keep the colon in its place. A volvulus of the cecum should be considered as a possible cause of intestinal obstruction in the postoperative period following a right nephrectomy for WT, with a surgical intervention being the treatment of choice. The management of a cecal volvulus should be decided based on the viability of the bowel. A simple detorsion might be sufficient for the successful management of the condition, but we believe that closing the lateral peritoneum helped minimize the movement of the right colon during our second intervention. Although based on the evidence we cannot conclude that closure of the lateral peritoneum is mandatory, we do believe that this might have played a huge role in the effectiveness of the procedure.

believe that closing the lateral peritoneum helped minimize the movement of the right colon during our second intervention. Although based on the evidence we cannot conclude that closure of the lateral peritoneum is mandatory, we do believe that this might have played a huge role in the effectiveness of the procedure. New Insights and the Importance for the Pediatric Surgeon When performing a nephrectomy it might be important to consider closing the lateral peritoneum as it might play a role in avoiding possible post-operative cecal volvulus. Acknowledgment The author would like to acknowledge Dr Rodrigo Gonzalez-Urquijo and the Instituto Tecnologico y de Estudios Superiores de Monterrey, Medical Research Department. Conflict of Interest None.

Introduction Ectopic adrenocortical tissue (EAT) along the spermatic cord is an uncommon finding in children who undergo surgical groin exploration with an overall incidence of 1 to 9.3%. 1 The majority of patients are younger than 2 years of age and the occurrence seems significantly increased with undescended testes. 2 In a case study of 935 groin explorations, Sullivan et al reported a prevalence of 0.7% at inguinal herniotomy, 4.1% at ligation of the patent processus vaginalis for communicating hydrocele, and 3.3% at exploration for undescended testes. 3 The ectopic adrenal tissue appears as a small (1–5 mm) bright yellow soft nodule, clearly different in color and consistency from fat, and embedded between cremasteric fibers. With the progressive increase in laparoscopy for inguinal hernia repair and nonpalpable testes in children, as in open inguinal surgery. Pediatric surgeons should expect to find EAT during these procedures. The author reports on a case of EAT in a child who underwent laparoscopic exploration of a nonpalpable left testis at his institution. Case Report

The ectopic adrenal tissue appears as a small (1–5 mm) bright yellow soft nodule, clearly different in color and consistency from fat, and embedded between cremasteric fibers. With the progressive increase in laparoscopy for inguinal hernia repair and nonpalpable testes in children, as in open inguinal surgery. Pediatric surgeons should expect to find EAT during these procedures. The author reports on a case of EAT in a child who underwent laparoscopic exploration of a nonpalpable left testis at his institution. Case Report A 2-year-old boy had neonatal diagnosis of a nonpalpable left testicle. After a delay due to uncertainty by his parents, the boy came to us for laparoscopic exploration. The patient underwent standard laparoscopy with a reusable transumbilical 5-mm port with a 0-degree optic. Laparoscopic exploration showed a closed left internal ring, hypotrophic vessels, and a vas deferens entering the internal inguinal ring. A bright yellow nodule of approximately 4 mm stood out over the vessels 3 cm from the inner inguinal ring ( Fig. 1 ). Suspecting the presence of EAT, the author decided to go ahead with the excision of the nodule. An additional contralateral right 3 mm trocar was introduced on the right side of the abdomen; a small window was created in the peritoneum, overlapping the nodule, and the nodule was easily isolated and excised electrocoagulating its thin vessels (not recommended in case of vital testis). Histopathological examination showed an oval-shaped nodule consisting of a small adrenal cortical nodule, with no medullary tissue or atypical cells and some microcalcifications ( Fig. 2 ). At the end of the laparoscopy, given that the internal ring was closed, the testicular nubbin was excised through a small inguinal incision. 4 The histological exam revealed fibrous–muscular–adipose tissue.

mall adrenal cortical nodule, with no medullary tissue or atypical cells and some microcalcifications ( Fig. 2 ). At the end of the laparoscopy, given that the internal ring was closed, the testicular nubbin was excised through a small inguinal incision. 4 The histological exam revealed fibrous–muscular–adipose tissue. Fig. 1 Laparoscopic appearance of ectopic adrenocortical tissue covered by peritoneum and its excision using a 3-mm operative trocar. Fig. 2 Histologic section of the excised nodule, showing encapsulated adrenal cortical tissue and focal calcifications (hematoxylin and eosin, HE, ×50).Inset: detail of ectopic adrenocortical tissue showing no medullary tissue or atypical cells (HE ×150). Discussion

Fig. 1 Laparoscopic appearance of ectopic adrenocortical tissue covered by peritoneum and its excision using a 3-mm operative trocar. Fig. 2 Histologic section of the excised nodule, showing encapsulated adrenal cortical tissue and focal calcifications (hematoxylin and eosin, HE, ×50).Inset: detail of ectopic adrenocortical tissue showing no medullary tissue or atypical cells (HE ×150). Discussion In 1740, Morgagni first described ectopic adrenal tissue in the vicinity of the adrenal gland, and in 1885, Dagonest observed the presence of adrenocortical tissue in the spermatic cord of an infant. 5 6 These remnants are usually found in the pediatric age but are also described in adults. 7 8 Ectopic adrenal cortical tissue can be found in the upper abdomen or anywhere along the track of the gonadal descent. The most frequent sites are celiac axis area (32%), broad ligament (23%), adnexa of the testes (7.5%), kidney (subcapsular upper pole) (0.1–6%), and spermatic cord (1–9.3%). 9 10 These anatomic locations can be explained on an embryologic basis given the close spatial relationship between the developing kidneys and adrenal glands. There are even unusual anatomic sites where one can find these EAT, such as placenta, liver, lung, and intracranial cavity. 11 General occurrence in various publications refers to patients who have experienced surgical groin explorations (1.2%). In a recent review, Mendez et al found EAT in 13 out of 1,120 surgical groin exploration (1.16%) including 6 inguinal hernias, 5 undescended testes, and 2 communicating hydroceles. Moreover, in their review of the literature until 2006, less than 100 cases were described near the genital structures. 1 Some cases of EAT have also been reported in girls, although the lower incidence in girls is not easy to explain. Oğuz et al, 12 in a multicenter study, reported an incidence of EAT of 2.02% out of a total of 296 male patients who had inguinoscrotal surgery between 2009 and 2011. Vaos et al 13 reported a comprehensive incidence of 2.5% of EAT in 316 inguinoscrotal operations performed on 289 consecutive patients (0.7% in girls and 1.8% in boys). In this report, the nodules were located along the spermatic cord, in the apex of the hernial sac, and between the testis and epididymis. Some authors have estimated that these remnants may be present in 50% of newborns but most of them become atrophic by adult life.

nsecutive patients (0.7% in girls and 1.8% in boys). In this report, the nodules were located along the spermatic cord, in the apex of the hernial sac, and between the testis and epididymis. Some authors have estimated that these remnants may be present in 50% of newborns but most of them become atrophic by adult life. 14 Other authors 2 suggest that there is a significant increased incidence of EAT in patients with undescended testis: out of 1,069 patients (159 girls and 910 boys), the incidence of EAT totaled 1.63% in the cases of hernia and hydrocele of cord, with 5.1% in cases of undescended testis. No EAT was found in girls. To our knowledge, this is the first published case of EAT found laparoscopically in a case of vanishing testis. Laparoscopic exploration for nonpalpable testis highlighted EAT along the hypotrophic spermatic vessels in their intra-abdominal course. Macroscopically, the appearance of ectopic adrenal tissue was characteristic: a round, yellow nodule, firm in consistency, standing out clearly on the floor of the peritoneum almost superimposed on the testicular vessels. This finding is quite different from what is usually found during groin surgery where the EAT, when present, appears firmly embedded in the cremasteric fibers that surround the spermatic elements, resembling a fat lobule. In fact, these remnants may undergo hyperplasia in conditions associated with excessive adrenocorticotropic hormone production and may occasionally give rise to neoplasms. 15 Although the occurrence of neoplasm in ectopic adrenal nodules is far from common, pheochromocytoma, Leydig cell tumor, and adrenal adenoma have been reported. 16 17 18 Moreover, primary neoplastic tissue in the spermatic cord is also rare but paragangliomas have been described. 19 In conclusion, on the basis of this observation, the author thinks it is important for the surgeon to expect the possibility of a nodule consistent with EAT during groin procedures and also laparoscopy for nonpalpable testis. These remnants should be removed to prevent hormone production or malignant transformation.

19 In conclusion, on the basis of this observation, the author thinks it is important for the surgeon to expect the possibility of a nodule consistent with EAT during groin procedures and also laparoscopy for nonpalpable testis. These remnants should be removed to prevent hormone production or malignant transformation. New Insights and the Importance for the Pediatric Surgeon This is the first report on a case of ectopic adrenocortical tissue found and treated laparoscopically in a case of vanishing testis in a child. Conflict of Interest None.

Introduction Isolated rectal atresia is a rare condition with a reported incidence of 1% of all anorectal malformations. 1 2 The exact pathogenesis of rectal atresia is not known, but developmental, embryological, thickened Houston's valves, genetic, infective and environmental theories have been postulated. 1 Most authors believe it to be an acquired lesion due to the lack of associated congenital anomalies. 3 A broad search of the English literature was performed to screen for relevant papers that examined other anomalies associated with rectal atresia, including Down's syndrome, congenital cardiac and renal diseases, tracheoesophageal fistula, choanal atresia, skeletal anomalies, midgut malrotation, presacral masses, pouch colon anomalies, vaginal atresia and vesical, urethral, double urethral, vestibular and labial fistulas of the rectum. 1 2 3 4 5 6 7 8 9 10 11 Unlike rectal atresia, hypothyroidism exhibits a high rate of associated anomalies. 12 13 14 15 The association of rectal atresia with congenital hypothyroidism has not been reported to date in either rectal atresia or congenital hypothyroidism studies in the literature. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 In this study, a unique case of rectal atresia that presented as congenital hypothyroidism with isolated rectal atresia is reported in a newborn. The patient was treated successfully by a three stage posterior sagittal approach. This case is presented to demonstrate an uncommon and important therapeutic approach. Case Report

In this study, a unique case of rectal atresia that presented as congenital hypothyroidism with isolated rectal atresia is reported in a newborn. The patient was treated successfully by a three stage posterior sagittal approach. This case is presented to demonstrate an uncommon and important therapeutic approach. Case Report A 1-day-old male neonate was admitted due to failure to pass meconium and abdominal distention. At that time, prenatal ultrasonic investigations and the family history were reported as normal. During follow-up, the mother was revealed to have had a high-risk pregnancy. A prenatal ultrasonic investigation at 10 weeks of gestation revealed fetal death of one of twin fetuses. A decision was then made to closely monitor the pregnancy to preserve the remaining fetus. After that, pregnancy went well and no pathologic futures were reported in the surviving baby' ultrasounds. The surviving twin was delivered with a birth weight of 2300 g via elective caesarian section to a 23-year-old primigravida mother after a full-term pregnancy. The patient exhibited progressive abdominal distension, vomiting, and failure to pass meconium. A physical examination revealed a normal appearance of the perineum, genitalia and a normally located anal opening; funnel anus was not detected and no signs of fistula were present. After examination of the anal opening with a firm catheter, a blind-ending anal canal was diagnosed. An upside-down invertogram was performed, and a Hegar dilator was passed through the anal opening, which revealed an air column in the distal intestine ( Fig. 1A ).

was not detected and no signs of fistula were present. After examination of the anal opening with a firm catheter, a blind-ending anal canal was diagnosed. An upside-down invertogram was performed, and a Hegar dilator was passed through the anal opening, which revealed an air column in the distal intestine ( Fig. 1A ). Fig. 1 ( A ) Invertogram showing that the dilator contacted the air column in the distal intestine and the air-fluid levels at 28 hours of age. ( B ) Diverting sigmoid colostomy when the patient was 8 months old. ( C ) Distal cologram showing the rectal atresia without any fistula with an ∼1.5 cm atretic gap. Plain X-rays, echocardiography, ultrasound examinations of the newborn did not reveal any associated cardiac, spinal, urinary, or skeletal anomalies; the sacrum and sacral ratio were normal and there was no presacral mass on spinal ultrasound. A diverting descending colostomy was performed on the second day of life ( Fig. 1B ). Biopsy was taken for Hirschsprung's disease and result confirm the presence of ganglion cells. Newborn screening tests identified congenital hypothyroidism, which was confirmed by the results of the blood-free thyroxin (T4) and thyroid-stimulating hormone tests. Therefore, on the 12 days of life thyroid hormone replacement was started at a dose of 11.0 μg/kg/day with levothyroxine.

he presence of ganglion cells. Newborn screening tests identified congenital hypothyroidism, which was confirmed by the results of the blood-free thyroxin (T4) and thyroid-stimulating hormone tests. Therefore, on the 12 days of life thyroid hormone replacement was started at a dose of 11.0 μg/kg/day with levothyroxine. A definitive operation was performed at the age of 9 months via the posterior sagittal approach. Before the operation, a distal colostogram revealed rectal atresia without any fistula, and the atretic gap length of the rectum was determined to be ∼1.5 cm ( Fig. 1C ). The continuity of the rectum was established with circumferential anastomosis using interrupted absorbable sutures and a posterior sagittal approach ( Fig. 2A – E ). A muscle stimulator was used during the entire procedure, and good muscle contractions were observed equally on both sides ( Video 1 ). Closure of the colostomy was performed after completion of a dilatation program at the age of 1 year. Thyroid hormone replacement continued throughout the follow-up period. The patient is currently well with a follow-up period of 4.5 years and his rectal examination is normal. There is no fecal incontinence or soiling, but he suffers from constipation and requires stool softeners for this condition. Bowel function was assessed according to the Rintala bowel function score; it is classified as a good outcome with 15 points. The hypothyroidism follow-up is continued by the pediatric endocrinology clinic.

fecal incontinence or soiling, but he suffers from constipation and requires stool softeners for this condition. Bowel function was assessed according to the Rintala bowel function score; it is classified as a good outcome with 15 points. The hypothyroidism follow-up is continued by the pediatric endocrinology clinic. Video 1 A muscle stimulator was used during the operation, and good muscle contractions were observed equally on both sides. Online content including video sequences viewable at: www.thieme-connect.com/products/ejournals/html/10-1055-s-0037-1612610-EJPSR-17-0353-v1.mp4 . Fig. 2 ( A ) Intraoperative image showing the patient in a prone position with the pelvis elevated for the posterior sagittal approach. The patient exhibited normal perineum, genitalia, and a normally located anal opening. ( B ) Exposure of the defect with a Hegar dilator in the distal pouch. ( C ) The fibrous tissue separating the two rectal pouches (the incision asymmetry is due to the asymmetric position of the instrument). ( D ) The upper pouch of the rectum is held with sutures and opened. ( E ) A simple end-to-end circumferential anastomosis with interrupted long-term absorbable sutures. Discussion

Fig. 2 ( A ) Intraoperative image showing the patient in a prone position with the pelvis elevated for the posterior sagittal approach. The patient exhibited normal perineum, genitalia, and a normally located anal opening. ( B ) Exposure of the defect with a Hegar dilator in the distal pouch. ( C ) The fibrous tissue separating the two rectal pouches (the incision asymmetry is due to the asymmetric position of the instrument). ( D ) The upper pouch of the rectum is held with sutures and opened. ( E ) A simple end-to-end circumferential anastomosis with interrupted long-term absorbable sutures. Discussion Several classifications are used to define anorectal malformations. Rectal atresia is generally categorized as complex and unusual. 16 Rectal atresia has also been referred as a colonic atresia. 17 Moreover, rectal atresia itself is classified in different manners. Sharma and Gupta updated and revised the rectal atresia classification to incorporate the various types observed and provide increased detail. The present case, which consisted of a gap between the proximal and distal ends, is classified as type four according to their publications and is extremely rare. 1

ent manners. Sharma and Gupta updated and revised the rectal atresia classification to incorporate the various types observed and provide increased detail. The present case, which consisted of a gap between the proximal and distal ends, is classified as type four according to their publications and is extremely rare. 1 Congenital hypothyroidism is the most common endocrine disorder, affects ∼1:2,000 to 1:4,000 newborns worldwide and exhibits a high prevalence of extrathyroidal malformations. 12 Common symptoms include goiter, poor feeding, constipation, hypothermia bradycardia, and prolonged jaundice. 12 Infants exhibiting congenital hypothyroidism and extrathyroidal congenital malformations were reported with prevalence rates ranging from 8.4% to 28.2%. The majority of these patients exhibited cardiac features, dysmorphic features, neurologic abnormalities, genitourinary malformations, and Down's syndrome. 12 13 14 15 To the best of our knowledge, the association of hypothyroidism and rectal atresia has not been described so far. Although several explanations of the relationship between hypothyroidism and congenital malformations have been proposed hitherto, none is considered adequate. 13 14 15 Therefore, the coexistence of the hypothyroidism and rectal atresia is related to coincidence or association remains questionable.

en described so far. Although several explanations of the relationship between hypothyroidism and congenital malformations have been proposed hitherto, none is considered adequate. 13 14 15 Therefore, the coexistence of the hypothyroidism and rectal atresia is related to coincidence or association remains questionable. Distal intestinal atresia is difficult to diagnose prenatally, and this condition is a very rare cause of intestinal obstruction. 18 Management depends on the level of atresia and the presence of associated anomalies. In cases of rectal atresia with a normal anal opening, a delay in diagnosis and potential complications, such as pneumoperitoneum and mortality, may occur. 11 On examination, anus sometimes appears skin lined, narrowed, and funnel shaped. It is known as a “funnel anus” and highly suggestive for rectal atresia and rectal stenosis. 2 19 Preoperative workup includes plain and cross table or upside-down X-rays, echocardiography, and ultrasonography of the urologic system and spine. Presacral mass should not be overlooked. Hamrick et al reported a 29% prevalence of presacral masses in a rectal atresia/stenosis series. 2 Rectal atresia cases require a preliminary diverting colostomy to minimize postoperative complication. 2 Biopsy may be taken at the time of making a colostomy to rule out associated Hirschsprung's disease. 1 A distal cologram is helpful to exclude fistula and confirm the length of the atretic gap. 1 2 Obstructive uropathies are common in these neonates and warrant urgent decompression of the urinary tract as well as a colostomy. 6

y be taken at the time of making a colostomy to rule out associated Hirschsprung's disease. 1 A distal cologram is helpful to exclude fistula and confirm the length of the atretic gap. 1 2 Obstructive uropathies are common in these neonates and warrant urgent decompression of the urinary tract as well as a colostomy. 6 The optimal surgical care for patients with rectal atresia begins with appropriate decision making during the critical newborn period. Many operative approaches are used to correct this unique malformation. 2 3 19 20 An alternative option consisting of treatment of rectal atresia with anastomosis using magnets has been reported. 21 However, the selection of the surgical technique depends on the presence of associated anomalies, presacral mass, pouch colon, and fistula. 2 3 7 8 9 11 Posterior sagittal approach is a useful technique for the surgical correction of rectal atresia according to the largest reported series of rectal atresia/stenosis patients. 1 16 A midline posterior sagittal incision exposes the rectal pouch, which is mobilized from the surrounding muscle fibers, and a direct, end-to-end anastomosis between the blind tips of the anus and rectum can be performed. 16 This technique has been successfully used in cases of rectal atresia with a gap between the two pouches. 1 After the colostomy is closed following dilatation, patients with rectal atresia have appropriate bowel control for their age. 2

o-end anastomosis between the blind tips of the anus and rectum can be performed. 16 This technique has been successfully used in cases of rectal atresia with a gap between the two pouches. 1 After the colostomy is closed following dilatation, patients with rectal atresia have appropriate bowel control for their age. 2 Postoperative assessment is done with standardized questionnaires by Rintala and Lindahl at the age of 4.5 years. 22 Constipation in anorectal malformations is extremely common, particularly in the lower types with good prognosis for bowel control which include rectal atresia or stenosis. 2 23 However, among the symptoms caused by hypothyroidism, constipation is foremost, but some patients do not experience complete resolution of hypothyroid symptoms when treated with sufficient hormone therapy. 12 The present patient was continent, but he suffered from constipation. Both hypothyroidism and anorectal malformations are causes of congenital constipation. Therefore, the cause of constipation in our patient on long-term follow-up remains unclear and maybe multifactorial.

en treated with sufficient hormone therapy. 12 The present patient was continent, but he suffered from constipation. Both hypothyroidism and anorectal malformations are causes of congenital constipation. Therefore, the cause of constipation in our patient on long-term follow-up remains unclear and maybe multifactorial. Conclusion Rectal atresia is very rare, mostly nonsyndromic and occasionally associated with other anomalies. The association of congenital hypothyroidism and rectal atresia is unknown and has not been previously reported. This case report presents the first instance of congenital hypothyroidism and rectal atresia in a male neonate, and the subsequent treatments of both conditions are described. End-to-end rectoanal anastomosis via a posterior sagittal approach is a safe and effective technique for the surgical correction of rectal atresia. New Insights and the Importance for the Pediatric Surgeon A unique case of type four rectal atresia with hypothyroidism was treated with meticulous preoperative examination and PSARP method. Acknowledgement Author wishes to thank Prof. Dr. Ayşenur Celayir. Conflict of Interest None.

Introduction The occurrence of a sternal cleft (SC) is very rare in children, 1 2 and may be associated with several clinical problems; 3 most importantly, respiratory dysfunction secondary to disturbances in respiratory mechanics. Only a very few reports have been published that reported a combination of SC with aortic aneurysms (AA). 4 5 6 7 8 Here, we present a female neonate with an AA who underwent SC repair. Case Report We report on a term female newborn with a prenatally diagnosed isolated fetal aneurysm of the ascending aorta, but without further organ-specific diagnostic workup. As there was no evidence for an infection in this child, TORCH serology was not performed as per protocol of our center. Initial cardiopulmonary adaption was normal (APGAR scores of 8/9/9 at 1, 5, and 10 minutes). Physical examination revealed a SC with a palpable, short osseous bridge near the xiphoid process (XP) with visualization of the pulsating aorta [ Video 1 (online only)]. Moreover, a reddish, padded strand extended from the XP to the umbilical cord ( Fig. 1 ). Otherwise, the physical examination was unrevealing. Due to secondary onset of respiratory insufficiency within the first hour of life, the girl was intubated and ventilated; attempts for extubation failed because of the instability of the anterior chest wall. Fig. 1 Presentation of the strand with extension from the xiphoid process to the umbilical cord.

Initial cardiopulmonary adaption was normal (APGAR scores of 8/9/9 at 1, 5, and 10 minutes). Physical examination revealed a SC with a palpable, short osseous bridge near the xiphoid process (XP) with visualization of the pulsating aorta [ Video 1 (online only)]. Moreover, a reddish, padded strand extended from the XP to the umbilical cord ( Fig. 1 ). Otherwise, the physical examination was unrevealing. Due to secondary onset of respiratory insufficiency within the first hour of life, the girl was intubated and ventilated; attempts for extubation failed because of the instability of the anterior chest wall. Fig. 1 Presentation of the strand with extension from the xiphoid process to the umbilical cord. Video 1 Pulsating ascending aorta under the skin. Online content including video sequences viewable at: www.thieme-connect.com/products/ejournals/html/10-1055-s-0037-1612619-EJPSR-17-0327cg-v2.mp4 . In the following diagnostic workup, echocardiography and CT [ Supplementary Fig. S1 (online only)] confirmed the diagnosis of an aneurysm of the ascending aorta (diameter of 14 mm, z-score +4.75) and SC. Genetic analysis was negative for the examined 25 genes causing Marfan syndrome and other thoracic AA and aortic dissection related-syndromes as well as Ehlers–Danlos syndrome: ACTA2, CBS, COL1A1, COL1A2, COL3A1, COL5A1, COL5A1, EFEMP2, FBN1, FLNA, MAT2A, MFAP5, MYH11, MYLK, NOTCH1, PLOD1, PRKG1, SKI, SLC2A10, SMAD3, TGFB2, TGFB3, TGFBR1, TGFBR2, TNXB.

the examined 25 genes causing Marfan syndrome and other thoracic AA and aortic dissection related-syndromes as well as Ehlers–Danlos syndrome: ACTA2, CBS, COL1A1, COL1A2, COL3A1, COL5A1, COL5A1, EFEMP2, FBN1, FLNA, MAT2A, MFAP5, MYH11, MYLK, NOTCH1, PLOD1, PRKG1, SKI, SLC2A10, SMAD3, TGFB2, TGFB3, TGFBR1, TGFBR2, TNXB. On the day 6 of life, staged surgical closure of the SC was staged by our thoracic and pediatric surgeons. Intraoperatively, the cartilage bridge including the XP was resected. Afterward, the SC was approximated by sutures [3–0 monofilament absorbable suture (Polydioxanone, PDS II, Ethicon, Somerville, NJ United States)]. By this maneuver, the lower two-thirds of the cleft were approximated. The removed part of the lower sternum including the XP was used as an autologous graft to close the upper part of the remaining cleft. In so doing, complete closure of the SC was achieved. However, immediately after closure of the cleft the neonate developed cardiopulmonary instability. Therefore, the XP was moved to the mid of the cleft and fixed by sutures (3–0 monofilament absorbable suture; Polydioxanone, PDS II, Ethicon, Somerville, NJ United States) to stabilize the thoracic anterior wall and to improve thoracic motility with no recurrence of cardiovascular instability ( Fig. 2 ). Fig. 2 Intraoperative situs with autologous graft in the upper part of the SC ( cranial, thymus, sternum, ★ autologous graft).

On the day 6 of life, staged surgical closure of the SC was staged by our thoracic and pediatric surgeons. Intraoperatively, the cartilage bridge including the XP was resected. Afterward, the SC was approximated by sutures [3–0 monofilament absorbable suture (Polydioxanone, PDS II, Ethicon, Somerville, NJ United States)]. By this maneuver, the lower two-thirds of the cleft were approximated. The removed part of the lower sternum including the XP was used as an autologous graft to close the upper part of the remaining cleft. In so doing, complete closure of the SC was achieved. However, immediately after closure of the cleft the neonate developed cardiopulmonary instability. Therefore, the XP was moved to the mid of the cleft and fixed by sutures (3–0 monofilament absorbable suture; Polydioxanone, PDS II, Ethicon, Somerville, NJ United States) to stabilize the thoracic anterior wall and to improve thoracic motility with no recurrence of cardiovascular instability ( Fig. 2 ). Fig. 2 Intraoperative situs with autologous graft in the upper part of the SC ( cranial, thymus, sternum, ★ autologous graft). The postoperative period was uneventful. The girl was self-ventilating on room air with only subtle recessions in the jugular fossa [ Video 2 (online only)]. The AA was not of relevance at this early stage, thus only medical treatment with β-blockers was initiated. The actual development of weight and growth is fine at the age of 6 months. There is no increased susceptibility for pulmonary infections, cardiac function is nearly normal, and the diameter of the ascending aorta decreased in size (now z-score of +2.6).

stage, thus only medical treatment with β-blockers was initiated. The actual development of weight and growth is fine at the age of 6 months. There is no increased susceptibility for pulmonary infections, cardiac function is nearly normal, and the diameter of the ascending aorta decreased in size (now z-score of +2.6). Video 2 Recessions in the jugular fossa after extubation. Online content including video sequences viewable at: www.thieme-connect.com/products/ejournals/html/10-1055-s-0037-1604049-EJPSR-17-0322-CG-v1.mp4 . Discussion Successful surgical repair of SC by primary closure was first described by Maier and Bertone in 1949. 9 Several other surgical procedures have been reported since then including the use of costal cartilage grafts taken from the ribs, 10 autologous cartilage transfer, 11 or the bilateral sternal bar turnover flaps. 12 Also prosthetic materials like Teflon, silicone, or titanium have been used to close and stabilize the defect. 13

other surgical procedures have been reported since then including the use of costal cartilage grafts taken from the ribs, 10 autologous cartilage transfer, 11 or the bilateral sternal bar turnover flaps. 12 Also prosthetic materials like Teflon, silicone, or titanium have been used to close and stabilize the defect. 13 In our patient, primary repair of the SC by direct approximation of the sternal bars as described by Shamberger and Welch 14 was planned. However, complete closure may not always be possible due to intraoperative onset of cardiocirculatory disturbances as in our patient. Thus, when complete surgical correction in SC is not feasible, the use of an autologous graft for stabilization should be considered. This can be seen as a bridging procedure, a first step of the correction of the SC and to gain time. Our case report demonstrates that stabilization of the anterior thoracic wall can improve respiratory mechanics with the restoration of adequate respiratory function.

logous graft for stabilization should be considered. This can be seen as a bridging procedure, a first step of the correction of the SC and to gain time. Our case report demonstrates that stabilization of the anterior thoracic wall can improve respiratory mechanics with the restoration of adequate respiratory function. Since this is an extremely rare case, there are no guidelines or recommendations regarding the correct time frame. Given the clinical course in our opinion, definitive closure of the SC in our patient should be postponed until the end of the first year of life. It can be expected that the cleft itself will not grow in size, thus when approximating both sternal bars, the intrathoracic pressure will not increase in the same manner as in the initial surgical procedure, and will be better tolerated. Permanent stabilization of the sternum can be achieved by a transsternally placed pectus bar or wires—comparable to surgical repair of pectus excavatum. 15 The AA was not of relevance at this early stage and has demonstrated a relative decrease in size (z-score), thus only nonsurgical medical treatment (use of β-blockers to prevent further progression of the AA) was initiated as recommended in adult patients. 16 Conclusion A stepwise correction of a SC with the use of an autologous graft for stabilization may improve respiratory function and should be considered when complete surgical correction is not feasible, but complete closure of the SC should be realized early in infancy when the thoracic wall is still malleable.

The AA was not of relevance at this early stage and has demonstrated a relative decrease in size (z-score), thus only nonsurgical medical treatment (use of β-blockers to prevent further progression of the AA) was initiated as recommended in adult patients. 16 Conclusion A stepwise correction of a SC with the use of an autologous graft for stabilization may improve respiratory function and should be considered when complete surgical correction is not feasible, but complete closure of the SC should be realized early in infancy when the thoracic wall is still malleable. Acknowledgments Thanks to the parents for the permission to publish this case report. Funding None. Supplementary Material Supplementary Figure Supplementary Figure

Introduction Pectus excavatum (PE) is the most common anterior chest wall deformity. 1 2 3 The indication of surgical correction is based on the severity of the deformity, its progression, symptoms, functional testing, and the psychosocial effects on the patient. 4 5 Although the age of repair has been considered controversial, currently the indication of repair at any age is based primarily on the degree of the deformity and symptoms with evidence of cardiac and/or pulmonary compression. 6 The minimally invasive repair for pectus excavatum (MIRPE) was first described by Nuss in 1998. 4 7 8 Since then, its use has spread worldwide, modifications have been issued, and it is currently the procedure of choice in the correction of PE. However, some surgeons are still skeptic about its role in the surgical correction of extremely deep cases of PE. The aim of this article is to describe the case of a patient with an extremely severe PE that underwent a minimally invasive approach, as well as the difficulties in assessing PE severity. Case Presentation

Pectus excavatum (PE) is the most common anterior chest wall deformity. 1 2 3 The indication of surgical correction is based on the severity of the deformity, its progression, symptoms, functional testing, and the psychosocial effects on the patient. 4 5 Although the age of repair has been considered controversial, currently the indication of repair at any age is based primarily on the degree of the deformity and symptoms with evidence of cardiac and/or pulmonary compression. 6 The minimally invasive repair for pectus excavatum (MIRPE) was first described by Nuss in 1998. 4 7 8 Since then, its use has spread worldwide, modifications have been issued, and it is currently the procedure of choice in the correction of PE. However, some surgeons are still skeptic about its role in the surgical correction of extremely deep cases of PE. The aim of this article is to describe the case of a patient with an extremely severe PE that underwent a minimally invasive approach, as well as the difficulties in assessing PE severity. Case Presentation An 8-year-old girl with severe sternum depression and a history of exercise intolerance, fatigue, and shortness of breath on exertion was assessed. The patient also referred nocturnal dyspnea. Family history revealed an older sibling with asymptomatic severe PE that required surgical correction (Nuss procedure) at 7 years of age. Our patient's sternal depression was visible since birth and symptoms had started earlier in life (6–7 years of age), followed by rapid progression of symptoms which were more notable in the past 6 months. Genetic tests were performed to rule out Marfan syndrome as well as other abnormalities. The physical examination showed a prepubescent female with a height of 131 cm and weight of 22 kg. Her chest revealed a deep, asymmetric PE deformity ( Fig. 1 ).

progression of symptoms which were more notable in the past 6 months. Genetic tests were performed to rule out Marfan syndrome as well as other abnormalities. The physical examination showed a prepubescent female with a height of 131 cm and weight of 22 kg. Her chest revealed a deep, asymmetric PE deformity ( Fig. 1 ). Fig. 1 Patient prior to surgical intervention. Patient is an 8-year-old girl; image shows lateral and anteroposterior image at the time of the first outpatient clinic visit. An asymmetrical severe pectus excavatum is observed. As part of the assessment protocol, a computed tomography (CT) scan was ordered (see Fig. 2 ). Chest CT evaluation showed that the sternum depression was posterior to the anterior vertebral column rendering the Haller and correction index, to the best of our knowledge unmeasurable. Other severity indexes were calculated: sternal rotation angle was 40° and the asymmetry index of 58%, indicating a marked left-sided asymmetry. Fig. 2 Patient's chest imaging prior to surgical correction. Chest computed tomography showing the sternum depression is below anterior vertebral column line (red line). As part of the functional evaluation, a spirometry and echocardiogram were indicated. The preoperative spirometry indicated an obstructive ventilation pattern (forced expiratory volume in 1 second = 74.4%). The echocardiogram revealed a dilated inferior vena cava, mitral valve prolapse with normal ventricular function, all signs of ventricular compression.

a spirometry and echocardiogram were indicated. The preoperative spirometry indicated an obstructive ventilation pattern (forced expiratory volume in 1 second = 74.4%). The echocardiogram revealed a dilated inferior vena cava, mitral valve prolapse with normal ventricular function, all signs of ventricular compression. After multidisciplinary committee evaluation, considering the severity of the symptoms and available resources, the indication of surgical repair was offered to the family. A MIRPE approach was designed. Right thoracoscopic visualization was achieved with a 0° and 10mm Storz endoscope placed at the fourth intercostal space. Although an angled scope offers a better view, it was not available at the time of the repair. This was followed by placement of a midsternum 7.5 mm cannulated bone screw (Laffitte). This would generate upward traction that would allow better visualization of the substernal dissection plane. After careful dissection, two 9-inch steel bars were slipped under the sternum through the fifth and seventh intercostal space following the Nuss procedure indications. Two steel bars and stabilizers on both sides of the bar were placed to fully correct the deformity and to prevent bar displacement.

l dissection plane. After careful dissection, two 9-inch steel bars were slipped under the sternum through the fifth and seventh intercostal space following the Nuss procedure indications. Two steel bars and stabilizers on both sides of the bar were placed to fully correct the deformity and to prevent bar displacement. Postoperative care was uneventful allowing for a painless and quick recovery. At the 2 months' spirometry evaluation, an improvement in FEV1 and other parameters was observed (see Table 1 ), and cardiac compression signs disappeared on cardiac ultrasound. The patient referred that most symptoms had disappeared by the 3-month follow-up (nocturnal dyspnea and shortness of breath upon slight exertion), with great improvement in quality of life. Exercise was restricted during the initial 4 months, after which normal activity other than contact-sports were allowed. The desired sternum shape was achieved, with slight overcorrection noticed around 18 months postoperatively. The steel bars were removed after a 2-year period. At the 3-month follow-up, all of the patient's exercise intolerance had disappeared, and she was now more physically active. Postoperative bar removal imaging showed an acceptable correction of the deformity (see Figs. 3 and 4 ). During follow-up, the patient is content with the aesthetic result of the procedure; also the patient can carry out normal exercise.

results of repair in children over 3 years, with +600 cases of children under the age of five. Although follow-up is currently around 10 years, the recurrence rate remains low (0.4%) without statistical differences in morbidity or recurrence rate when compared with those patients who underwent repair at a later age. 11 There was also controversy as how to evaluate such a deformity. A debate was raised as to how to measure a Haller and correction index when the sternal depression is beyond the anterior vertebral column. Is it possible to measure the Haller or correction index in these patients? The correction index described by St Peter et al 12 used to obtain a quantitative measure of the severity of the deformity also requires the estimation of the minimal distance between the sternum depression and the anterior vertebral column. How do we adequately measure that centimeter that is beyond this line? Add a negative to the value? Unfortunately, this case was evaluated prior to the introduction of cardiac magnetic resonance imaging (MRI) as part of our institution's assessment protocol of PE in 2015. 13 14 A chest CT with high radiation exposure has now been replaced by a cardiac MRI which offers inspiration/expiration imaging and shows the dynamics of the malformation, as well as a more integral assessment of its effect in cardiac function. 13 14 In this case, the calculation of other PE indexes was necessary to allow for proper measurement. The asymmetry index and angle rotation showed the magnitude of the deformity. 2 3 12 15 16

n imaging and shows the dynamics of the malformation, as well as a more integral assessment of its effect in cardiac function. 13 14 In this case, the calculation of other PE indexes was necessary to allow for proper measurement. The asymmetry index and angle rotation showed the magnitude of the deformity. 2 3 12 15 16 Other apprehensions were raised as the surgical plan was drawn. The main fear was the substantial risk of heart injury during substernal dissection and bar placement due to the severity of the malformation. To minimize this risk, available means were used to exert upward traction of the sternum that allowed better visualization. The vacuum bell, that is, now our current practice, was not available; hence, a bone screw was placed midsternum. 17 Nowadays, there are different tools that can be used for this purpose: vacuum bell, Uemura hooks, Dr. Park's Crane, Rultract retractor, among others. 5 18 de Campos and Tedde 18 describe several techniques that have allowed the upward traction of the sternum to make the repair safer. These procedures may be used not only in the case of severe PE but also in mild or moderate PE.

s purpose: vacuum bell, Uemura hooks, Dr. Park's Crane, Rultract retractor, among others. 5 18 de Campos and Tedde 18 describe several techniques that have allowed the upward traction of the sternum to make the repair safer. These procedures may be used not only in the case of severe PE but also in mild or moderate PE. Another major concern was the risk of bar migration. Several studies have described this complication with rates ranging from 1% to 18%. 4 19 20 21 This was the main reason for placing two steel bars as well as stabilizers. Another technique described for this purpose is Park et al 22 bridge technique for PE bar fixation, in which one stabilizer is used to fixate both bars. Our choice was to place two steel bars and stabilizers on both sides of the bar to fully correct the deformity and to prevent bar displacement. However, our present practice is to place stabilizers on the left side of the bar (if the patient is right-handed) and to use pericostal sutures with Endoclose on the other side to achieve further stabilization. Bar removal is usually performed after a 2- to 4-year period. 4 In this case, bar removal was scheduled after a 2-year period. This decision was made due to the slight overcorrection observed, and signs of bar impingement on the chest wall.

Another major concern was the risk of bar migration. Several studies have described this complication with rates ranging from 1% to 18%. 4 19 20 21 This was the main reason for placing two steel bars as well as stabilizers. Another technique described for this purpose is Park et al 22 bridge technique for PE bar fixation, in which one stabilizer is used to fixate both bars. Our choice was to place two steel bars and stabilizers on both sides of the bar to fully correct the deformity and to prevent bar displacement. However, our present practice is to place stabilizers on the left side of the bar (if the patient is right-handed) and to use pericostal sutures with Endoclose on the other side to achieve further stabilization. Bar removal is usually performed after a 2- to 4-year period. 4 In this case, bar removal was scheduled after a 2-year period. This decision was made due to the slight overcorrection observed, and signs of bar impingement on the chest wall. Conclusion PE is the most common anterior thoracic deformity. A MIRPE is widespread and has replaced open procedure for PE repair; however, extremely severe cases of PE continue to challenge surgeons. We believe that with current advances in imaging and surgical maneuvers, the repair of extremely severe PE with MIRPE is both feasible and safe. In the evaluation of this case, we are left with the uncertainty of how to measure the severity of PE since Haller and correction index in particularly deep PE cannot be calculated. In the meantime, we believe a negative Haller index and correction index describe the uniqueness and severity of this case.

ible and safe. In the evaluation of this case, we are left with the uncertainty of how to measure the severity of PE since Haller and correction index in particularly deep PE cannot be calculated. In the meantime, we believe a negative Haller index and correction index describe the uniqueness and severity of this case. Conflict of Interest None.

Introduction Primary repair or the technically more demanding patching by thoracoscopy is increasingly used for the treatment of diaphragmatic hernia in neonates and children. This minimal invasive approach not only lessens postoperative pain and hospital stay, but also seems to be associated with a better cosmetic outcome and considerably less psychological, anatomical, and functional consequences of a thoracotomy, especially in young patients. 1 2 3 This procedure requires advanced surgical skills and a high level of training using miniature endoscopic instruments, especially when treating the neonatal population. Limited working space and time may result in poor fixation leading to hernia recurrence which remains a major concern for thoracoscopic surgeons operating on these challenging conditions. 1 2 This complication, namely inappropriate fixation and hernia recurrence, is believed to significantly affect morbidity and mortality. 4 In recent years, the advent of new instrumentation and techniques, such as nonabsorbable tack devices, has had a significant impact on surgical practice, notably in the field of hernia in adults (inguinal/umbilical/incisional). 5 6 The ProTack 5mm fixation device is a single-use tool for fastening prosthetic material, such as hernia mesh, to soft tissue. The tack is helical and made of titanium.

absorbable tack devices, has had a significant impact on surgical practice, notably in the field of hernia in adults (inguinal/umbilical/incisional). 5 6 The ProTack 5mm fixation device is a single-use tool for fastening prosthetic material, such as hernia mesh, to soft tissue. The tack is helical and made of titanium. In this article, we report two cases in which most of the challenges posed by thoracoscopic prosthetic repair of congenital diaphragmatic hernia (CDH) were easily solved with the use of the ProTack device. The overall procedure was similar to the thoracoscopic prosthetic repair of CDH described in the literature. 2 3 4 This report highlights the possibility of adding spiral tacks to the thoracoscopic pediatric surgeon' armamentarium as an alternative to suturing when patching is necessary. Case Reports Case 1 A male newborn was delivered vaginally at 36 weeks of gestation. Routine antenatal ultrasound was normal. The baby was placed on a respirator 20 hours after birth because of respiratory distress. A plain radiograph (RX) demonstrated a large left-sided CDH with air-filled loops of bowel filling the entire left thoracic cavity, leading to a significant right mediastinal shift.

tation. Routine antenatal ultrasound was normal. The baby was placed on a respirator 20 hours after birth because of respiratory distress. A plain radiograph (RX) demonstrated a large left-sided CDH with air-filled loops of bowel filling the entire left thoracic cavity, leading to a significant right mediastinal shift. The patient was stabilized on a ventilator and operated on 48 hours later. He was set in the right lateral-decubitus position, and three 5-mm trocars were placed in a triangular shape ( Fig. 1 ). Low-pressure insufflation at 4 mm Hg was applied. The diaphragmatic defect was posterolateral. The entire small bowel as well as the spleen was included in the hernia, and all contents were easily lowered into the peritoneal cavity. Fig. 1 Sites of port placement.

The patient was stabilized on a ventilator and operated on 48 hours later. He was set in the right lateral-decubitus position, and three 5-mm trocars were placed in a triangular shape ( Fig. 1 ). Low-pressure insufflation at 4 mm Hg was applied. The diaphragmatic defect was posterolateral. The entire small bowel as well as the spleen was included in the hernia, and all contents were easily lowered into the peritoneal cavity. Fig. 1 Sites of port placement. The posterior edge of the diaphragm was mobilized by incising the overlying peritoneum, and a primary repair was attempted by means of 4–0 braided polyester interrupted sutures knotted intracorporeally ( Fig. 2 ). However, primary approximation of the diaphragm failed because of excessive tension, and the use of a prosthetic patch (Parietex Composite Mesh, Covidien, Ireland [Protack 5 mm, Covidien,Mansfield, USA]) was deemed necessary. Once adjusted in size to match the defect, the mesh was secured to the diaphragm and directly to the ribs (where the diaphragm was completely absent) using seven nonabsorbable spiral tacks (ProTack 5mm, Covidien) ( Figs. 3 and 4 ). No bleeding from the adjacent vessels and no fracture of the ribs were observed during tack application and a right firing angle was obtained. A chest drain was left in the thoracic cavity at the end of the procedure. Operating time was ∼110 minutes. Fig. 2 Diaphragmatic defect, partially repaired by thoracoscopic suturing. Fig. 3 Use of an appropriately sized mesh to close the diaphragmatic defect. The mesh is fixated to the diaphragm using spiral tacks.

The posterior edge of the diaphragm was mobilized by incising the overlying peritoneum, and a primary repair was attempted by means of 4–0 braided polyester interrupted sutures knotted intracorporeally ( Fig. 2 ). However, primary approximation of the diaphragm failed because of excessive tension, and the use of a prosthetic patch (Parietex Composite Mesh, Covidien, Ireland [Protack 5 mm, Covidien,Mansfield, USA]) was deemed necessary. Once adjusted in size to match the defect, the mesh was secured to the diaphragm and directly to the ribs (where the diaphragm was completely absent) using seven nonabsorbable spiral tacks (ProTack 5mm, Covidien) ( Figs. 3 and 4 ). No bleeding from the adjacent vessels and no fracture of the ribs were observed during tack application and a right firing angle was obtained. A chest drain was left in the thoracic cavity at the end of the procedure. Operating time was ∼110 minutes. Fig. 2 Diaphragmatic defect, partially repaired by thoracoscopic suturing. Fig. 3 Use of an appropriately sized mesh to close the diaphragmatic defect. The mesh is fixated to the diaphragm using spiral tacks. Fig. 4 Technique of anchorage of the mesh. It is crucial to secure circumferentially the mesh and deploy tacks perpendicular to the tissue, which can sometimes be challenging.

Fig. 2 Diaphragmatic defect, partially repaired by thoracoscopic suturing. Fig. 3 Use of an appropriately sized mesh to close the diaphragmatic defect. The mesh is fixated to the diaphragm using spiral tacks. Fig. 4 Technique of anchorage of the mesh. It is crucial to secure circumferentially the mesh and deploy tacks perpendicular to the tissue, which can sometimes be challenging. The postoperative course was uneventful. The size of the left lung gradually increased, and the mediastinal shift resolved ( Fig. 5 A1 , A2 ). The patient was discharged from the hospital 3 weeks after the operation. He progressed well throughout the follow-up period, presenting a normal chest RX, and the patient remains asymptomatic 4 years and 3 months after surgery. Fig. 5 Postoperative chest radiograph: (A1) Immediate postoperative and (A2) Two days postoperatively. The size of the left lung gradually increased, and the mediastinal shift resolved.

The postoperative course was uneventful. The size of the left lung gradually increased, and the mediastinal shift resolved ( Fig. 5 A1 , A2 ). The patient was discharged from the hospital 3 weeks after the operation. He progressed well throughout the follow-up period, presenting a normal chest RX, and the patient remains asymptomatic 4 years and 3 months after surgery. Fig. 5 Postoperative chest radiograph: (A1) Immediate postoperative and (A2) Two days postoperatively. The size of the left lung gradually increased, and the mediastinal shift resolved. Case 2 A 6-month-old boy was admitted to the hospital with acute respiratory distress. He was delivered at 38 weeks of gestation with a birth weight of 3,100 g. Notable medical history included an antenatally diagnosed left-sided CDH associated with extreme pulmonary hypoplasia. He underwent fetal endoscopic tracheal occlusion (Fetendo-PLUG) with limited response. His diaphragmatic defect was repaired on the fifth day of life in another institution and included placement of a patch over a large left posterolateral defect through a classical subcostal left laparotomy. A Nissen fundoplication with gastrostomy was also performed at 3 months of age because of significant gastroesophageal reflux and failure to thrive.

ed on the fifth day of life in another institution and included placement of a patch over a large left posterolateral defect through a classical subcostal left laparotomy. A Nissen fundoplication with gastrostomy was also performed at 3 months of age because of significant gastroesophageal reflux and failure to thrive. At 6 months of age, acute respiratory distress with cyanosis, tachypnea, lethargy, and opisthotonos were observed at the arrival in the emergency room. After cardiopulmonary resuscitation and artificial ventilation, a chest RX and a three-dimensional computed tomography (CT scan) 2 days later revealed a recurrence of the left CDH with small bowel loops filling the left thoracic cavity. A three trocar thoracoscopy was scheduled after stabilization and performed as in case 1. With the help of the capnothorax, the hernia contents were gently lowered into the abdomen and pieces of the former patch were extracted. A primary closure seemed impossible due to both a too large posterolateral diaphragmatic defect and very inflamed edges. A nonabsorbable Parietex mesh (Parietex Composite Mesh, Covidien) was carefully positioned and secured in place to cover the diaphragmatic defect. The patch was completely secured to the costal margins and diaphragm with spiral titanium tacks (ProTack 5mm, Covidien). In this case, ∼15 tacks were needed to achieve attachment. No chest tube was left in situ in this case.

esh, Covidien) was carefully positioned and secured in place to cover the diaphragmatic defect. The patch was completely secured to the costal margins and diaphragm with spiral titanium tacks (ProTack 5mm, Covidien). In this case, ∼15 tacks were needed to achieve attachment. No chest tube was left in situ in this case. The infant recovered well and was discharged from the hospital 1 month after the operation with no recurrence of the hernia on CT scan. After a few months, he was on complete oral feeds. A chest X-ray 1 year post-repair demonstrated a normal thorax, and no evidence of recurrence. Over a 5 ½-year period of follow-up, no subsequent episodes of recurrence occurred, and growth of the child was normal, without complaints. Discussion CDH is a complex, life-threatening malformation which still presents a high morbidity and mortality rate, with survival rate ranging between 50% and 80% in the different studies. 7 In the last decades, prodigious effort has been made to improve the treatment of patients with CDH. 7 8 Laparoscopy was, naturally, the first minimal invasive approach for CDH repair. 9 To date, no studies comparing laparoscopic and thoracoscopic techniques have been performed, but the latter offers advantages in terms of space and easy reduction in the abdominal content, and has been preferred for Bochdalek hernias repair by many authors. 9 10 With thoracoscopy, one can directly visualize the herniated viscera, gently push it into the peritoneal cavity, and then observe and repair the diaphragmatic defect with or without patching. 9

of space and easy reduction in the abdominal content, and has been preferred for Bochdalek hernias repair by many authors. 9 10 With thoracoscopy, one can directly visualize the herniated viscera, gently push it into the peritoneal cavity, and then observe and repair the diaphragmatic defect with or without patching. 9 Despite progress in instrumentations and improved surgeon' skills, repair of large CDH defects through thoracoscopic prosthetic repair is still significantly challenging due to the difficulty of intrathoracic suturing. Excessive perioperative hypercapnia and prolonged postoperative low brain oxygenation, along with difficult patch fixation and hernia recurrence, remain the most important factors affecting postoperative morbidity and mortality. 4 9 11 Different types of intra or extracorporeal knots, interrupted or running sutures, rib-anchoring stitches with or without skin incisions, and the Reverdin needle are some of the suturing methods available. Each one of them has been reported to have advantages and disadvantages. 12 13 The ideal tool that provides a quick and safe patch fixation, without suturing difficulties or failures, has yet to be found.

horing stitches with or without skin incisions, and the Reverdin needle are some of the suturing methods available. Each one of them has been reported to have advantages and disadvantages. 12 13 The ideal tool that provides a quick and safe patch fixation, without suturing difficulties or failures, has yet to be found. In this report, nonabsorbable spiral tacks were used for mesh fixation during the thoracoscopic repair of large congenital diaphragmatic defects, allowing for easy, quick, and efficient anchoring of the patch to the edges of the defect or ribs. This method has been applied mainly in laparoscopic inguinal, incisional, and ombilical hernia repair in adults. Dapri et al recently described a case of a nontraumatic left lateral diaphragmatic hernia repair by single-incision laparoscopy (SILS) with mesh reinforcement using spiral tacks in a 45-year-old male. 14 A case of thoracoscopic repair of recurrent diaphragmatic hernia using spiral tacks to fix the mesh in a neonate was also reported recently by Riquelme et al. 15 In our cases, no intraoperative complications, bleeding, or abnormal positioning occurred during tack application. In addition, no displacement of the tacks and no hernia recurrence were observed in the immediate postoperative period suggesting that the anchoring of the mesh was reliable. The presence of the helicoidal tacks in the anatomic position of the diaphragmatic defect without any damage to adjacent structures was confirmed through imaging studies during the follow-up period. In the remaining pulmonary parenchyma, no eventration or herniation was seen up to 4 years and 3 months, and 5 ½ years postoperatively for the first and second cases, respectively, indicating that the repair of the diaphragm was definitive and the placement of the tacks appropriate.

studies during the follow-up period. In the remaining pulmonary parenchyma, no eventration or herniation was seen up to 4 years and 3 months, and 5 ½ years postoperatively for the first and second cases, respectively, indicating that the repair of the diaphragm was definitive and the placement of the tacks appropriate. In both cases, we placed the spiral tacks with care to ensure their stability and to minimize protrusion. This procedure was challenging because of the small size of the operating field. It was sometimes necessary to change the position of the ProTack device, to achieve better placement of the tacks. As these devices are seldom used in the thorax, we used suggestions and information in the literature from adults laparoscopic procedures using ProTacks. Despite the widespread use of tacker mesh fixation in the repair of hernias worldwide in adults, only a few complications are reported, such as adhesion, small bowel obstruction, and perforation or volvulus. 16 17 Erosion, perforation, displacement, and hemorrhage could be potential complications in our cases. Careful placement of foreign bodies to ensure their stability and to minimize protrusion or contact with important and fragile structures seems to be the key to decrease the risk of displacement or erosion of the hardware, which could lead to serious complications. Newly designed deployment instruments incorporate an articulating shaft or a hinge mechanism allowing for improved access to different parts of the cavity, and delivering perpendicular placement of tacks more easily. 18 Ideally, the size of these devices should be adapted to the pediatric population.

ous complications. Newly designed deployment instruments incorporate an articulating shaft or a hinge mechanism allowing for improved access to different parts of the cavity, and delivering perpendicular placement of tacks more easily. 18 Ideally, the size of these devices should be adapted to the pediatric population. Absorbable tacks offer an alternative to the nonabsorbable metal ones. 5 The risk of recurrence after mesh fixation by absorbable tacks has been found to be similar to that of nonabsorbable devices by some reports, but it was increased in other series. 5 6 With resorbable devices, the foreign material, which is potentially associated with severe complications, does not remain in the body permanently. Only one case reported a complication due to absorbable tackers used in thoracoscopic CDH repair, which was probably due to suboptimal application. 19 In an experimental study, spiral titanium tacks provided better fixation than absorbable tacks at both perpendicular and acute angles and should be strongly considered when perpendicular tack deployment cannot be achieved like in diaphragmatic hernia repair. 20 Glue mesh fixation is also well known in laparoscopic inguinal hernia repair. In a meta-analysis, glue mesh fixation was compared with spiral tacks in 1,001 patients. 21 There were fewer risks for developing chronic groin pain in the “glue” group. Our two patients have not complained of any pain. The use of this material has been already reported in neonatal cases as sealants over esophageal anastomosis and was found to be both safe and effective. 22

spiral tacks in 1,001 patients. 21 There were fewer risks for developing chronic groin pain in the “glue” group. Our two patients have not complained of any pain. The use of this material has been already reported in neonatal cases as sealants over esophageal anastomosis and was found to be both safe and effective. 22 Conclusion This report of the two cases indicates that mesh fixation using spiral tacks by means of thoracoscopy in the repair of large CDH in very young children is technically feasible even in case of CDH recurrence. The ProTack device offers a simple, quick, and safe method to achieve excellent fixation. Although no major complications were observed in our cases, a larger experience with this device is needed to recommend its use. Conflict of Interest None.

Introduction Pediatric Horner's syndrome is rare, occurring in 1.42 per 100,000 children below the age of 19 years. 1 It is a combination of symptoms that occur when the oculosympathetic pathway is interrupted. Horner's syndrome causes the clinical triad of miosis, ptosis, and anhidrosis on the ipsilateral side of the lesion. The location of the lesion can be central (first neuron lesion), preganglionic (second neuron), or postganglionic (third neuron). 2 In children, mainly the preganglionic nerves are affected. 3 The etiology of pediatric Horner's syndrome can be divided into acquired and congenital causes. Acquired causes comprise surgery of the head, neck, and chest (42%). Horner's syndrome can also be caused by trauma, vascular malformations, neoplasms (e.g., neuroblastoma), and infections. About 15% of cases suffer from acquired causes other than surgery. 4 Congenital causes comprise birth trauma, neoplasms, infections, and carotid malformations, but often the cause of the lesion remains undefined. 5 In congenital Horner's syndrome or Horner's syndrome which occurred very early in life, heterochromia, which is a lighter colored iris in the affected eye, as well as Harlequin's sign, an asymmetric flush with hidrosis and redness of the face on the nonaffected side of the head, occur. 6

the lesion remains undefined. 5 In congenital Horner's syndrome or Horner's syndrome which occurred very early in life, heterochromia, which is a lighter colored iris in the affected eye, as well as Harlequin's sign, an asymmetric flush with hidrosis and redness of the face on the nonaffected side of the head, occur. 6 If Horner's syndrome is suspected, a cocaine test will confirm the diagnosis. To locate the lesion, a Paredrine test containing 4-hydroxyamphetamine, which normally results in noradrenaline release, is used. If the lesion is postganglionic, there is no dilatation because the transmitter vesicles cannot be released. In central or preganglionic lesions, mydriasis occurs because the postganglionic nerves remain intact and are able to produce noradrenaline. Because cocaine also blocks uptake of hydroxyamphetamine, these two tests should not be done on the same day. Alternative methods to detect the location of the lesion comprise sweat test (searching for anhidrosis) and magnetic resonance imaging (MRI) scan. In congenital Horner's syndrome, pharmacological testing is difficult and often remains inconclusive. Thus, a contrast-enhanced MRI scan of the head, neck, and chest and an urinary catecholamine metabolite test to exclude the presence of neuroblastoma are often the only diagnostic tests applied. 6 7

imaging (MRI) scan. In congenital Horner's syndrome, pharmacological testing is difficult and often remains inconclusive. Thus, a contrast-enhanced MRI scan of the head, neck, and chest and an urinary catecholamine metabolite test to exclude the presence of neuroblastoma are often the only diagnostic tests applied. 6 7 Change of hair texture caused by Horner's syndrome is a rare phenomenon and has only been described sporadically in the literature. 8 All human hair fibers exhibit the same basic structure. However, it is the three-dimensional structure of the entire fiber that determines whether the hair appears straight or curled. According to recent research, hair shape is determined by genetic and biological factors. 9 A curled hair has an elliptical or “D”-like cross-section. During hair growth, the ellipse changes to form a coil. Since the follicle of a highly curled hair appears curved, it is suggested that a curly follicle produces a curled hair, and asymmetry of the follicle results in curled hair formation regardless of ethnicity. However, it remains unknown what causes curved follicles and which factors are responsible for asymmetry of the hair follicle. 9 10 We report a rare case of ipsilateral straight hair in association with acquired Horner's syndrome in an infant and discuss the possible mechanisms for this phenomenon.

Since the follicle of a highly curled hair appears curved, it is suggested that a curly follicle produces a curled hair, and asymmetry of the follicle results in curled hair formation regardless of ethnicity. However, it remains unknown what causes curved follicles and which factors are responsible for asymmetry of the hair follicle. 9 10 We report a rare case of ipsilateral straight hair in association with acquired Horner's syndrome in an infant and discuss the possible mechanisms for this phenomenon. Case Report A Caucasian baby girl, weighing 3225 g, was delivered in the 37th week of gestation by cesarean section after amniotic fluid was lost. Ultrasonic examination during pregnancy showed a huge cervical mass. The diagnosis of a giant lymphangioma of the neck was suspected after MRI. Ultrasonic and MRI scans showed the mass to be composed of multiple cysts of different size with a few solid parts ( Fig. 1A , B ). It was located predominantly on the right side of the infant's neck, extending over the midline anteriorly. The tumor caused a tilting of her neck to the left. Because there was no massive compression of the trachea, we decided to postpone surgery beyond the neonatal period. The girl was discharged from hospital at the age of 6 days with home pulsoxymetric surveillance. Regular visits to the outpatient clinic were arranged.

The tumor caused a tilting of her neck to the left. Because there was no massive compression of the trachea, we decided to postpone surgery beyond the neonatal period. The girl was discharged from hospital at the age of 6 days with home pulsoxymetric surveillance. Regular visits to the outpatient clinic were arranged. Fig. 1 ( A ) Duplex sonography of the right neck region obtained on the first day of life showing the vertebral artery and multiple hypoechogenic cysts of different size representing a mixed-type lymphangioma. ( B ) Magnetic resonance imaging scan of the neck region showing multiple cysts of different size extending from the skull base to the upper thoracic aperture in the anterior region of the neck. Operative Intervention During the operation at week 5 of the infant's life, we noted that the main vessels and nerves on the right side of the neck were completely entrapped by the multicystic lymphangioma. We undertook a subtotal excision of the mixed-type lymphangioma, due to the presence of dense adhesions between the lymphangioma and vessels and nerves at the right side of the neck. Small remnants of the cystic lymphangioma in the area of the upper thoracic aperture and in close vicinity to the trachea, skull base, and recurrent laryngeal nerve were not removed. We preserved the trunk of the transverse cervical nerve, but severed some minor peripheral nerve branches to facilitate the removal of the central part of the lymphangioma.

ngioma in the area of the upper thoracic aperture and in close vicinity to the trachea, skull base, and recurrent laryngeal nerve were not removed. We preserved the trunk of the transverse cervical nerve, but severed some minor peripheral nerve branches to facilitate the removal of the central part of the lymphangioma. Eight days after surgery, the girl was able to breath and drink without any difficulties and was discharged from hospital. The surgical wound healed well. The patient's parents reported that her voice was moderately weakened. We opted against a laryngoscopic examination because the weakening of the voice improved steadily. Postoperatively, we noted that the girl's right eyelid was positioned lower than her left eyelid, and her right palpebral fissure was smaller when compared with the left ( Fig. 2 ). We diagnosed Horner's syndrome and referred her to the ophthalmologists for diagnostic workup and treatment. Horner's syndrome was confirmed. The infant showed partial ptosis of her right eyelid, and the diameter of the right pupil was smaller when compared with the left eye, with preserved pupillary light reflex. In addition, we prescribed physiotherapy to improve the moderate limitation of active elevation and abduction of her right shoulder and arm and to correct the tilting of her neck. Fig. 2 Photograph taken 4 months after subtotal resection of the lymphangioma. Miosis and ptosis of the eye affected by Horner's syndrome. There is no heterochromia of the iris.

Postoperatively, we noted that the girl's right eyelid was positioned lower than her left eyelid, and her right palpebral fissure was smaller when compared with the left ( Fig. 2 ). We diagnosed Horner's syndrome and referred her to the ophthalmologists for diagnostic workup and treatment. Horner's syndrome was confirmed. The infant showed partial ptosis of her right eyelid, and the diameter of the right pupil was smaller when compared with the left eye, with preserved pupillary light reflex. In addition, we prescribed physiotherapy to improve the moderate limitation of active elevation and abduction of her right shoulder and arm and to correct the tilting of her neck. Fig. 2 Photograph taken 4 months after subtotal resection of the lymphangioma. Miosis and ptosis of the eye affected by Horner's syndrome. There is no heterochromia of the iris. At the age of 3 months, the residual swelling of the infant's neck on the right side had subsided spontaneously, and her voice had recovered. However, Horner's syndrome was still present but showed further improvement. Movement of her right arm and shoulder had improved. When the girl was 6 months old, her parents reported that her hair texture had changed. At birth, her hair was brown and straight. After birth, the color of the hair turned from brownish to blond, and the hair texture became more curled on both sides of the head. Four months after surgery, the hair on the left side of her head was still curly, while the hair on the right side had turned straight ( Figs. 3 , 4A , B ).

r hair was brown and straight. After birth, the color of the hair turned from brownish to blond, and the hair texture became more curled on both sides of the head. Four months after surgery, the hair on the left side of her head was still curly, while the hair on the right side had turned straight ( Figs. 3 , 4A , B ). Fig. 3 Photograph taken 6 months after the operation. Straight hair is visible on the right side of the head. The right side of the head is affected by Horner's syndrome. The original hair texture of the infant is retained on the left side of the head. Fig. 4 Photograph taken at 16 months. ( A ) Straight hair on the right side of her head which is affected by Horner's syndrome. ( B ) The original hair texture of the infant is retained on the left side of the head which is not affected by Horner's syndrome. At the age of 9.5 months, the ophthalmologist reported that ptosis had improved and the girl's vision had remained stable with no noticeable difference in the visual acuity between her eyes. We noted a minimal facial asymmetry due to her lymphangioma on the right side. Movement of her right shoulder and arm showed minimal restriction of elevation and abduction. Her psychomotor development was normal for age. Follow-up MRI scan of the neck at the age of 1 year revealed a small residual part of the lymphangioma in close vicinity of the larynx, and we therefore opted against sclerotherapy. The residual cysts became smaller spontaneously.

We noted a minimal facial asymmetry due to her lymphangioma on the right side. Movement of her right shoulder and arm showed minimal restriction of elevation and abduction. Her psychomotor development was normal for age. Follow-up MRI scan of the neck at the age of 1 year revealed a small residual part of the lymphangioma in close vicinity of the larynx, and we therefore opted against sclerotherapy. The residual cysts became smaller spontaneously. At the age of 2.5 years, ultrasonic imaging showed several cysts measuring 3 mm that were localized close to her right thyroid lobe. Horner's syndrome had improved further but moderate ptosis of the right eyelid was still present. There was no impairment of her visual acuity. Her hair had normalized, and the hair texture was curly on both sides of her head. The tilting of the neck had subsided, and we noted no scoliosis. At this point, we stopped physiotherapy. Discussion Our patient showed some complications after surgery in the neck region. The transient weakening of the voice had most likely occurred due to the preparation of the laryngeal recurrent nerve on the affected side. 11 The laryngeal recurrent nerve was running in close vicinity of the medial circumference of the lymphangioma. The weakening of the voice improved steadily with time.

neck region. The transient weakening of the voice had most likely occurred due to the preparation of the laryngeal recurrent nerve on the affected side. 11 The laryngeal recurrent nerve was running in close vicinity of the medial circumference of the lymphangioma. The weakening of the voice improved steadily with time. The motor impairment of the ipsilateral shoulder was caused by partial weakness of the upper part of the trapezius muscle. This part is innervated mainly by the spinal accessory nerve and branches of the cervical plexus. 12 Muscle function improved with physiotherapy over time. We, therefore, did not conduct neurophysiologic examination. The infant developed unilateral Horner's syndrome after subtotal excision of a large cervical lymphangioma of the neck. In addition to the usual symptoms, our patient experienced a striking symptom: her curly hair turned nearly straight on the side of the head affected by Horner's syndrome. We hypothesized that this phenomenon was associated with Horner's syndrome and was not due to spontaneous change of hair texture. The change in hair structure was not obvious until a few months after surgery. Other authors did not mention at what time point the change of hair structure appeared. 2 3 8 We do not believe that this phenomenon was due to spontaneous change of hair texture during growth of the child because this would take longer than just a few months. 10

ucture was not obvious until a few months after surgery. Other authors did not mention at what time point the change of hair structure appeared. 2 3 8 We do not believe that this phenomenon was due to spontaneous change of hair texture during growth of the child because this would take longer than just a few months. 10 We are not aware of the exact pathophysiological mechanism that caused the unilateral change of hair curliness in our patient. Shewmon 13 speculated that the reason for ipsilateral straight hair might be the loss of trophic effect of sympathetic stimulation. Similarly, the loss of trophic effect on the melanocytes in the iris results in a paler color of the iris.

cal mechanism that caused the unilateral change of hair curliness in our patient. Shewmon 13 speculated that the reason for ipsilateral straight hair might be the loss of trophic effect of sympathetic stimulation. Similarly, the loss of trophic effect on the melanocytes in the iris results in a paler color of the iris. Another investigation, partly based on a study in sheep, suggested that the arrectores pilorum muscles may cause curly hair by rhythmic contractions. These muscles are innervated by sympathetic neurons. Thus, it seems reasonable that sympathetic denervation is associated with the occurrence of straight hair. 14 We consider this hypothesis somewhat unlikely since Thibaut et al 15 demonstrated, in 2007, that the arrectores pilorum muscles are not involved in the formation of curly hair. As part of the study, they separated the hair follicles from the muscles and sebaceous glands and implanted them in vitro, but the hair still grew curly. These authors concluded that the human hair shape is probably programmed by the hair bulb located at the basal area of the hair follicle. 15 Thus, the way how sympathetic nerves influence hair structure at the level of the hair follicles remains undefined. Similarly, it is not known at what age a lesion in the sympathetic pathway can lead to a change in hair curliness.

s probably programmed by the hair bulb located at the basal area of the hair follicle. 15 Thus, the way how sympathetic nerves influence hair structure at the level of the hair follicles remains undefined. Similarly, it is not known at what age a lesion in the sympathetic pathway can lead to a change in hair curliness. Reports in the medical literature exclusively describe children aged between 3 weeks and 2 years suffering from this hair texture phenomenon, and in contrast to our patient, all children suffered from congenital Horner's syndrome. Congenital Horner's syndrome is defined as a disorder manifesting in the first month after birth. 8 However, we cannot exclude the possibility that Horner's syndrome was caused by the lymphangioma in our patient. Occurrence of Horner's syndrome due to neuroblastoma has been described in the literature. 16 Our patient showed no sign of Horner's syndrome before surgery nor was her iris involved which represents a typical symptom of congenital Horner's syndrome. 16 Previously described children with change of hair texture exhibited similar features, with the exception of a 10-month old girl. The girl suffered from right-sided Horner's syndrome and was later diagnosed with neuroblastoma. 17 After thoracoscopic resection and chemotherapy, the patient developed alopecia. Six months after treatment start, her hair had partially regrown, but only on the left side of her head. To our knowledge, this is the first report of unilateral hair growth in a patient suffering from Horner's syndrome. 17

euroblastoma. 17 After thoracoscopic resection and chemotherapy, the patient developed alopecia. Six months after treatment start, her hair had partially regrown, but only on the left side of her head. To our knowledge, this is the first report of unilateral hair growth in a patient suffering from Horner's syndrome. 17 We found one literature report on a mild phenotype of giant axonal neuropathy which resulted in a change of hair texture in affected children. 18 This phenomenon in giant axonal neuropathy may be linked to a change of hair texture in acquired Horner's syndrome in young children.

euroblastoma. 17 After thoracoscopic resection and chemotherapy, the patient developed alopecia. Six months after treatment start, her hair had partially regrown, but only on the left side of her head. To our knowledge, this is the first report of unilateral hair growth in a patient suffering from Horner's syndrome. 17 We found one literature report on a mild phenotype of giant axonal neuropathy which resulted in a change of hair texture in affected children. 18 This phenomenon in giant axonal neuropathy may be linked to a change of hair texture in acquired Horner's syndrome in young children. To the best of our knowledge, there are no reports on adults who experienced change in hair texture due to acquired Horner's syndrome. Therefore, we assume that the loss of sympathetic stimulation has no significant influence on hair texture in adults. However, change of hair texture from curly to straight hair has been described after the treatment of hepatitis C virus infection and alopecia. 19 20 Inhibition or mutation of the epidermal growth factor receptor has been reported to change the hair texture and causing eyelash trichomegaly 21 In adults, a change of hair texture occurs very rarely, and causes are unrelated to Horner's syndrome. We report the rare case of an infant in whom Horner's syndrome occurred after surgical resection of a giant lymphangioma of the neck region. Horner's syndrome was accompanied by an ipsilateral change in hair texture. The change of hair texture showed gradual regression, and also Horner's syndrome improved with time. The pathway by which Horner's syndrome influences hair texture remains poorly understood. Further investigations are required to examine the cause of this phenomenon.

ccompanied by an ipsilateral change in hair texture. The change of hair texture showed gradual regression, and also Horner's syndrome improved with time. The pathway by which Horner's syndrome influences hair texture remains poorly understood. Further investigations are required to examine the cause of this phenomenon. Conclusion Pediatricians should be aware that unilateral straight hair in children may be a symptom of Horner's syndrome. Conflict of Interest None.

Introduction Marfan's syndrome (MS) is a systemic connective tissue disorder which mainly involves ocular, skeletal, and cardiovascular systems. 1 Antoine Marfan described the first case in 1896 2 and its current frequency is estimated to be 1:5,000 to 1:10,000, without enrichment in any ethnic or racial group and no gender preference. It is caused by an autosomal dominant mutation of FBN1 with a high degree of clinical variability, ranging from isolated features to severe and rapidly progressive disease in multiple organ systems. 3 Gastrointestinal symptoms are usually rare. Most of the time, this system only gets involved by noncomplicated inguinal hernias which are the most frequently reported hernias in MS. 2 4 However, despite being really rare, hiatal hernia, paraesophageal hernia, and intrathoracic stomach have all been reported in MS and are thought to be due to the abnormality of gastric ligaments and diaphragm. 4 5 These entities become important because of their tendency to relapse and the possibility of triggering extremely rare but critical situations such as a gastric volvulus. 1 6 Various types of hernias are seen in MS, such as inguinal hernias and an occasional hiatus hernia. Classically, MS diagnosis has been based on family history and the observation of its characteristic findings. In fact, ectopia lentis and aortic aneurysm have a special clinical significance because of their specificity and frequency. 3 7 Instead, the sensitivity of molecular genetic testing of FBN1 is substantial yet incomplete because of its large heterogeneity. 1

y history and the observation of its characteristic findings. In fact, ectopia lentis and aortic aneurysm have a special clinical significance because of their specificity and frequency. 3 7 Instead, the sensitivity of molecular genetic testing of FBN1 is substantial yet incomplete because of its large heterogeneity. 1 MS rarely presents with symptoms in the neonatal period. 2 6 Our objective is to report one of these rare cases of MS that debuted with the complete herniation of the stomach into the thorax through a large hiatal hernia. Clinical Report A 12-day-old male infant was admitted with recurrent nonbilious vomiting. The patient was born to a 35-old-gravida by spontaneous vaginal delivery at 40 weeks, weighing 2,868 g, after a controlled pregnancy and a threat of preterm delivery at 30 + 6 weeks solved with tocolytic and corticoid treatment. The Appearance, Pulse, Grimace, Activity, and Respiration scores were 9 at 1 minute and 10 at 5 minutes. The mother and the patient displayed features of MS. The patient was a small, thin infant with “senile” appearance ( Fig. 1 ), micrognathia, crumpled ears, and reduced muscle mass and subcutaneous tissue. The fingers were long, overlapping, and mildly hyperextensible and his wrists were deviated ulnarly. He showed flexion deformities with difficulty in extending the elbows, hips, and knees. Left inguinal hernia was also present. Fig. 1 Patient's senile appearance. The parents reported that the maternal grandfather had a similar phenotype but none of them had previous cardiac events.

The mother and the patient displayed features of MS. The patient was a small, thin infant with “senile” appearance ( Fig. 1 ), micrognathia, crumpled ears, and reduced muscle mass and subcutaneous tissue. The fingers were long, overlapping, and mildly hyperextensible and his wrists were deviated ulnarly. He showed flexion deformities with difficulty in extending the elbows, hips, and knees. Left inguinal hernia was also present. Fig. 1 Patient's senile appearance. The parents reported that the maternal grandfather had a similar phenotype but none of them had previous cardiac events. A nasogastric tube was placed, obtaining nonbile stained gastric content. Ultrasound showed no stomach at its normal position, but a compatible structure was observed through the right diaphragm and an intrathoracic gas bubble with the nasogastric tube inside was seen ( Fig. 2 ). Blood test was completely normal. The diagnostic workup completed by an upper gastrointestinal water-soluble contrast study, confirming the complete herniation of the stomach into the right thorax ( Fig. 3 ). The images evinced a partially volvulating stomach with pylorus on top and the duodenum crossing the hiatus, making the passage of the content to the intestine much more difficult. Fig. 2 Chest X-ray showing an intrathoracic gas bubble compatible with stomach. Fig. 3 Upper gastrointestinal contrast study confirming the complete herniation of the stomach into the left thorax. Subsequent echocardiography documented mildly dilated ascending aorta. Ophthalmic examination was normal.

A nasogastric tube was placed, obtaining nonbile stained gastric content. Ultrasound showed no stomach at its normal position, but a compatible structure was observed through the right diaphragm and an intrathoracic gas bubble with the nasogastric tube inside was seen ( Fig. 2 ). Blood test was completely normal. The diagnostic workup completed by an upper gastrointestinal water-soluble contrast study, confirming the complete herniation of the stomach into the right thorax ( Fig. 3 ). The images evinced a partially volvulating stomach with pylorus on top and the duodenum crossing the hiatus, making the passage of the content to the intestine much more difficult. Fig. 2 Chest X-ray showing an intrathoracic gas bubble compatible with stomach. Fig. 3 Upper gastrointestinal contrast study confirming the complete herniation of the stomach into the left thorax. Subsequent echocardiography documented mildly dilated ascending aorta. Ophthalmic examination was normal. The decision for surgical exploration was made through a longitudinal supraumbilical laparotomy. We confirmed the presence of a large stomach, volvulating over its mesenteroaxial axis and almost completely herniated ( Fig. 4 ) through a wide diaphragmatic hiatus ( Fig. 5 ). The transverse colon was also forming part of the hernia. A hiatal dissection was performed after reducing the organs into the abdomen by pulling them carefully with atraumatic surgical instruments. Using a large nasogastric tube into the esophagus and stomach, the hiatus was repaired with an absorbable mesh reinforcement (Vicryl, Ethicon). A modified Thal fundoplication was performed. To guarantee the fixation of the stomach on its normal position, a gastrostomy (Stamm's technique) was realized.

ical instruments. Using a large nasogastric tube into the esophagus and stomach, the hiatus was repaired with an absorbable mesh reinforcement (Vicryl, Ethicon). A modified Thal fundoplication was performed. To guarantee the fixation of the stomach on its normal position, a gastrostomy (Stamm's technique) was realized. Fig. 4 Large and partially volvulating stomach, almost completely herniated into the thorax. Fig. 5 Wide diaphragmatic hiatus. After surgery, the infant was admitted to the neonatal intensive care unit. The postoperative course was uneventful. Oral feedings were commenced on the third day, being gradually advanced and tolerated. He was discharged with an adequate and exclusive oral tolerance after 7 days. He was readmitted 3 months later for left inguinal hernia repair and gastrostomy withdrawal. During the later life, genetic consultation confirmed the diagnosis of MS in the patient and his mother. After 2 years of follow-up, the patient remains asymptomatic maintaining an adequate oral tolerance and an appropriate weight gain. Discussion Although the diagnosis of MS is more common in adolescence and adulthood because of the typical body habitus and the development of the most typical complications (mitral valve prolapse and other cardiological events), 2 it is important to know that it may also debut in the neonatal period.

After 2 years of follow-up, the patient remains asymptomatic maintaining an adequate oral tolerance and an appropriate weight gain. Discussion Although the diagnosis of MS is more common in adolescence and adulthood because of the typical body habitus and the development of the most typical complications (mitral valve prolapse and other cardiological events), 2 it is important to know that it may also debut in the neonatal period. We should suspect this condition in a newborn with abnormal habitus, heart valve insufficiency, cutis laxa, crumpled ears, joint contractures, muscle hypoplasia, congenital anomalies of the eye, or pulmonary hypoplasia. This might occur with a negative family history for MS. Various types of hernias are also seen in MS, such as inguinal hernias 4 and an occasional sliding hiatal hernia because of the unusual elasticity of tissues. 2 4 5 8 Lax gastric ligaments (gastrohepatic, gastrophrenic, gastrosplenic, gastrocolic) and a deficient diaphragmatic hiatus predisposes to it. 2 5 8 In this case, patients usually display symptoms of preduodenal intestinal obstruction like nonbilious vomiting 4 6 8 or respiratory distress and a Marfan's phenotype or familiar history. 5 8 We should consider this entity in patients that present these characteristics because prompt recognition can facilitate management and counseling. After confirmation of the diagnosis, it is essential to reposition the stomach into the abdomen to avoid gastric necrosis by a complex gastric volvulus. 5 8 9

In this case, patients usually display symptoms of preduodenal intestinal obstruction like nonbilious vomiting 4 6 8 or respiratory distress and a Marfan's phenotype or familiar history. 5 8 We should consider this entity in patients that present these characteristics because prompt recognition can facilitate management and counseling. After confirmation of the diagnosis, it is essential to reposition the stomach into the abdomen to avoid gastric necrosis by a complex gastric volvulus. 5 8 9 After the hiatal repair, we chose to place an absorbable mesh to induce fibrosis and therefore local reinforcement. We chose a Vicryl mesh to avoid the presence of a foreign material in the long term. Nevertheless, a funduplication and gastrostomy must be considered as a part of the surgical approach to prevent reflux and recurrence. 5 8 9 It is important to emphasize that gastrostomy is only performed to fix the stomach. If possible, it should not be used for feeding to preserve the correct development of the feeding/swallowing mechanism in the newborn. It might be withdrawn after 3 to 6 months if the patient's clinical condition allows it. In summary, MS should be considered in any infant with hiatal/paraesophageal hernia, which should be repaired early to avoid gastric volvulus. These infants require long-term multidisciplinary follow-up to detect recurrences and other early complications of MS. 8 9 10 New Insights and the Importance for a Pediatric Surgeon Prompt recognition of this entity can facilitate management and counseling, avoiding ischemic necrosis by a complex gastric volvulus.

In summary, MS should be considered in any infant with hiatal/paraesophageal hernia, which should be repaired early to avoid gastric volvulus. These infants require long-term multidisciplinary follow-up to detect recurrences and other early complications of MS. 8 9 10 New Insights and the Importance for a Pediatric Surgeon Prompt recognition of this entity can facilitate management and counseling, avoiding ischemic necrosis by a complex gastric volvulus. Conflict of Interest None.

Introduction Establishing vascular access during cardiopulmonary resuscitation is essential due to the need for immediate administration of drugs and the possibility of volume therapy. Routine peripheral vein cannulation may be difficult in several situations, including extensive multiple trauma with hemorrhagic shock and centralized circulation or extensive burns. One of the possible alternatives is intraosseous (IO) access, a relatively quick and easy option for the administration of drugs or larger amounts of fluid. The authors present a very serious complication in this IO access that occurred during resuscitation. The aim of the work presented here is to point out that even a commonly used method for establishing vascular access can lead to very severe complications. Case Report

Introduction Establishing vascular access during cardiopulmonary resuscitation is essential due to the need for immediate administration of drugs and the possibility of volume therapy. Routine peripheral vein cannulation may be difficult in several situations, including extensive multiple trauma with hemorrhagic shock and centralized circulation or extensive burns. One of the possible alternatives is intraosseous (IO) access, a relatively quick and easy option for the administration of drugs or larger amounts of fluid. The authors present a very serious complication in this IO access that occurred during resuscitation. The aim of the work presented here is to point out that even a commonly used method for establishing vascular access can lead to very severe complications. Case Report The emergency medical service (EMS) was called by the parents of a 2.5-month-old infant. The parents of a previously healthy infant reported a sudden loss of consciousness and respiratory arrest. The ambulance crew arrived 8 minutes after the initial call and found the infant in cardiac arrest. The parents had started resuscitation according to instructions from the EMS dispatcher over the phone (∼9 minutes). Cardiopulmonary resuscitation was immediately commenced by the EMS physician. After an unsuccessful attempt to insert a peripheral venous catheter, IO access into the left tibial tuberosity was performed to provide vascular access. The heart rate was restored ∼2 minutes after the start of medical resuscitation. The infant was intubated using a nasotracheal tube and brought to the emergency unit and later to the intensive care and resuscitation unit (ICRU) of the Pediatric Department at the University Hospital. Upon admission to the ICRU, the patient was described as being nonreactive, with mydriatic pupils and unmeasurable blood pressure. Inotropic support (initiated at the scene) was continued with a high-dose adrenaline. During admission, a swelling of the left lower limb (the side with inserted IO cannula) was immediately appreciated. Due to the suspected extravasation and extramedullary distribution of catecholamines and crystalloids, identical IO access was established on the lower right limb and the IO needle on the left leg was removed. A central venous catheter was later introduced via the left subclavian vein. Baseline laboratory tests revealed leukocytosis with a left shift, procalcitonin elevation, laboratory signs of severe hepatopathy and coagulopathy. Clinical signs of multiple organ failure were present. After ∼120 minutes, the infant's condition was stabilized, and acute computed tomography scan (CT brain scan + the whole body CT scan) was performed, which revealed extensive thrombosis of the cerebral veins and generalized visceral and limb vasoconstriction. About 3 hours after admission, a vascular surgeon was contacted due to increased edema and livid discoloration of the lower left leg.

uted tomography scan (CT brain scan + the whole body CT scan) was performed, which revealed extensive thrombosis of the cerebral veins and generalized visceral and limb vasoconstriction. About 3 hours after admission, a vascular surgeon was contacted due to increased edema and livid discoloration of the lower left leg. The vascular surgeon evaluated the limb as a combination of iatrogenic compartment syndrome (due to extravasation of catecholamines and crystalloids) and limb ischemia. He described a hard swelling of the entire lower leg and livid discoloration of almost the entire limb ( Fig. 1 ). Urgent fasciotomy of all muscle beds was indicated as the only way to save the limb with two incisions as usual from two skin incisions (medially and laterally to the tibial edge [ Fig. 2] ). The muscles extruded immediately after cutting the fascia. Large areas of inferior and posterior groups of the muscles were grayish and not likely vital. An incision was also made in the dorsal foot area, where marked swelling was also present. The fasciotomy wounds were covered with a lubricated, moist dressing. A dressing change was scheduled at 48 hours post fasciotomy. During these 2 days, the infant remained in very serious condition, and was on permanent circulatory support. An EEG scan revealed a severely abnormal electroencephalogram, with a very depressed background with periodic occurrence of burst suppression EEG pattern. The EEG pattern corresponds to an unfavorable prognosis. Two days later, the dressing on the lower left limb was changed and clearly necrotic tissues were removed. The vitality of most muscles was still very unlikely. In the following period, the patient's condition stabilized, but the cause of circulatory arrest remained unknown and severe consciousness disorder continued. The dressing on the lower left leg was changed every 2 days in the operating room. Full conservative anticoagulation and vasodilation therapy were still indicated. The finding on the limb gradually deteriorated, and the patient developed skin necrosis ( Fig. 3 ) that was gradually removed along with a part of the muscles. While in comprehensive intensive care, the infant's overall condition and consciousness disorder improved, but the local finding on the left lower limb significantly progressed and resulted in above knee amputation on day 19 after the admission ( Fig. 4 ).

) that was gradually removed along with a part of the muscles. While in comprehensive intensive care, the infant's overall condition and consciousness disorder improved, but the local finding on the left lower limb significantly progressed and resulted in above knee amputation on day 19 after the admission ( Fig. 4 ). The amputation stump healed without complications, the overall condition continued to improve, and the infant was discharged to home care on day 51 after the admission. During the hospitalization, no clear explanation was found for the primary cause of the cardiac arrest. No congenital developmental defects and no mitochondrial defects were found. As a result of postischemic brain damage, recurrent epileptic attacks occur. A magnetic resonance imaging scan of the brain performed (1 month after the event) showed clear postischemic changes. The extent of brain tissue damage will be revealed during further development of the infant. Fig. 1 Left lower limb on admission, a visible insertion site after intraosseous access. Fig. 2 Fasciectomies performed. Fig. 3 Limb gangrene. Fig. 4 Above knee amputation. Discussion

The amputation stump healed without complications, the overall condition continued to improve, and the infant was discharged to home care on day 51 after the admission. During the hospitalization, no clear explanation was found for the primary cause of the cardiac arrest. No congenital developmental defects and no mitochondrial defects were found. As a result of postischemic brain damage, recurrent epileptic attacks occur. A magnetic resonance imaging scan of the brain performed (1 month after the event) showed clear postischemic changes. The extent of brain tissue damage will be revealed during further development of the infant. Fig. 1 Left lower limb on admission, a visible insertion site after intraosseous access. Fig. 2 Fasciectomies performed. Fig. 3 Limb gangrene. Fig. 4 Above knee amputation. Discussion Immediate intravenous (IV) access for the administration of drugs or infusions is absolutely necessary during resuscitation and more generally in urgent or intensive medicine. Access to the bone marrow may be beneficial when IV access cannot be established for any reason. The IO administration route was first introduced by Drinker in 1922. Through IO access, infusions may be administered at the same rate as IV administration. Some intensive care guidelines recommend using IO access after repeated failure of attempts to establish IV access. During the resuscitation, IO access can be established even after the first failed IV access attempt. 1 Several systems available on the market differ by method (punching, drilling, etc.). The most common sites for intramedullary access are the proximal tibia (suitable for children), humeral head (more common in adults), or sternal manubrium. The most commonly reported complications of IO access are infection at the injection site, which may result in severe osteomyelitis, damage to the growth plate, and fat embolism, 2 which have been reported in adults. Dislocation of the needle followed by extravasation should also be considered (analogy of IV cannula dislocation 3 ). In this case report, very severe complications occurred after the malposition of the IO needle during the resuscitation, and subsequent extravasation of both the crystalloid infusion and inotropic support. In our opinion, two mechanisms contributed to the development of these serious findings in our case: a combination of a mechanical insult due to extravasation of the crystalloid and the vasoconstrictor mechanism. In the infant patient, total visceral and limb vasoconstriction occurred. The local influence of adrenalin leakage further complicated the situation. In the medical facility, the malposition of the IO needle was immediately detected and the needle was removed. Complications associated with the dislocation of the IO needle are more likely in obese people. Another risk factor is the necessity of extensive manipulation of the patient, such as during the resuscitation.

In the medical facility, the malposition of the IO needle was immediately detected and the needle was removed. Complications associated with the dislocation of the IO needle are more likely in obese people. Another risk factor is the necessity of extensive manipulation of the patient, such as during the resuscitation. The precise identification of the site of insertion is essential to avoid any complications of IO access. The most commonly used access site is the proximal tibial region; this is located 1 to 2 cm medially to the tibial tuberosity in adults and 1 cm medially and 1 cm distally to the tibial tuberosity in children. For a proximal humerus, this is located ∼2 cm above the surgical neck. The selection of an appropriate intramedullary needle size is also crucial. In newborns and infants/toddlers, limb compartment syndrome is very rare and is always associated with trauma or some invasive intervention. The most common site of occurrence is on the leg. 4 5 6 7 Late diagnosis of this complication is not uncommon and logically has worse outcomes. The consequences of this complication may be very serious; the loss of the limb is rare. The infant patient in our case, unfortunately, developed a combination of limb ischemia due to adrenaline extravasation, centralization due to circulatory instability, and compartment syndrome due to extravasation of crystalloids. The question is how to prevent such serious consequences in this complication of IO access, where early diagnosis and immediate intervention are more difficult due to the overall poor condition of the patient. In our case, even very fast diagnosis and surgical intervention could not prevent irreversible damage to the soft tissues of the limb.

revent such serious consequences in this complication of IO access, where early diagnosis and immediate intervention are more difficult due to the overall poor condition of the patient. In our case, even very fast diagnosis and surgical intervention could not prevent irreversible damage to the soft tissues of the limb. New Insights and the Importance for the Pediatric Surgeon Common use of intraosseous needle in newborn can have severe complication. Needle malposition can cause severe compartment syndrome and limb ischemia. Conflict of Interest None.

Introduction Single port access (single-incision pediatric endosurgery [SIPES]) for laparoscopic appendectomy is increasingly being used. 1 2 3 However, special ports to perform SIPES appendectomy may not be affordable in developing countries. 4 5 6 Moreover, commercially available SIPES ports are known to have a limited range of motion of the surgical instruments. 7 8 Therefore, alternatives to overcome both obstacles are warranted. In this technical report, we present our way to set up and carry out SIPES via a homemade glove port. Case Report

Single port access (single-incision pediatric endosurgery [SIPES]) for laparoscopic appendectomy is increasingly being used. 1 2 3 However, special ports to perform SIPES appendectomy may not be affordable in developing countries. 4 5 6 Moreover, commercially available SIPES ports are known to have a limited range of motion of the surgical instruments. 7 8 Therefore, alternatives to overcome both obstacles are warranted. In this technical report, we present our way to set up and carry out SIPES via a homemade glove port. Case Report A 10-year-old female presented to the emergency room with diffuse abdominal pain for 1 day, and two episodes of vomiting. The clinical and laboratory findings were consistent with appendicitis. Therefore, the girl was taken to the operating room and surgical table set up for SIPES appendectomy, while glove port was prepared ( Fig. 1 ). A 2-cm vertical incision was made in the fascia underlying the umbilicus to enter the peritoneal cavity. A wound retractor (Alexis, Size XS, Applied Medical Resources Corp., Rancho Santa Margarita, CA) was placed directly through the fascia, and a 6.5 size latex sterile powder-free surgical glove was connected to it ( Fig. 2 ). The thumb of the glove was cut off and a 5-mm trocar (Karl Storz, Germany) was introduced in the abdomen for CO 2 insufflation and introduction of the monopolar hook and tied to the wound retractor to prevent dislocation ( Fig. 3 ). A 5-mm 45-cm scope (Stryker Endoscopy, San Jose, CA) was connected to the light cord using a 90° angulated light adapter (Karl Storz) and introduced through a 2-mm incision in one of the finger tips. With standard reusable 5-mm straight laparoscopic instruments, introduced in the same technique as the camera, the appendix was identified, and the mesoappendix divided. The appendix was grabbed, the capnoperitoneum was deflated, and the appendix exteriorized and amputated over a polyglactin suture ligation extracorporeally. The fascial incision was approximated with a running 2–0 polyglactin suture. Finally, the skin incision was closed using interrupted subcuticular 4–0 poliglecaprone sutures ( Video 1 ). Histological examination confirmed the diagnosis of appendicitis. There were no intra- or postoperative complications. The patient was discharged on postoperative day 2.

ed with a running 2–0 polyglactin suture. Finally, the skin incision was closed using interrupted subcuticular 4–0 poliglecaprone sutures ( Video 1 ). Histological examination confirmed the diagnosis of appendicitis. There were no intra- or postoperative complications. The patient was discharged on postoperative day 2. Fig. 1 Equipment used for single-incision pediatric endosurgery with glove port. Fig. 2 Hitch stich to facilitate removal of the Alexis-Port. Fig. 3 The fixation of 5-mm trocar between the trocar valve and the Alexis-Port. Video 1 Creation of highly effective glove port and subsequently appendectomy procedure using the single-incision pediatric endosurgery (SIPES) technique. Online content including video sequences viewable at: www.thieme-connect.com/products/ejournals/html/10-1055-s-0038-1667140-EJPSR-18-0378-v1.mp4 . Discussion

Fig. 3 The fixation of 5-mm trocar between the trocar valve and the Alexis-Port. Video 1 Creation of highly effective glove port and subsequently appendectomy procedure using the single-incision pediatric endosurgery (SIPES) technique. Online content including video sequences viewable at: www.thieme-connect.com/products/ejournals/html/10-1055-s-0038-1667140-EJPSR-18-0378-v1.mp4 . Discussion SIPES provides additional advantages over traditional 3-port laparoscopy such as improved cosmetic and the ability to perform multiple procedures without the need for additional ports. 2 9 10 Glove ports rather than traditional ports can be used as a cost-effective method with improved ergonomics to circumvent the limitations of the previous single ports. 4 11 12 13 14 Furthermore, the elasticity and pliability of the surgical glove port compared with commercially available ports allows reasonable range of movements and achieves enough triangulation ( Fig. 4 ). This has been also shown by others. 14 However, despite several advantages of homemade glove port, the application for SIPES is not approved and can be an issue for some medical institutions. For patients with a potential for developing a latex allergy, nonlatex surgical gloves should be used. The current expenses for SIPES ports range from 318 to 1,378 USD 15 16 17 compared with glove ports based on equipment available for around 30 euro. 4 Fig. 4 Comparison of ranges of motion between homemade glove port and a commercially available device (Tri-Port, Olympus, Center Valley, PA).

SIPES provides additional advantages over traditional 3-port laparoscopy such as improved cosmetic and the ability to perform multiple procedures without the need for additional ports. 2 9 10 Glove ports rather than traditional ports can be used as a cost-effective method with improved ergonomics to circumvent the limitations of the previous single ports. 4 11 12 13 14 Furthermore, the elasticity and pliability of the surgical glove port compared with commercially available ports allows reasonable range of movements and achieves enough triangulation ( Fig. 4 ). This has been also shown by others. 14 However, despite several advantages of homemade glove port, the application for SIPES is not approved and can be an issue for some medical institutions. For patients with a potential for developing a latex allergy, nonlatex surgical gloves should be used. The current expenses for SIPES ports range from 318 to 1,378 USD 15 16 17 compared with glove ports based on equipment available for around 30 euro. 4 Fig. 4 Comparison of ranges of motion between homemade glove port and a commercially available device (Tri-Port, Olympus, Center Valley, PA). With the aim to further improve the technique of setting up the glove port, we established the following modifications: The fixation of a 5-mm trocar to the wound retractor prevents dislocation and allows the introduction of sharp instruments through the port as the tip of the port cannot violate the glove.

Fig. 4 Comparison of ranges of motion between homemade glove port and a commercially available device (Tri-Port, Olympus, Center Valley, PA). With the aim to further improve the technique of setting up the glove port, we established the following modifications: The fixation of a 5-mm trocar to the wound retractor prevents dislocation and allows the introduction of sharp instruments through the port as the tip of the port cannot violate the glove. All other instruments (2nd grasper and the camera) can be introduced through the 2-mm incisions in the finger tips, therefore time-consuming connection of special cannulas or trocars as suggested by others is not necessary. 14 18 The hitch stich, the wound retractor, allows easy removal at the end of the procedure. The bariatric scope in combination with the 90° angled light connector (Karl Storz) avoids clashing of instruments and the instruments and the telescope/camera head. Conclusion SIPES appendectomy using a homemade glove port is a low-cost alternative to commercially available ports and it is easy to set up and use. Moreover, the high flexibility of the glove and the wide range of motion of the instruments make SIPES more comfortable than using most SIPES ports currently available in the market. Therefore, this device is a good option even in highly equipped laparoscopic centers.

y available ports and it is easy to set up and use. Moreover, the high flexibility of the glove and the wide range of motion of the instruments make SIPES more comfortable than using most SIPES ports currently available in the market. Therefore, this device is a good option even in highly equipped laparoscopic centers. New Insights and the Importance for a Pediatric Surgeon The surgical equipment to perform single-incision pediatric endosurgery (SIPES) does not have to be expensive. The glove port presented in this technical report is not only highly cost effective but allows a wide range of motion of the surgical instruments. Therefore, homemade glove ports should have a role even in highly developed countries. Conflict of Interest None.

Introduction Congenital diaphragmatic hernia (CDH) is a birth defect of the diaphragm caused by an insufficient closure of the pleuroperitoneal canal during fetal development. 1 About 80% of these malformations are located on the left posterolateral side and are therefore called Bochdalek hernias. 2 Most patients with CDH are diagnosed prenatally or immediately after birth. 3 However, children with small defects may be asymptomatic postnatally and present later in life, when an increased intra-abdominal pressure facilitates herniation of organs into the chest. 4 5 6 Some cases of CDH develop gastrointestinal symptoms due to gastric ischemia or injury or volvulus. 3 7 We report on a child with previously unknown Bochdalek hernia and life-threatening presentation due to tension pneumothorax. Case Report A 3-year-old boy presented to an outside hospital with a 5-day history of progressing respiratory distress and retching. The initial chest X-ray showed a left-sided tension pneumothorax with mediastinal shift and the suspicion of bowel loops in the left lower hemithorax ( Fig. 1 ). Therefore, the patient was transferred to our institution. Fig. 1 Chest X-ray at presentation (this image is provided by courtesy of Pediatric Clinic, Fachkrankenhaus Hubertusburg, Wermsdorf, Sachsen, Germany).

A 3-year-old boy presented to an outside hospital with a 5-day history of progressing respiratory distress and retching. The initial chest X-ray showed a left-sided tension pneumothorax with mediastinal shift and the suspicion of bowel loops in the left lower hemithorax ( Fig. 1 ). Therefore, the patient was transferred to our institution. Fig. 1 Chest X-ray at presentation (this image is provided by courtesy of Pediatric Clinic, Fachkrankenhaus Hubertusburg, Wermsdorf, Sachsen, Germany). On admission, he showed severe dyspnea, a temperature of 39.5°C and tachycardia of 200/min. After immediate endotracheal intubation, a thoracic computed tomography (CT) scan was performed, which confirmed a left-sided enterothorax with mediastinal shift ( Fig. 2 ). A left-sided chest tube was inserted, which drained a fluid that was initially considered to be old blood. Due to the sudden onset of symptoms and a normal chest X-ray which was available from the age of 1 year ( Fig. 3 ), a diaphragmatic rupture was considered as a differential diagnosis. The boy was therefore taken to the operation room (OR) immediately. On diagnostic laparoscopy, a left-sided Bochdalek hernia was detected with herniation of the small intestine, spleen, and stomach into the chest ( Fig. 4 ). Bile-stained fluid was found in the thorax and abdomen. After repositioning of the herniated organs into the abdomen, a gastric perforation at the lesser curvature was detected ( Fig. 5 ), explaining the pneumothorax. The surgeon felt that the gastric perforation could not be closed safely laparoscopically; therefore, a conversion to laparotomy was performed with closure of the gastric perforation and repair of the CDH with interrupted stitches. After extubation on the fourth postoperative day, a retrovesical abscess was drained 30 days after the surgery. Due to a gastroparesis, the boy showed a prolonged recovery and was finally discharged after 4 to 5 weeks in good condition. After a follow-up of 2 years, the boy is asymptomatic and is doing well.

stitches. After extubation on the fourth postoperative day, a retrovesical abscess was drained 30 days after the surgery. Due to a gastroparesis, the boy showed a prolonged recovery and was finally discharged after 4 to 5 weeks in good condition. After a follow-up of 2 years, the boy is asymptomatic and is doing well. Fig. 2 Thoracic CT scan on admission: left-sided tension pneumothorax with mediastinal shift. No further signs of a traumatic etiology. Fig. 3 Chest X-ray at 1-year of age with no signs of diaphragmatic hernia. Fig. 4 Diagnostic laparoscopy: herniation of stomach, spleen and bowel into the chest. Fig. 5 Gastric perforation after repositioning of herniated organs into the abdomen. Discussion

plication of both virtual reconstruction technology and the printing of physical models in pediatric oncological cases where complex tumor resection are required is a useful tool for improving outcome and will take a relevant place in the near future for planning and surgical training in this type of complex surgeries. New Insights and the Importance for a Pediatric Surgeon 3D virtual reconstruction and 3D model printing is a developing technology that has multiple potential applications in the field of surgery regarding to planning, guiding and training surgical procedures. We report our experience using this technology in pediatric surgical oncology. Conflict of Interest None.

Fig. 2 Thoracic CT scan on admission: left-sided tension pneumothorax with mediastinal shift. No further signs of a traumatic etiology. Fig. 3 Chest X-ray at 1-year of age with no signs of diaphragmatic hernia. Fig. 4 Diagnostic laparoscopy: herniation of stomach, spleen and bowel into the chest. Fig. 5 Gastric perforation after repositioning of herniated organs into the abdomen. Discussion Intestinal symptoms of late presenting CDH can be nonspecific and may include abdominal pain or respiratory symptoms. 3 8 On radiologic imaging, heart murmurs and dilated bowel loops or intrathoracic cysts 3 are not found infrequently. We present the second case of a CDH with tension pneumothorax due to gastric perforation. CDH in association with gastric morbidity has been described in children of different age groups. In a systemic review on 362 children with late-presenting CDH, 46% of these were <1 year old, 32% aged 1 to 5 years, and 22% over 5 years old. 6 Therefore, the patient presented here is in the middle spectrum of these age groups. Gastric complications of CDH include gastric volvulus 7 and an incarceration of the stomach with or without perforation. 4 Baglai et al reported on gastric volvulus being the leading cause of gastric morbidity in late-presenting CDH in 45% followed by gastric incarceration with (35%) and without (20%) perforation. 6 In our case, we speculate that gastric ischemia with perforation in the absence of gastric volvulus was most likely the pathogenesis. However, we cannot rule out a combination of a previous volvulus and subsequent gastric perforation.

in 45% followed by gastric incarceration with (35%) and without (20%) perforation. 6 In our case, we speculate that gastric ischemia with perforation in the absence of gastric volvulus was most likely the pathogenesis. However, we cannot rule out a combination of a previous volvulus and subsequent gastric perforation. Unusual in our case was the clinical presentation with tension pneumothorax, which has been described before; Ozkan et al 5 reported a 5-year-old girl in whom the initial chest X-ray showed a thoracic herniation of the stomach, which was misinterpreted as a lung cyst at the left lower lobe. Two days after discharge, she was readmitted for a left-sided tension pneumothorax. This complication is extremely rare in CDH. In hindsight, as the chest tube in our patient drained the same fluid as did the nasogastric (NG) tube, we could have already thought of a hollow visceral perforation preoperatively. As the second imaging modality, a thoracic CT scan was performed as rupture of the diaphragm was considered a possible differential diagnosis. However, it can be discussed whether the CT scan was necessary, since it did not add additional information to the findings revealed by a conventional X-ray. CDH may be asymptomatic at birth and may present at a later age. The herniation of the viscera into the chest leads to respiratory symptoms. In case of both, enterothorax and tension pneumothorax, a gastric or bowel perforation in combination with CDH must be taken into consideration.

Unusual in our case was the clinical presentation with tension pneumothorax, which has been described before; Ozkan et al 5 reported a 5-year-old girl in whom the initial chest X-ray showed a thoracic herniation of the stomach, which was misinterpreted as a lung cyst at the left lower lobe. Two days after discharge, she was readmitted for a left-sided tension pneumothorax. This complication is extremely rare in CDH. In hindsight, as the chest tube in our patient drained the same fluid as did the nasogastric (NG) tube, we could have already thought of a hollow visceral perforation preoperatively. As the second imaging modality, a thoracic CT scan was performed as rupture of the diaphragm was considered a possible differential diagnosis. However, it can be discussed whether the CT scan was necessary, since it did not add additional information to the findings revealed by a conventional X-ray. CDH may be asymptomatic at birth and may present at a later age. The herniation of the viscera into the chest leads to respiratory symptoms. In case of both, enterothorax and tension pneumothorax, a gastric or bowel perforation in combination with CDH must be taken into consideration. New Insights and the Importance for a Pediatric Surgeon CDH may be asymptomatic at birth and may present at a later age. The herniation of the viscera into the chest leads to respiratory symptoms. In case of both, enterothorax and tension pneumothorax, a gastric or bowel perforation in combination with CDH must be taken into consideration.

Importance for a Pediatric Surgeon CDH may be asymptomatic at birth and may present at a later age. The herniation of the viscera into the chest leads to respiratory symptoms. In case of both, enterothorax and tension pneumothorax, a gastric or bowel perforation in combination with CDH must be taken into consideration. Acknowledgment We acknowledge support from the German Research Foundation (DFG) and Leipzig University within the program of Open Access Publishing. Conflict of Interest None.

Introduction Circumcision is one of the most frequent procedures performed by pediatric surgeons. However, after surgery some patients represent to the emergency department due to bleeding, pain, swelling, redness, local infection, and decreased urinary output. Most of this morbidity is minor and can be easily managed. 1 In contrast, ischemia or even necrosis of the glans is a rare complication; in the past 15 years, nine pediatric cases have been reported in the literature. 2 3 4 5 6 7 8 We report the case of a boy with ischemia of the glans penis after circumcision at an outside hospital. Case Report

Circumcision is one of the most frequent procedures performed by pediatric surgeons. However, after surgery some patients represent to the emergency department due to bleeding, pain, swelling, redness, local infection, and decreased urinary output. Most of this morbidity is minor and can be easily managed. 1 In contrast, ischemia or even necrosis of the glans is a rare complication; in the past 15 years, nine pediatric cases have been reported in the literature. 2 3 4 5 6 7 8 We report the case of a boy with ischemia of the glans penis after circumcision at an outside hospital. Case Report A 16-year-old boy with phimosis underwent elective circumcision at an outside hospital. Anesthesia was performed via dorsal penile nerve block (DPNB) (15 mL of 0.25% bupivacaine). On the first postoperative day, he was referred to our hospital due to pain, black discoloration, and swelling of the glans. Voiding was possible. On clinical examination, the distal glans showed severe ischemia ( Fig. 1 ). All laboratory results including blood count, lactate, D-dimer, and clotting profile were within normal limits. Color Doppler ultrasound of the penis showed good cavernosal artery flow to the glans. After transferral to our pediatric intensive care unit, a caudal block was performed to reduce sympathetic tone and improve arterial blood flow. Five hours after admission, the patient underwent digital subtraction angiography (DSA) under sedation. After overwiew of the pelvic arteries and the left internal iliac artery, the internal pudendal artery was explored selectively via microcatheter (Progreat 2.7F, Terumo) but no vasospasm or thrombus was detected. A sufficient arterial perfusion as well as normal venous drainage of the glans was confirmed ( Fig. 2 and 3 ). To use all therapeutic options, intra-arterial spasmolysis with a bolus of 5 µg alprostadil and 150 µg nitroglycerine was sequentially given. Eight hours after admission, systemic therapy with sildenafil (1 mg/kg orally once a day), L-arginine-hydrochlorid (0.1 mg/kg/hour), and unfractionated heparin (15 units/kg/hour, up to 20 units/kg/hour depending on partial thromboplastin time) were initiated and given for 3 days.

nitroglycerine was sequentially given. Eight hours after admission, systemic therapy with sildenafil (1 mg/kg orally once a day), L-arginine-hydrochlorid (0.1 mg/kg/hour), and unfractionated heparin (15 units/kg/hour, up to 20 units/kg/hour depending on partial thromboplastin time) were initiated and given for 3 days. Fig. 1 Glans with severe ischemia on the first postoperative day (admission). Fig. 2 Digital subtraction angiography (DSA) of the left internal pudendal artery (*) via microcatheter (Progreat 2.7F, Terumo) confirmed a sufficient arterial perfusion of the glans with good contrast filling of the dorsal artery of the penis (**). Fig. 3 Venous phase of the digital subtraction angiography (DSA) of the left internal pudendal artery (*) showed a sufficient venous drainage of the glans. After 3 days of systemic vasodilatative therapy, the darkish color of the glans changed to a brownish appearance. A surgical intervention was not necessary and ischemia resolved completely. The boy was discharged on the seventh postoperative day without adverse events ( Fig. 4 ). Fig. 4 After treatment, glans ischemia resolved completely (seventh postoperative day after angiography). Discussion

After 3 days of systemic vasodilatative therapy, the darkish color of the glans changed to a brownish appearance. A surgical intervention was not necessary and ischemia resolved completely. The boy was discharged on the seventh postoperative day without adverse events ( Fig. 4 ). Fig. 4 After treatment, glans ischemia resolved completely (seventh postoperative day after angiography). Discussion Glans ischemia is an extremely rare complication after circumcision. In most cases, the etiology remains unclear. Apart from hematoma, tight suture lines, or excessive use of monopolar cautery, DPNB has been suggested to be the most frequent cause of this complication. 4 9 In a large cohort from Singapore analyzing 3,909 DPNB, the total complication rate was reported to be as low as 0.23%. 10 Other authors also report on ischemia of the glans penis following DPNB for circumcision. 6 7 11 As an alternative for DPNB, topical anesthetics have been evaluated but did not provide sufficient perioperative analgesia. 12 Kaplanian et al suggested to limit the volume of local anesthetic solution to an amount of 0.2 mL/kg (up to maximum of 10 mL). 7 In our patient, the primary surgeons exceeded this dose and applied 15 mL of plain bupivacaine on each side. Therefore, this could have led to a transient vasospasm.

rioperative analgesia. 12 Kaplanian et al suggested to limit the volume of local anesthetic solution to an amount of 0.2 mL/kg (up to maximum of 10 mL). 7 In our patient, the primary surgeons exceeded this dose and applied 15 mL of plain bupivacaine on each side. Therefore, this could have led to a transient vasospasm. In case of glans ischemia following DPNB, the main goal of treatment is the reestablishment of sufficient blood flow to the penis. Several therapeutic approaches have been described including pentoxifylline, hyperbaric therapy, iloprost, enoxaparin, anticoagulation, local testosterone, and peridural anesthesia. 2 3 4 5 6 7 8 In our case, we started treatment by performing a caudal epidural block to improve arterial supply and to reduce sympathetic tone and pain. This is in line with a report of a group from Australia, who successfully applied this technique in a 9-year-old boy. 7

In case of glans ischemia following DPNB, the main goal of treatment is the reestablishment of sufficient blood flow to the penis. Several therapeutic approaches have been described including pentoxifylline, hyperbaric therapy, iloprost, enoxaparin, anticoagulation, local testosterone, and peridural anesthesia. 2 3 4 5 6 7 8 In our case, we started treatment by performing a caudal epidural block to improve arterial supply and to reduce sympathetic tone and pain. This is in line with a report of a group from Australia, who successfully applied this technique in a 9-year-old boy. 7 Thereafter, we focused on treating the suspected vascular origin of glans ischemia. In adult urologic departments, DSA is frequently used in the diagnostic workup for arteriogenic impotence and percutaneous endovascular revascularization to treat vasculogenic erectile dysfunction (penile artery stenosis) 13 14 15 or in the treatment of high-flow priapism. 16 To the best of our knowledge, we applied invasive angiography for the first time in a patient with glans ischemia. We observed a sufficient arterial perfusion as well as a normal venous drainage of the glans. A vasospasm or arterial obstruction, as well as a venous outflow problem, was ruled out during this procedure. Additionally, potent vasodilators nitroglycerine (nitric oxide as cyclic guanosine monophosphate activator) and alprostadil (prostaglandin E1 analog) were applied intra-arterially to test if this could further improve the perfusion, which was not the case.

a venous outflow problem, was ruled out during this procedure. Additionally, potent vasodilators nitroglycerine (nitric oxide as cyclic guanosine monophosphate activator) and alprostadil (prostaglandin E1 analog) were applied intra-arterially to test if this could further improve the perfusion, which was not the case. The nonselective phosphodiesterase inhibitor pentoxifylline (PTX) is a hemorheological drug, which improves peripheral blood flow by reducing whole blood viscosity. It works by relaxing smooth muscle of the corpus cavernosum. Two case reports on a 3- and 10-year-old boy combined the therapy of caudal block with PTX and described good outcomes. 4 6 However, indication and dosage of PTX in children remain controversial. 3 4 6 8 11 Hence, in our patient we used the selective phosphodiesterase inhibitor (PDE 5) sildenafil as a vasodilative agent with success and did not appreciate side effects. Efe et al suggested a monotherapy with low molecular weight heparin (enoxaparin) as the treatment of choice for ischemia of the glans in a 7-year-old boy. After 5 days of anticoagulation, the black discoloration of the glans disappeared. 5 In our patient, we used the protocol of unfractionated heparin (15–20 units/kg/hour) according to the protocol of Sara and Lowry ([bupivacaine 0.5%] with low-dose heparin infusion [25 units/kg/hour] for 4 days). 9 Other therapeutic options include hyperbaric therapy, which has been evaluated in adults only. 17 Therefore, we did not consider this therapeutic option.

Efe et al suggested a monotherapy with low molecular weight heparin (enoxaparin) as the treatment of choice for ischemia of the glans in a 7-year-old boy. After 5 days of anticoagulation, the black discoloration of the glans disappeared. 5 In our patient, we used the protocol of unfractionated heparin (15–20 units/kg/hour) according to the protocol of Sara and Lowry ([bupivacaine 0.5%] with low-dose heparin infusion [25 units/kg/hour] for 4 days). 9 Other therapeutic options include hyperbaric therapy, which has been evaluated in adults only. 17 Therefore, we did not consider this therapeutic option. Conclusion Ischemia of the glans is a rare complication after circumcision after DPNB. Although the cause of the transient ischemia could not be proven, we speculate that DPNB might have caused severe vasospasm. In the current patient, the multimodal treatment resulted in complete recovery of the glans without adverse events. Our case emphasizes the role of DSA in the diagnostic workup and the therapeutic possibilities of local spasmolysis, systemic vasodilatation, and anticoagulation. We acknowledge support from the German Research Foundation (DFG) and Leipzig University within the program of Open Access Publishing. Conflict of Interest None.

Introduction Biliary atresia and choledochal cyst (CC) are two of the most common causes of obstructive jaundice in infants. Infantile variant of CC can mimic cystic biliary atresia in clinical presentation leading to diagnostic dilemma. 1 2 3 Biliary atresia associated with CC is also reported in literature. Here, we present an unusual case of CC that does not fit into the current classification of CCs, and can be called an atretic variant of CC. Case Report A 73-day-old boy was brought to our hospital with the complaint of progressively increasing jaundice. This boy was conceived by in vitro fertilization, the second among twins, through cesarean section. Antenatal check-ups were unremarkable. Until 15 days of life, this boy was healthy, taking breastfeeds well and passing yellow/greenish stool. Later he developed symptoms: passing clay colored stool, yellowish discoloration of sclera and body. Initial evaluation done in other hospitals revealed cholestatic jaundice. Finally, when the patient was brought to our hospital, he was deeply icteric. The patient was underweight compared with his elder twin. Liver, with smooth margin and soft consistency, was palpable 3 cm below costal margin. Patient had elevated liver enzymes (AST–64 U/L, ALT–129 U/L, ALP–1,000 U/L) and hyperbilirubinemia (Total bilirubin–8.74 mg /dL, direct–4.9 mg/dL). Gamma glutamyl transpeptidase (GGT) was elevated (1,006 U/L). Hemogram, PT/INR, C-reactive protein, and thyroid profile were normal.

was palpable 3 cm below costal margin. Patient had elevated liver enzymes (AST–64 U/L, ALT–129 U/L, ALP–1,000 U/L) and hyperbilirubinemia (Total bilirubin–8.74 mg /dL, direct–4.9 mg/dL). Gamma glutamyl transpeptidase (GGT) was elevated (1,006 U/L). Hemogram, PT/INR, C-reactive protein, and thyroid profile were normal. Ultrasonography of abdomen revealed dilated intrahepatic biliary radicles with dilated common bile duct (CBD) till mid part of CBD. A hyperechoic soft calculus without distal acoustic shadowing was seen in the lumen of distal intrapancreatic CBD. Gall bladder (GB) was normal in size with well-defined walls ( Fig. 1 ). Fig. 1 ( a , b , and c ) Ultrasound images showing a well-defined gall bladder (thin yellow arrow), dilated common bile duct (CBD; thick yellow arrow), an echogenic calculus in lower CBD (white arrow). Further, Magnetic resonance cholangiopancreatographic (MRCP) imaging revealed tubular cystic dilatation of common hepatic duct and confirmed intrahepatic biliary radicles dilatation. Cystic duct was unusually draining quite distally. Abrupt narrowing was seen at the junction of common hepatic duct (CHD) and CBD which was hypothesized to be due to a possible stricture at this level. A calculus (8 mm) was evident in the lower CBD extending into intrapancreatic part of CBD. Cystic duct was draining just above the calculus ( Figs. 2 and 3 ). Main pancreatic duct was not dilated.

t the junction of common hepatic duct (CHD) and CBD which was hypothesized to be due to a possible stricture at this level. A calculus (8 mm) was evident in the lower CBD extending into intrapancreatic part of CBD. Cystic duct was draining just above the calculus ( Figs. 2 and 3 ). Main pancreatic duct was not dilated. Fig. 2 MRI images of a 3-month-old infant with cholestatic jaundice. ( a , b ) 2D MRCP showing cystic dilatation of common hepatic duct (thick yellow arrow), and gall bladder (thin yellow arrow). ( c , d ) Axial 3D MRCP and axial T1-weighted MR images depicting course and drainage of cystic duct (red arrow) unusually distally and continuing as CBD which shows a T2 hypointense ( c ), T1 hyperintense ( d ) calculus (white arrow) within it respectively. 2D, two dimensional; 3D, three dimensional; CBD, common bile duct; MRCP, Magnetic resonance cholangiopancreatography; MRI, magnetic resonance imaging. Fig. 3 ( a ) 2D MRCP showing blind ending dilated common hepatic duct. Line diagram ( b ) depicting the final preoperative diagnosis of infantile choledochal cyst associated with atresia of common hepatic duct distally resulting in dilated common hepatic duct proximally (thick yellow arrow), normal size of GB (thin yellow arrow), cystic duct insertion in lower part (red arrow) and a calculus in lower CBD (blue arrow), as correlated with Coronal T2-weighted MRI image. CBD, common bile duct; GB, gall bladder; MRI, magnetic resonance imaging.

Introduction Childhood tuberculosis (TB) is a public health problem around the world. According to the World Health Organization (WHO), the percentage of TB in children varies from 3% to over 25%, depending on the country. 1 The abdominal localization comes in fourth position after pulmonary, ganglionic, pleural, and osteoarticular manifestation. 2 If the abdomen is affected, peritoneal TB is the most common, followed by an infection of the intestines. Although in industrialized countries, the frequency of mycobacterium TB-associated infection is mainly related to human immunodeficiency virus; in sub-Saharan Africa, peritoneal TB is not an exceptional case in pediatrics. Nevertheless, even in endemic countries, peritoneal TB remains extremely rare in infants with a predominance in the older child. 3 4 5 We report on two cases with abdominal TB and discuss the diagnostic difficulties related to this localization in young children. Observations Case 1

Childhood tuberculosis (TB) is a public health problem around the world. According to the World Health Organization (WHO), the percentage of TB in children varies from 3% to over 25%, depending on the country. 1 The abdominal localization comes in fourth position after pulmonary, ganglionic, pleural, and osteoarticular manifestation. 2 If the abdomen is affected, peritoneal TB is the most common, followed by an infection of the intestines. Although in industrialized countries, the frequency of mycobacterium TB-associated infection is mainly related to human immunodeficiency virus; in sub-Saharan Africa, peritoneal TB is not an exceptional case in pediatrics. Nevertheless, even in endemic countries, peritoneal TB remains extremely rare in infants with a predominance in the older child. 3 4 5 We report on two cases with abdominal TB and discuss the diagnostic difficulties related to this localization in young children. Observations Case 1 A 6-month-old female infant (6000 g; 3rd percentile) with an unremarkable medical history was transferred to our pediatric ward because of postprandial vomiting for 5 days. On clinical examination, she was subfebrile (38°C) and had a distended but soft abdomen. No mass was palpable. Abdominal ultrasound suggested the possibility of intussusception. As an enema to reduce intussusception was no therapeutic option at our center, the abdomen was surgically explored. On laparotomy, blood-stained but clear ascites was found as well as intestinal adhesions. Moreover, we saw diffuse granulations, mesenteric lymphadenopathy, and ischemia of the terminal ileum ( Figs. 1 and 2 ). Peritoneal cultures were harvested, appendectomy was performed, and the intestinal lymph nodes were biopsied. Postoperatively, tuberculin intradermal reaction (IDRt) was positive at 9 mm and cultures grew tuberculous contagium. The Anti-TB treatment administered was: rifampicin, isoniazid, pyrazinamide, ethambutol (RHZE) for 2 months, then RH the following 4 months. This regimen was applied before receiving the biopsy results which showed tuberculoid follicular lesions ( Figs. 3 and 4 ). The patient was declared cured after this treatment. She had no abdominal symptoms after 2 years of follow-up.

ing in dilated common hepatic duct proximally (thick yellow arrow), normal size of GB (thin yellow arrow), cystic duct insertion in lower part (red arrow) and a calculus in lower CBD (blue arrow), as correlated with Coronal T2-weighted MRI image. CBD, common bile duct; GB, gall bladder; MRI, magnetic resonance imaging. HIDA (hepatobiliary iminodiacetic acid scan) scan revealed no radioisotope excretion into gut even after 24 hours. Presumptive diagnosis of choledochal cyst was made before taking up the patient for laparotomy. Intraoperatively, hepatomegaly normal size GB, and a 3 × 2.5 cm cyst in subhepatic region were found ( Fig. 4 ). Bile was seen on needle aspiration from cyst. Mobilization of GB was done and cystic duct was found to be opening into duodenum, without any communication with the cyst or CHD. The cyst was mobilized and looped with a feeding tube. On further dissection, cyst was found to have a blind ending distally ( Fig. 5 ). Right and left hepatic ducts were patent and opening into the cyst. Both hepatic ducts were irrigated with saline. Excision of cyst and hepaticodocho-jejunostomy was done. Distally, ligation and division of cystic duct at the entry into duodenum, and cholecystectomy completed the procedure. Postoperatively oral feeds were started after 48 hours. Patient passed normal colored stool and showed normal weight gain. Jaundice had subsided and he was discharged after 5 days. Fig. 4 Intraoperative image showing normal sized gall bladder, dilated common hepatic duct (cyst) and cystic duct noncommunicating with common hepatic duct.

Intraoperatively, hepatomegaly normal size GB, and a 3 × 2.5 cm cyst in subhepatic region were found ( Fig. 4 ). Bile was seen on needle aspiration from cyst. Mobilization of GB was done and cystic duct was found to be opening into duodenum, without any communication with the cyst or CHD. The cyst was mobilized and looped with a feeding tube. On further dissection, cyst was found to have a blind ending distally ( Fig. 5 ). Right and left hepatic ducts were patent and opening into the cyst. Both hepatic ducts were irrigated with saline. Excision of cyst and hepaticodocho-jejunostomy was done. Distally, ligation and division of cystic duct at the entry into duodenum, and cholecystectomy completed the procedure. Postoperatively oral feeds were started after 48 hours. Patient passed normal colored stool and showed normal weight gain. Jaundice had subsided and he was discharged after 5 days. Fig. 4 Intraoperative image showing normal sized gall bladder, dilated common hepatic duct (cyst) and cystic duct noncommunicating with common hepatic duct. Fig. 5 Intraoperative image showing blind ending dilated common hepatic duct (cyst) noncommunicating with cystic duct. Histopathological examination of cyst (cuboidal epithelium, stromal mononuclear infiltrates) and liver (normal lobular architecture and no evidence of fibrosis, giant cells) were consistent with choledochal cyst. Follow-up at 6 weeks was done. The patient was healthy, anicteric, taking feeds well, passing normal colored stool, and gained weight. Discussion

Histopathological examination of cyst (cuboidal epithelium, stromal mononuclear infiltrates) and liver (normal lobular architecture and no evidence of fibrosis, giant cells) were consistent with choledochal cyst. Follow-up at 6 weeks was done. The patient was healthy, anicteric, taking feeds well, passing normal colored stool, and gained weight. Discussion Todani modification of Alonso-Lej classification classifies CC into five types ( Table 1 ). 3 CC in infants needs to be differentiated from cystic biliary atresia due to their similar clinical presentation. 2 Nondilatation of intrahepatic biliary radicles and absence of biliary epithelial lining of cyst; differentiates cystic biliary atresia from CC in infants. 1 2 Contrary to CC, cystic biliary atresia does not have any continuity with biliary tree and aspirate of cyst is nonbilious. HIDA scan in cystic biliary atresia does not reveal any excretion of radioisotope into the gut. Our case had radiological, intraoperative and histopathological findings of CC but the unusual intraoperative finding was the completely atretic distal end of CC and noncommunication of cystic duct with the cyst. This finding does not fit into any of the present classification of CC. 3 4 Though distal stenosis of CC and association of CC with biliary atresia are well described in literature, an atretic variant of CC is not yet described, to the best of our knowledge.

end of CC and noncommunication of cystic duct with the cyst. This finding does not fit into any of the present classification of CC. 3 4 Though distal stenosis of CC and association of CC with biliary atresia are well described in literature, an atretic variant of CC is not yet described, to the best of our knowledge. Table 1 Todani modification of Alonso-Lej classification for choledochal cyst Type I Fusiform or cystic dilatation of CBD Type II Bile duct diverticulum Type III Choledochocele Type IV Multiple extrahepatic and intrahepatic dilation of duct IV a both intra and extrhepatic ducts are affected IV b involves only extraheptic bile duct Type V Fusiform or saccular dilation of intrahepatic bile ducts (Caroli disease)

or cystic dilatation of CBD Type II Bile duct diverticulum Type III Choledochocele Type IV Multiple extrahepatic and intrahepatic dilation of duct IV a both intra and extrhepatic ducts are affected IV b involves only extraheptic bile duct Type V Fusiform or saccular dilation of intrahepatic bile ducts (Caroli disease) This new entity also raises curiosity to its embryological basis. Various hypotheses have been proposed to explain the development of CC. Most often cited theory is anomalous pancreaticobiliary junction with reflux of pancreatic juice into the bile duct causing weakness and dilatation of duct wall. 5 Other theories include distal stenosis, sphincter of oddi dysfunction, faulty remodeling of the embryonic ductal plate, motility disorders and viral damage to the ganglion cells of bile duct. 4 5 6 Our case was not associated with other congenital anomalies so the inciting insult was probably late perinatal event instead of early embryological one which is usually associated with congenital anomalies of other organs developing at the same time. 7 The finding of distal atresia of the bile duct with discontinuity of cystic duct from bile duct is similar to the type-3 jejunoileal atresia (JIA). It is widely accepted that jejunoileal atresia results from vascular insult late in foetal life. Similar to JIA, late perinatal ischemic insult can best explain the pathogenesis of this atretic variant of CC. 8 9

e duct with discontinuity of cystic duct from bile duct is similar to the type-3 jejunoileal atresia (JIA). It is widely accepted that jejunoileal atresia results from vascular insult late in foetal life. Similar to JIA, late perinatal ischemic insult can best explain the pathogenesis of this atretic variant of CC. 8 9 Conclusion Atretic variant of CC is a newly described entity that can mimic cystic biliary atresia and other variants of CC in clinical presentation. It requires early surgical intervention to prevent irreversible changes in the liver. In addition, this variant is expected to behave like other variants of CC with excellent prognosis if timely intervention is done. New Insights and the Importance for the Pediatric Surgeon An atretic variant of choledochal cyst is described as a variant that needs early surgical intervention to prevent irreversible damage to liver. Conflict of Interest None.

Introduction Surgical resection is one of the cornerstones in the multidisciplinary treatment of pediatric solid tumors. Preoperative tumor evaluation and surgical strategy planning are commonly based on conventional imaging techniques: ultrasound, nuclear magnetic resonance imaging (MRI), and computed tomography (CT). These tests give us images in two dimensions. Virtual three-dimensional (3D) reconstruction using specialized software provides a better understanding of the spatial relationships between the tumor and adjacent organs and allows exploring in an interactive form the surgical anatomy preoperatively. They also allow a fast and simple representation of high-definition 3D images, using files digital image and communication in medicine (DICOM) from CT or MRI. 1 2 3 This helps to evaluate more accurately the conditions under which the operation will be performed and to anticipate possible intraoperative complications. 3D printing models, also called additive manufacturing models, are the result of the transformation of 3D digital files into tangible models. 4 5 6 This technology began in the 80s when Charles W. Hull designed the first stereolithography machine. Although there are different types of 3D printer, all are based on the fusion or deposition of materials layer by layer until the production of the desired structure. 6 7 8 Recently, numerous studies have been published documenting the usefulness of 3D technology in medicine both from the educational point of view and for planning and surgical training. 5 6 7 8 9

This technology began in the 80s when Charles W. Hull designed the first stereolithography machine. Although there are different types of 3D printer, all are based on the fusion or deposition of materials layer by layer until the production of the desired structure. 6 7 8 Recently, numerous studies have been published documenting the usefulness of 3D technology in medicine both from the educational point of view and for planning and surgical training. 5 6 7 8 9 In this article, we present four cases of pediatric patients affected from solid tumor lesions in which 3D reconstruction and printing technology was used in surgical planning, as they were cases in which the surgery was technically complex and or the complete resection presented an important forecast implication. Materials and Methods We conduct a case series study. Data about complex oncological cases in which the 3D reconstruction and printing technology was implemented were collected from January 2016 to January 2018. The data for the 3D reconstruction were obtained in the DICOM format from the preoperative tests performed on the patients, MRI in three cases and CT in one, without any additional imaging tests. MRI was performed by 1.5-T scanner obtaining coronal and axial sections. Fast Spin Echo T2-fat-suppressed sequences were selected. The slice thickness was 4 to 5 mm. CT images were acquired by high-resolution multiplanar scan of 0.5-mm slice thickness.

in three cases and CT in one, without any additional imaging tests. MRI was performed by 1.5-T scanner obtaining coronal and axial sections. Fast Spin Echo T2-fat-suppressed sequences were selected. The slice thickness was 4 to 5 mm. CT images were acquired by high-resolution multiplanar scan of 0.5-mm slice thickness. Subsequently, the specialized team of engineers of Cella Medical Solutions (Murcia, Spain) performed the processing of two-dimensional images using previously validated medical image treatment advanced algorithms. Initially, a noise reduction algorithm (anisotropic diffusion filter) was applied, followed by segmentation algorithms of the anatomical structures of interest and creation of the 3D image of each structure. These images were exported in sterolitographic file (stl). Finally, the post-processing of the virtual reconstruction was performed. After virtual reconstruction was completed, the models were printed by fused deposit modeling (FDM) and injection printing 3D technology (ROVA3D, BCN3D, ORD Solutions, BCN technologies©, and FORM 3D©) ( Fig. 1 ). Different plastic derived materials were used such as polylactic acid, acrylonitrile butadiene styrene (ABS), polyvinyl, polyurethane, and polystyrene. Once the printing was finished, post-processing procedures were performed by removing support structures, clearing coating, and dyeing structures when necessary for getting the most optimal final model.

s were used such as polylactic acid, acrylonitrile butadiene styrene (ABS), polyvinyl, polyurethane, and polystyrene. Once the printing was finished, post-processing procedures were performed by removing support structures, clearing coating, and dyeing structures when necessary for getting the most optimal final model. Fig. 1 Different steps of 3D printing are shown. ( A ) FDM printer; ( B ) printed structure still with support material. ( C ) Result after post-processing the piece. FDM, fused deposit modeling; 3D, three-dimensional. The printed models were made on real scale, including both the tumor and adjacent organs considered of interest. Special importance was given to the reconstruction of the vascularization involved. Results Between January 2016 and January 2018, data about four cases of patients affected by complex solid tumors were collected, due to both, the location and the relationships of the tumor, as the therapeutic implications of the surgical resection.

The printed models were made on real scale, including both the tumor and adjacent organs considered of interest. Special importance was given to the reconstruction of the vascularization involved. Results Between January 2016 and January 2018, data about four cases of patients affected by complex solid tumors were collected, due to both, the location and the relationships of the tumor, as the therapeutic implications of the surgical resection. Case 1 : A 7-month-old infant was diagnosed with bilateral Wilms tumor. Neoadjuvant chemotherapy treatment was initiated according to the protocol of the International Society of Pediatric Oncology (SIOP) with little response to chemotherapy. In the preoperative MRI, a large tumor dependent on the left kidney with limited renal parenchyma was observed. Right renal tumor presented smaller size with largely conserved parenchyma. Initially, left radical nephrectomy and right nephron-sparing surgery were planned. The volumetric reconstruction ( Fig. 2 ) of both the tumor and the healthy renal tissue proved that the left kidney parenchyma free of nephroblastoma was greater than the bidimensional imaging tests showed, considering feasible to perform bilateral nephron-sparing surgery. Using 3D printing technology, 1:1 scale models were obtained providing information on the volume of both tumors in relation to the size of the kidneys and the small size of the renal vascularization. The 3D physical models were consulted during the procedure due to doubts regarding the vascularization of both tumors ( Fig. 3 ). Surgical intervention was accomplished without incidents, but during the postoperative period, the patient presented thrombosis of the right renal artery that was tried to be solved by endovascular treatment, in spite of which atrophy of the right kidney developed. Currently, the patient is in remission of his disease, he maintains normal renal function values although he has hypertension controlled with amlodipine.

patient presented thrombosis of the right renal artery that was tried to be solved by endovascular treatment, in spite of which atrophy of the right kidney developed. Currently, the patient is in remission of his disease, he maintains normal renal function values although he has hypertension controlled with amlodipine. Fig. 2 Case 1. ( A ) Coronal MRI section showing both kidneys affected by Wilms tumor, confirming the large size of the left tumor mass. ( B and C ) Volumetric reconstructions of both tumors. Healthy renal parenchyma susceptible to nephron sparing surgery was proved. LK, left kidney; MRI, magnetic resonance imaging; RK, right kidney. Fig. 3 Case 1. The images ( A – C ) show different moments of the surgical intervention. After bilateral nephron-sparing surgery, coagulation was performed with adhesive matrix and plication of the renal parenchyma. In the image ( D ), the resected specimens are shown together with their corresponding three-dimensional models, objectifying the great similarity between both.

ts of the surgical intervention. After bilateral nephron-sparing surgery, coagulation was performed with adhesive matrix and plication of the renal parenchyma. In the image ( D ), the resected specimens are shown together with their corresponding three-dimensional models, objectifying the great similarity between both. Case 2 : A 3-year-old girl was diagnosed with multiple bilateral pulmonary metastases secondary to unilateral, stage III, intermediate risk Wilms tumor. After receiving chemotherapy for metastatic recurrence according to the SIOP protocol, 3 four residual lesions were verified in the control CT; three of them were located in the right lung (two in the right upper lobe and one in the posterior segment of the right lower lobe) and one in the upper lobe of the left lung. Fig. 4 shows the reconstruction of the metastatic lesions and the comparison with their location in the CT scan. The 3D reconstruction allowed the 360-grade vision of the metastases within the pulmonary parenchyma, providing relevant information for a more precise location of the lesions. Fig. 4 Case 2. ( A ) Three-dimensional reconstruction performed from the preoperative CT. There are three metastases in the right lung and one in the left one. ( B and C ) Cross-sections of the thoracic CT. Lesions marked by arrow correspond to those marked with the same color in the virtual reconstruction. CT, computed tomography.

. ( A ) Three-dimensional reconstruction performed from the preoperative CT. There are three metastases in the right lung and one in the left one. ( B and C ) Cross-sections of the thoracic CT. Lesions marked by arrow correspond to those marked with the same color in the virtual reconstruction. CT, computed tomography. Case 3 : An infant of 13 months was diagnosed at 7 months of right abdominal neuroblastoma. In MRI, an extensive mass was observed that encompassed the regional aorta, the exit of the celiac trunk, the mesenteric artery, and the renal arteries, exerting mass effect on the inferior vena cava and displacing both the liver and the right kidney. Percutaneous puncture was performed with an anatomopathological diagnosis of poorly differentiated neuroblastoma, classified as Group “D” (low) of the International Neuroblastoma Risk Group (INRG) classification (stage L2, age less than 18 months, MYCN not amplified and 11q not deleted). The abdominal mass was considered unresectable, and an expectant attitude was adopted initially. During follow-up, hypertension and increased compression of the inferior vena cava were found. These symptoms were considered life-threatening; therefore, chemotherapy was initiated according to the European Low and Intermediate Risk Neuroblastoma protocol. The non-response to chemotherapy and the persistence of symptoms led to proposing surgery. Fig. 5 shows an axial section of the MRI showing the large size of the neuroblastoma and the correspondence with the virtual reconstruction in which it is clearly visualized how the tumor includes the superior mesenteric artery and the renal artery, making possible to know the disfigured anatomy by the large abdominal mass. Three different physical models were printed, two models that recreated the tumor, the right kidney and the relevant vessels (vena cava and portal vein, aorta, celiac trunk, and superior mesenteric artery), one of them with the tumor printed in transparent polyurethane for visualization of the vasculature embraced within the tumor. In the other model, liver, spleen and left kidney were also included. In addition to planning the surgical approach, these models were also consulted several times as a guide during the intervention ( Fig. 6 ). Surgery was performed in one of the spanish national reference centers for neuroblastoma obtaining complete resection of the tumor without incidents during the intervention.

uded. In addition to planning the surgical approach, these models were also consulted several times as a guide during the intervention ( Fig. 6 ). Surgery was performed in one of the spanish national reference centers for neuroblastoma obtaining complete resection of the tumor without incidents during the intervention. Fig. 5 Case 3. ( A ) MRI showing a large abdominal mass that moves the liver upward. ( B ) Virtual reconstruction of the tumor (yellow), liver (green), both kidneys (gray), and spleen (brown). The involved vasculature was also reconstructed (aorta in red, vena cava in blue, and portal vein in purple). ( C ) Modified virtual reconstruction, observing the tumor of semitransparent consistency, which allows the visualization of the renal artery and vein within the tumor. The kidney is displaced inferiorly with loss of its morphology due to the mass effect. MRI, magnetic resonance imaging. Fig. 6 Case 3. ( A ) Different printed models representing the abdominal neuroblastoma. The superior model includes tumor (yellow), aorta (red) and vena cava (blue) with its respective branches, liver (blue), both kidneys (green), and spleen (brown). The different structures are assembled by a magnetization system. The other two models represent the tumor and the relevant vascularity in different textures. ( B ) The model was consulted several times during the surgery. In the image, the correspondence between the model and the surgical anatomy is observed.

(brown). The different structures are assembled by a magnetization system. The other two models represent the tumor and the relevant vascularity in different textures. ( B ) The model was consulted several times during the surgery. In the image, the correspondence between the model and the surgical anatomy is observed. Case 4 : A 5-year-old girl was suffering from left paravertebral neuroblastoma. She was diagnosed prenatally of pleural effusion, and admission to the neonatal intensive care unit (ICU) was needed at birth due to respiratory insufficiency and thoracocentesis with diagnosis of chylothorax. During admission, a large cervico-thoracic tumor compatible with neuroblastoma was detected. The tumor included the left common carotid artery and left internal jugular vein without infiltrating them. Percutaneous biopsy revealed the diagnosis of low-risk undifferentiated neuroblastoma (group D) of the INRG classification (stage L2, age less than 18 months, MYCN not amplified, and 11q not deleted). The tumor was considered unresectable (L2), and given the symptomatology of the patient, chemotherapy treatment was initiated with significant reduction in tumor volume. After that, expectant attitude was decided upon, while the patient remained asymptomatic. In the last year, a progressive increase in tumor size was observed, so a new biopsy was performed with a diagnosis of ganglioneuroma, and surgical intervention was considered. Fig. 7 shows the comparison between MRI and virtual reconstruction. It is seen how the trachea, the internal jugular vein and the carotid artery arte desplaced by the tumor, and how the vertebral artery is encased whithin it. The vertebral artery is encompassed by the tumor. Currently, the printed models are being prepared, and the intervention will be performed soon.

l reconstruction. It is seen how the trachea, the internal jugular vein and the carotid artery arte desplaced by the tumor, and how the vertebral artery is encased whithin it. The vertebral artery is encompassed by the tumor. Currently, the printed models are being prepared, and the intervention will be performed soon. Fig. 7 Case 4. Virtual reconstruction of cervicothoracic paravertebral ganglioneuroma and comparison with images obtained by MRI (A-B coronal view, C-D transverse view). The tumor displaces the left carotid artery, the internal jugular vein, and the airway. The vertebral artery is encased by the tumor. MRI, magnetic resonance imaging. Discussion

Fig. 7 Case 4. Virtual reconstruction of cervicothoracic paravertebral ganglioneuroma and comparison with images obtained by MRI (A-B coronal view, C-D transverse view). The tumor displaces the left carotid artery, the internal jugular vein, and the airway. The vertebral artery is encased by the tumor. MRI, magnetic resonance imaging. Discussion 3D technology has potential applications in medicine among which is the creation of anatomical models to plan surgeries. In this article, we report our experience in the implementation of a new high-fidelity tool for, among other specialties, pediatric oncological surgery. The 3D virtual reconstruction and prototypes, designed from both MR and CT images in the form of DICOM files, have been useful in all four cases of complex oncological resections, not only when the surgery was being planned, but have also proved to be a useful tool during the surgical intervention. 3D virtual recreation computer software allows a 360-grade view of the tumor and the relevant adjacent structures in a clear colorimetric scale, as well as modified the consistency of the structures and removes or reintroduces them as the user desired. These kinds of software calculate precisely the volume of the tumor and the normal parenchyma as well. Real-scale printed models are able to be consulted and manipulated, to corroborate the distorted and particular anatomy and vascularization of each case. In the first case (bilateral Wilms tumor), the volumetric reconstruction was decisive at the time of the planning of the intervention, proving the existence of enough remaining renal tissue, which made nephron-sparing surgery in both kidneys posible 10 (avoiding nephrectomies, one of the goals of the surgery). In this case, surgery planning with visualization and manipulation of the 3D model to achieve the greatest preservation of nephrons was an optimal tool.

he existence of enough remaining renal tissue, which made nephron-sparing surgery in both kidneys posible 10 (avoiding nephrectomies, one of the goals of the surgery). In this case, surgery planning with visualization and manipulation of the 3D model to achieve the greatest preservation of nephrons was an optimal tool. Regarding case 3, in which the celiac trunk was involved and both the liver and the portal vein are displaced, the usefulness of visualization and manipulation at the pre-surgical time and during surgery was also demonstrated. This set of technologies has been widely described in in liver surgery where precise evaluations of the remaining volume of the liver and anatomical variation are needed for the preoperative planning of safe curative hepatectomy. In surgical planning, a better understanding of spatial relationships can be obtained by exploring the surgical site interactively in a 3D format; preoperatively, this helps to evaluate more accurately the conditions under which the operation must be performed as well as to predict and prevent possible complications. 11 12 13 14 15 In addition to 3D reconstruction, the impression, manipulation, and assembly maneuvers of each structure allow to later recognize the anatomy and “not feel lost” during the dissection of unique structures, such as those found in complex solid tumors.

well as to predict and prevent possible complications. 11 12 13 14 15 In addition to 3D reconstruction, the impression, manipulation, and assembly maneuvers of each structure allow to later recognize the anatomy and “not feel lost” during the dissection of unique structures, such as those found in complex solid tumors. There are already numerous publications and surgical series in which virtual reconstruction and 3D printing prior to surgery are routinely used in multiple specialties (tumors, placement of endovascular, endobronchial prostheses, etc.). 16 17 18 19 20 New challenges are also created, such as the possibility of producing several models in series, the plasticity of the material, and its operability, etc. To summarized, it is a field open to surgery of the future, in which you can manipulate a tumor model so similar to reality that surgery could be performed in the printed model prior to the real surgery, reducing the surgical times and improving safety significantly As an added value, 3D models the patient and his family's understanding of the intervention that will be performed, improving doctor–patient communication. 20 Our experience has been highly positive, and we believe that the application of both virtual reconstruction technology and the printing of physical models in pediatric oncological cases where complex tumor resection are required is a useful tool for improving outcome and will take a relevant place in the near future for planning and surgical training in this type of complex surgeries.

niazid, pyrazinamide, ethambutol (RHZE) for 2 months, then RH the following 4 months. This regimen was applied before receiving the biopsy results which showed tuberculoid follicular lesions ( Figs. 3 and 4 ). The patient was declared cured after this treatment. She had no abdominal symptoms after 2 years of follow-up. Fig. 1 Granulations on the ileum (see arrow). Fig. 2 Granulations on the abdominal wall (see arrow). Fig. 3 Appendiceal mucosa presenting an inflammatory granuloma involving tuberculous follicle in the submucosa. ( a ) Giant cells. ( b ) Epithelïoid cells. ( c ) Appendiceal mucosa. Fig. 4 Ganglionic parenchyma site of inflammatory granulomas. ( a ) Epithelïoid cells. ( b ) Giant cells in concentric arrangement surrounded by lymphocytes. ( c ) Signs of starting necrosis.

Fig. 3 Appendiceal mucosa presenting an inflammatory granuloma involving tuberculous follicle in the submucosa. ( a ) Giant cells. ( b ) Epithelïoid cells. ( c ) Appendiceal mucosa. Fig. 4 Ganglionic parenchyma site of inflammatory granulomas. ( a ) Epithelïoid cells. ( b ) Giant cells in concentric arrangement surrounded by lymphocytes. ( c ) Signs of starting necrosis. Case 2 An 8-month-old (7000 g; 3rd percentile) boy from Mauritania with no particular pathological history was transferred from a health center near the Senegal–Mauritanian border for the management of peritonitis. The clinical picture had evolved for 15 days, with the appearance of abdominal bloating associated with vomiting and cessation of bowel movements. On clinical examination, he had a temperature of 38°C, and was tachycardic at 148 beats per minute. He showed nutritional edema and flank dullness. His abdomen was distended and had peritonitis on palpation. Therefore, the boy was taken to the operating room. Surgical exploration revealed clear ascites associated with small intestinal granulations and adhesions. We performed adhesiolysis, biopsies, and drainage of the peritoneal cavity. Postoperatively, GeneXpert tests were performed on the peritoneal fluid and the IDRt was negative. Anatomopathological examination of the operative specimens revealed the presence of caseous necrosis compatible with peritoneal TB. The medical treatment and the evolution were identical to Case 1. Discussion

Case 2 An 8-month-old (7000 g; 3rd percentile) boy from Mauritania with no particular pathological history was transferred from a health center near the Senegal–Mauritanian border for the management of peritonitis. The clinical picture had evolved for 15 days, with the appearance of abdominal bloating associated with vomiting and cessation of bowel movements. On clinical examination, he had a temperature of 38°C, and was tachycardic at 148 beats per minute. He showed nutritional edema and flank dullness. His abdomen was distended and had peritonitis on palpation. Therefore, the boy was taken to the operating room. Surgical exploration revealed clear ascites associated with small intestinal granulations and adhesions. We performed adhesiolysis, biopsies, and drainage of the peritoneal cavity. Postoperatively, GeneXpert tests were performed on the peritoneal fluid and the IDRt was negative. Anatomopathological examination of the operative specimens revealed the presence of caseous necrosis compatible with peritoneal TB. The medical treatment and the evolution were identical to Case 1. Discussion The most common digestive tract localization of pediatric TB is the peritoneal serosa. 6 Malnutrition is the leading predisposition for the reactivation of a latent peritoneal focus. 2 This comorbidity was noted in our second patient. In our center, the leading cause of an acute abdomen in infants with low-grade fever and disturbances in bowel mobility is intussusception. It is difficult to diagnose peritoneal TB because of clinical polymorphism and the absence of specific radiological findings. In infants, this diagnostic challenge is more obvious because of the high prevalence of digestive tract infections with infectious organisms other than TB. Febrile ascites is the most common clinical manifestation. The diagnostic difficulties of peritoneal TB have been reported by several authors. 7 8 9 The definitive diagnosis of abdominal TB is made by histology of bioptic specimens. 4 7 Some authors recommend to obtain these biopsies by laparoscopy which allows good exploration of the peritoneal cavity and a sensitivity close to 100%. 4 5 6 7 8 9 However, the long time spent to obtain the results of the pathological examination justifies the use of a certain number of diagnostic arguments (clear ascites, granulations, IDRt, GeneXpert) and allow us to start the treatment before the histology results. IDRt does not provide the diagnosis of peritoneal TB. 4 5 However, combined with other clinical and epidemiological parameters, it can be an important element in decreasing the treatment period. In our first patient, the discovery of diffuse granulations throughout the abdomen associated with a clear ascites, the positive cultures for tuberculous contagium, in combination with a positive IDRt allowed to start the medical treatment before the results of the anatomic pathological examination were available. The accuracy GeneXpert is affected by the sampling times and the conditions during transport. The sensitivity has been reported to be as low as 60 to 80%. 10 The first patient was operated at night and the laboratory that performs this examination is not open at night which explains why the test was not performed in this patient.

y GeneXpert is affected by the sampling times and the conditions during transport. The sensitivity has been reported to be as low as 60 to 80%. 10 The first patient was operated at night and the laboratory that performs this examination is not open at night which explains why the test was not performed in this patient. In the second patient the geneXpert is negative because the sensitivity of this test is not 100%. Conclusion Peritoneal TB is rare in infants, and diagnosis can be challenging. In children with abdominal pain, low-grade fever, and ascites, this differential diagnosis should be taken into consideration, especially in endemic arias. Medical treatment is usually effective. New Insights and the Importance for the Pediatric Surgeon Pectus excavatum (PE) is the most common malformation of the anterior chest wall. The use of minimally invasive repair for pectus excavatum in extremely severe cases of PE is still controversial and adequate severity assessment indexes should be ensued. Conflict of Interest None.

Introduction Transverse testicular ectopia (TTE) is a rare anomaly in which both testes descend or migrate through a single inguinal canal into the same hemiscrotum. Often, the diagnosis is made during surgical exploration (65%). 1 2 This condition is usually associated with other abnormalities such as persistent Mullerian duct syndrome (PMDS), true hermaphroditism, inguinal hernia, hypospadias, pseudohermaphroditism, and scrotal anomalies. About 100 cases of TTE have been reported in the literature. 1 However, in most of cases the diagnosis cannot be made preoperatively. We report on two consanguineous children. Case Reports Two first degree cousins presented with a right nonpalpable testis which could not be visualized on pelvic ultrasound (US). Therefore, they were referred for abdominopelvic conventional magnetic resonance imaging (MRI) plus diffusion-weighted imaging. General physical examination was unremarkable, no other congenital anomalies were found, and no family history for the same condition. On examination, the right testis was nonpalpable inguinally with an empty right hemiscrotum. The MRIs were performed with a 1.5-T MRI system (Achiva, Philips Medical Systems, Best, Netherlands) using a body coil under sedation. Case 1 Physical examination showed the phenotype of a 3-year-old boy with a normal size penis. The testis was palpable in the left groin and a nonpalpable undescended testis was found on the right side.

The MRIs were performed with a 1.5-T MRI system (Achiva, Philips Medical Systems, Best, Netherlands) using a body coil under sedation. Case 1 Physical examination showed the phenotype of a 3-year-old boy with a normal size penis. The testis was palpable in the left groin and a nonpalpable undescended testis was found on the right side. On MRI, the left testis was located in the left hemiscrotum, was 1.3 × 0.6 cm in size, and associated with mild hydrocele. Another testis measuring 1.2 × 0.6 cm was noted on the ipsilateral side within the left inguinal canal ( Fig. 1 ). Fig. 1 Coronal short tau inversion recovery (STIR) image. Coronal diffusion-weighted image with a b-value of 800. The figure shows the presence of one testis in the left hemiscrotum with hydrocele and another one noted in the left inguinal canal which show hyperintense signal in STIR sequence and restriction in diffusion-weighted image. Exploration of the left groin revealed the presence of both testes within the same side one above the other. They had separate vasa deferentia and testicular vessels for each testis and a common cremasteric muscle and tunical covering. After herniotomy, the ectopic testis was fixed transseptally to the right hemiscrotum ( Fig. 2 ). Fig. 2 Crossed ectopic testes with separate vas and vessel and a hernia sac. Case 2 Physical examination showed a 1-year-old male with a normal size penis. The testis was felt in the left groin and a nonpalpable undescended testis was found on the right side. In comparison to the right side, the left scrotum was well developed.

Fig. 2 Crossed ectopic testes with separate vas and vessel and a hernia sac. Case 2 Physical examination showed a 1-year-old male with a normal size penis. The testis was felt in the left groin and a nonpalpable undescended testis was found on the right side. In comparison to the right side, the left scrotum was well developed. On MRI, the left testis was located in the left hemiscrotum, was 1.2 × 0.6 cm in size, and associated with mild hydrocele. Another testis measuring 1.3 × 0.6 cm was noted at the same side just above the first one near the neck of the scrotum ( Fig. 3a and b ). At this point, the diagnosis of left side TTE was obvious. There was no evidence of inguinal hernia or Mullerian duct structures. Left groin exploration showed a testis over the other near the scrotal neck. Both cords were released followed by bilateral orchidopexy in a subdartos pouch after passing one of them through the median raphe onto the right hemiscrotum. Fig. 3 Coronal short tau inversion recovery (STIR) image. Coronal diffusion-weighted image with a b-value of 800. The figure shows the presence of one testis in the left hemiscrotum with hydrocele and another one noted in the left scrotal neck which shows hyperintense signal in STIR sequence and restriction in diffusion-weighted image. The suspected diagnosis of a right undescended testis was changed to a left-sided TTE.

Fig. 3 Coronal short tau inversion recovery (STIR) image. Coronal diffusion-weighted image with a b-value of 800. The figure shows the presence of one testis in the left hemiscrotum with hydrocele and another one noted in the left scrotal neck which shows hyperintense signal in STIR sequence and restriction in diffusion-weighted image. The suspected diagnosis of a right undescended testis was changed to a left-sided TTE. Left groin exploration showed a testis over the other near the scrotal neck. Both having common coverings. Both cords were released followed by bilateral orchiopexy using a subdartos pouch after passing one of them through the median raphe onto the right hemiscrotum ( Fig. 4 ). Fig. 4 ( A ) Intraoperative finding of two cords. ( B ) Subdartos scrotal fixation of both testes. Discussion

Left groin exploration showed a testis over the other near the scrotal neck. Both having common coverings. Both cords were released followed by bilateral orchiopexy using a subdartos pouch after passing one of them through the median raphe onto the right hemiscrotum ( Fig. 4 ). Fig. 4 ( A ) Intraoperative finding of two cords. ( B ) Subdartos scrotal fixation of both testes. Discussion Ectopic testis may be located in different sites including the superficial inguinal pouch, suprapubic, femoral, perineal, and base of the penis. TTE is a rare anatomical abnormality where both gonads migrate toward the same hemiscrotum. It was first described by Von Lenhossek in 1886. 3 Several theories have been proposed in an attempt to explain the etiology of this rare anomaly. These include the PMDS which may result from the failure of synthesis or release of Mullerian duct inhibitory factor (MIF), the failure of end organs to respond to MIF, or a defect in the timing of the release of MIF. It seems possible that the mechanical effect of the persistent Mullerian duct structures prevents a normal testicular descent. As a result, both testicles descend toward the same hemiscrotum with the appearance of TTE. 2 4 5 6 Despite this variety of theories, the exact etiology of this condition is still not fully understood.

Ectopic testis may be located in different sites including the superficial inguinal pouch, suprapubic, femoral, perineal, and base of the penis. TTE is a rare anatomical abnormality where both gonads migrate toward the same hemiscrotum. It was first described by Von Lenhossek in 1886. 3 Several theories have been proposed in an attempt to explain the etiology of this rare anomaly. These include the PMDS which may result from the failure of synthesis or release of Mullerian duct inhibitory factor (MIF), the failure of end organs to respond to MIF, or a defect in the timing of the release of MIF. It seems possible that the mechanical effect of the persistent Mullerian duct structures prevents a normal testicular descent. As a result, both testicles descend toward the same hemiscrotum with the appearance of TTE. 2 4 5 6 Despite this variety of theories, the exact etiology of this condition is still not fully understood. The mean age of presentation is 4 years 1 2 7 and the clinical presentation generally includes an ipsilateral inguinal hernia and a contralateral or sometimes a bilateral cryptorchidism. 5 The diagnosis of TTE can be made preoperatively by clinical examination, use of ultrasonography by an experienced sonographer, and MRI. 8 Although laparoscopy is considered the gold standard, 9 10 MRI may have additional diagnostic value. 11 Our two cases of TTE in first degree cousins are unusual as there was no clinical evidence for an inguinal hernia or other anomalies indicating TTE.

The mean age of presentation is 4 years 1 2 7 and the clinical presentation generally includes an ipsilateral inguinal hernia and a contralateral or sometimes a bilateral cryptorchidism. 5 The diagnosis of TTE can be made preoperatively by clinical examination, use of ultrasonography by an experienced sonographer, and MRI. 8 Although laparoscopy is considered the gold standard, 9 10 MRI may have additional diagnostic value. 11 Our two cases of TTE in first degree cousins are unusual as there was no clinical evidence for an inguinal hernia or other anomalies indicating TTE. Patients with TTE are at increased risk of malignant transformation, Mullerian remnants, and subsequent malignancy. In one study, a yolk sack tumor developed in a patient who presented with transverse ectopic testes. 12 Wood and Elder showed that the risk of malignancy in undescended testicles is decreased if orchiopexy is performed before the age of 10 to 12 years. 13 Once the diagnosis of TTE is made, orchiopexy is recommended for the preservation of fertility and to prevent malignant transformation. 13 . Management of testicular ectopia includes either transseptal or extraperitoneal transposition orchiopexy. Our two cases were managed by transseptal orchiopexy with preservation of the vas deferens and testicular vessels. TTE associated with fused vas deferens is extremely rare. 14 15 16 This condition may hinder the testis from being placed into the scrotum during orchiopexy. In cases of fused vas deferens, a transseptal orchiopexy is recommended.

Management of testicular ectopia includes either transseptal or extraperitoneal transposition orchiopexy. Our two cases were managed by transseptal orchiopexy with preservation of the vas deferens and testicular vessels. TTE associated with fused vas deferens is extremely rare. 14 15 16 This condition may hinder the testis from being placed into the scrotum during orchiopexy. In cases of fused vas deferens, a transseptal orchiopexy is recommended. Our follow-up plan includes a physical examination after 1 month postorchidopexy and then after 6 and 12 months. Conclusion TTE is a rare anomaly with unknown pathogenesis. Hereditary factors may play a role. It should be suspected in patients presenting with impalpable undescended testis on one side and cryptorchidism on the other side. Also, a thorough clinical examination and laparoscopy imaging such as US and MRI are useful for establishing the preoperative diagnosis of TTE and associated anomalies. New Insights and the Importance for the Pediatric Surgeon Crossed testicular ectopia (CTE)/transverse testicular ectopia (TTE) is a rare condition occurring in only 1 in 4 million male patients so it's needed to be documented, it's presence in consanguineous boys enhance role of hereditary factors in addition to use of DW-MRI to confirm diagnosis and associated anomalies. Conflict of Interest An informed consent of key informants was obtained and confidentiality of data was ensured.

Introduction Hirschsprung's disease (HD) is defined by the absence of enteric neurons in the distal part of the bowel. Neonates with HD usually present with a distended abdomen, feeding intolerance with bilious vomiting, and delayed passage of meconium. Failure to promptly diagnose HD can result in death secondary to recurrent enterocolitis. 1 2 Although its etiology is still unknown, several gene defects have been discovered. 3 Intestinal duplication (ID) is a rare congenital condition usually involving the small intestine, located on the mesenteric side of the bowel, with which it may share blood supply. Although it is symptomatic at birth in only 30% of cases, diagnosis is made before 2 years of life in up to 70% of patients. Symptoms vary as ID can affect any part of the gastrointestinal tract. 4 Many hypotheses have been made about its etiology, such as intrauterine hypoxia, partial twinning, split notochord syndrome, and remnants of embryological diverticula. 5 6 7 To date, no cases of intestinal obstructions simultaneously caused by both HD and ID have been reported. 8 9 Our study presents a unique case, where appropriate diagnosis and treatment were delayed due to the misleading presence of both ID and HD. Case Report

Intestinal duplication (ID) is a rare congenital condition usually involving the small intestine, located on the mesenteric side of the bowel, with which it may share blood supply. Although it is symptomatic at birth in only 30% of cases, diagnosis is made before 2 years of life in up to 70% of patients. Symptoms vary as ID can affect any part of the gastrointestinal tract. 4 Many hypotheses have been made about its etiology, such as intrauterine hypoxia, partial twinning, split notochord syndrome, and remnants of embryological diverticula. 5 6 7 To date, no cases of intestinal obstructions simultaneously caused by both HD and ID have been reported. 8 9 Our study presents a unique case, where appropriate diagnosis and treatment were delayed due to the misleading presence of both ID and HD. Case Report A full-term male neonate born at our Institute by induced vaginal delivery presented with bilious vomiting and abdominal distension starting from day 2 of life. X-ray revealed substantial distension of the small bowel, and abdominal ultrasound showed a simple cystic mass in the right hypochondrium (38 × 27 mm). Explorative laparotomy found an occlusive duplication at the ileocecal junction ( Fig. 1A ). Since resection of the duplication was not possible, we performed exeresis of the ileocecal region and end-to-end anastomosis ( Fig. 1B ). Gross pathology reaffirmed the diagnosis of ID and confirmed obstruction of the intestinal lumen. Fig. 1 ( A ) Occlusive duplication at the ileocecal junction. (B) Resected ileocecal region including intestinal duplication.

A full-term male neonate born at our Institute by induced vaginal delivery presented with bilious vomiting and abdominal distension starting from day 2 of life. X-ray revealed substantial distension of the small bowel, and abdominal ultrasound showed a simple cystic mass in the right hypochondrium (38 × 27 mm). Explorative laparotomy found an occlusive duplication at the ileocecal junction ( Fig. 1A ). Since resection of the duplication was not possible, we performed exeresis of the ileocecal region and end-to-end anastomosis ( Fig. 1B ). Gross pathology reaffirmed the diagnosis of ID and confirmed obstruction of the intestinal lumen. Fig. 1 ( A ) Occlusive duplication at the ileocecal junction. (B) Resected ileocecal region including intestinal duplication. Postsurgical course was characterized by recurrent bilious vomiting, abdominal distension, and constipation. At 1 month of life, due to exacerbation of his symptoms, the child underwent explorative relaparotomy: anastomotic stenosis and fibrous bands were excluded as the cause of occlusion. Instead, we found massive intestinal adherences, not apparently due to the previous surgery, as they were generalized and not localized, and were not causing bowel obstruction.

of his symptoms, the child underwent explorative relaparotomy: anastomotic stenosis and fibrous bands were excluded as the cause of occlusion. Instead, we found massive intestinal adherences, not apparently due to the previous surgery, as they were generalized and not localized, and were not causing bowel obstruction. Despite a second surgical procedure, abdominal distension persisted, associated with the absence of spontaneous evacuation and impaired weight gain. Since postsurgical complications had already been excluded at the second laparotomy, we started suspecting HD. Another explorative laparotomy was planned a month later: ileostomy was performed along with multiple intestinal biopsies, which confirmed the diagnosis of short-segment HD (length of aganglionic bowel of ∼10 cm from the anal verge). At nine months of life, the child successfully underwent transanal endorectal pull-through (De La Torre). Intestinal recanalization followed 3 months later. At 1 year follow-up, the child had normal bowel function, and no more episodes of abdominal distension have occurred. Discussion

Despite a second surgical procedure, abdominal distension persisted, associated with the absence of spontaneous evacuation and impaired weight gain. Since postsurgical complications had already been excluded at the second laparotomy, we started suspecting HD. Another explorative laparotomy was planned a month later: ileostomy was performed along with multiple intestinal biopsies, which confirmed the diagnosis of short-segment HD (length of aganglionic bowel of ∼10 cm from the anal verge). At nine months of life, the child successfully underwent transanal endorectal pull-through (De La Torre). Intestinal recanalization followed 3 months later. At 1 year follow-up, the child had normal bowel function, and no more episodes of abdominal distension have occurred. Discussion Only two cases of co-occurring HD and ID are reported in the literature in English. The first, published in 2012, is that of a 35-year-old woman with a medical history of HD treated with Soave procedure at 29 months of life presenting with an adenocarcinoma originating from a rectal duplication. 8 The second, published in 2017, is that of a full-term female newborn in whom an ileal duplication was accidentally found when she underwent exploratory laparotomy to perform multiple full-thickness biopsies for HD. 9 We also found one case of association of cystic bowel duplication and intestinal neuronal dysplasia described in 1983. 10 Our case is the first in which co-occurring ID and HD have constituted a clinical challenge rather than an incidental finding since they both caused clinical bowel obstruction. The presence of one masked the presence of the other, delaying diagnosis and immediate appropriate therapy. In hindsight, the massive presence of adhesions encountered during the second laparotomy could have prompted suspicion of HD, as adhesions form secondary to Hirschsprung-associated enterocolitis. As far as etiopathogenesis is concerned, both pathologies have unclear causal factors, and many hypotheses have been proposed. One advanced for both conditions is intrauterine hypoxia, postulated in the pathogenesis of ID, 5 6 7 8 9 10 11 but also suggested as an interfering factor in ganglion cell migration in HD. 10 Although it is highly possible that the association of HD and ID is coincidental, it might be speculated that the coexistence of HD and ID is secondary to ischemic damage that occurs during embryogenesis and that interferes with both intestinal canalization and neural crest migration.

ganglion cell migration in HD. 10 Although it is highly possible that the association of HD and ID is coincidental, it might be speculated that the coexistence of HD and ID is secondary to ischemic damage that occurs during embryogenesis and that interferes with both intestinal canalization and neural crest migration. Conclusion The coexistence of ID and HD is extremely rare, and only two cases have been described in the English literature. As shown in our report, the presence of HD and ID together can mislead the diagnostic work-up and delay appropriate therapy. New Insights and the Importance for a Pediatric Surgeon When treating neonates, pediatric surgeons should always be alert to the possibility of unusual combinations of pathologies with a similar clinical presentation, where one condition may mask the other. Conflict of Interest None.

Introduction Congenital diaphragmatic hernia (CDH) is a congenital anomaly characterized by a defect in the diaphragm, lung hypoplasia, and abnormal vascular remodeling. 1 This condition can lead to pulmonary hypertension (PH) and fatal respiratory failure. The impact of pulmonary vascular disease starts in utero and can also contribute to postnatal systolic and diastolic cardiac dysfunction in patients with CDH. It is well-known that severe PH complicates the course of CDH and is associated with poor survival. Despite medical and surgical advances, PH remains a major problem in CDH patients, leading to progressive deterioration in pulmonary function, impaired oxygenation, and respiratory failure. 2 3 Although PH may present (or relapse) at an older age, little is known about delayed presentation of PH in CDH patients and its treatment, in particular the potential role of lung or heart–lung transplantation in these patients. 3 The aim of this paper is to report a patient with CDH who underwent lung transplantation (Ltx) 15 years after CDH repair for delayed presentation of PH and required heart-lung retransplantation (H-Ltx) at 17 years of age for chronic rejection. Case Report

Although PH may present (or relapse) at an older age, little is known about delayed presentation of PH in CDH patients and its treatment, in particular the potential role of lung or heart–lung transplantation in these patients. 3 The aim of this paper is to report a patient with CDH who underwent lung transplantation (Ltx) 15 years after CDH repair for delayed presentation of PH and required heart-lung retransplantation (H-Ltx) at 17 years of age for chronic rejection. Case Report Our patient was born at 33 weeks of gestation by cesarean section to a 36-year-old gravida 3, para 2 woman after a prenatal diagnosis of left CDH (31 weeks' gestation). Postnatal chest X-rays confirmed the left CDH, with the liver in the thorax. Initial echocardiogram revealed only a small patent ductus arteriosus. There were no signs of PH at this time. At 24 hours of life, she underwent surgical repair of a huge diaphragmatic defect (type D according to the CDH Study Group staging system) 4 that required a silastic patch closure. Weaning from mechanical ventilation was achieved on postoperative day (POD) 4, but 3 days later she was reintubated for recurrent respiratory distress. Respiratory support was finally stopped on POD 26, and she was discharged home on POD 58. Predischarge echocardiography was unremarkable. The infancy was uneventful and the quality of life was excellent. She underwent periodic follow-up visits that revealed gastroesophageal reflux (GER) and initial mild scoliosis. At the age of 10, she was admitted for abdominal pain, vomiting, and dyspnea, and GER disease and severe PH were diagnosed. Heart ultrasound showed an estimated systolic right ventricular pressure of 130 mm Hg, with a systolic blood pressure of 100/50 mm Hg. Medical treatment for PH was initiated, including sildenafil, bosentan, and furosemide. Since PH progressively worsened despite maximal medical treatment, leading to end-stage respiratory failure, at the age of 14, she was listed for H-Ltx, and 3 months later she received the new organs. Postoperative course was characterized by short-term complications such as severe central venous line-related infections, massive pleural effusion and ascites, and delayed complications, including CDH recurrence with intestinal obstruction, requiring laparotomy and ileostomy formation. In addition, she suffered from recurrent respiratory infections, severe malnutrition, and drug-induced diabetes, significantly impacting her quality of life. With aging, scoliosis and GER also aggravated, requiring posterior vertebral arthrodesis and Nissen's fundoplication at 16 years of age.

and ileostomy formation. In addition, she suffered from recurrent respiratory infections, severe malnutrition, and drug-induced diabetes, significantly impacting her quality of life. With aging, scoliosis and GER also aggravated, requiring posterior vertebral arthrodesis and Nissen's fundoplication at 16 years of age. At 17 years of age, she developed a further episode of pneumonia-related respiratory failure and required a tracheostomy for multiple failed attempts of extubation. Bronchiolitis obliterans deteriorated her respiratory function, and the same year she was relisted for Ltx and underwent her second bipulmonary transplantation. Despite the second transplant, her general conditions rapidly deteriorated, she developed chronic lung rejection, and she eventually died at the age of 18, 4 and 1.5 years after the first and the second Ltx, respectively. Discussion We report a patient who underwent H-Ltx and subsequent Ltx for CDH-related end-stage PH.

At 17 years of age, she developed a further episode of pneumonia-related respiratory failure and required a tracheostomy for multiple failed attempts of extubation. Bronchiolitis obliterans deteriorated her respiratory function, and the same year she was relisted for Ltx and underwent her second bipulmonary transplantation. Despite the second transplant, her general conditions rapidly deteriorated, she developed chronic lung rejection, and she eventually died at the age of 18, 4 and 1.5 years after the first and the second Ltx, respectively. Discussion We report a patient who underwent H-Ltx and subsequent Ltx for CDH-related end-stage PH. Along with our patient, eight children with CDH who underwent Ltx or H-Ltx have been described ( Table 1 ). 1 2 3 5 6 Two were reported among 31 cases of children who underwent Ltx for a so-called diffuse lung disease and have very few data available. 6 Therefore, for most data, we will refer to the six with available data. All, except one, had a prenatal diagnosis of CDH and five had left side defect. The CDH repair was performed between 3 and 19 days after birth. Four patients underwent CDH repair during extracorporeal membrane oxygenation (ECMO). A diaphragmatic patch was required in four cases: two underwent primary closure, whereas the modality of closure was not specified in the remaining two patients. One patient had major associated anomalies (double-inlet left ventricle, transposition of the great arteries, subaortic stenosis, coarctation of the aorta). In all patients, early (at birth) or delayed (in adolescence) PH was the indication for Ltx or H-Ltx. Three patients had an H-Ltx, one had a single Ltx and transplant pneumonectomy 3 years later, one had a bilateral Ltx in a single step, one underwent sequential lungs transplantation, and two had unspecified Ltx. In all patients who had ECMO, Ltx was performed before 6 months of age (in the neonatal period in 2), whereas indication to Ltx developed in adolescence in the remaining two. In the early transplantation group, the time in the waiting list for emergency transplantation was 8 to 22 days. Median age at transplant was 105 days (range: 0–17 years). The donor's lung reduction was required in one patient only, and a right middle lobectomy was performed.

eloped in adolescence in the remaining two. In the early transplantation group, the time in the waiting list for emergency transplantation was 8 to 22 days. Median age at transplant was 105 days (range: 0–17 years). The donor's lung reduction was required in one patient only, and a right middle lobectomy was performed. Table 1 Literature review of CDH patients who underwent lung or heart-lung transplantation Author Year No. of patients Prenatal diagnosis ECMO CDH side Type of Tx Age at Tx Outcome Follow-up Van Meurs et al 1994 1 No Yes R Lung 17 d Alive 4 y Lee et al 2002 1 18 wk Yes L Lung 36 d Died 51 d post-Tx – Lee et al 2002 2 27 wk Yes L Lung 105 d Alive 3 y Lee et al 2002 3 18 wk Yes L Heart–lung 19 d Died 84 d post-Tx – Rama et al 2010 1 ns ns ns Lung ns ns ns Rama et al 2010 2 ns ns ns Lung ns ns ns Schmidt et al 2013 1 ns Yes L Lung 10 y Died 109 d post-Tx – Iacusso et al (this study) 2017 1 31 wk No L Heart–lung Lung 12 y 17 y Died 4 y after first Tx – Abbreviations: CDH, congenital diaphragmatic hernia; ECMO, extracorporeal membrane oxygenation; ns, not significant; Tx, transplantation.

2 ns ns ns Lung ns ns ns Schmidt et al 2013 1 ns Yes L Lung 10 y Died 109 d post-Tx – Iacusso et al (this study) 2017 1 31 wk No L Heart–lung Lung 12 y 17 y Died 4 y after first Tx – Abbreviations: CDH, congenital diaphragmatic hernia; ECMO, extracorporeal membrane oxygenation; ns, not significant; Tx, transplantation. Our patient shows the dark side of CDH-associated PH that may develop or recur late after a substantial period of well-being. In CDH patients, PH may persist or recur despite negative echocardiographic findings. In a recent study on pulmonary blood flow and vascular resistance, Zussman et al found that five out of eight patients with higher pulmonary artery pressure and vascular resistance and reduced blood flow as compared with controls did not have signs of PH on their echocardiogram. 7 Late or recurrent PH may represent a significant and severe morbidity in CDH patients. Burgos et al found that two out of seven late mortalities in CDH patients were related to persistent or recurrent PH. 8 In one patient, PH recurred at 9 years of age, following an asymptomatic period of several years free from medications. This patient is similar to our own patient who was well and medication-free up to 10 years of age when a diagnosis of severe PH was made. These data suggest that follow-up of CDH patients should carefully address the issue of persistent/recurring PH, finding a reasonable balance between the aggressiveness of investigations and the risks of missing the diagnosis.

ho was well and medication-free up to 10 years of age when a diagnosis of severe PH was made. These data suggest that follow-up of CDH patients should carefully address the issue of persistent/recurring PH, finding a reasonable balance between the aggressiveness of investigations and the risks of missing the diagnosis. Our case and the few others reported show that Ltx may represent a last-resort, life-prolonging option for end-stage PH in CDH patients, although associated with a final ominous prognosis. The first Ltx in a CDH patient was described by van Meurs et al in 1994. 2 They performed a lobar Ltx in a patient in whom other available strategies had failed. The patient underwent ECMO as a bridge to transplantation, and organ transplantation as a bridge to recovery of normal function in the paired organ. The Ltx was not considered as a definitive therapy because long-term immunosuppression, with its risks, may be avoided if the contralateral lung develops adequately. At 4 years of age, the patient underwent a transplant pneumonectomy for severe side effects of chronic immunosuppression 1 that was tolerated well. No data are available on further follow-up. Lee et al report on three patients with CDH who underwent Ltx at 19, 36, and 105 days of age, respectively. 3 The patient who underwent Ltx at 105 days of age was alive at 3 years of follow-up. Patients who underwent Ltx at a younger age died after 84 and 51 days, respectively, suggesting that age at Ltx may influence survival. It is possible that CDH patients with worse lung function, bad general status, and higher PH required transplant earlier and had poorer prognosis and thus worse outcome. On the other hand, the patient reported by van Meurs et al was transplanted at 19 days of age and was alive for 4 years after Ltx. 2 Therefore, it is not possible from these scant available data to detect any pattern of survival based on the age at transplant. Overall, in this small collected series of 6 CDH patients who underwent Ltx and available data, we found only two survivors. Three died shortly after transplant (one had a brain hemorrhage while on ECMO, one could not be weaned off ECMO, and one due to adenovirus infection waiting for re-Ltx), and our patient died 1.5 years after the second transplant due to chronic lung rejection. Only two patients can be considered as long-term survivors, although data are available only up to 3 and 4 years of follow-up, respectively.

ne could not be weaned off ECMO, and one due to adenovirus infection waiting for re-Ltx), and our patient died 1.5 years after the second transplant due to chronic lung rejection. Only two patients can be considered as long-term survivors, although data are available only up to 3 and 4 years of follow-up, respectively. These figures seem worse than the data from the overall pediatric Ltx population, where a 5-year survival rate of 50% is reported. 9 In CDH patients, early Ltx was proposed as a bridge in patients with severe PH and hypoplasia, waiting for pulmonary parenchyma to grow. 2 3 However, very few studies address the issue of Ltx in CDH patients. It is possible that Ltx in very young infants is technically more demanding or it may be more complex to obtain donor organs, preventing Ltx in small CDH patients. Accordingly, less than 5% of all pediatric Ltx reported in the Registry of the International Society for Heart and Lung Transplantation between January 2008 and June 2016 were performed in infants aged less than 1 year. 10

demanding or it may be more complex to obtain donor organs, preventing Ltx in small CDH patients. Accordingly, less than 5% of all pediatric Ltx reported in the Registry of the International Society for Heart and Lung Transplantation between January 2008 and June 2016 were performed in infants aged less than 1 year. 10 In conclusion, our case confirms the possibility of late PH occurrence or recurrence in CDH survivors even after a period of well-being and its ominous prognosis. The literature on the issue of Ltx in CDH patients is very scant and heterogeneous maybe due to the lack of available donor organs for small CDH infants and the rarity of late occurrence/recurrence of PH. Despite this limitation in the evidence, Ltx may be considered as a last-resort therapy for patients with CDH and severe PH unresponsive to conventional treatment, conceding time for the lung to grow and allow adequate oxygenation to the patient. New Insights and the Importance for the Pediatric Surgeon In patients with CDH, persistent/late onset pulmonary hypertension is rare but potentially ominous. Long-term follow-up is necessary. In CDH patients with end-stage respiratory failure due to pulmonary hypertension lung transplantation may be considered as a last resort treatment. Single lung transplantation, followed by transplant pneumonectomy, may represent a bridge to allow contralateral lung development, without the long term effects of immunosuppression. Conflict of Interest None.

Introduction Retrocaval ureter is a rare congenital anomaly where the ureter passes posterior to the inferior vena cava (IVC) resulting in extrinsic obstruction and corresponding progressive ureterohydronephrosis. 1 It is usually encountered on the right side. Left retrocaval ureter is an extremely rare condition, and is associated with situs inversus, duplicated or single left IVC. The treatment of choice is surgery. Laparoscopy offers good results. 2 We report a case of a left retrocaval ureter with consequent ureterohydronephrosis in an adolescent girl with situs inversus abdominalis. A laparoscopic transperitoneal approach was used for correction of the anomaly. Case Report A 16-year-old girl presented with a history of intermittent colicky left lumbar pain. The acute episodes of pain were related to excessive hydration, physical activity, or diuretic drinks. There was no history of urinary tract infection or hematuria. Laboratory urinary tests revealed normal values. Ultrasonography showed dilatation of the left renal pelvis and proximal ureter, with normal renal parenchymal thickness and no calculus; an abdominal situs inversus. A computed tomography confirmed the abdominal situs inversus, and described left ureterohydronephrosis, and left retrocaval ureter. The anteroposterior renal pelvis diameter (APD) was 20 mm and the proximal ureter had a diameter of 18 mm; parenchymal index was normal ( Fig. 1 ).

Case Report A 16-year-old girl presented with a history of intermittent colicky left lumbar pain. The acute episodes of pain were related to excessive hydration, physical activity, or diuretic drinks. There was no history of urinary tract infection or hematuria. Laboratory urinary tests revealed normal values. Ultrasonography showed dilatation of the left renal pelvis and proximal ureter, with normal renal parenchymal thickness and no calculus; an abdominal situs inversus. A computed tomography confirmed the abdominal situs inversus, and described left ureterohydronephrosis, and left retrocaval ureter. The anteroposterior renal pelvis diameter (APD) was 20 mm and the proximal ureter had a diameter of 18 mm; parenchymal index was normal ( Fig. 1 ). Fig. 1 Abdominal computed tomography scan with contrast injection. ( A ) Axial view, dorsal decubitus position: abdominal situs inversus. ( B ) Axial view, lateral decubitus position: proximal dilated ureter with retrocaval path. ( C ) 3D reconstruction: “hook-fish” sign. VCI, vena cava inferior. As the patient was symptomatic, she was taken into surgery. We used the laparoscopic transperitoneal approach.

Fig. 1 Abdominal computed tomography scan with contrast injection. ( A ) Axial view, dorsal decubitus position: abdominal situs inversus. ( B ) Axial view, lateral decubitus position: proximal dilated ureter with retrocaval path. ( C ) 3D reconstruction: “hook-fish” sign. VCI, vena cava inferior. As the patient was symptomatic, she was taken into surgery. We used the laparoscopic transperitoneal approach. After induction of general endotracheal anesthesia, a nasogastric tube and a Foley's catheter were inserted. The patient was placed in a modified flank position (45 degrees right lateral decubitus position) with overextension. A 5 mm umbilical port was placed for the 30-degree laparoscope, using an open technique. Two additional 5 mm ports were carefully placed. The line of Toldt's was incised and the left colon was retracted medially to permit access to the renal and retroperitoneal space. Dissection was carried using blunt and sharp instruments, exposing the renal pelvis, precaval dilated ureter, preureteral segment of the IVC, and postcaval ureter. After mobilizing the retrocaval ureteral segment, no intrinsic stricture was noted. The ureter was transected at the most caudal point of the dilated segment, mobilized, uncrossed, and placed lateral to IVC. After spatulation of the distal ureter and antegrade placement double-J ureteral stent (Charrière 4.7) we performed a ureteroureterostomy over a using a 5–0 monofilament, resorbable suture ( Fig. 2 ). An intraperitoneal drain was placed close to the anastomosis. Operative time was 150 minutes; no significant blood loss was noted.

lation of the distal ureter and antegrade placement double-J ureteral stent (Charrière 4.7) we performed a ureteroureterostomy over a using a 5–0 monofilament, resorbable suture ( Fig. 2 ). An intraperitoneal drain was placed close to the anastomosis. Operative time was 150 minutes; no significant blood loss was noted. Fig. 2 Intraoperative images. ( A ) Retrocaval disposition of the left ureter. ( B , C ) Transection and spatulation of the distal ureter. ( D ) Intraoperative placement of a J–J ureteral stent. ( E ) Final aspect of the anastomosis. VCI, vena cava inferior. The postoperative course was uneventful; the lumbar drain was removed on the 2nd postoperative day. The girl was discharged home after 5 days. The double-J stent was removed after 5 weeks. During a 20-month follow-up the patient was symptom-free. Ultrasonographic re-evaluation showed marked decrease of APD (7 mm). Discussion Retrocaval ureter, also known as circumcaval ureter or preureteral vena cava, is a rare congenital anomaly due to the abnormal development of the inferior vena cava. The embryological event causing the malformation is the persistence of the right posterior cardinal vein instead of the right subcardinal vein as the renal segment of the cava vein. 1 The term preureteral vena cava is preferred to retrocaval ureter, as the anomaly generating this condition is a vascular one.

vena cava. The embryological event causing the malformation is the persistence of the right posterior cardinal vein instead of the right subcardinal vein as the renal segment of the cava vein. 1 The term preureteral vena cava is preferred to retrocaval ureter, as the anomaly generating this condition is a vascular one. It was first described by Hochstetter in 1893. 3 Its incidence is 1 in 1,500 cadavers, and the sex ratio is 3:1, male to female. The first reported ureteropyeloplasty was performed by Anderson and Hynes in 1949, in a 10–year- old girl, with this anomaly. 4 Based on imaging, two types of retrocaval ureter have been described. The radiological classification of Bateson and Atkinson distinguishes type I–obstructive–“low loop” (ureter passes behind the IVC at the level of 3rd lumbar vertebra, with a fish-hook [S–shape] appearance), and type II–nonobstructive–“high loop” (retrocaval segment is at the level or the renal pelvis). 5 Symptoms usually develop in the third or fourth decade of life, children are less often diagnosed with this condition. There are also asymptomatic forms which do not require surgical treatment; type II, nonobstructive cases (approximately 10%). 5

e–“high loop” (retrocaval segment is at the level or the renal pelvis). 5 Symptoms usually develop in the third or fourth decade of life, children are less often diagnosed with this condition. There are also asymptomatic forms which do not require surgical treatment; type II, nonobstructive cases (approximately 10%). 5 If the subcardinal vein persists at a left vena cava system, left circumcaval ureter is encountered. It is an exceptionally rare anomaly and associated with either complete or partial abdominal situs inversus, duplicated IVC or single left IVC. There are only nine cases of left retrocaval ureter published in the medical literature 6 7 8 9 10 11 12 13 14 ( Table 1 ). Among them, there is only one pediatric case: a 10-month-old girl with Goldenhar's syndrome, diagnosed during routine urological screening. 14 Table 1 Published cases of left retrocaval ureter No. Author Patient's age Associated anomaly 1 Brooks, 1962 6 43 y Total situs inversus 2 Pierro, 1990 7 34 y Left IVC transposition 3 Watanabe, 1991 8 68 y Partial situs inversus 4 Ishitoya, 1997 14 10 mo Left IVC transposition 5 Rubinstein, 1999 9 45 y Duplicated IVC 6 Gramegna, 2003 10 19 y Left IVC transposition 7 Kozyrakis, 2012 11 75 y Left IVC transposition 8 Kim, 2013 12 24 y Duplicated IVC 9 Thirugnanasambandam, 2015 13 84 y Left IVC transposition Abbreviation: IVA, inferior vena cava.

4 Ishitoya, 1997 14 10 mo Left IVC transposition 5 Rubinstein, 1999 9 45 y Duplicated IVC 6 Gramegna, 2003 10 19 y Left IVC transposition 7 Kozyrakis, 2012 11 75 y Left IVC transposition 8 Kim, 2013 12 24 y Duplicated IVC 9 Thirugnanasambandam, 2015 13 84 y Left IVC transposition Abbreviation: IVA, inferior vena cava. The treatment includes ureteral division, relocation, and ureteroureterostomy with or without ureteral segmental resection. It can be performed as open surgery or using minimal invasive techniques. Laparoscopic transperitoneal treatment for this condition was first described in adults by Baba et al in 1994 15 and retroperitoneal approach was first used in 1999. 16 17 In children, laparoscopic approach has been used in 2002. 18 The first case of robot-assisted laparoscopic correction of a retrocaval ureter was done in a child in 2006. 19 Resection of the retrocaval segment is described and may be needed, due to fibrosis, dysplasia, or narrowing of the retrocaval ureteral segment. We did not perform any ureteral resection, as we did not encounter any intrinsic obstruction. Spatulation of the distal ureter allowed us to perform a wide anastomosis, using separate stitches, over an ureteral stent. Ureteroureterostomy was the most challenging and time-consuming part of the intervention. Running-sutures may provide a faster intracorporeal suture. Transperitoneal intracorporeal suturing is less time-consuming and easier than retroperitoneal suturing. 20 A minilaparotomy to perform the anastomosis, after finishing the laparoscopic dissection and transection of the ureter, can be used to reduce operative time. 21

sutures may provide a faster intracorporeal suture. Transperitoneal intracorporeal suturing is less time-consuming and easier than retroperitoneal suturing. 20 A minilaparotomy to perform the anastomosis, after finishing the laparoscopic dissection and transection of the ureter, can be used to reduce operative time. 21 Conclusions There is no consensus or surgical gold standard to treat this malformation. The laparoscopic transperitoneal approach to treat retrocaval ureter is a feasible technique with an excellent postoperative result in children. New Insights and the Importance for the Pediatric Surgeon Retrocaval ureter is a rare malformation that can be efficiently treated by laparoscopic surgery. Left retrocaval ureter is exceptionally rare and is associated with caval vein anomaly. Conflict of Interest None.

Introduction Langerhans's cell histiocytosis (LCH) is a rare disease characterized by abnormal infiltration of certain organs by dendritic cells. 1 Pulmonary involvement in LCH (PLCH) is more common in adults and mostly occurs in smokers. 2 The estimated incidence of LCH is 4 to 9 cases per 1 million in children. 3 4 Pulmonary involvement as an isolated entity is extremely rare in children and usually can be seen as part of a systemic disease. Pathogenesis of PLCH differs from that of adults where most of the affected adult cases are cigarette smokers. However, no causative relationship has been proven between smoking and pediatric PLCH, passive smoking may considered in the etiology. An uncontrolled immune response to an unknown stimulus or antigen is suggested in the pathogenesis in children. 5 The clinical findings of PLCH are nonproductive cough and dyspnea. Spontaneous pneumothorax (SP) is resulting from a rupture of subpleural bullae and cystic lesions occur in 10 to 20% of cases. 6 Pleurodesis, cysts excision, and partial or total pleurectomy are common surgical options. Extensive parenchymal destruction due to fibrosis may lead to respiratory failure and end-stage pulmonary disease. 7 There is no consensus on the optimum treatment of bilateral PLCH in children. A 3-year-old boy with SP due to bilateral PLCH is presented to discuss the surgical options of recurrent and refractory PLCH cases in children. Case Report

The clinical findings of PLCH are nonproductive cough and dyspnea. Spontaneous pneumothorax (SP) is resulting from a rupture of subpleural bullae and cystic lesions occur in 10 to 20% of cases. 6 Pleurodesis, cysts excision, and partial or total pleurectomy are common surgical options. Extensive parenchymal destruction due to fibrosis may lead to respiratory failure and end-stage pulmonary disease. 7 There is no consensus on the optimum treatment of bilateral PLCH in children. A 3-year-old boy with SP due to bilateral PLCH is presented to discuss the surgical options of recurrent and refractory PLCH cases in children. Case Report A 3-year-old boy with severe respiratory distress admitted to emergency department. He had pneumothorax at right chest and underwent tube thoracostomy ( Fig. 1A ). In his past medical history, he has free of symptoms and had no chronic disease. None of the family members had lung disease and none of them were smokers. At admission, his vital signs were within normal limits except high respiratory rate (45/minute). Total blood count, liver, and renal function tests were also unremarkable. On physical examination, a 2 × 1 cm palpable mass was noted at right side of neck. Computed tomography (CT) of chest revealed bilateral air cysts in varying sizes with ground glass appearance ( Fig. 1B ). Pneumothorax was also detected. The patient underwent biopsy form the neck mass and diagnosed as LCH confined to right salivary gland. The histopathologic features include polymorph nuclear leucocytes and atypical histocytes with oval nucleus with pale granular cytoplasm. Immunohistochemical staining with CD1a (cluster of differentiation 1 a) and S100 were positive. Chemotherapy including vinblastine and prednisone was initiated. During follow-up, pneumothorax persisted and another chest tube was inserted on the left side. Since the patient was unresponsive to medical treatment 2 weeks after admission, chemical pleurodesis with talc (2 cc) was performed. The pleurodesis was performed through tube thoracostomy. No intrapleural analgesics were used. The tube was clamped for 1 hour and unclamped at the end of procedure. During the clamped period, patients recommended to rotate on each side for 10 minutes. At the end of 10 days follow-up, patient developed bilateral pneumothorax with subcutaneous emphysema despite tube thoracostomy. Thoracoscopic bullae excision with pleural decortication was performed 10 days after talc pleurodesis ( Fig. 2 ). Histopathological evaluation of pleural biopsies obtained by thoracoscopy revealed inflammation and foreign bodies (talc). No lung biopsy was sampled during bullae excision. After surgical excision of subpleural bullae, the patient was symptom free for only 1 week and after developed respiratory insufficiency.

ig. 2 ). Histopathological evaluation of pleural biopsies obtained by thoracoscopy revealed inflammation and foreign bodies (talc). No lung biopsy was sampled during bullae excision. After surgical excision of subpleural bullae, the patient was symptom free for only 1 week and after developed respiratory insufficiency. Although total pleurectomy was considered for the patient, due to nosocomial pulmonary infection and severe respiratory distress, mechanical ventilation was initiated. Despite high-frequency ventilation, the oxygenation was inadequate and higher pressures are required. Five weeks after the bullae excision, extracorporeal membranous oxygenation (ECMO) support is initiated with vein-venous catheterization via internal jugular vein. At the 24th day of ECMO, he developed cranial bleeding and ventricular herniation and died of bleeding complications. Fig. 1 ( A ) Chest X-ray of the patient showing pneumothorax on right side. ( B ) Computed tomography revealing bilateral cysts and ground glass appearance. Fig. 2 Thoracoscopic image of cystic lesion. Discussion

Although total pleurectomy was considered for the patient, due to nosocomial pulmonary infection and severe respiratory distress, mechanical ventilation was initiated. Despite high-frequency ventilation, the oxygenation was inadequate and higher pressures are required. Five weeks after the bullae excision, extracorporeal membranous oxygenation (ECMO) support is initiated with vein-venous catheterization via internal jugular vein. At the 24th day of ECMO, he developed cranial bleeding and ventricular herniation and died of bleeding complications. Fig. 1 ( A ) Chest X-ray of the patient showing pneumothorax on right side. ( B ) Computed tomography revealing bilateral cysts and ground glass appearance. Fig. 2 Thoracoscopic image of cystic lesion. Discussion Langerhans's cell histiocitosis (LCH) is a rare disease occurring in 4 to 9 cases per 1 million children. 8 It encopresis a heterogeneous spectrum of clinical findings and may involve either a single organ or present as multisystem disease. Pulmonary involvement is seen in 25% of all pediatric cases and usually as part of a multiorgan LCH. Isolated PLCH is reported only 1% of pediatric cases. 9 Bilateral PLCH is also extremely rare in children and may have an unpredictable course and lethal outcome. It predominantly affects young adults between the ages 20 and 40 years, and there is no gender predilection. Pediatric PLCH peaks between 1 and 3 years of age. The exact incidence of PLCH in children is unknown because significant numbers of cases are asymptomatic and may resolve spontaneously. 10 Although it constitutes 4 to 5% of all pediatric lung biopsies, histopathologic diagnosis of PLCH is difficult in advanced disease.

ediatric PLCH peaks between 1 and 3 years of age. The exact incidence of PLCH in children is unknown because significant numbers of cases are asymptomatic and may resolve spontaneously. 10 Although it constitutes 4 to 5% of all pediatric lung biopsies, histopathologic diagnosis of PLCH is difficult in advanced disease. The pathogenesis of PLCH in adults is attributed to cigarette smoking and 95% of affected adults are smokers. In children, immune response to an unknown antigen and/or stimulus is suggested as the underlying cause of LCH. PLCH presents with tachypnea, chest pain, dyspnea, cyanosis, chronic, and persistent cough. SP resulting rupture from cystic lesions may occur as initial presentation. Our patient was also admitted to emergency department with SP and dyspnea. The diagnosis of PLCH is characteristically based on the radiologic findings. It may consist of cystic lesions varying in size and frequently involves lower lobes in children. CT typically shows nodular and reticular lesions in early stages of the disease and cystic lesions in advanced disease. CT scan of our patient also suggested advanced PLCH showing cystic lesions with ground glass appearance. The ground glass opacities are very rare radiologic findings of PLCH and correspond to desquamated interstitial pneumonia like changes. 11 According to the stage of disease, different radiologic findings can be seen. Nodular and ground glass appearance also exhibit regression of PLCH, while emphysema and fibrotic changes suggest persistence and progression. Other nodular and interstitial lung diseases should be differentiated from PLCH. The silicosis and sarcoidosis may have similar nodular lesions in chest scans but very rare in children. Also, different from the infectious causes, PLCH has no lymph nodes in perihilar areas.

c changes suggest persistence and progression. Other nodular and interstitial lung diseases should be differentiated from PLCH. The silicosis and sarcoidosis may have similar nodular lesions in chest scans but very rare in children. Also, different from the infectious causes, PLCH has no lymph nodes in perihilar areas. Children with progressive PLCH can be treated with chemotherapy including vinblastine, prednisone, methotrexate, cyclophosphamide, and etoposide. 12 The clinical response to chemotherapy is unpredictable in children and requires long-term follow-up. Vinblastine and prednisone was the choice of chemotherapy in our patient and partial response was obtained during the medical treatment. The role of passive cigarette smoking in the development of PLCH is controversial in children. However, there is no prospective data suggesting improvement of outcome by stopping smoke exposure; it is highly recommended.

oice of chemotherapy in our patient and partial response was obtained during the medical treatment. The role of passive cigarette smoking in the development of PLCH is controversial in children. However, there is no prospective data suggesting improvement of outcome by stopping smoke exposure; it is highly recommended. The treatment option of recurrent pneumothoraces is tube thoracotomy in most cases. Other surgical interventions are needed when there is no improvement in respiratory functions after 12-week course of chemotherapy; persistent pneumothorax due to chest tubes or worsening of CT scan findings during follow-up. 13 The conservative treatment of recurrent pneumothoraces in PLCH usually fails since the underlying lungs are very fragile and tend to collapse easily. The recurrence rate of pneumothorax treated with chest tube is only 58%. 14 Chemical pleurodesis has limited rate of success in the treatment of extensive PLCH and not recommended if there is a possibility of lung transplantation. At the early stages of disease, we used chemical pleurodesis with talc after full extension of lungs with tube thoracostomy. However, talc pleurodesis was failed and did not prevent recurrent pneumothoraces. Abdul Aziz et al reported that mechanical pleurodesis is superior to chemical pleurodesis. 13 It can be performed by either with thoracotomy or thoracoscopy. It is not considered as a contraindication for transplantation. Therefore, thoracscospic excision of cysts with partial pleurectomy was performed. In extensive PLCH, partial pleurectomy and/or abrasion of pleura may not be sufficient to eradicate persistent pneumothorax. Total pleurectomy is an aggressive surgical alternative that can be used in recurrent pneumothorax. Although better outcomes are reported, it should be the last resort of surgical treatment in children. For our patient, authors discussed to perform bilateral total pleurectomy as last surgical option to avoid recurrence. But, due to nosocomial respiratory infection and need of ventilator support, it was not possible to perform total pleurectomy.

reported, it should be the last resort of surgical treatment in children. For our patient, authors discussed to perform bilateral total pleurectomy as last surgical option to avoid recurrence. But, due to nosocomial respiratory infection and need of ventilator support, it was not possible to perform total pleurectomy. Lung transplantation is a therapeutic option in selected cases with progressive disease. 7 It should be considered when severe pulmonary hypertension exists and not response to vasodilator treatment. Surgical pleurodesis did not preclude lung transplantation. ECMO has been shown to be a valid alternative as a bridge for lung transplantation in PLCH. 15 It was not considered as an alternative treatment in our case because of severe deterioration of the patient. The prognosis of PLCH is variable and unpredictable in pediatric age group. The poor outcome of the PLCH is related with extremes age, prolonged worse conditions, multiorgan involvement, reduced diffusion capacity, extensive cysts and honeycombing on imaging, recurrent pneumothoraces, pulmonary hypertension, and prolonged treatment. 16 Death may occur due to pulmonary hypertension and/or severe respiratory insufficiency. Our patient had most of the poor prognostic factors and died due to severe pulmonary insufficiency and ECMO complications.

sts and honeycombing on imaging, recurrent pneumothoraces, pulmonary hypertension, and prolonged treatment. 16 Death may occur due to pulmonary hypertension and/or severe respiratory insufficiency. Our patient had most of the poor prognostic factors and died due to severe pulmonary insufficiency and ECMO complications. Conclusion In conclusion, despite the use of chemotherapy and surgical excision of cystic lesions, bilateral PLCH in children may have lethal outcome. Other treatment options including respiratory support with ECMO and lung transplantation should be kept in mind in persistent cases. New Insights and the Importance for the Pediatric Surgeon Despite the use of chemotherapy and surgical excision of cystic lesions, bilateral pulmonary Langerhans's cell histiocytosis in children may have lethal outcome. Other treatment options including respiratory support with extracorporeal membranous oxygenation and lung transplantation should be considered in persistent cases. Acknowledgement Authors would like to thank to Professor Alev Özön for her kind contribution to organize a multidisciplinary approach for the patient. Conflict of Interest None. Note This case report is presented in the Joint Congress of 36th National Congress of Turkish Association of Pediatric Surgeons and International Pediatric Endosurgery Group (IPEG), Middle East Chapter, October 24 to 27, 2018, İzmir, Turkey.

Introduction Mesenteric cysts are rare intra-abdominal benign tumors with no classical clinical feature. 1 They were first reported in 1507 by the Italian anatomist Benevieni after an autopsy of an 8 years old girl. 2 The frequency is 1 in 100, 000 to 350, 000 adult hospital admissions 3 4 and 1 in 20,000 to 35,000 pediatrics hospital admissions. 4 It mostly arises from the mesenteric border of small intestine. The size varies from 8 to 35 cm. 5 Commonly patients complain of abdominal swelling, abdominal pain, early satiety, vomiting, diarrhea, and even present with an acute abdomen. Abdominal computed tomography (CT) scan, magnetic resonance imaging (MRI) and abdominal ultrasound are diagnostic. Surgical complete excision of the cyst is the treatment of choice with a very low recurrence rate. Right sided syndrome is a congenital absence of right kidney and right testis occurring in a single patient. Unilateral renal agenesis may be associated with anomalies of the genitourinary system, such as absence of testis, epididymis, and uterine anomalies. Again, abdominal ultrasound and MRI are diagnostic. 6

Mesenteric cysts are rare intra-abdominal benign tumors with no classical clinical feature. 1 They were first reported in 1507 by the Italian anatomist Benevieni after an autopsy of an 8 years old girl. 2 The frequency is 1 in 100, 000 to 350, 000 adult hospital admissions 3 4 and 1 in 20,000 to 35,000 pediatrics hospital admissions. 4 It mostly arises from the mesenteric border of small intestine. The size varies from 8 to 35 cm. 5 Commonly patients complain of abdominal swelling, abdominal pain, early satiety, vomiting, diarrhea, and even present with an acute abdomen. Abdominal computed tomography (CT) scan, magnetic resonance imaging (MRI) and abdominal ultrasound are diagnostic. Surgical complete excision of the cyst is the treatment of choice with a very low recurrence rate. Right sided syndrome is a congenital absence of right kidney and right testis occurring in a single patient. Unilateral renal agenesis may be associated with anomalies of the genitourinary system, such as absence of testis, epididymis, and uterine anomalies. Again, abdominal ultrasound and MRI are diagnostic. 6 Case Presentation A 15 years old male presented with the compliant of progressive abdominal swelling since 10 years which had further progressed within the preceding 12 months.” Furthermore he experienced early satiety, unable to run, difficulty walking, shortness of breath in lying position, and significant subjective weight loss in 3 months duration. He was a grade VII student but discontinued school due to walking difficulty for the last 3 months. Two years prior to the current presentation he was admitted for with chylous ascites diagnosed by abdominal ultrasound at our hospital; however, a CT or MRI was not done at this time and a planned treatment with octreotide could not be performed as the patient left the hospital prior to that.

At admission she presented mild dyspnea and breath sounds were present only in the left hemithorax, while heart sounds could be heard on the right hemithorax. A chest X-ray confirmed the right atelectasis and no cardiac shadow was visible in the left hemithorax. Echocardiography revealed a right cardiac shift rather than dextrocardia and a severely hypoplastic right pulmonary artery. The newborn required noninvasive ventilation with nasal continuous positive airway pressure (nCPAP) with FiO 2 0.21 to maintain an arterial oxygen saturation of 98% with a good respiratory dynamic. On day 3 of her life, a chest computed tomography (CT) scan ( Fig. 1A–C ) was performed and revealed an anomalous origin of the right main bronchus from the distal esophagus at the level of T9, while the trachea ended directly into the left main bronchus at the level of T4. The CT scan also confirmed the presence of severe right pulmonary artery hypoplasia. The right lung was partially air filled possibly after retrograde air inflation from the digestive tract, mainly due to the nCPAP. An associated pulmonary airway malformation could not be excluded by the CT scan. The diagnosis of right esophageal lung was made and confirmed by an esophageal contrast study performed on the same day ( Fig. 1D ).

lty for the last 3 months. Two years prior to the current presentation he was admitted for with chylous ascites diagnosed by abdominal ultrasound at our hospital; however, a CT or MRI was not done at this time and a planned treatment with octreotide could not be performed as the patient left the hospital prior to that. On physical examination he was looking chronically sick, the vital signs were within normal limits. The abdomen was significantly distended ( Fig. 1 ) with dullness on percussion over the whole abdomen with a positive fluid thrill. The right scrotum was empty. The complete blood cell count, renal and liver function tests, and serum albumin and stool examination were normal. Abdominal ultrasound and abdominal CT scan showed a 20 × 32 cm giant mesenteric cyst, absent right kidney and left moderate hydronephrosis due to the compression effect from the mass ( Fig. 2 ). On exploratory laparotomy through a midline abdominal incision a huge retroperitoneal cyst from duodenum to sacrum was found. The cyst was completely excised and right orchiectomy was done for intra-abdominal testis to prevent testicular germ cell malignancy. Gram stain, culture, and gene expert of the cystic fluid was negative with 7/mm 3 white blood cells. A postoperative abdominal ultrasound postoperative day 7 showed a mild left sided hydronephrosis. The patient recovered uneventfully and was discharged 1 day later. Histology of the resected testis showed an atrophic testis without sign of malignant transformation. Follow-up on 20th post surgery was unremarkable.

ells. A postoperative abdominal ultrasound postoperative day 7 showed a mild left sided hydronephrosis. The patient recovered uneventfully and was discharged 1 day later. Histology of the resected testis showed an atrophic testis without sign of malignant transformation. Follow-up on 20th post surgery was unremarkable. Fig. 1 Significantly distended abdomen at presentation. Fig. 2 ( A and B ) Axial post contrast CT scan of the abdomen, 20 × 32 cm huge cystic mass displacing the entire visceral organt to the back, absent right kidney and dilatation of renal pelvis and calyces of left kidney due to mass effect. CT, computed tomography. Discussion Mesenteric cysts can arise from jejunum to the rectum, mostly from mesenteric border of the ileal mesentery. The possible etiology includes a benign proliferation of ectopic mesenteric lymphatic vessels which lack communication with the remaining lymphatic system, 7 trauma, surgery, and neoplasms. 8 It can occur at any age and approximately one-third of cases are found in children younger than 15 years. 9 Mesenteric cysts in pediatrics age group are seen often in males (62.5%). The majority of the patients are younger than 10 years, and 75% younger than 5 years of age. 7 The clinical presentation is nonspecific and depends on the size and the site of the cyst.

hird of cases are found in children younger than 15 years. 9 Mesenteric cysts in pediatrics age group are seen often in males (62.5%). The majority of the patients are younger than 10 years, and 75% younger than 5 years of age. 7 The clinical presentation is nonspecific and depends on the size and the site of the cyst. Commonly, patients complain of abdominal swelling with or without pain, early satiety, vomiting, diarrhea, dyspepsia, and constipation. In extreme cases, infection or rupture associated with an acute abdomen has been reported. 10 11 Our patient showed abdominal painful swelling, early satiety and walking difficulty. The 10-year duration of symptoms is explained by the slow growth of the cyst and diagnostic difficulty of mesenteric cysts by primary health care providers. Abdominal CT scan, abdominal ultrasound, and MRI are diagnostic for mesenteric cysts. 10 12 Surgical complete excision of the cyst is the treatment of choice 13 14 with very low recurrence rates and prevents malignant transformation into adenocarcinoma. 15 16 Simple drainage and marsupialization are also the treatment options with unacceptable recurrence and infection. 7

Commonly, patients complain of abdominal swelling with or without pain, early satiety, vomiting, diarrhea, dyspepsia, and constipation. In extreme cases, infection or rupture associated with an acute abdomen has been reported. 10 11 Our patient showed abdominal painful swelling, early satiety and walking difficulty. The 10-year duration of symptoms is explained by the slow growth of the cyst and diagnostic difficulty of mesenteric cysts by primary health care providers. Abdominal CT scan, abdominal ultrasound, and MRI are diagnostic for mesenteric cysts. 10 12 Surgical complete excision of the cyst is the treatment of choice 13 14 with very low recurrence rates and prevents malignant transformation into adenocarcinoma. 15 16 Simple drainage and marsupialization are also the treatment options with unacceptable recurrence and infection. 7 The term right sided syndrome is used when the absence of the right kidney and right testis occurs in a single patient. 17 The kidney and the testis are derived from the intermediate mesoderm during embryogenesis. Renal agenesis may be unilateral or bilateral occurring from failure of induction by the ureteric bud or errors in development of the mesonephric duct. 18 Unilateral renal agenesis is a developmental defect associated with anomalies of the genitourinary system, such as absence of testis (cryptorchidism), epididymis, and anal, vertebral, or uterine anomaly. 17 18 The gold standard for diagnosis of a solitary kidney after detection on ultrasound is MRI. 19 Individuals who have solitary kidney should be informed about the case and regular follow-up must be adjusted. All possible nephrotoxic drugs have to avoid as much as possible.

s, and anal, vertebral, or uterine anomaly. 17 18 The gold standard for diagnosis of a solitary kidney after detection on ultrasound is MRI. 19 Individuals who have solitary kidney should be informed about the case and regular follow-up must be adjusted. All possible nephrotoxic drugs have to avoid as much as possible. In our case, abdominal ultrasound was suggestive for huge mesenteric cyst and abdominal CT scan showed 20 × 32 cm giant mesenteric cyst, absent right kidney, and left moderate hydronephrosis due to compression effect from the cyst. Createnine and blood urea nitrogen were normal. The patient underwent open surgery through midline access. The cyst excised completely and right orchiectomy was done and the patient recovered uneventfully. The probability of a malignant neoplasm developing in an undescended testicle is approximately 20 to 48 times greater than a normally descended testicle and higher (approximately 5%) for intra-abdominal testicles. 20 21 Testicular tumor in intra-abdominal testis can occur as early as 1st year of life and higher frequency starting from puberty. 22 In cases of testicular atrophy, it must be considered whether orchiectomy, a testicular prosthesis or a close follow-up regime for detecting carcinoma-in-situ-testis or invasive testicular neoplasia are options of treatment. 23 24 In our patient the testis was atrophied and orchiectomy was done as there is no prosthesis and limitation of close follow-up and early detection of neoplasms. 25 The left hydronephrosis resolved postoperatively evidenced on subsequent ultrasounds. To the best of our knowledge, there is no case report of giant mesenteric cyst and right sided syndrome in a single patient.

omy was done as there is no prosthesis and limitation of close follow-up and early detection of neoplasms. 25 The left hydronephrosis resolved postoperatively evidenced on subsequent ultrasounds. To the best of our knowledge, there is no case report of giant mesenteric cyst and right sided syndrome in a single patient. Conclusion In conclusion, we present the first coincidental finding of a right sided syndrome and giant mesenteric cyst. Whether this observation represents a true association between two diseases remains unknown. However, in children with renal agenesis and ipsilateral cryptorchidism surgeons may also expect additional abdominal pathology, such as mesenteric cysts. New Insights and the Importance for the Pediatric Surgeon Giant mesenteric cyst can be symptomatic for prolonged duration more than 12 months (for10 years) without life threatening complication. Right sided syndrome and giant mesenteric cyst can occur in a single patient with unknown association which need for explanation. Acknowledgments The authors would like to acknowledge Dr. Laway Beza and all those involved in the management of the patient. Conflict of Interest None.

Introduction Infantile fibrosarcoma (IFS) is a very rare condition occurring in approximately five new cases per 1 million children. 1 IFS accounts for less than 1% of childhood cancers and for 24% of soft tissue sarcomas. This is a rare nonrhabdomyosarcoma soft tissue tumor which develops at the expense of connective tissue. IFS accounts for 5 to 10% of soft tissue sarcoma before 1 year. 2 The average age at diagnosis is 3 months but 40% of tumors are present at birth. 2 Only 10% of IFS occurs in the abdomen and few of them involve the gastrointestinal tract. 3 We present a case of neonatal intestinal fibrosarcoma diagnosed prenatally as a meconium cyst. Case Report A child of unrelated parents was born at 37 gestation weeks (GW) by Caesarean section (weight 2,630 g). At 30 GW, fetal ultrasound (US) showed peritoneal ascites with septations and an echogenic mass adjacent to a right dilated bowel loop with an echogenic content ( Fig. 1 ). Antenatal magnetic resonance (MR) imaging at 33 GW confirmed the diagnosis ( Fig. 2 ) and demonstrated normal jejunal loops in the left flanck. This prenatal imaging was compatible with a bowel atresia, Meconium peritonitis (MP), and associated pseudocyst (cystic meconium peritonitis). Fig. 1 Prenatal ultrasound, transverse slice at the level just below the liver: visualization of a hyperechogenic mass (white solid arrows) adjacent to a dilated digestive loop (white dotted arrows), surrounded by anechogenic ascites (asterisk [*]) in the right hemiabdomen.

A child of unrelated parents was born at 37 gestation weeks (GW) by Caesarean section (weight 2,630 g). At 30 GW, fetal ultrasound (US) showed peritoneal ascites with septations and an echogenic mass adjacent to a right dilated bowel loop with an echogenic content ( Fig. 1 ). Antenatal magnetic resonance (MR) imaging at 33 GW confirmed the diagnosis ( Fig. 2 ) and demonstrated normal jejunal loops in the left flanck. This prenatal imaging was compatible with a bowel atresia, Meconium peritonitis (MP), and associated pseudocyst (cystic meconium peritonitis). Fig. 1 Prenatal ultrasound, transverse slice at the level just below the liver: visualization of a hyperechogenic mass (white solid arrows) adjacent to a dilated digestive loop (white dotted arrows), surrounded by anechogenic ascites (asterisk [*]) in the right hemiabdomen. Fig. 2 Fetal MRI, coronal slices (T2-sequence on the left side, T1 on the right side): the abdominal mass (white arrows) appears as an intermediate hyperintense T2-lesion and a hypointense to isointense T1-lesion. Visualization of some adjacent dilated bowel loops with meconial content (tri-line arrow). The dotted arrows show the liver. MRI, magnetic resonance imaging.

e left side, T1 on the right side): the abdominal mass (white arrows) appears as an intermediate hyperintense T2-lesion and a hypointense to isointense T1-lesion. Visualization of some adjacent dilated bowel loops with meconial content (tri-line arrow). The dotted arrows show the liver. MRI, magnetic resonance imaging. The prenatal investigation of the amniotic fluid was normal (karyotype and digestives enzymes) and cystic fibrosis was ruled out. At birth, the abdomen was distended. Contrast enema demonstrated a micro colon. The US revealed echogenic ascites with septations, a large epigastric dilated digestive loop with echogenic content, surrounded by a large, hyperechoic mass, with slight vascularization on the Doppler. At surgery, MP with a small bowel stenosis (25 cm proximal to the ileocaecal junction) was confirmed along with a soft tissue mass adjacent to the liver and the posterior peritoneum. Taken together, these observations suggested the presence of a meconium cyst. The mass was resected with difficulties related to the close relation with the liver, and anastomosis was performed. Due to extensive adhesiolysis, the resection was complicated by a haemorrhagic shock and required a cardiopulmonary resuscitation (37 minutes and 10 adrenaline doses). Due to extensive adhesiolysis, the resection was complicated by a hemorrhagic shock, that was characterized by a hemoglobin level lower than 8 g/dL, and below normal coagulation factor levels (platelets, aPTT [activated partial thromboplastin time], and factors V, and VIII) that barely reached 30% of the expected values. We performed a cardiopulmonary resuscitation that lasted 37 minutes and required the injection of 10 adrenaline doses and the transfusion of 100 mL/kg of packed red blood cells (PRCB), platelets, and 2 units of fresh frozen plasma (FFP). Despite medical treatment, including the administration of albumin and vasoactive drugs, the noncontrolled bleeding persisted, requiring a second surgery to place a perihepatic packing. It was removed 5 days later.

usion of 100 mL/kg of packed red blood cells (PRCB), platelets, and 2 units of fresh frozen plasma (FFP). Despite medical treatment, including the administration of albumin and vasoactive drugs, the noncontrolled bleeding persisted, requiring a second surgery to place a perihepatic packing. It was removed 5 days later. The histopathologic examination revealed a dense cellular tumor in the ileal intestinal wall with spindle-shaped cells in clusters from submucosa to subserosa. The tumor cells had oval nuclei and blurred cytoplasmic limits with high mitotic activity and frequent apoptosis. They were arranged in interlacing fascicles following a characteristic herringbone pattern. The blood vessels were sheathed in the tumor and their walls were thickened. Moreover, inside the segment named “meconium cyst,” there was a lumen which was full of meconium and bordered by an intestine mucosa infiltrated with tumor cells meaning that the “cyst” was a part of the tumor ( Fig. 3 ). Fig. 3 Intestinal fibrosarcoma (hematoxylin and eosin: original magnification ×25 and ×250 in inset), dense spindle cells proliferation occurring in the submucosa and extending to the whole intestinal wall.

The histopathologic examination revealed a dense cellular tumor in the ileal intestinal wall with spindle-shaped cells in clusters from submucosa to subserosa. The tumor cells had oval nuclei and blurred cytoplasmic limits with high mitotic activity and frequent apoptosis. They were arranged in interlacing fascicles following a characteristic herringbone pattern. The blood vessels were sheathed in the tumor and their walls were thickened. Moreover, inside the segment named “meconium cyst,” there was a lumen which was full of meconium and bordered by an intestine mucosa infiltrated with tumor cells meaning that the “cyst” was a part of the tumor ( Fig. 3 ). Fig. 3 Intestinal fibrosarcoma (hematoxylin and eosin: original magnification ×25 and ×250 in inset), dense spindle cells proliferation occurring in the submucosa and extending to the whole intestinal wall. Immunohistochemistry showed that tumor cells only expressed vimentin, to the exclusion of markers like desmin, catenin, and transmembrane phosphoglycoprotein protein encoded by the CD34 gene (cluster of differenciation 34 founded in hematopoietic cells) (CD34). Many nuclei expressed K i -67. Cytogenetic examination with fluorescence in situ hybridization (FISH) analysis did not confirmed a rearrangement of the ETV6 region, and an translocation of genetic material between the ETV6 gene located on the short arm of chromosome 12 at position p13. and the NTRK3 gene located on the long arm of chromosome 15 at position q25.3 to create the (12;15)(p13;q25) fusion gene, ( ETV6-NTRK3 ) fusion transcript was not detected using Reverse Transcriptase - Polymerase Chain Reaction (RT-PCR).

l between the ETV6 gene located on the short arm of chromosome 12 at position p13. and the NTRK3 gene located on the long arm of chromosome 15 at position q25.3 to create the (12;15)(p13;q25) fusion gene, ( ETV6-NTRK3 ) fusion transcript was not detected using Reverse Transcriptase - Polymerase Chain Reaction (RT-PCR). Because untreatable and fatal neurological sequelae were diagnosed, secondary to the hemorrhagic shock, palliative care was provided to the patient who died at 63 days of life. Discussion The interest of this case lies in its location and its diagnostic timeline. Clinical Presentation

l between the ETV6 gene located on the short arm of chromosome 12 at position p13. and the NTRK3 gene located on the long arm of chromosome 15 at position q25.3 to create the (12;15)(p13;q25) fusion gene, ( ETV6-NTRK3 ) fusion transcript was not detected using Reverse Transcriptase - Polymerase Chain Reaction (RT-PCR). Because untreatable and fatal neurological sequelae were diagnosed, secondary to the hemorrhagic shock, palliative care was provided to the patient who died at 63 days of life. Discussion The interest of this case lies in its location and its diagnostic timeline. Clinical Presentation In 71% of the cases, IFS develops in the limbs. Nevertheless, several ectopic cases were observed, particularly in the trunk 4 5 or the neck. 4 Some of the described axial cases are located in the abdominal region and derive from the intestine. 3 They are associated with either a digestive perforation or a bowel obstruction. Some of the described axial cases are located in the abdominal region and derive from the intestine. 3 The study of the Berrebi cohort show that abdominal IFS are frequently associated with either a MP complicating a digestive perforation on the mesenteric side of the tumor wall (54% of the cases), or a bowel obstruction (30% of the cases). 3 A big mass could lead to a perforation and stenosis that is susceptible to hide the primitive cause and thus complicate the diagnosis. In our case, the cyst was closely related to the liver; therefore, the surgical dissection and separation of the cyst from the surrounding structures were extensive and leading significant blood loss and ultimately hemorrhagic shock. This finding must make think to another diagnosis than a usual meconium cyst related to an atresia.

the cyst was closely related to the liver; therefore, the surgical dissection and separation of the cyst from the surrounding structures were extensive and leading significant blood loss and ultimately hemorrhagic shock. This finding must make think to another diagnosis than a usual meconium cyst related to an atresia. When IFS is located in the abdomen, differential diagnosis, such as all intra-abdominal tumors, any remainder malignant mesenchymal tumors or sarcomas, 2 hemangioma or infantile fibromatosis, 2 and Meckel's diverticulum, 3 5 should be ruled out first. In our case, all the difficulties came from the unknown presurgical diagnosis. Imaging On imaging IFS have nonspecific features. On US, they usually appear as a heterogeneous, well-defined soft tissue single mass, 4 6 most often hypoechogenic with an anechoic part (cystic). 4 On the Doppler, the blood flow is increased, especially at the periphery. 4 Magnetic resonance imaging (MRI) is the imaging of choice. The expected imaging results for IFS are well-defined heterogeneous soft tissue mass, with T1-low signal and T2-hyper signal. 4 7 8 Sometimes an hemorrhagic or necrotic content can be observed. 4 Internal fibrous septa have also been described. 7 High-flow vascular structures can be seen and after contrast injection, intense enhancement is usually depicted, which can be heterogeneous and predominant at the periphery. 4 8 However, we found a heterogeneous mass to the exclusion of any other criteria. This nonspecific observation led us to diagnose a meconial cyst.

igh-flow vascular structures can be seen and after contrast injection, intense enhancement is usually depicted, which can be heterogeneous and predominant at the periphery. 4 8 However, we found a heterogeneous mass to the exclusion of any other criteria. This nonspecific observation led us to diagnose a meconial cyst. In our case, the MP associated to a distal bowel atresia was misleading and the mesenteric lesion visualized on imaging was not taken into consideration at first, despite the Doppler showed a slightly increased vascularization but without preferential location and an heterogeneous mass which evoked a solid lesion. Fetal MRI was not helpful and postnatal MRI was not performed. Perhaps additional MRI should be performed when the diagnosis is ambiguous which was not done. Genetic and Anatomopathology Pathological analyses of IFS show a tumoral lesion with regular walls, made of layers of closely packed spindle-shaped cells arranged in bundles and fascicles, resulting in a herringbone pattern. The mitotic activity is high and the nucleus is hyperchromatic. The vascularization is rich, and it is possible to find hemorrhagic, necrotic, or inflamed areas. 4 7 In immunohistochemistry, tumor cells express only vimentin whereas muscular markers, such as desmin, are negative 4 9 as found in our clinical report. The ETV6-NTRK3 fusion transcript (FT) associated with the t(12;15)(p13;q25) translocation is a good biomarker for diagnosis, and allowed us to differentiate IFS from adult fibrosarcoma, infantile fibromatosis, and other tumors with spindle cells.

Pathological analyses of IFS show a tumoral lesion with regular walls, made of layers of closely packed spindle-shaped cells arranged in bundles and fascicles, resulting in a herringbone pattern. The mitotic activity is high and the nucleus is hyperchromatic. The vascularization is rich, and it is possible to find hemorrhagic, necrotic, or inflamed areas. 4 7 In immunohistochemistry, tumor cells express only vimentin whereas muscular markers, such as desmin, are negative 4 9 as found in our clinical report. The ETV6-NTRK3 fusion transcript (FT) associated with the t(12;15)(p13;q25) translocation is a good biomarker for diagnosis, and allowed us to differentiate IFS from adult fibrosarcoma, infantile fibromatosis, and other tumors with spindle cells. The genetic tests did not reveal the presence of an ETV6-NTRK3 fusion transcript, which doesn't mean it was absent because this test has a 9% false negative rate 9 . Given the consistency of the above listed histological criteria, this finding didn't alter our diagnosis. Treatment and Prognosis The main treatment of neonatal IFS is the surgery with resection with as wide margins as possible, except if there is a risk of mutilation, in which case chemotherapy is used first. IFS indeed is a chemosensitive tumor that can be treated by the association vincristine-actinomycin (VA), a combination that demonstrated its efficacy with a response rate of 71%. 1 2

gery with resection with as wide margins as possible, except if there is a risk of mutilation, in which case chemotherapy is used first. IFS indeed is a chemosensitive tumor that can be treated by the association vincristine-actinomycin (VA), a combination that demonstrated its efficacy with a response rate of 71%. 1 2 The global survival rate varies between 80 to 100%. 9 Metastases are rare (less than 5% of cases) and mainly axial (localized on the trunk). 2 The main risk is local relapse, 2 9 with a rate between 5 and 40% according to groups and localization. 9 In our case, no alternative therapeutic could be considered because we never thought about a differential tumoral diagnosis. During the surgery, as the tumoral diagnosis never mentioned, the surgeon thought removing a cyst, usual complication about meconial peritonitis; thus, the surgery is the only alternative and performing chemotherapy was not an option. Maybe we found in this example, a reason of the wait and see approach before 3 months, because of the dramatic outcomes of the surgery while the tumoral evolution is not aggressive. However, the surgery was required because of the bowel stenosis. After surgery, chemotherapy was not an option since the cerebral lesions were too important

this example, a reason of the wait and see approach before 3 months, because of the dramatic outcomes of the surgery while the tumoral evolution is not aggressive. However, the surgery was required because of the bowel stenosis. After surgery, chemotherapy was not an option since the cerebral lesions were too important Conclusion Cystic meconium peritonitis (CMP) is a result of in-utero bowel perforation. It will lead to secondary inflammation to the peritoneal cavity resulting in fibrosis, calcification, and cyst formation. Difficult surgery is expected as a result of the fibroadhesion and inflammation. Any unexpected solid lesion should raise the suspicion of complicated bowel tumoral lesion. If a neoplastic lesion is suspected extensive surgery may be postponed until the final diagnosis is made New Insights and the Importance for the Pediatric Surgeon We present a brief report describing a rare case of neonatal fibrosarcoma in an unusual localization diagnosed prenatally as a meconium cyst. The surgery was complicated by hemorrhagic shock and it highlights the importance of combining prenatal and postnatal imaging and genetic test to try to reach the diagnosis. Conflict of Interest None declared.

Introduction Pancreatic pseudocyst (PP) is a localized collection of pancreatic secretions, rich in amylase and other enzymes that is lined by fibrous tissue and do not have an epithelial lining. Only few cases had been reported in childhood in literature. Mediastinal extension of a PP is even rarer. Depending on the mass effect it exerts, and it can cause pericardial or pleural effusion, dysphagia, or odynophagia. 1 We present a case of mediastinal pseudopancreatic cyst (MPP) in a 7-year-oldchild who present to our department without a prior history of acute pancreatitis or significant trauma. After clinical examination and imaging, it turned out to be a pseudopancreatic cyst extending to the mediastinum through the diaphragm. The patient underwent cystogastrostomy with uneventful postoperative course. Case Report

Pancreatic pseudocyst (PP) is a localized collection of pancreatic secretions, rich in amylase and other enzymes that is lined by fibrous tissue and do not have an epithelial lining. Only few cases had been reported in childhood in literature. Mediastinal extension of a PP is even rarer. Depending on the mass effect it exerts, and it can cause pericardial or pleural effusion, dysphagia, or odynophagia. 1 We present a case of mediastinal pseudopancreatic cyst (MPP) in a 7-year-oldchild who present to our department without a prior history of acute pancreatitis or significant trauma. After clinical examination and imaging, it turned out to be a pseudopancreatic cyst extending to the mediastinum through the diaphragm. The patient underwent cystogastrostomy with uneventful postoperative course. Case Report A 7-year-old male child was presented to the Pediatric Department in Shatbi University Hospital with recurrent chest infections since 1 year. Repeated chest X-ray showed left pleural effusion. The child was managed conservatively and discharged from the pediatric department. During the course of follow-up for the respiratory condition an abdominal ultrasound was ordered to investigate a new onset minor abdominal discomfort. An abdominal cyst was found which, otherwise, was not clinically palpable during abdominal examination. Subsequent computed tomography (CT) scanning of the abdomen and pelvis with intravenous contrast revealed a retroperitoneal thick-walled fluid filled mass, measuring approximately 11.5 × 13 cm in close relation to the main pancreatic duct with inflammation of the adjacent pancreatic tissue. Provisional reports indicated a pseudo-pancreatic cyst with further extension into the posterior mediastinum through one of the diaphragmatic hiatus ( Fig. 1 ). Serum amylase and lipase were markedly elevated (amylase: 45,630 U/I and lipase: 180,000 U/I).

reatic duct with inflammation of the adjacent pancreatic tissue. Provisional reports indicated a pseudo-pancreatic cyst with further extension into the posterior mediastinum through one of the diaphragmatic hiatus ( Fig. 1 ). Serum amylase and lipase were markedly elevated (amylase: 45,630 U/I and lipase: 180,000 U/I). Fig. 1 CT abdomen showing fluid containing cystic mass behind the stomach around the pancreas extending up to the chest. CT, computed tomography.

reatic duct with inflammation of the adjacent pancreatic tissue. Provisional reports indicated a pseudo-pancreatic cyst with further extension into the posterior mediastinum through one of the diaphragmatic hiatus ( Fig. 1 ). Serum amylase and lipase were markedly elevated (amylase: 45,630 U/I and lipase: 180,000 U/I). Fig. 1 CT abdomen showing fluid containing cystic mass behind the stomach around the pancreas extending up to the chest. CT, computed tomography. The decision after surgical consultation was to perform laparotomy. The child was prepared for the operation. Through an upper midline incision, the abdomen was explored. This revealed a large, smooth, and fluctuant mass behind the stomach extending up to the posterior mediastinum through the esophageal hiatus, mostly arising from the pancreas. Aspiration of the coffee ground contents was done followed by a drainage procedure by anastomosing the posterior wall of the stomach to the anterior wall of the cyst wall (cystogastrostomy) using running 4/0 vicryl sutures. A Penrose drain was inserted in the left upper quadrant followed by a layered closure. The postoperative course was uneventful. Nasogastric suction and intravenous fluids were continued for 5 days, after which oral feedings were gradually given. The drain was removed after 5 days and the child was discharged from the hospital on the 9th postoperative day. Follow-up abdominal ultrasound was done after 2 weeks and revealed dramatic improvement regarding the size of the cyst. Serum amylase gradually fell to the normal limits. A repeat ultrasound confirmed complete resolution of the cyst after 6 months. The patient is doing well after 1 year of regular follow-up visits.

ay. Follow-up abdominal ultrasound was done after 2 weeks and revealed dramatic improvement regarding the size of the cyst. Serum amylase gradually fell to the normal limits. A repeat ultrasound confirmed complete resolution of the cyst after 6 months. The patient is doing well after 1 year of regular follow-up visits. Discussion PP is a major and common complication of pancreatitis, though few cases are reported in children. Approximately 80% of PPs are located within the head and the body of the pancreas, while the rest lie extrapancreatic in areas, such as mediastinum, liver, spleen, pelvis, and neck. 2 Since the first description of a case of MPP in an adult patient, only few cases had been reported in children. To our best knowledge, only eight cases had been reported in addition to ours ( Table 1 ). It is postulated that during the acute phase of pseudocyst formation, the fluid may track along the path of least resistance to gain access into the mediastinum via aortic or esophageal hiatus of the diaphragm hiatus. Later, the outer wall gets organized to form a pseudocyst. 3 The fluids mostly travel through the esophageal and aortic hiatus into the posterior mediastinum 4 However, If the extension was through the vena caval hiatus or the foramen of Morgagni, MPP will be located in the middle and anterior mediastinum, respectively. 5 Table 1 Cases of MPP in children reported in literature Study Age Etiology Main complaint History of pancreatitis Abdominal mass Route of extension Management

PP is a major and common complication of pancreatitis, though few cases are reported in children. Approximately 80% of PPs are located within the head and the body of the pancreas, while the rest lie extrapancreatic in areas, such as mediastinum, liver, spleen, pelvis, and neck. 2 Since the first description of a case of MPP in an adult patient, only few cases had been reported in children. To our best knowledge, only eight cases had been reported in addition to ours ( Table 1 ). It is postulated that during the acute phase of pseudocyst formation, the fluid may track along the path of least resistance to gain access into the mediastinum via aortic or esophageal hiatus of the diaphragm hiatus. Later, the outer wall gets organized to form a pseudocyst. 3 The fluids mostly travel through the esophageal and aortic hiatus into the posterior mediastinum 4 However, If the extension was through the vena caval hiatus or the foramen of Morgagni, MPP will be located in the middle and anterior mediastinum, respectively. 5 Table 1 Cases of MPP in children reported in literature Study Age Etiology Main complaint History of pancreatitis Abdominal mass Route of extension Management Laird and Clagett 6 15 y Post traumatic Anorexia, nausea, vomiting No No Esophageal hiatus Puestow procedure Galligan and Williams 7 10 y Idiopathic Anorexia, nausea, vomiting No Yes Esophageal hiatus Cystogastrostomy Kirchner et al 8 7 mo Idiopathic Dyspnea No No Foramen of Morgagni Roux-en-Y cysto-jejunostomy Sharma et al 9 8 y Post traumatic Chest pain, dyspnea No No Traumatic diaphragmatic hernia Cysto-gastrostomy

Laird and Clagett 6 15 y Post traumatic Anorexia, nausea, vomiting No No Esophageal hiatus Puestow procedure Galligan and Williams 7 10 y Idiopathic Anorexia, nausea, vomiting No Yes Esophageal hiatus Cystogastrostomy Kirchner et al 8 7 mo Idiopathic Dyspnea No No Foramen of Morgagni Roux-en-Y cysto-jejunostomy Sharma et al 9 8 y Post traumatic Chest pain, dyspnea No No Traumatic diaphragmatic hernia Cysto-gastrostomy Crombleholme et al 10 2 y Ductal anomaly Vomiting No No Esophageal hiatus Roux-en-Y cysto-jejunostomy 7 y Ductal anomaly Abdominal pain Yes No Esophageal hiatus Puestow's procedure Bonnard et al 11 11 y Ductal anomaly – – – Not given Thoracoscopic drainage Nabi et al 12 11 y Idiopathic Abdominal pain Yes No Not given Transgastric endoscopic drainage Current case 7 y Idiopathic Dyspnea No No Esophageal hiatus Cystogastrostomy As MPP decompresses through the diaphragm, it is usually not felt while performing abdominal examination. It can present with nonspecific manifestations caused by compression by the cyst. This includes abdominal pain, gastrointestinal upsets, chest pain, dyspnea, dysphagia, odynophagia, and symptoms of pericardial or pleural effusion like our case. 13

iaphragm, it is usually not felt while performing abdominal examination. It can present with nonspecific manifestations caused by compression by the cyst. This includes abdominal pain, gastrointestinal upsets, chest pain, dyspnea, dysphagia, odynophagia, and symptoms of pericardial or pleural effusion like our case. 13 Owing to the vague nature of symptoms, imaging remains the cornerstone of diagnosis of such a rare disease. While chest radiography is not diagnostic but can illustrate lower mediastinal widening, retro or paracardiac well-defined opacity in addition to associated features, such as pleural or pericardial effusion. 14 Although ultrasound is accurate in diagnosing PP, it cannot detect the existing mediastinal extension. 15 Contrast-enhanced CT is a valuable modality not just in identifying the pseudocyst but also establishing its relationship with surrounding structures. It appears as a thin-walled low attenuation peripherally enhancing cyst. 16 MRCP (magnetic resonance cholangiopancreatography) is as effective as ERCP (endoscopic retrograde cholangiopancreatography) to demonstrate the track between the abdominal cyst and the mediastinal cyst, as well as ductal morphology. 17

ructures. It appears as a thin-walled low attenuation peripherally enhancing cyst. 16 MRCP (magnetic resonance cholangiopancreatography) is as effective as ERCP (endoscopic retrograde cholangiopancreatography) to demonstrate the track between the abdominal cyst and the mediastinal cyst, as well as ductal morphology. 17 The approach to treatment of MPP is individualized depending on the underlying etiology, ductal anomaly, and whether the patient is symptomatic or not. Indications for intervention include increasing pseudocyst size or persistence over 4 to 6 weeks. 18 Surgical treatment has often been used for therapeutic management of patients with mediastinal pseudocyst and these can vary from pancreatic resections to external or internal drainage, targeting the abdominal component to reduce the pressure that causes patency of any communicating tract. 19 In children, three cases underwent cystogastrostomy, roux-en-Y cystojejunostomy was done in two cases and similarly the Puestow's procedure. Each of the endoscopic ultrasound (EUS) assisted endoscopic drainage through transgastric approach and thoracoscopic drainage were done in one case and claimed by the authors to obviate the need for cystenterostomy. 11 12 Conclusion Although extremely rare, the diagnosis of MPP should be considered in the differential diagnosis of any cystic mediastinal mass. The finding of a thin-walled cystic mass in the posterior or middle mediastinum in continuity with pancreas in addition to an elevated serum amylase level can establish the definitive diagnosis.

hough extremely rare, the diagnosis of MPP should be considered in the differential diagnosis of any cystic mediastinal mass. The finding of a thin-walled cystic mass in the posterior or middle mediastinum in continuity with pancreas in addition to an elevated serum amylase level can establish the definitive diagnosis. New Insights and the Importance for the Pediatric Surgeon Pseudopancreatic cyst is uncommon in children and mediastinal extenstion is extremely rare with few cases reported in literature. It represents a diagnostic challenge and should be included in the differential diagnosis of cystic mediastinal swellings. Conflict of Interest None.

Introduction Esophageal lung is an extremely rare malformation characterized by an anomalous origin of one of the main bronchi, which arises from the esophagus. Only few cases have been reported in the literature and no standardized guidelines exist for its treatment. 1 We report a case of right esophageal lung diagnosed in a neonate and discuss our management and possible alternative treatment strategies. Case Report A female newborn was referred to our center from a peripheral hospital on day 2 of her life after onset of respiratory distress, right atelectasis at the chest X-ray, and elevation of inflammatory indexes. She was delivered at 37 5/7 weeks of gestation by an elective cesarean section for maternal indications. The pregnancy was uneventful apart from the suspect of fetal dextrocardia. At admission she presented mild dyspnea and breath sounds were present only in the left hemithorax, while heart sounds could be heard on the right hemithorax. A chest X-ray confirmed the right atelectasis and no cardiac shadow was visible in the left hemithorax. Echocardiography revealed a right cardiac shift rather than dextrocardia and a severely hypoplastic right pulmonary artery. The newborn required noninvasive ventilation with nasal continuous positive airway pressure (nCPAP) with FiO 2 0.21 to maintain an arterial oxygen saturation of 98% with a good respiratory dynamic.

lly air filled possibly after retrograde air inflation from the digestive tract, mainly due to the nCPAP. An associated pulmonary airway malformation could not be excluded by the CT scan. The diagnosis of right esophageal lung was made and confirmed by an esophageal contrast study performed on the same day ( Fig. 1D ). Fig. 1 Upper row: CT scan on day 3 of life. ( A ) Transverse section showing the anomalous origin of the right bronchus; ( B , C ) three-dimensional anatomy reconstruction showing the trachea ending with the left bronchus in the left lung and the right bronchus originating from the esophagus. Lower row: esophageal contrast studies ( D ) Esophageal contrast study on day 3 of life. The oral contrast evidences the anomalous origin of the right bronchus from the esophagus. ( E ) Contrast study on the 3 rd post-operative day after closure of the anomalous bronchus with clips. ( F ) Evidence of partial re-canalization of the anomalous bronchus at 5 months of age. CT, computed tomography. On day 4 of her life, the patient was taken to the operating theater for endoscopic examination of airways and esophagus to better characterize the anatomy and make a treatment plan. The tracheobronchoscopy showed no signs of tracheomalacia, the carina was absent, and the trachea ended directly into the left main bronchus. Esophagoscopy showed a tubular cartilaginous structure arising from the right wall of the distal third of the intrathoracic esophagus.

rize the anatomy and make a treatment plan. The tracheobronchoscopy showed no signs of tracheomalacia, the carina was absent, and the trachea ended directly into the left main bronchus. Esophagoscopy showed a tubular cartilaginous structure arising from the right wall of the distal third of the intrathoracic esophagus. We decided to proceed with a thoracoscopic approach straight after the endoscopic examination. The aim was closing the anomalous bronchus to avoid pneumonia, while leaving the lung in place and delaying the pneumonectomy later in time to reduce the risk of acute mediastinal shift and consequent postpneumonectomy syndrome. 2 The neonate was placed on her left decubitus and three trocars were placed: a 3 mm one for the camera was placed in the 5th intercostal space below the tip of the scapula, two operative ports were placed—a 5 mm one on the 6th intercostal space on the posterior axillary line, and a 3 mm one on the 4th intercostal space on the anterior axillary line. Almost complete pulmonary atelectasis and right lung infarction was evident. A window in the mediastinal pleura was created and the esophageal bronchus arising from the right wall of the distal esophagus was detected. The anomalous bronchus was isolated and closed with two 5 mm titanium endoclips ( Fig. 2 ). Fig. 2 Intraoperative images of the anomalous bronchus arising from the esophagus before and after ligation with titanium endo-clips.

The neonate was placed on her left decubitus and three trocars were placed: a 3 mm one for the camera was placed in the 5th intercostal space below the tip of the scapula, two operative ports were placed—a 5 mm one on the 6th intercostal space on the posterior axillary line, and a 3 mm one on the 4th intercostal space on the anterior axillary line. Almost complete pulmonary atelectasis and right lung infarction was evident. A window in the mediastinal pleura was created and the esophageal bronchus arising from the right wall of the distal esophagus was detected. The anomalous bronchus was isolated and closed with two 5 mm titanium endoclips ( Fig. 2 ). Fig. 2 Intraoperative images of the anomalous bronchus arising from the esophagus before and after ligation with titanium endo-clips. The immediate postoperative course was uneventful. On the 3rd postoperative day, after a negative esophageal contrast study ( Fig. 1E ), feeding was started and well tolerated. We decided to perform an early contrast study to confirm the complete interruption of the aerodigestive communication to start an early oral feeding. The patient was discharged on the 15th postoperative day with an antibiotic prophylaxis and a regular follow-up program. We scheduled the pneumonectomy after 6 months of life.

cided to perform an early contrast study to confirm the complete interruption of the aerodigestive communication to start an early oral feeding. The patient was discharged on the 15th postoperative day with an antibiotic prophylaxis and a regular follow-up program. We scheduled the pneumonectomy after 6 months of life. In the following months she presented twice with fever that was successfully treated with antibiotics. At the third episode, at 5 months of age, she was admitted to our neonatal intensive care unit (ICU). An esophageal contrast study was performed which revealed a partial recanalization of the right main bronchus with a small leakage toward the right lung past the endoclips ( Fig. 1E ). Chest X-ray showed a relevant cardiac shift to the right side despite the native lung was still in the thoracic cavity; however, the patient didn't present symptoms related to mediastinal shift. Therefore, we decided to proceed with the pneumonectomy. A right posterolateral thoracotomy was made. The pulmonary hilum was isolated, and the pulmonary artery and veins were ligated and divided. The ectopic right bronchus was then identified and divided at its origin. The esophageal wall was repaired with an interrupted single–layer, long-term absorbable suture. Considering the absence of symptoms, despite the presence of mediastinal shift, no thoracic expander was placed into the chest. A chest drain was left in situ.

ic right bronchus was then identified and divided at its origin. The esophageal wall was repaired with an interrupted single–layer, long-term absorbable suture. Considering the absence of symptoms, despite the presence of mediastinal shift, no thoracic expander was placed into the chest. A chest drain was left in situ. The postoperative course was characterized by a minor esophageal leakage diagnosed by a contrast study performed on the 6th post-operative day due to salivary leak from the chest drain. It was successfully treated conservatively with fasting and antibiotics. Histological examination of the right lung revealed complex pulmonary airways malformation with squamous metaplasia of segmental bronchial epithelium and histiocytosis of locoregional lymphnodes. A multidisciplinary follow-up was organized comprehensive of regular visits in our thoracic surgery outdoor clinic and in the follow-up service of the neonatal unit. The patient is now 5 years old and has a normal life, needing respiratory physiotherapy cycles for high airways resistance. Discussion

The postoperative course was characterized by a minor esophageal leakage diagnosed by a contrast study performed on the 6th post-operative day due to salivary leak from the chest drain. It was successfully treated conservatively with fasting and antibiotics. Histological examination of the right lung revealed complex pulmonary airways malformation with squamous metaplasia of segmental bronchial epithelium and histiocytosis of locoregional lymphnodes. A multidisciplinary follow-up was organized comprehensive of regular visits in our thoracic surgery outdoor clinic and in the follow-up service of the neonatal unit. The patient is now 5 years old and has a normal life, needing respiratory physiotherapy cycles for high airways resistance. Discussion In case of esophageal lung, interrupting the communication between the esophagus and the bronchus is essential to allow oral intake without developing airway infections. The subsequent surgical options include either the connection of the unventilated lung to the respiratory tree through a bronchotracheal anastomosis, or a pneumonectomy. In most case report, the treatment of choice is pneumonectomy, 1 2 3 4 5 6 7 8 but there are reports of successful tracheobronchial reconstructions. 9 10 11 In our case, we judged that a tracheobronchial anastomosis was unfeasible due to the long tracheobronchial gap, in fact the position of the right main bronchus was very low in the thorax with a gap of four vertebral bodies between its origin and the end of the trachea, as it is well evidenced by the three-dimensional CT reconstruction of the anatomy of our patient ( Fig. 1B–C ). Furthermore, the patient presented severe right pulmonary artery hypoplasia that represented a contraindication to the preservation of the lung. For these reasons we decided to perform the pneumonectomy.

hea, as it is well evidenced by the three-dimensional CT reconstruction of the anatomy of our patient ( Fig. 1B–C ). Furthermore, the patient presented severe right pulmonary artery hypoplasia that represented a contraindication to the preservation of the lung. For these reasons we decided to perform the pneumonectomy. Concerning the timing, we were concerned of a possible postpneumonectomy syndrome which, although rare, is caused by mediastinal shift leading to kinking of the trachea and of the main heart vessels with respiratory and circulatory consequences. The literature concerning this dangerous complication is scant, consisting mainly of case reports. Fatal cases are reported after pneumonectomy in infants 12 13 and severe mediastinal shift is especially related to right pneumonectomy due to the normal mediastinal anatomy with a left-sided aortic arch. 14 15 Even if not definitively demonstrated, the risk of postpneumonectomy syndrome is considered to be higher in the pediatric population 15 due to the softer mediastinal connective tissue and consequently even higher in the neonates. It is on this basis that we decided to postpone the pneumonectomy and preserved the native lung as a natural expander to eliminate the need of prosthetic material. Initial closure of the bronchus, delayed pneumonectomy, and administration of antibiotic prophylaxis allowed us to reach our therapeutic goals: feeding, prevention of pneumonias, and prevention of the complication, following pneumonectomy. Nonetheless, the patient presented with pneumonia after the first operation. The right main bronchus was clipped but not divided. The infective episodes were a consequence of a reestablished communication between the esophagus and the right lung. There are no reports about partial recanalization in esophageal lung, since there are no case reports of a “two-stage” approach to this malformation. However, dislocation of metal clips have been reported in tracheoesophageal fistula (TEF) repair. 16 Shortly after the presented case, we had a case of isolated TEF that we closed with metal clip and that developed a recurrence of the fistula, following clip dislocation. For these reasons, from that moment on we started using self-locking clips such as “Hem-o-lok ® ” or transfixed suture and division of the fistula with scissor and up to now we haven't had other similar complications.

we closed with metal clip and that developed a recurrence of the fistula, following clip dislocation. For these reasons, from that moment on we started using self-locking clips such as “Hem-o-lok ® ” or transfixed suture and division of the fistula with scissor and up to now we haven't had other similar complications. However, in the presented case, the recanalization occurred without any clip dislocation, as assessed during the second operation. Therefore, retrospectively, we think that closing the bronchus with a self-locking clip, dividing it at its origin from the esophagus, and suturing the esophageal wall might have prevented the recurrent infections. Once we evidenced the partial recanalization of the anomalous bronchus, and as the chest X-ray showed an important mediastinal shift despite the presence of the native lung, the decision to proceed with the pneumonectomy without placing a thoracic expander seemed reasonable, and, at present, with a 4-year follow-up, the patient is doing well. One can claim that an early pneumonectomy could have prevented the recurrent infections determining a definitive treatment in only one step, but we cannot state that the native lung failed in preventing early postpneumonectomy syndrome; it may have been effective by avoiding the immediate shift of the mediastinum and allowing a slow adaptation to the new anatomy.

ld have prevented the recurrent infections determining a definitive treatment in only one step, but we cannot state that the native lung failed in preventing early postpneumonectomy syndrome; it may have been effective by avoiding the immediate shift of the mediastinum and allowing a slow adaptation to the new anatomy. Conclusion The management of esophageal lung is challenging. We believe that, if feasible, the treatment of choice should be the tracheobronchial anastomosis with the preservation of the lung to allow the best quality of life to the affected child. However, if this approach is not possible for anatomical reasons, such as a long trachealbronchial gap, vascular anomalies, or pulmonary malformation, in those cases, we believe that a reasonable treatment plan is an early mini-invasive closure and division of the esophageal bronchus, followed by delayed pneumonectomy of the esophageal lung. This allows a slow adaptation of thoracic anatomy to reduce the risk of postpneumonectomy syndrome. New Insights and the Importance for the Pediatric Surgeon The management of esophageal lung is a challenging subject. The proposed minimally-invasive, two-step approach may be of some usefulness in cases of esophageal lung with long tracheobronchial gap and associated pulmonary and vascular anomalies. Conflict of Interest None.

Introduction Head and neck tumors are rare entities in neonates. They mainly consist of vascular malformations, other developmental lesions, and rarely other benign or malignant soft tissue tumors. 1 Cystic hygromas on the neck are the most common vascular malformations. They are classically found in the left posterior triangle of the neck. Large cystic hygromas can cause fetal hydrops and neonatal respiratory difficulties. 2 Ex utero intrapartum treatment (EXIT) procedure can be performed for urgent management of severe airway obstructions caused by the detected giant neck mass. 3 We report on a neonate with cervical mass which turned out to be a hamartoma arising from ectopic thyroid tissue. Case Report A full-term 1-day-old male neonate with a birth weight of 3,420 g was admitted to our department with a left sided cervical mass. The patient had a history of fetal tachycardia and maternal fever during the pregnancy. However, the cervical lesion was not detected on antenatal ultrasounds. On physical examination, the patient had a painless mobile mass on the left side of the neck at the level of the thyroid gland, 3 to 4 cm in diameter ( Fig. 1 ). Fig. 1 Solid mass on the neck extending from the left side to the midline (arrows). Otherwise no abnormality was detected.

Case Report A full-term 1-day-old male neonate with a birth weight of 3,420 g was admitted to our department with a left sided cervical mass. The patient had a history of fetal tachycardia and maternal fever during the pregnancy. However, the cervical lesion was not detected on antenatal ultrasounds. On physical examination, the patient had a painless mobile mass on the left side of the neck at the level of the thyroid gland, 3 to 4 cm in diameter ( Fig. 1 ). Fig. 1 Solid mass on the neck extending from the left side to the midline (arrows). Otherwise no abnormality was detected. On magnetic resonance imaging (MRI) a large mass was seen in the soft tissue of the neck extending from the left side to the midline. The heterogeneous enhancing lobulated tumor slightly dislocated the hypopharynx, larynx, and trachea, causing mild tracheal dislocation and compression. The major vessels of the neck were also mildly dislocated. The morphology of the mass on MRI was not specific for any type of tumor. The thyroid gland was of normal size, shape, and structure. No pathologic lymph nodes or signs of invasion of surrounding tissues were detected ( Fig. 2A–C ). Fig. 2 ( A, B ) Sagittal T2 ( A ) and T1-weighted ( B ), MRI images, showing a lobulated tumor (asterisks) at the level of the thyroid gland, extending from the left side to the midline. ( C ) Axial, T2-weighted MRI demonstrates a tumor (arrows) on the left side of the neck at the level of the thyroid gland, dislocating the hypopharynx, larynx, trachea, and major vessels.

), MRI images, showing a lobulated tumor (asterisks) at the level of the thyroid gland, extending from the left side to the midline. ( C ) Axial, T2-weighted MRI demonstrates a tumor (arrows) on the left side of the neck at the level of the thyroid gland, dislocating the hypopharynx, larynx, trachea, and major vessels. On the 10th day of life, the tumor underwent complete removal via Kocher's incision. The excision of the tumor was straightforward; the tumor was excised within its capsule without any injuries of the cervical structures ( Fig. 3 ). Fig. 3 The completely removed tumor. The perioperative period was uneventful. No recurrence was detected during the 6-month follow-up. Pathological Finding On gross examination, the 1 cm mass was lobulated, firm in consistency, gray–white in color, and well demarcated. Microscopic examination revealed a cell-rich, immature mesenchymal tissue with spindle cells, and nodules of hypercellular hyaline cartilage without evidence of malignancy. Scarce mitotic activity (1/10 high power fields) was noted in the spindle cell component which demonstrated collagen fibers and myxoid areas. Focally thyroid follicles were noted within the mass. The spindle cells were S100, smooth muscle actin (SMA), neurofilament (NF), and paired box gene 8 (PAX8) all negative, but showed vimentin and weak thyroid transcription factor 1 (TTF1) staining. The thyroid follicles showed cytokeratin (CK), PAX8, and TTF1 expression ( Fig. 4A–D ).

ollicles were noted within the mass. The spindle cells were S100, smooth muscle actin (SMA), neurofilament (NF), and paired box gene 8 (PAX8) all negative, but showed vimentin and weak thyroid transcription factor 1 (TTF1) staining. The thyroid follicles showed cytokeratin (CK), PAX8, and TTF1 expression ( Fig. 4A–D ). Fig. 4 Histology of the specimen. ( A, B ) Large lobules of hyaline cartilage are visible separated by spindle cells and scattered thyroid follicles (×5 magnification, hematoxylin-eosin staining). ( C ) Thyroid follicles and spindle cells show strong and weak TTF1 staining, respectively (×20 magnification). ( D ) Only thyroid follicles show PAX8 staining (×20 magnification). The findings were consistent with a chondromesenchymal hamartoma arising in ectopic thyroid tissue. Discussion

Fig. 4 Histology of the specimen. ( A, B ) Large lobules of hyaline cartilage are visible separated by spindle cells and scattered thyroid follicles (×5 magnification, hematoxylin-eosin staining). ( C ) Thyroid follicles and spindle cells show strong and weak TTF1 staining, respectively (×20 magnification). ( D ) Only thyroid follicles show PAX8 staining (×20 magnification). The findings were consistent with a chondromesenchymal hamartoma arising in ectopic thyroid tissue. Discussion The differential diagnosis of neck masses in children is based on clinical, pathological, and imaging characteristics. In childhood, most lesions are either congenital or inflammatory in origin and only a few neoplasms occur in the neck. The most common congenital developmental masses in the neck include thyroglossal duct cysts, branchial cysts, dermoid cysts, and vascular malformations. Inflammatory lesions can be a result of infectious lymphadenitis, reactive lymphadenopathy, or Kawasaki's disease. The differential diagnosis for neoplastic neck masses include benign tumors, such as haemangiomas, teratomas, plexiform neurofibromas, juvenile nasopharyngeal angiofibromas (JNA), and Langerhans cell histiocytosis (LCH), as well as malignant tumors, such as Hodgkin's lymphoma (HL) and non-Hodgkin's lymphomas (NHL), rhabdomyosarcoma (RMS), thyroid malignances, nasopharyngeal carcinomas (NPC), salivary gland tumors, neuroblastoma, and metastasis. 4 5 6

juvenile nasopharyngeal angiofibromas (JNA), and Langerhans cell histiocytosis (LCH), as well as malignant tumors, such as Hodgkin's lymphoma (HL) and non-Hodgkin's lymphomas (NHL), rhabdomyosarcoma (RMS), thyroid malignances, nasopharyngeal carcinomas (NPC), salivary gland tumors, neuroblastoma, and metastasis. 4 5 6 Teratoma is a subtype of germ cell tumors (GCT) derived from more than one of the three germinal layers. They are more common in the testes and ovaries, but can present in many different regions in the midline, including the sacral region, retroperitoneum, mediastinum, and brain. Mature teratomas are generally bening, immature teratomas in young children also tend to behave as benign tumors. In patients, older than 15 years, immature teratomas can manifest as highly devastating malignancies. 7 Hamartomas are benign congenital nonneoplastic abnormalities, but can be locally aggressive leading to complications. Ectopic thyroid tissue is another congenital developmental defect that may present as a tumor. The prevalence is 1 in 100,000 to 300,000; 90% of cases are localized to the base of the tongue. However, rare cases may appear in the cervical region resulting from abnormal migration and fusion of the lateral thyroid anlage. 8

opic thyroid tissue is another congenital developmental defect that may present as a tumor. The prevalence is 1 in 100,000 to 300,000; 90% of cases are localized to the base of the tongue. However, rare cases may appear in the cervical region resulting from abnormal migration and fusion of the lateral thyroid anlage. 8 Chondromesenchymal hamartomas are rare lesions, most occur nasally and are referred to as nasal chondromesenchymal hamartoma (NCMH). 9 Infants and children are predominantly involved but it occurs in adults as well. It presents mainly with sleep-disordered breathing due to nasal obstruction, feeding difficulties in infants, recurrent sinusitis, serous otitis media, epistaxis and watery rhinorrhea, decreased sense of smell, or frontal headache with no nasal symptoms. Orbital involvement and intracranial expansion may result in additional severe symptoms. In 2015, a systematic review was identified among 48 cases in the literature in adults and children but only in the nasal or cranial region. All patients underwent operative resection of the NCMH. Although NCMH is a benign lesion, malignant transformation has been reported in the literature. 4 9 10 11 12 13 14 15

toms. In 2015, a systematic review was identified among 48 cases in the literature in adults and children but only in the nasal or cranial region. All patients underwent operative resection of the NCMH. Although NCMH is a benign lesion, malignant transformation has been reported in the literature. 4 9 10 11 12 13 14 15 The authors report the first case of chondromesenchymal hamartoma arising in ectopic thyroid tissue in neonate. Chondromesenchymal hamartomas usually arise from the nasal mucosa, a thyroid chondromesenchymal hamartoma is an exceedingly rare lesion, reported only in a few cases in the literature. 16 No cases of ectopic thyroid tissue developing chondromesenchymal hamartoma have been reported, so far. Considering the wide extension of the hamartoma, MRI was performed for preoperative planning and complete surgical excision. The specimen revealed no evidence of malignant transformation. New Insights and the Importance for the Pediatric Surgeon The authors report the first case of chondromesenchymal hamartoma arising in ectopic thyroid tissue in neonate. Acknowledgment The first author (A.SZ.) of the case report would like to thank Prof. Andrew B. Pinter for his support in writing and reviewing the article. Conflict of Interest None.

Introduction Anorectal Anomalies (ARA) represent a major category in pediatric surgery with different phenotypes in the male and female. 1 Successive classifications have been proposed trying to include the wide diversity of the spectrum; and lastly, the Krickenbeck conference identified ARA as seven major clinical groups and other rare variants. 2 Here, we present a special case of ARA in which the clinical picture was further complicated by the presence of complete tubular colonic duplication. Case Presentation A 2-year-old girl presented by abnormal passage of stools through her vestibule. She had a history of operation (anorectoplasty) during the neonatal period. The patient operative files revealed a limited sagittal anorectoplasty performed (without colostomy) for a rectoperineal fistula, with uneventful postoperative recovery.

ar-old girl presented by abnormal passage of stools through her vestibule. She had a history of operation (anorectoplasty) during the neonatal period. The patient operative files revealed a limited sagittal anorectoplasty performed (without colostomy) for a rectoperineal fistula, with uneventful postoperative recovery. The history would suggest an iatrogenic rectovaginal fistula, and, therefore, the patient was scheduled for examination under anesthesia with the possibility of performing a diverting colostomy. Examination under anesthesia revealed a well-positioned neoanus with good caliber; however, the vestibule showed a shallow common urogenital sinus with a septated vagina. The common sinus was short and wide that the anatomy was well-exposed for inspection upon labial retraction. Stools were seen emerging out of the vagina upon applying pressure on the lower abdomen. A decision was taken for fecal diversion as a first step for a staged repair. An oblique left lower abdominal incision was made to perform a pelvic (sigmoid) colostomy which revealed the presence of double colon sharing a common mesentery. A pelvic colostomy was done for the double colon that consisted of two proximal stomas and two distal mucous fistulae.

on as a first step for a staged repair. An oblique left lower abdominal incision was made to perform a pelvic (sigmoid) colostomy which revealed the presence of double colon sharing a common mesentery. A pelvic colostomy was done for the double colon that consisted of two proximal stomas and two distal mucous fistulae. Diagnostic workup before definitive repair: we performed contrast X-ray studies ( Fig. 1A ) and pelvic magnetic resonance imaging (MRI; Fig. 1B and C ). Injection of contrast through the proximal stomas (each at a time) revealed complete colonic duplication up to a double cecum (with failure of contrast to pass into neither the ileum nor the appendix). Injection of contrast through the distal mucous fistulae revealed one posterior colon and rectum (R1) terminating by the neoanus, and another anterior colon and rectum (R2) terminating via a rectovaginal fistula ( Fig. 1A ). MRI studies revealed no associated spinal anomalies; and helped to complete the picture by defining the three-dimensional (3D) orientation of the double colon and rectum in relation to other pelvic soft tissue structures ( Fig. 1B and C ).

terior colon and rectum (R2) terminating via a rectovaginal fistula ( Fig. 1A ). MRI studies revealed no associated spinal anomalies; and helped to complete the picture by defining the three-dimensional (3D) orientation of the double colon and rectum in relation to other pelvic soft tissue structures ( Fig. 1B and C ). Fig. 1 A 2-year-old girl with complete colonic duplication on top of anorectal anomaly. The upper row demonstrates investigations performed before reconstruction: ( A ) contrast studies through the two distal “colostomy” stomas demonstrating one posterior colon and rectum (R1) terminating by the neo-anus, and another anterior colon and rectum (R2) terminating into the vagina (Vg); ( B ) and ( C ) midsagittal and axial MRI (T2-WI), respectively, demonstrating the orientation of both recti (R1 and R2) in relation to each other and to other pelvic organs (UB, urinary bladder). Note the injection of gel through both recti to facilitate their identification in MRI by the hyperintense signal of the gel in T2-WI. The lower row demonstrates pelvic MRI anatomy following reconstruction (after excision of anterior rectum R2, and closure of colostomy); ( D ) midsagittal plane; ( E ) axial plane. MRI, magnetic resonance imaging; WI, weighted image. The sacral ratio was calculated in the antero–posterior X-ray film of the sacrum; its value was 0.53, which indicated a fair prognosis regarding the continence potential. 3

Fig. 1 A 2-year-old girl with complete colonic duplication on top of anorectal anomaly. The upper row demonstrates investigations performed before reconstruction: ( A ) contrast studies through the two distal “colostomy” stomas demonstrating one posterior colon and rectum (R1) terminating by the neo-anus, and another anterior colon and rectum (R2) terminating into the vagina (Vg); ( B ) and ( C ) midsagittal and axial MRI (T2-WI), respectively, demonstrating the orientation of both recti (R1 and R2) in relation to each other and to other pelvic organs (UB, urinary bladder). Note the injection of gel through both recti to facilitate their identification in MRI by the hyperintense signal of the gel in T2-WI. The lower row demonstrates pelvic MRI anatomy following reconstruction (after excision of anterior rectum R2, and closure of colostomy); ( D ) midsagittal plane; ( E ) axial plane. MRI, magnetic resonance imaging; WI, weighted image. The sacral ratio was calculated in the antero–posterior X-ray film of the sacrum; its value was 0.53, which indicated a fair prognosis regarding the continence potential. 3 Operative management: our surgical plan was to remove the distal colon and rectum (R2) communicating with the vagina, while keeping the posterior distal colon and rectum (R1) as a single exit for fecal stream after reanastomoses with proximal double colon ( Fig. 2D ).

The sacral ratio was calculated in the antero–posterior X-ray film of the sacrum; its value was 0.53, which indicated a fair prognosis regarding the continence potential. 3 Operative management: our surgical plan was to remove the distal colon and rectum (R2) communicating with the vagina, while keeping the posterior distal colon and rectum (R1) as a single exit for fecal stream after reanastomoses with proximal double colon ( Fig. 2D ). Fig. 2 Intraoperative photos and diagram representing extra-mucosal excision of the antimesenteric distal colon (C2) and rectum. ( A ) The distal colon to be excised (C2) was incised along its free antimesenteric border; ( B ) submucosal injection of adrenaline/saline solution (1/200,000) to control bleeding and elevate the mucosa (hydrodissection); ( C ) stripping of the mucosa off this distal colon and rectum; ( D, E ) the continuity of the fecal stream was then restored by anastomosing the proximal double colonic lumens with the retained distal colon (C1) in a Y-shape fashion. Note the limited terminal septotmy for the common wall between the proximal double colonic lumens in ( D ).

mucosa off this distal colon and rectum; ( D, E ) the continuity of the fecal stream was then restored by anastomosing the proximal double colonic lumens with the retained distal colon (C1) in a Y-shape fashion. Note the limited terminal septotmy for the common wall between the proximal double colonic lumens in ( D ). As expected, separation of one colon from the other was unfeasible due to their common mesentery, so we went for just mucosal excision of the “antimesenteric” distal colon and rectum (R2). The distal colon to be excised was incised along its free antimesenteric border ( Fig. 2A ), followed by submucosal injection of adrenaline/saline solution (1/200,000) to control bleeding and elevate the mucosa (hydrodissection; Fig. 2B ). Stripping the mucosa off this distal colon and rectum was then applied down into the pelvis; the stripped mucosa was transfixed and excised at its lower end communicating into the vagina ( Fig. 2C ). This was followed by seromuscular closure. The continuity of the fecal stream was then restored by anastomosing the proximal double colonic lumens with the retained distal colon and rectum in a Y -shape fashion ( Fig. 2D and E ). Before that, a 7 cm linear cutting stapler was applied to the distal end of the common wall between the proximal double colonic lumens (limited septotomy), converting it into a single wide lumen that could be anastomosed to the single distal colon ( Fig. 2D ). Postoperative recovery was uneventful, and the patient had normal defecation through a single neoanus. Few months later, the patient underwent partial urogenital sinus mobilization with division of the vaginal septum as a final step in the staged reconstruction. Partial urogenital sinus mobilization (PUM) is a procedure used to correct short common urogenital sinus anomalies which comprises dissection of the common urogenital sinus up to but without cutting through the pubourethral ligament. 4

on with division of the vaginal septum as a final step in the staged reconstruction. Partial urogenital sinus mobilization (PUM) is a procedure used to correct short common urogenital sinus anomalies which comprises dissection of the common urogenital sinus up to but without cutting through the pubourethral ligament. 4 The patient has been followed-up for the last 3 years, with a satisfactory functional and cosmetic outcome. She has voluntary fecal and urinary control; occasional constipation showed good response to stimulant laxatives. A follow-up MRI has been prescribed to check for postreconstruction pelvic anatomy ( Fig. 1D and E ). Discussion

The patient has been followed-up for the last 3 years, with a satisfactory functional and cosmetic outcome. She has voluntary fecal and urinary control; occasional constipation showed good response to stimulant laxatives. A follow-up MRI has been prescribed to check for postreconstruction pelvic anatomy ( Fig. 1D and E ). Discussion Complete colonic duplication is rare and usually occurs as a part of the caudal duplication syndrome. 5 In such cases, the diagnosis is clinically evident by the presence of two ani arranged side by side in the perineum which is commonly associated with duplication of the external genitalia as well (double phallus or double vestibule). 6 In this report, the diagnosis was initially missed due to the uncommon sagittal arrangement of duplicated rectum: one rectum was ending externally into the perineum by apparent rectoperineal fistula, while the other was hidden by its internal termination into the vagina. The patient was initially managed as a straight forward case of rectoperineal fistula by primary limited sagittal anorectoplasty. Later, the other hidden colon showed itself by passage of stools through the vagina, when we suspected an iatrogenic rectovaginal fistula complicating the primary operation. A decision for fecal diversion was taken, and, surprisingly, we detected the presence of double colon at time of colostomy creation. Conventional contrast studies confirmed the diagnosis; MRI provided anatomical soft tissue details and could have been useful for making the correct diagnosis prior to abdominal exploration.

ecision for fecal diversion was taken, and, surprisingly, we detected the presence of double colon at time of colostomy creation. Conventional contrast studies confirmed the diagnosis; MRI provided anatomical soft tissue details and could have been useful for making the correct diagnosis prior to abdominal exploration. Another point that was missed during the initial management of this case was the presence of associated urogenital anomaly in the form of a short common urogenital sinus and a septated vagina. This highlights the importance of proper exposure (labial retraction) during inspection that would help to identify all aspects of the anomaly. A short common channel in cloaca (< 1 cm) has been reported as a benign type with excellent prognosis. 7 This can be managed either by simple introitoplasty without dissection around the urethra 7 or by partial urogenital sinus mobilization (PUM). 4 On the other hand, division of vaginal septum has been recommended to be performed early during anorectal reconstruction to avoid possible psychological concerns of such procedure when performed during adolescence. 8

oitoplasty without dissection around the urethra 7 or by partial urogenital sinus mobilization (PUM). 4 On the other hand, division of vaginal septum has been recommended to be performed early during anorectal reconstruction to avoid possible psychological concerns of such procedure when performed during adolescence. 8 Our final diagnosis for this case was a variant of anorectal anomaly in the female, which was associated with complete colonic duplication. One colon (which was in the free mesenteric border) terminated anteriorly into the vagina as a part of a ‘short common channel’ cloaca (with rectovaginal communication); 9 while the other colon terminated via a rectoperineal fistula. Although the anomaly seems to be rather complex and confusing, yet our case was associated with an excellent outcome due to the benign type of anorectal anomalies (rectoperineal fistula and “short common channel” cloaca) 7 and absence of significant sacral dysplasia; 3 in addition to adequate identification of the abnormal anatomy by appropriate investigations and the staged approach for surgical reconstruction. New Insights and the Importance for the Pediatric Surgeon The present article describes how to manage a rare case of anorectal anomaly associated with complete colonic duplication. Conflict of Interest None.

Introduction Since the 1980s, minimally invasive surgery has provided technological advances in different areas of surgery. The current use of new techniques has allowed the recent introduction of indocyanine green (ICG), which has facilitated the approach and the prevention of intraoperative complications in adults. It provides greater clarity and depth image visualization and reduces surgical time. 1 Regarding cholecystectomy, it helps to better identify the bile duct anatomy, and in case of urological or oncological surgery, it allows to define the vascular anatomy, reducing the number of iatrogenic lesions 2 The experience with the use of ICG fluorescence in adults has shown multiple applications in recent years (colorectal, vascular, hepatobiliary, or tumor surgery) 3 ; however, the experience and bibliography described in pediatric cases are specific. We present our experience in different laparoscopic procedures performed in children using ICG fluorescence imaging. Case Reports A high-definition camera (10 mm) (Stryker) equipped with a visible infrared light source (800 nm) was used. The laparoscopy camera used includes 3 CMOS chip technology, 1920 × 1080 p resolution, DVI, and S-VHS outputs and interval of 1/60 (1/50)-1/50000 seconds. The ENV (endoscopic near-infrared visualization) mode is used as a light source. Case 1

Case Reports A high-definition camera (10 mm) (Stryker) equipped with a visible infrared light source (800 nm) was used. The laparoscopy camera used includes 3 CMOS chip technology, 1920 × 1080 p resolution, DVI, and S-VHS outputs and interval of 1/60 (1/50)-1/50000 seconds. The ENV (endoscopic near-infrared visualization) mode is used as a light source. Case 1 A 14-year-old girl presented with aortocoronary fistula, which caused a decreased coronary flow during diastole. Her clinical condition worsened during exercise. Right three-port (3 mm) thoracoscopy was performed in upright positions. The fistulous tract was readily identified and dissected on arrival at the right atrium. The presence of this rare vascular anomaly was confirmed by fluorescence by immediate injection of ICG (dose of 0.2 mg/kg), allowing better visualization and secure ligature ( Fig. 1 ). Fig. 1 Aortocoronary fistula ligation. The image shows vascular permeability of the fistula, clearly demonstrated with the uptake of indocyanine green through it. Case 2 A 13-year-old boy was scheduled for varicocelectomy. He had a clinical history of asymmetry and testicular pain. Umbilical, and right and left flank trocars (5 mm) were introduced for lens and instruments, respectively. After intravenous (IV) injection of ICG, the arterial vessels were initially visualized following by the venous vessels. Thereafter, ligation of the spermatic cord was performed in block, ensuring selection of all vessels and avoiding the section of lymphatics that are not filled in this phase ( Fig. 2 ).

Case 2 A 13-year-old boy was scheduled for varicocelectomy. He had a clinical history of asymmetry and testicular pain. Umbilical, and right and left flank trocars (5 mm) were introduced for lens and instruments, respectively. After intravenous (IV) injection of ICG, the arterial vessels were initially visualized following by the venous vessels. Thereafter, ligation of the spermatic cord was performed in block, ensuring selection of all vessels and avoiding the section of lymphatics that are not filled in this phase ( Fig. 2 ). Fig. 2 Ligation of spermatic vessels in varicocele. After the injection of the contrast, the vessels are filled (arterial and venous) and its correct ligature is verified, thanks to the infrared light of the fluorescence that indicates the vascular tree. Case 3 A 13-year-old girl was admitted because of cholelithiasis and recurrent abdominal pain. She required two previous hospital admissions. Laparoscopic cholecystectomy was scheduled few days after admission. Fifteen minutes after ICG IV injection, the biliary tree was perfectly drawn, allowing clear identification of cystic artery, common bile duct, and hepatic duct. Safe dissection of the bile duct and artery was performed, completing cholecystectomy with total control of all surgical maneuvers ( Fig. 3 ). Fig. 3 Cholecystectomy. Thanks to the fluorescence, contrast uptake can be observed initially in the cystic artery (A) and later in the cystic duct (C). In the image, we are in a late phase of fluorescence since both structures can be visualized. Cases 4 and 5

A 13-year-old girl was admitted because of cholelithiasis and recurrent abdominal pain. She required two previous hospital admissions. Laparoscopic cholecystectomy was scheduled few days after admission. Fifteen minutes after ICG IV injection, the biliary tree was perfectly drawn, allowing clear identification of cystic artery, common bile duct, and hepatic duct. Safe dissection of the bile duct and artery was performed, completing cholecystectomy with total control of all surgical maneuvers ( Fig. 3 ). Fig. 3 Cholecystectomy. Thanks to the fluorescence, contrast uptake can be observed initially in the cystic artery (A) and later in the cystic duct (C). In the image, we are in a late phase of fluorescence since both structures can be visualized. Cases 4 and 5 Two children aged 3 and 6 years, respectively, had steroid-resistant hypertension and renal failure. Nephrectomy was indicated in each of them, which was performed by retroperitoneal laparoscopy. In both cases, intraoperative injection of indocyanine dye allowed renal vascular anatomy to be identified with certainty, showing the peripheral vascularization of the ureter. This technique definitively facilitated safe dissection of the renal hilum ( Fig. 4 ). Fig. 4 Nephrectomy. The image shows the renal artery (A) and periureteral vessels (U). Without fluorescence, the differentiation between the ureter and the vessels is difficult. Thanks to the fluorescence, we can identify them more easily since the ureter does not present contrast uptake.

Two children aged 3 and 6 years, respectively, had steroid-resistant hypertension and renal failure. Nephrectomy was indicated in each of them, which was performed by retroperitoneal laparoscopy. In both cases, intraoperative injection of indocyanine dye allowed renal vascular anatomy to be identified with certainty, showing the peripheral vascularization of the ureter. This technique definitively facilitated safe dissection of the renal hilum ( Fig. 4 ). Fig. 4 Nephrectomy. The image shows the renal artery (A) and periureteral vessels (U). Without fluorescence, the differentiation between the ureter and the vessels is difficult. Thanks to the fluorescence, we can identify them more easily since the ureter does not present contrast uptake. In all cases, we initially administrated ICG dye through a peripheral venous access at a standard dose of 0.2 mg/kg. No adverse effects were present during or after IV ICG injections. All patients were observed for 30 minutes to 1 hour in the recovery room, except the patient with aortocoronary fistula who was in the pediatric intensive care unit overnight. Discussion ICG is an anionic molecule that is soluble in water, with a molecular mass of 776 daltons. After IV injection, ICG binds rapidly to plasma proteins, especially to lipoproteins (albumin). Under near-infrared light, the released fluorescence can be detected using a specifically designed camera. 1 Not every laparoscopic equipment includes or is compatible for usage of an infrared light source, nor all equipment have the same technology for doing that.

Discussion ICG is an anionic molecule that is soluble in water, with a molecular mass of 776 daltons. After IV injection, ICG binds rapidly to plasma proteins, especially to lipoproteins (albumin). Under near-infrared light, the released fluorescence can be detected using a specifically designed camera. 1 Not every laparoscopic equipment includes or is compatible for usage of an infrared light source, nor all equipment have the same technology for doing that. We advise the use of devices that allow a vision with gray-scale functionality compared with those that only have black-and-white vision. ICG has an exclusively biliary excretion; therefore, its most logical application in the visualization of biliary tree anatomy during laparoscopic cholecystectomy, 2 as shown in our case. After injection of ICG, the cystic artery could be initially observed, and 15 minutes later, the common hepatic, common bile duct, and cystic duct were identified. In addition, this allowed better visualization and anatomical dissection, avoiding injury to the biliary tree. This technique also avoids performing intraoperative cholangiography when bile duct injury is suspected during the procedure. 4 ICG has many other applications already described in the literature; it allows identification of sentinel node in breast tumors, melanoma, and prostate cancer among others. It also facilitates lymphadenectomy in tumors with lymphatic spread by local injection. 5

ICG has an exclusively biliary excretion; therefore, its most logical application in the visualization of biliary tree anatomy during laparoscopic cholecystectomy, 2 as shown in our case. After injection of ICG, the cystic artery could be initially observed, and 15 minutes later, the common hepatic, common bile duct, and cystic duct were identified. In addition, this allowed better visualization and anatomical dissection, avoiding injury to the biliary tree. This technique also avoids performing intraoperative cholangiography when bile duct injury is suspected during the procedure. 4 ICG has many other applications already described in the literature; it allows identification of sentinel node in breast tumors, melanoma, and prostate cancer among others. It also facilitates lymphadenectomy in tumors with lymphatic spread by local injection. 5 In colorectal surgery, it facilitates intestinal resections and is used to verify the adequate vascularization of the intestinal anastomoses, demonstrating a lower rate of postoperative complications. 6 7 Other applications have been described in surgery, such as liver resections, nephrectomies, and splenectomies. 8 9 In summary, ICG images are recommended for interventions in which visualization of the vascular anatomy is necessary to differentiate between anatomical and vascular variants, 3 10 as describe in our series of patients.

ions have been described in surgery, such as liver resections, nephrectomies, and splenectomies. 8 9 In summary, ICG images are recommended for interventions in which visualization of the vascular anatomy is necessary to differentiate between anatomical and vascular variants, 3 10 as describe in our series of patients. The ICG imaging system seems to be simple and safe. Its application in adult surgery is wide and contrasted. The ability to visualize the vascular structures or the bile duct anatomy allows us to approach laparoscopic techniques of different complexities with greater safety for the patient. We have verified its use in children. Larger and more specific studies are needed to confirm its applicability, expand its indications, and address its advantages and disadvantages. New Insights and the Importance for the Pediatric Surgeon The ability to visualize the vascular structures or the bile duct allows us to approach laparoscopic techniques of different complexities, with greater safety for the patient. We have verified its use in children. Conflict of Interest None.

Introduction The Nuss procedure and the modified Ravitch technique are the most commonly performed operations for pediatric anterior chest wall deformities. 1 The modified Ravitch procedure includes lower sternum exposure, removal of abnormal cartilages, and fixation of the sternum with a stainless steel bar which is left in place for at least a year and is removed afterwards. The metal strut displacement after the modified Ravitch procedure is extremely rare and can have intrathoracic or intraabdominal propagation. 2 3 4 5 6 7 There are less than 20 reports in the literature of patients whose postoperative course had been complicated by migration of the metal strut into the pericardium, right atrium, left and right ventricle, the abdomen, and left upper bronchus. 8 Our patient had been operated with two stainless steel 30 cm struts with end perforations; the technique being chosen due to the severity and asymmetry of the pectus excavatum (PE).

ated by migration of the metal strut into the pericardium, right atrium, left and right ventricle, the abdomen, and left upper bronchus. 8 Our patient had been operated with two stainless steel 30 cm struts with end perforations; the technique being chosen due to the severity and asymmetry of the pectus excavatum (PE). Case Report A 16-year-old boy with a severe deformity underwent a modified Ravitch procedure in an institution. Two metal struts were inserted to stabilize the deformity and were sewn to the ribs with heavy No. 2 multifilament nonabsorbable braided sutures. The position of the bars was such that the tension had been larger on the inferior strut due to the asymmetry of the PE. The position of the metal bars had been verified postoperatively by anteroposterior and lateral chest X-ray only. The displacement of one strut was noted after 4 months. The inferior strut migrated approximately 1cm rightward and threatened to perforate the skin. The mechanism of migration was straightening of the strut due to the severity of the intrathoracic deformity, its strong pulling force and consequent displacement. The dislocated bar was surgically removed and the other strut left in place. The postoperative course was uneventful and the position of the remaining bar checked only by radiography. At the time of initial surgery, the laboratory results were consistent with the findings of a healthy young adolescent.

nt displacement. The dislocated bar was surgically removed and the other strut left in place. The postoperative course was uneventful and the position of the remaining bar checked only by radiography. At the time of initial surgery, the laboratory results were consistent with the findings of a healthy young adolescent. The patient complained of limb pain 10 days post strut removal. With pain intensification, he was examined by a vascular surgeon about 2 months after the second intervention. Bilateral distal arterial and venous leg thromboses were diagnosed by the color Doppler. The transthoracic heart ultrasound showed the remaining metal strut perforating the anterior right ventricular (RV) wall, the intraventricular septum, ending its course in the left ventricular (LV) cavity with massive intracardiac biventricular thrombosis with no pericardial effusion. He was immediately transferred to a pediatric cardiac surgery center. On admission, the boy was neurologically intact, with no clinical signs of venous obstruction. The femoral arterial pulses were vaguely present, the popliteal were absent. An urgent multi detector computer tomography (MDCT) was performed. The brain scan was normal. The superior vena cava (SVC) pathway was clear, but the inferior vena cava (IVC) system was partially occluded with two long plaques below the cavoatrial junction ( Fig. 1 ). Fig. 1 ( A ) Preoperative severe pectus excavatum; ( B ) displacement of the inferior metal strut; ( C ) the postoperative position of the remaining strut.

On admission, the boy was neurologically intact, with no clinical signs of venous obstruction. The femoral arterial pulses were vaguely present, the popliteal were absent. An urgent multi detector computer tomography (MDCT) was performed. The brain scan was normal. The superior vena cava (SVC) pathway was clear, but the inferior vena cava (IVC) system was partially occluded with two long plaques below the cavoatrial junction ( Fig. 1 ). Fig. 1 ( A ) Preoperative severe pectus excavatum; ( B ) displacement of the inferior metal strut; ( C ) the postoperative position of the remaining strut. The portal venous system was unobstructed, there was a partial occlusion of the left femoral vein. The arterial circulation was severely damaged, partial occlusion of both femoral arteries with no circulation in the popliteal and posterior tibial arteries. An indication for urgent cardiac operation and strut removal was quoted to the parents with high intraoperative risks ( Fig. 2 ). Fig. 2 ( A ) Lower limb angiography showing partial occlusion of both femoral arteries and absent circulation in the popliteal arteries; ( B ) the lateral X-ray of the remaining strut. The operation was performed on cardio pulmonary bypass (CPB) in deep hypothermia and neck cannulation. A transoesophageal echocardiogram (TOE) was performed during the surgery. The sternum was severely adherent to the pericardium with the lower left sided ribs, completely detached from the sternum. After the cardiac arrest, the strut was removed without resistance ( Figs. 3 and 4 ).

eep hypothermia and neck cannulation. A transoesophageal echocardiogram (TOE) was performed during the surgery. The sternum was severely adherent to the pericardium with the lower left sided ribs, completely detached from the sternum. After the cardiac arrest, the strut was removed without resistance ( Figs. 3 and 4 ). Fig. 3 ( A ) Intracardial metal strut ending its course in the left ventricle “muffed” by thrombus; ( B ) iatrogenic ventricular septal defect after strut and thrombus removal. Fig. 4 Intraoperative moment of metal strut removal. The cardiac apex was macerated, the enterance hole on the parietal wall of the RV was “muffed” with thrombi and fibrin. The RV clots were removed through the undamaged tricuspid valve. The large iatrogenic ventricular septal defect (VSD) involved the cardiac apex with the surrounding muscular septum. Left-sided massive thrombi and fibrin detritus were evacuated transeptally and via the VSD. The mitral valve (MV) was intact. The VSD was closed with a 0.6 mm polytetrafluoroethylene (PTFE) patch and the cardiac apex reconstructed with direct pledgeted sutures ( Fig. 5 ). Fig. 5 VSD closed with a PTFE patch with repair of the RV wall. PTFE, polytetrafluoroethylene; RV, right ventricular; VSD, ventricular septal defect.

The cardiac apex was macerated, the enterance hole on the parietal wall of the RV was “muffed” with thrombi and fibrin. The RV clots were removed through the undamaged tricuspid valve. The large iatrogenic ventricular septal defect (VSD) involved the cardiac apex with the surrounding muscular septum. Left-sided massive thrombi and fibrin detritus were evacuated transeptally and via the VSD. The mitral valve (MV) was intact. The VSD was closed with a 0.6 mm polytetrafluoroethylene (PTFE) patch and the cardiac apex reconstructed with direct pledgeted sutures ( Fig. 5 ). Fig. 5 VSD closed with a PTFE patch with repair of the RV wall. PTFE, polytetrafluoroethylene; RV, right ventricular; VSD, ventricular septal defect. The postoperative TOE showed poor myocardial function with severe dyskinesia of the ventricular septum but no residual masses. He was resuscitated for 30 minutes postoperatively for refractive ventricular fibrillations in spite of aggressive antiarrhythmic therapy and pacemaker overdrive. The patient was in junctional ectopic tachycardia (JET), required high-inotropic support, but converted into sinus rhythm within 12 hours. Control MDCT showed ischemic brain lesions with no bleeding. The laboratory tests did not imply of an underlying coagulopathy. The improved cardiac status allowed extubation within a week but due to further neurological deterioriation with frequent generalized convulsions, he was discharged severely impaired and transferred to his local hospital. Migration of metal struts is a rare complication and, except in our case, had been dealt with successfully.

ved cardiac status allowed extubation within a week but due to further neurological deterioriation with frequent generalized convulsions, he was discharged severely impaired and transferred to his local hospital. Migration of metal struts is a rare complication and, except in our case, had been dealt with successfully. Discussion The mechanism of migration has not been clearly identified. It should be differentiated from strut dislocation when displacement occurs without organ injury. In our case, the strut migration into the heart followed the removal of a dislocated metal strut less than 6 months after the PE repair which is one of the earliest found in the literature (6 months to 37 years). 6 8 There are no valid comparisons of the modified Ravitch technique strut migrations versus the Nuss bar migrations. The exact mechanism of this painless heart perforation is not known. The postoperative position of the remaining strut, in our case, was checked by anterioroposterior and lateral X-rays and was found to be sufficient and adequate. Our presumption is that, in our case, the left-sided sternal joints of the abnormal and fused ribs detached shortly after the first strut removal. The ribs had stabbed the myocardium but did not protrude into the ventricle. The loss of anchor of the single metal strut and the severity and asymmetry of the PE combined with the increased pressure, allowed the respiratory chest movements to slowly and gradually impress the strut into the heart. Therefore, we conclude that the second strut migration is the consequence of the first strut removal.

loss of anchor of the single metal strut and the severity and asymmetry of the PE combined with the increased pressure, allowed the respiratory chest movements to slowly and gradually impress the strut into the heart. Therefore, we conclude that the second strut migration is the consequence of the first strut removal. We would advocate postoperative X-ray screenings at 1, 3, 6, and 12 months postsurgery. A cardiac/Doppler ultrasound examination should be performed before the hospital discharge in cases of cardiovascular symptoms or eventful postoperative courses. It is questionable that should both bars be removed if one would get dislocated and the other one had seemed to remain in place. We feel that the decision should be individually made depending on the patient's condition, experience of the surgeon and the possibilities of the medical center. Considering that the metal struts can generate pressure on the myocardium, antiaggregational therapy can also be debated as a possible postoperative standard in cases where there is evidence of clinical symptoms rather than only aesthetic deformity. The prevention of metal strut migrations can be prevented with longer devices which can include fixage of more than one rib.

We would advocate postoperative X-ray screenings at 1, 3, 6, and 12 months postsurgery. A cardiac/Doppler ultrasound examination should be performed before the hospital discharge in cases of cardiovascular symptoms or eventful postoperative courses. It is questionable that should both bars be removed if one would get dislocated and the other one had seemed to remain in place. We feel that the decision should be individually made depending on the patient's condition, experience of the surgeon and the possibilities of the medical center. Considering that the metal struts can generate pressure on the myocardium, antiaggregational therapy can also be debated as a possible postoperative standard in cases where there is evidence of clinical symptoms rather than only aesthetic deformity. The prevention of metal strut migrations can be prevented with longer devices which can include fixage of more than one rib. Conclusion The modified Ravitch procedure is predominantly an esthetic operation performed in children with marked PE deformity and is considered safe and an alternative to the Nuss procedure. About 20 cases in the literature have been described where the metal stabilizators have migrated and caused different organ injuries. Our case with a fatal outcome raises awareness for the follow-up of these patients and immediate action, especially in the situation of strut dislocations or migrations.

Nuss procedure. About 20 cases in the literature have been described where the metal stabilizators have migrated and caused different organ injuries. Our case with a fatal outcome raises awareness for the follow-up of these patients and immediate action, especially in the situation of strut dislocations or migrations. New Insights and the Importance for the Pediatric Surgeon This is a rare but fatal complication of an aesthetic operation in the pediatric age group. Very few reports have been made on this subject. We feel that we need to point the possibility of these complications. Conflict of Interest None.

Introduction Hirschsprung's disease (HSCR) is a disorder of migration of neural crest cells during embryonic development characterized by the absence of ganglia in the distal colon, resulting in a functional obstruction. The principles of management involve excision of the aganglionic segment and anastomosis of the ganglionated bowel above the dentate line. The level of aganglionosis is determined by performing multiple seromuscular biopsies on the antimesenteric border of the bowel. This practice is substantiated by Coventry's theory that vagal neural crest cells colonize the gut by intra- and extramural migration. 1 The intramural progression is from cranial to caudal, whereas the extramural migration occurs along the mesentery and circumferentially innervates the bowel from the mesenteric side. 1 Some authors also recommend the presence of ganglion cells and the absence of hypertrophies nerves on a full-thickness biopsy to be adequate to determine the level of pullthrough. 2 Herein, we describe a child in whom this practice would have failed because of the selective absence of ganglion cells in 30% of the circumference of the bowel along the mesenteric border for most of the transverse colon. To the best of our knowledge, this is the first report of such an occurrence and has significant diagnostic and therapeutic implications. Case Report

Hirschsprung's disease (HSCR) is a disorder of migration of neural crest cells during embryonic development characterized by the absence of ganglia in the distal colon, resulting in a functional obstruction. The principles of management involve excision of the aganglionic segment and anastomosis of the ganglionated bowel above the dentate line. The level of aganglionosis is determined by performing multiple seromuscular biopsies on the antimesenteric border of the bowel. This practice is substantiated by Coventry's theory that vagal neural crest cells colonize the gut by intra- and extramural migration. 1 The intramural progression is from cranial to caudal, whereas the extramural migration occurs along the mesentery and circumferentially innervates the bowel from the mesenteric side. 1 Some authors also recommend the presence of ganglion cells and the absence of hypertrophies nerves on a full-thickness biopsy to be adequate to determine the level of pullthrough. 2 Herein, we describe a child in whom this practice would have failed because of the selective absence of ganglion cells in 30% of the circumference of the bowel along the mesenteric border for most of the transverse colon. To the best of our knowledge, this is the first report of such an occurrence and has significant diagnostic and therapeutic implications. Case Report A boy baby born at 37 weeks of gestation was transferred for specialist care for abdominal distension and delayed passage of meconium at 36 hours of life with a significant maternal family history of HSCR. Plain abdominal X-ray showed free air ( Fig. 1 ). He underwent emergency laparotomy with repair of a perforation at the splenic flexure, ileostomy, multiple seromuscular biopsies of the bowel, a rectal washout, and full-thickness rectal biopsy. The biopsies showed the absence of ganglion cells distal to the perforation at the splenic flexure and ganglion cells present at and proximal to the perforation. He underwent a pullthrough at 5 months of age. Frozen section of the proximal doughnut showed ganglion cells and no hypertrophied nerves, but some parts of the circumference were difficult to assess. It was decided to bring the splenic flexure down to complete the pullthrough. The final biopsy completed 4 days later, however, found absent ganglion cells in 30% of the circumference of the doughnut on the mesenteric side ( Fig. 2 ). Findings were discussed with the family, and they wished for normal bowel to be pulled through. A redo pullthrough was performed 9 days after the original operation. Intraoperative biopsies at the revision pullthrough showed the absence of ganglion cells in 30% of the circumference along the mesenteric border in the entire transverse colon ( Fig. 3 ). The hepatic flexure was tumbled down. The postoperative recovery was uneventful, and the child remains well at follow-up except for frequent stooling. The genetic work-up is pending.

h showed the absence of ganglion cells in 30% of the circumference along the mesenteric border in the entire transverse colon ( Fig. 3 ). The hepatic flexure was tumbled down. The postoperative recovery was uneventful, and the child remains well at follow-up except for frequent stooling. The genetic work-up is pending. Fig. 1 Supine plain abdominal film with evidence of free air in the peritoneal cavity. Fig. 2 Histopathology of the doughnut showing ( a ) the presence of ganglion cells in 70% of the circumference on the antimesenteric side ( long arrows indicate the myenteric plexus, short arrows indicate the submucosal plexus) and ( b ) the absence of ganglion cells in 30% of the circumference on the mesenteric side ( arrowheads indicate the aganglionic myenteric plexus). Fig. 3 Pictorial representation of the pattern of aganglionosis. Discussion

Fig. 2 Histopathology of the doughnut showing ( a ) the presence of ganglion cells in 70% of the circumference on the antimesenteric side ( long arrows indicate the myenteric plexus, short arrows indicate the submucosal plexus) and ( b ) the absence of ganglion cells in 30% of the circumference on the mesenteric side ( arrowheads indicate the aganglionic myenteric plexus). Fig. 3 Pictorial representation of the pattern of aganglionosis. Discussion HSCR is thought to be the result of arrested enteric neural crest cell (ENCC) migration, which occurs rostrocaudally. The level of arrest determines the length of the aganglionic segment. Rare variations in the pattern of aganglionosis are well described and are categorized into “skip” lesions, in which there is a segment of ganglionated bowel surrounded proximally and distally by aganglionosis, occurring most commonly with total colonic aganglionosis. 3 Another variant described is the “zonal” aganglionosis, in which a segment of aganglionic bowel is surrounded by normal ganglionated bowel. The concept of “double zonal aganglionosis” is applied to HSCR with a skip lesion. 3 The presence of skip lesions is explained by the mesenteric migration of ENCCs that colonize a segment of bowel within the aganglionic segment ahead of the wavefront of distally migrating ENCCs. However, the pattern of aganglionosis in our patient is unique and defies the current understanding of the embryogenesis of HSCR.

presence of skip lesions is explained by the mesenteric migration of ENCCs that colonize a segment of bowel within the aganglionic segment ahead of the wavefront of distally migrating ENCCs. However, the pattern of aganglionosis in our patient is unique and defies the current understanding of the embryogenesis of HSCR. ENCC precursors originate in the vagal region (somites 1–7) and to a lesser extent from the thoracic and sacral regions of the neural tube. 3 They enter the gut mesenchyme by the third week of gestation and migrate in a craniocaudal pattern, proliferate, differentiate, and colonize the gut completely by the eighth week of gestation, forming the network of the enteric nervous system. This theory, however, does not explain the defect in our patient. We hypothesize the following various mechanisms that could cause the pattern of aganglionosis in our patient. Failure of transmesenteric migration: in a mouse model, Druckenbrod and Epstein studied the transmesenteric migration of ENCCs from the midgut to the hindgut during the time these organs are transiently juxtaposed. 4 This migratory process requires glial cell derived neurotrophic factor (GDNF) signaling. In the absence of this transmesenteric migration, the intramural migration may continue along the antimesenteric border and cause a defect such as in our patient. The failure of transmesenteric migration could be because of a lack of juxtaposition, inadequate numbers of ENCCs due to a preexisting genetic defect, or defects in GDNF signaling.

transmesenteric migration, the intramural migration may continue along the antimesenteric border and cause a defect such as in our patient. The failure of transmesenteric migration could be because of a lack of juxtaposition, inadequate numbers of ENCCs due to a preexisting genetic defect, or defects in GDNF signaling. Delayed migration: ENCC colonization has been shown to be a timed process. 5 Furthermore, ENCC migration is enhanced when the cells are in contact with each other along the wavefront. Isolated ENCCs do not migrate as quickly or as directionally as chains of cells. Mutations in L1 cell adhesion molecule (L1CAM), a protein that maintains such cell–cell contacts, reduce ENCC contact and can retard ENCC migration. 5 The microenvironment may no longer be permissive to colonization by the time they arrive. Genetic factors provide the framework for patterning and morphogenesis. However, it is now known that maternal and placental factors such as hypoxia, inflammation, drug intake, and nutrition can affect the survival of ENCC. 6 This concept shed new light on the existing theories of aganglionosis. The area of aganglionosis in our patient involved the watershed area of the intestine. Did a hostile environment due to transient hypoxia, inflammation, or nutrient deficiency destroy the colonized ENCCs and cause the defect? The pattern of aganglionosis in the transition zone is unusual, and a proper doughnut biopsy will avoid a transition zone pullthrough. 2

Genetic factors provide the framework for patterning and morphogenesis. However, it is now known that maternal and placental factors such as hypoxia, inflammation, drug intake, and nutrition can affect the survival of ENCC. 6 This concept shed new light on the existing theories of aganglionosis. The area of aganglionosis in our patient involved the watershed area of the intestine. Did a hostile environment due to transient hypoxia, inflammation, or nutrient deficiency destroy the colonized ENCCs and cause the defect? The pattern of aganglionosis in the transition zone is unusual, and a proper doughnut biopsy will avoid a transition zone pullthrough. 2 New Insights and the Importance for the Pediatric Surgeon A careful doughnut biopsy of the pullthrough segment is essential to avoid missing unusual patterns of aganglionosis Conflict of interest None.

Introduction Solid pseudopapillary tumors (SPTs) of the pancreas account for 8 to 17% of all pediatric pancreatic neoplasms. 1 2 The tumor was first described by the U.S. American pathologist Virginia Kneeland Frantz in 1959 as a lesion with low-grade malignancy. 3 Malignant behavior is rarely seen and associated with distinct histological findings, such as vascular invasion, peripancreatic infiltration, and lymph node metastases. 4 5 Therefore, complete surgical resection remains the mainstay of successful treatment. The ideal surgical approach mainly depends on the localization of the tumor. Lesions in the pancreatic body or the tail are usually treated by distal pancreatectomy. The technical feasibility of laparoscopic spleen-preserving distal pancreatectomy for Frantz tumors was first described in 1996. 6 However, few reports in the pediatric population are available about its long-term oncological safety. 7 8 Herein, we present the case of a female adolescent patient with a SPT which was treated laparoscopically with spleen-preserving distal pancreatectomy. Three years of follow-up showed neither recurrences nor signs of metastasis. Case Report A 17-year-old girl was presented to a regional hospital with abdominal pain for several months without emesis or weight loss. Abdominal ultrasonography (US) was performed and revealed a tumorous lesion in the upper abdomen giving the indication for referral to our department.

Herein, we present the case of a female adolescent patient with a SPT which was treated laparoscopically with spleen-preserving distal pancreatectomy. Three years of follow-up showed neither recurrences nor signs of metastasis. Case Report A 17-year-old girl was presented to a regional hospital with abdominal pain for several months without emesis or weight loss. Abdominal ultrasonography (US) was performed and revealed a tumorous lesion in the upper abdomen giving the indication for referral to our department. Upon clinical examination of the patient (body height = 163 cm, body weight = 51 kg), there was slight tenderness in the left upper quadrant without a palpable mass. US was repeated and showed a complex, spherical mass of 4.4 cm × 3.6 cm × 4 cm in diameter with partly complex cystic and solid components. The mass was located between the abdominal wall, the medial margin of the spleen, and the anterior margin of the kidney. Magnetic resonance imaging (MRI) showed a well encapsulated mass at the tail of the pancreas which presented with partially cystic and partially solid areas with contrast enhancement at the periphery of the mass but not in the central cystic part. The lesion was in close relation to the splenic vein superiorly, the medial surface of the spleen laterally, the transverse colon and mesocolon anteriorly, and in front of the left kidney and suprarenal gland. Neither vascular invasions nor lymphadenopathy were detected ( Fig. 1 ). Routine laboratory investigations and tumor markers (alpha fetoprotein [AFP], cancer antigen 125 [CA125], CA 19–9, carcinoembryonic antigen [CEA], and adrenocorticotropic hormone [ACTH]) were within normal ranges.

left kidney and suprarenal gland. Neither vascular invasions nor lymphadenopathy were detected ( Fig. 1 ). Routine laboratory investigations and tumor markers (alpha fetoprotein [AFP], cancer antigen 125 [CA125], CA 19–9, carcinoembryonic antigen [CEA], and adrenocorticotropic hormone [ACTH]) were within normal ranges. Fig. 1 Axial contrast-enhanced MRI of the abdomen showing the tumorous lesion (white arrow) and its relation to adjacent organs. MRI, magnetic resonance imaging. Due to the radiological findings and the negative tumor markers, a solid pseudopapillary tumor of the pancreas was suspected. The decision was taken to proceed with laparoscopy. With the patient in supine position, one 10 mm umbilical trocar and two additional 5 mm trocars in the left- and right-upper abdomen were placed. Laparoscopic distal pancreatectomy with preservation of the spleen was done using a 30-degree camera ( Fig. 2 ). Dissection was assisted with LigaSure and separation of the mass from the pancreas using Endo GIA Auto Suture Universal Stapler (Covidien, Minneapolis, MN, United States). A retrieval bag was used for removal of the mass. Operation time was 165 minutes. Fig. 2 ( A ) Dissection of the tumor; ( B ) distal pancreatectomy using a stapler; ( C ) the spherical tumor after complete separation; (D) operative bed with pancreatic stump.

Due to the radiological findings and the negative tumor markers, a solid pseudopapillary tumor of the pancreas was suspected. The decision was taken to proceed with laparoscopy. With the patient in supine position, one 10 mm umbilical trocar and two additional 5 mm trocars in the left- and right-upper abdomen were placed. Laparoscopic distal pancreatectomy with preservation of the spleen was done using a 30-degree camera ( Fig. 2 ). Dissection was assisted with LigaSure and separation of the mass from the pancreas using Endo GIA Auto Suture Universal Stapler (Covidien, Minneapolis, MN, United States). A retrieval bag was used for removal of the mass. Operation time was 165 minutes. Fig. 2 ( A ) Dissection of the tumor; ( B ) distal pancreatectomy using a stapler; ( C ) the spherical tumor after complete separation; (D) operative bed with pancreatic stump. Histopathological examination revealed a completely resected pseudopapillary tumor of the pancreas with 5.5 cm in diameter. The proliferation fraction (Ki67 staining) within the tumor tissue was below 5%. Immunohistochemical examination was negative for keratin, chromogranin, CEA, and S-100 and positive for (neuron specific enolase) NSE, β-catenin, and α-1 antitrypsin. Additionally, single cells were positive for vimentin und synaptophysin. The postoperative course was uneventful except for a temporary peripancreatic fluid collection managed conservatively. Repeated ultrasound examinations were performed until complete resolution without pseudopancreatic cyst formation was seen 7 months postoperatively.

Histopathological examination revealed a completely resected pseudopapillary tumor of the pancreas with 5.5 cm in diameter. The proliferation fraction (Ki67 staining) within the tumor tissue was below 5%. Immunohistochemical examination was negative for keratin, chromogranin, CEA, and S-100 and positive for (neuron specific enolase) NSE, β-catenin, and α-1 antitrypsin. Additionally, single cells were positive for vimentin und synaptophysin. The postoperative course was uneventful except for a temporary peripancreatic fluid collection managed conservatively. Repeated ultrasound examinations were performed until complete resolution without pseudopancreatic cyst formation was seen 7 months postoperatively. Sonographic and clinical follow-up examinations, every 3 months during the first year and every 6 months thereafter, were unremarkable. In addition, MRI was performed twice a year in the first 2 postoperative years and at the end of the third year without evidence of a residual tumor or a recurrence. Moreover, body weight, as well as endocrine and exocrine function of the pancreas, was normal 3 years following removal of the tumor. Discussion SPTs, also termed as Frantz tumors, are rare low-grade malignant pancreatic tumors that typically occur in females aged between 20 and 40 years. 8 The present case describes an adolescent female with a SPT located in the pancreatic tail which was managed by laparoscopic spleen preserving distal pancreatectomy. Neither complications nor recurrence were encountered during 3 years of follow-up.

c tumors that typically occur in females aged between 20 and 40 years. 8 The present case describes an adolescent female with a SPT located in the pancreatic tail which was managed by laparoscopic spleen preserving distal pancreatectomy. Neither complications nor recurrence were encountered during 3 years of follow-up. Due to the rarity of these lesions, reports describing SPT of the pancreas only including pediatric patients are confined to case reports or case series. 8 9 Crocoli and coworkers have recently reported one of the largest series including detailed information of 43 pediatric patients with SPT. 10 The authors have found the typical female preponderance and the median age at diagnosis was 13.2 years.

ncluding pediatric patients are confined to case reports or case series. 8 9 Crocoli and coworkers have recently reported one of the largest series including detailed information of 43 pediatric patients with SPT. 10 The authors have found the typical female preponderance and the median age at diagnosis was 13.2 years. The clinical presentation is variable and ranges between asymptomatic incidental discovery, abdominal discomfort and pain, palpable mass, nausea, vomiting, and weight loss. 8 However, there seems to be a difference in the clinical manifestation between children and adults. In an article published in 2008, clinical features of 15 pediatric cases were compared with 47 adult patients. 11 In adults, the diagnosis was usually made incidentally during screening by detection of a mass. However, all of the included children were symptomatic. 11 In contrast, 21% of the children described in another study, by Crocoli et al, had an incidental diagnosis and the remaining patients were diagnosed on abdominal ultrasound performed due to abdominal pain or had a palpable mass. 10 A rare case with hemoperitoneum due to traumatic tumor rupture has also been described in the literature. 12 Taken together, prompt abdominal ultrasound in pediatric patients with unspecific abdominal pain seems to be a prerequisite for rapid diagnosis.

rasound performed due to abdominal pain or had a palpable mass. 10 A rare case with hemoperitoneum due to traumatic tumor rupture has also been described in the literature. 12 Taken together, prompt abdominal ultrasound in pediatric patients with unspecific abdominal pain seems to be a prerequisite for rapid diagnosis. The tumor can arise from any part of the pancreas (head, body, or tail). Moreover, rare cases of extrapancreatic manifestations, such as the adrenal gland, have been reported. 13 Regarding the most prevalent localization of the tumor in the pancreas, there are contradictory findings in the literature. While some reports have found that the body/tail was most often affected, 10 others describe the pancreatic head as the most common localization 11 or an even distribution between head and tail. 8 In our patient, the mass arose from the tail of the pancreas in close relation to the spleen, left kidney, and adrenal gland. US examinations of SPTs usually show a homogeneous, hypoechogenic mass with a hyerpechogenic rim. 14 Computed tomography features include an encapsulated mass with varying solid and cystic components secondary to hemorrhagic degeneration. 14 At the periphery of the mass, both solid areas and calcifications can be seen. 15 Typical MRI findings are a large, well-defined, encapsulated lesion with heterogeneous high-or low-signal intensity on T1-weighted and heterogeneous high signal intensity on T2-weighted images. 16

secondary to hemorrhagic degeneration. 14 At the periphery of the mass, both solid areas and calcifications can be seen. 15 Typical MRI findings are a large, well-defined, encapsulated lesion with heterogeneous high-or low-signal intensity on T1-weighted and heterogeneous high signal intensity on T2-weighted images. 16 The use of preoperative biopsies is discussed controversially in the literature. While it may be useful in verifying the diagnosis 17 it could result in tumor dissemination in case of malignancy. 18 Additionally, it has been shown that biopsies have limited diagnostic accuracy of 56%. 19 This is mirrored by a comprehensive review of 718 patients where only 2.7% underwent biopsies. 2 To achieve excellent long-term outcome, surgery is the mainstay of SPT treatment. Since local recurrences and metastases are reported following incomplete resection, 4 5 20 complete resection of the tumor with preservation of as much pancreatic tissue as possible is warranted. The ideal surgical approach depends on the localization of the lesion and consists of pancreaticoduodenectomy with or without pyloric preservation for lesions located in the pancreatic head and of distal pancreatectomy with or without splenectomy for lesions in the pancreatic body or tail. 8 Moreover, cases of duodenum preserving pancreatic head resections and resection of the tumors have been described. 1 21

aticoduodenectomy with or without pyloric preservation for lesions located in the pancreatic head and of distal pancreatectomy with or without splenectomy for lesions in the pancreatic body or tail. 8 Moreover, cases of duodenum preserving pancreatic head resections and resection of the tumors have been described. 1 21 The use of minimally invasive surgery (MIS) for oncological procedures is debated in the literature due to the risk of tumor rupture and subsequent spillage. For SPT, Coelho and coworkers have reported 20 patients with SPT of whom 7 underwent laparoscopic resection with one intraoperative tumor rupture. 22 Crocoli et al described a series of 43 children with one case of tumor rupture (spillage). 10 However, it is unclear whether this patient was treated by open surgery or MIS. Nevertheless, laparoscopic procedures, like distal pancreatectomy, have been considered safe and feasible for SPT. 7 23 Recently, also a case of robotic pylorus preserving pancreatoduodenectomy for a solid pseudopapillary tumor of the pancreas in a 10-year-old child has been published. 24 In our case, the tumor had the characteristic imaging of SPT without signs of lymphadenopathy or invasion of adjacent vessels or organs. Therefore, the decision for a minimally invasive approach was made. Meticulous follow-up examinations consisting of clinical examination, ultrasound, and MRI did not show any signs of recurrence in our patient, 3 years postoperatively.

g of SPT without signs of lymphadenopathy or invasion of adjacent vessels or organs. Therefore, the decision for a minimally invasive approach was made. Meticulous follow-up examinations consisting of clinical examination, ultrasound, and MRI did not show any signs of recurrence in our patient, 3 years postoperatively. Conclusion In conclusion, laparoscopy in an adolescent patient with an SPT allowed complete resection following basic oncological principles, preservation of splenic and pancreatic function, and avoidance a major laparotomy. While data about the technical feasibility of a laparoscopic approach to pancreatic SPT are already available, this pediatric case report adds a long-term oncological and functional success to the available literature. New Insights and the Importance for the Pediatric Surgeon Laparoscopy in an adolescent patient with a solid pseudopapillary tumor located in the pancreatic tail allowed complete resection following basic oncological principles, preservation of splenic and pancreatic function, and avoidance a major laparotomy. Conflict of Interest None declared.

Introduction Sternal cleft is a congenital failure of sternal fusion during the embryological development. It is often associated with other malformations, such as cardiac anomaly. 1 Several classifications have been proposed based either on the anatomical level 2 or on the extension of the cleft process. 3 4 To our knowledge, Down's syndrome is usually not associated to this type of sternal malformation. Indication of surgical closure are mainly for the protection of mediastinum and underlying organs, improvement of breathing, and to avoid recurrent respiratory infections. 5 Because of higher compliance of the thoracic cage associated to a minimal compression of underlying structures in young children, early surgery is usually preferred. 6 However, when sternal cleft closure has been delayed, the best approach for surgical treatment remains unclear. A lot of clinical cases using various surgical technics are reported, but it is difficult to draw conclusion about the gold standard technique. 1 5 Bioceramics were originally used as vertebra cages and for complex orthopaedic procedures. Recently, a ceramic sternal prosthesis was designed to replace sternum when removed for tumor or infectious reasons. 7 We describe this new surgical option to fix this sternal defect.

Sternal cleft is a congenital failure of sternal fusion during the embryological development. It is often associated with other malformations, such as cardiac anomaly. 1 Several classifications have been proposed based either on the anatomical level 2 or on the extension of the cleft process. 3 4 To our knowledge, Down's syndrome is usually not associated to this type of sternal malformation. Indication of surgical closure are mainly for the protection of mediastinum and underlying organs, improvement of breathing, and to avoid recurrent respiratory infections. 5 Because of higher compliance of the thoracic cage associated to a minimal compression of underlying structures in young children, early surgery is usually preferred. 6 However, when sternal cleft closure has been delayed, the best approach for surgical treatment remains unclear. A lot of clinical cases using various surgical technics are reported, but it is difficult to draw conclusion about the gold standard technique. 1 5 Bioceramics were originally used as vertebra cages and for complex orthopaedic procedures. Recently, a ceramic sternal prosthesis was designed to replace sternum when removed for tumor or infectious reasons. 7 We describe this new surgical option to fix this sternal defect. Case Report A 9-year-old girl (height = 120 cm, weight = 16 kg) with Down's syndrome was referred to our institution with a butterfly-shaped congenital sternal cleft. The patient had been operated for a congenital heart defect (cor triatriatum) in the 1st week of life which left this unhealed congenital sternal cleft. As often in neonatal cardiac surgery, chest closure was delayed a few days and then, sternal rims were just sealed and the cleft was left open because of the cardiac surgery.

ient had been operated for a congenital heart defect (cor triatriatum) in the 1st week of life which left this unhealed congenital sternal cleft. As often in neonatal cardiac surgery, chest closure was delayed a few days and then, sternal rims were just sealed and the cleft was left open because of the cardiac surgery. Cardiac echocardiogram was normal at readmission. A midline defect with visible chest pulsations caused serious esthetical concerns and hard school integration ( Video 1 ). Because of intellectual impairment related to Down's syndrome, respiratory function testing was not available. Video 1 Preoperative visit. As shown in Fig. 1A , the previous cardiac surgery scar went on a skin raphe, as sometimes described in this rare congenital defect (failure of midline development). Clinical findings were confirmed by chest tomodensitometry ( Fig. 1B ). To preserve chest wall stability, optimize growth evolution, and avoid some pitfall of other techniques, we choose a ceramic prosthesis (Sternum Ceramil, I.Ceram, Limoges, France) for surgical repair. As at this time, the European Certification was not yet obtained, the surgery was performed thanks to a waiver from the French Agency for Health Security (ANSM) and the patient's parents who gave their consent for this surgery. Since this surgery this device has been CE marked. Fig. 1 ( A ) Preoperative view; ( B ) preoperative CT-scan 3D reconstruction. 3D, three-dimensional; CT, computed tomography.

As shown in Fig. 1A , the previous cardiac surgery scar went on a skin raphe, as sometimes described in this rare congenital defect (failure of midline development). Clinical findings were confirmed by chest tomodensitometry ( Fig. 1B ). To preserve chest wall stability, optimize growth evolution, and avoid some pitfall of other techniques, we choose a ceramic prosthesis (Sternum Ceramil, I.Ceram, Limoges, France) for surgical repair. As at this time, the European Certification was not yet obtained, the surgery was performed thanks to a waiver from the French Agency for Health Security (ANSM) and the patient's parents who gave their consent for this surgery. Since this surgery this device has been CE marked. Fig. 1 ( A ) Preoperative view; ( B ) preoperative CT-scan 3D reconstruction. 3D, three-dimensional; CT, computed tomography. At surgery, prior cutaneous scar was excised as much as possible. Pectoralis major muscle was elevated on either side from their sternal origin.

As shown in Fig. 1A , the previous cardiac surgery scar went on a skin raphe, as sometimes described in this rare congenital defect (failure of midline development). Clinical findings were confirmed by chest tomodensitometry ( Fig. 1B ). To preserve chest wall stability, optimize growth evolution, and avoid some pitfall of other techniques, we choose a ceramic prosthesis (Sternum Ceramil, I.Ceram, Limoges, France) for surgical repair. As at this time, the European Certification was not yet obtained, the surgery was performed thanks to a waiver from the French Agency for Health Security (ANSM) and the patient's parents who gave their consent for this surgery. Since this surgery this device has been CE marked. Fig. 1 ( A ) Preoperative view; ( B ) preoperative CT-scan 3D reconstruction. 3D, three-dimensional; CT, computed tomography. At surgery, prior cutaneous scar was excised as much as possible. Pectoralis major muscle was elevated on either side from their sternal origin. Costal cartilages were gently and softly excavated to leave space for prosthetic sternum implantation. This stage was simplified using the trial implant which allowed removing only the necessary amount of cartilages. The trial implant has the same shape and size as the ceramic but is in stainless steel. It is used to choose the size and to prepare the area of implantation. The preperforated prosthetic ceramic sternum (size 1) was anchored to cartilages with eight nonabsorbable 3/0 polyester sutures ( Fig. 2A ). Closed suction drains were placed above and below the sternum. The pectoralis major muscles were approximated medially and the skin was closed with subcutaneous and cutaneous continuous sutures.

ed prosthetic ceramic sternum (size 1) was anchored to cartilages with eight nonabsorbable 3/0 polyester sutures ( Fig. 2A ). Closed suction drains were placed above and below the sternum. The pectoralis major muscles were approximated medially and the skin was closed with subcutaneous and cutaneous continuous sutures. Fig. 2 ( A ) Peroperative view of I.Ceram porous alumina sternal prosthesis insertion with sutures; ( B ) postoperative view (month 12). Chest drains were removed on day 3 and the patient was discharged on day 7. Early and long-term postoperative pain was limited. At the last follow-up visit (month 12), the patient was doing well and the scar was totally healed, clean, and dry ( Fig. 2B , Video 2 ), as it was since the first outpatient visit at day 10. Video 2 Post operative visit (month 6). Discussion Cleft sternum is a rare congenital abnormality, less than 1 among 100,000 births, in which there is no sternal bone fusion. Surgery is not mandatory 8 but it reduces the risk of respiratory infections, paradoxical respiratory movement, damages of mediastinum, and organs. Three main surgical strategies have been described for congenital cleft closure: (1) direct closure, (2) closure using autologous tissue, or (3) artificial material. 1 5 8 9 However, no gold standard has emerges to date as the number of cases is very small.

Surgery is not mandatory 8 but it reduces the risk of respiratory infections, paradoxical respiratory movement, damages of mediastinum, and organs. Three main surgical strategies have been described for congenital cleft closure: (1) direct closure, (2) closure using autologous tissue, or (3) artificial material. 1 5 8 9 However, no gold standard has emerges to date as the number of cases is very small. When surgery is performed early, device-free surgical approach is recommended with approximation of sternal wedges. After 3 months, elasticity of thoracic cage decreases and direct closure might be hazardous. It is difficult to say whether an early surgery is better than a delayed one as no trial compared these two options. However, in our case, as an early surgery was not possible due to the cardiac surgery, the second option was mandatory. In older patients, the use of various autografts (ribs, costal cartilage, etc.) or prosthetic materials (titanium plates, silicone, etc.) has been described. 1 4 5 9 These techniques may lead to residual pain and exposes patients to infectious complications. Seromas have also been frequently seen. Harvesting bone graft can be challenging and using mesh can result in a chest instability and paradoxical motion. 5

materials (titanium plates, silicone, etc.) has been described. 1 4 5 9 These techniques may lead to residual pain and exposes patients to infectious complications. Seromas have also been frequently seen. Harvesting bone graft can be challenging and using mesh can result in a chest instability and paradoxical motion. 5 This prosthesis is a porous ceramic made of pure alumina (Al 2 O 3 ). It is fully biocompatible and nonabsorbable. This alumina sternum has been implanted in adults 11 times before this surgery. These surgeries were performed in other hospitals in France for adult patients with a mean age of 58 years (range, 38–79 years). 7 Indications were sternal cancer involvement ( n = 8) or refractory deep sternal wound infection ( n = 3). At the time of this surgery, the mean follow-up of these patients was 18 months with the older one dating from 3 years. No complications were observed. Bioceramic provides a new approach for tissue engineering. Porous characteristics (with pores mainly ranging from 100 to 900 µm) allow attachment of osteoblasts and chondroblasts to the ceramic. Its biocompatibility has been well demonstrated with more than 5,000 implantations of cervical cages and tibial wedges. 10 11 The risk of infection is lower than with metal. 12 13 Long-term follow-up showed no case of local/systemic effect and alumina ceramics are classified as inert. 14

d chondroblasts to the ceramic. Its biocompatibility has been well demonstrated with more than 5,000 implantations of cervical cages and tibial wedges. 10 11 The risk of infection is lower than with metal. 12 13 Long-term follow-up showed no case of local/systemic effect and alumina ceramics are classified as inert. 14 The resistance of the ceramic while compressed is superior to 20 MPa (thrice the one of cancellous bone). Its ovoid shape is particularly adapted and five different sizes are routinely available associated to three half shape to replace only the manubium part. For those attractive reasons and the adult experience, 7 we decided to try this new material in this rare condition. The rigidity of the ceramic with the smooth anchorage using suture threads avoids chest instability while giving elasticity. We hypothesized that this porous alumina ceramic could provide a good long-term option, with a preserved growth potential. Indeed, preserving the rib cartilages will allow a growth of the thoracic cage. Finally, we deplore the final aspect of the scar, which resulted from the first neonatal surgical procedure, and the delayed chest closure. Conclusion Thanks to an easy surgical technique and a long follow-up (> 12 months) the use of the I.Ceram porous alumina sternal prosthesis is a good option in this rare congenital anomaly. New Insights and the Importance for the Pediatric Surgeon We present an innovative technique for sternal cleft closure, using a porous alumina prosthesis. This is the first use in this indication. Outcomes are excellent. Conflict of Interest None.

Introduction Harlequin syndrome (HS) is a very rare and seemingly benign condition characterized by unilateral loss of facial sympathetic functions. It was first described by Lance, an Australian professor of neurology, in 1988 in adults who showed unilateral flushing and sweating. He hypothesized that these syndromes occurred due to an idiopathic ipsilateral affection of the sympathetic outflow of the third root. 1 Since then, several reports and reviews have been published on this disorder. The cause of HS can be idiopathic and iatrogenic in both adults and children. 1 2 3 4 Most of the pediatric cases are related to a certain cause, for example, surgical procedure close to the autonomous nervous system in the upper chest, whereas the pathogenesis of congenital cases of the syndrome is still unclear. Leading to disturbance of autonomous functions like sweating and thermoregulation of the skin HS can be regarded as autonomous dysautonomia. It is different from other partial dysautonomias, although etiological and clinical overlap seems possible. 5 We report the third case of postoperative HS following thoracoscopic repair of a tracheoesophageal fistula (TEF). 6 7 8 Case Report

Since then, several reports and reviews have been published on this disorder. The cause of HS can be idiopathic and iatrogenic in both adults and children. 1 2 3 4 Most of the pediatric cases are related to a certain cause, for example, surgical procedure close to the autonomous nervous system in the upper chest, whereas the pathogenesis of congenital cases of the syndrome is still unclear. Leading to disturbance of autonomous functions like sweating and thermoregulation of the skin HS can be regarded as autonomous dysautonomia. It is different from other partial dysautonomias, although etiological and clinical overlap seems possible. 5 We report the third case of postoperative HS following thoracoscopic repair of a tracheoesophageal fistula (TEF). 6 7 8 Case Report A Caucasian baby girl of healthy parents was delivered spontaneously in the 41th week of gestation with a birth weight of 3,480 g, height of 50 cm, and head circumference of 37 cm. Postnatal she was diagnosed to have esophageal atresia (EA) type C (TEF). On the second day of life (DOL) and after bronchoscopy, the thoracoscopy was performed in left-sided half prone position. A type C (gross) EA with distal fistula (TEF) was confirmed. The azygos vein was electrically transected and the vagal nerve was preserved. After ligation of the fistula using a polyglactin 4.0 suture, a well-perfused, tension-less primary anastomosis was created with polyglactin 5.0 interrupted stiches and splinted with an 8 Charrière nasogastric feeding tube. A 12 Charrière chest-drain was placed. Operating time was 105 minutes. The girl returned to neonatal intensive care unit (NICU) and the postoperative course was uneventful. On the 2nd postoperative day (POD), nutrition was started via the feeding tube. On the 4th POD, the girl was extubated, and continuous positive airway pressure ventilation was performed until the 7th DOL. Oral feeds were started at the 7th DOL. At the 10th DOL, she was transferred to the pediatric surgical ward. The nasogastric feeding tube was removed at the 22nd DOL. The VACTERL workup revealed no associated malformations. A screening for metabolic diseases was unremarkable. The patient was discharged on the 25th DOL. The further development at home was uneventful. At the age of 1 year, the first endoscopic follow-up was performed and showed a wide esophageal lumen without stenosis. The girl had a normal height (P91) and weight (P81). The second endoscopic follow-up after 2 years was unremarkable too with a pH study showing slightly higher acid exposition without remarkable reflux episodes. At the age of 2 years, the girl presented with no complications, normal ingestion, normal weight, and normal height. However, her parents reported that the right side of her face remained pale and was not sweating during agitation ( Fig. 1 ). This was recognized for the first time at her second birthday.

flux episodes. At the age of 2 years, the girl presented with no complications, normal ingestion, normal weight, and normal height. However, her parents reported that the right side of her face remained pale and was not sweating during agitation ( Fig. 1 ). This was recognized for the first time at her second birthday. A detailed retrospective study of her NICU patient data and previous consultations revealed that this condition had not been observed before. The neurological and behavioral development of the patient was in accordance with her age. After 1 year of follow-up, the symptom of asymmetric sweating and paleness remains unchanged. Fig. 1 Girl with a loss of facial flushing and warming of the right side after exertion (age of 3 years; picture shown with parental approval). Discussion

A detailed retrospective study of her NICU patient data and previous consultations revealed that this condition had not been observed before. The neurological and behavioral development of the patient was in accordance with her age. After 1 year of follow-up, the symptom of asymmetric sweating and paleness remains unchanged. Fig. 1 Girl with a loss of facial flushing and warming of the right side after exertion (age of 3 years; picture shown with parental approval). Discussion We report a case of HS that occurred 2 years after thoracoscopic repair of a child with TEF. HS is a repeatedly reported dysautonomia of the sympathetic innervation leading to loss of hemifacial flushing, sweating, and other sympathetic functions. It has to be separated from the Harlequin color change, which happens due to a paroxysmal change of vasomotor condition of the skin in the first DOLs, leading to alternating hemi-sided flushing. 9 In adults, the majority of cases cannot be related to any medical cause, while one-third of the patients appear due to expanding neoplasms or iatrogenic damage. 10 There are much fewer cases reported in children compared with adults. To date the majority of these patients have been described as having a secondary cause, although the limited number does not allow reliable conclusions. 3 11

, while one-third of the patients appear due to expanding neoplasms or iatrogenic damage. 10 There are much fewer cases reported in children compared with adults. To date the majority of these patients have been described as having a secondary cause, although the limited number does not allow reliable conclusions. 3 11 To our knowledge, the report presented here describes the third patient of HS in a girl with EA (gross type C) and successful thoracoscopic treatment. This raises the question whether HS is a side effect of the thoracoscopic technique. Studies comparing the long-term complications after thoracoscopic versus open approaches for EA show no differences. 6 7 However, in 2009 Cozzi et al reported a case of hemifacial flushing and sweating after open repair of EA. And there has been a second report after open repair in 2018. 12 The other two cases of HS after repair of EA using thoracoscopy were reported in 2015. Interestingly, the first appearance of the hemifacial flushing happened between 1 and 3 years of age in all described patients. 8 12 13 Therefore, an intraoperative complication or lesion of the sympathetic nervous structures is unlikely due to the uneventful postoperative course and late onset of symptoms. The previously mentioned electrical division of the azygos vein is routinely performed by a lot of surgeons during the thoracoscopic approach to better expose the TEF. 14 As in the previously published case reports, the thoracoscopic repair of the EA did not include electric division of the azygos vein HS that cannot be explained by this operative step either. 8 Moreover, the relevant sympathetic nervous structures are localized further cranial from the surgical field during thoracoscopic EA repair. In reported cases of iatrogenic damage, the sympathetic structures were injured in the apex of the lung or even further cranial. 2 4 15

e explained by this operative step either. 8 Moreover, the relevant sympathetic nervous structures are localized further cranial from the surgical field during thoracoscopic EA repair. In reported cases of iatrogenic damage, the sympathetic structures were injured in the apex of the lung or even further cranial. 2 4 15 The pathogenesis of primary and secondary HS is still not sufficiently understood. The loss of hemifacial flushing and sweating most likely occurs due to a loss of function or dysregulation of the third-order neuron of the vaso- and sudomotor fibers of the ipsilateral sympathetic nervous pathway. 1 3 In our case and in previously reported ones, an obvious correlation between the surgical procedure and occurrence of symptoms cannot be found. This leads to the question whether there is a primary “pre-surgical” connection between HS and EA or the appearance in these cases is completely coincidental. 12

us pathway. 1 3 In our case and in previously reported ones, an obvious correlation between the surgical procedure and occurrence of symptoms cannot be found. This leads to the question whether there is a primary “pre-surgical” connection between HS and EA or the appearance in these cases is completely coincidental. 12 The theory of neurocristopathy describes the connection between disturbed migration of neural crest cells and maturational dysautonomia. Maldevelopment of the autonomic nervous system during embryogenesis can result in dysregulated sympathetic innervation. There is evidence for an association of EA and autonomic disturbances such as hyperhidrosis during feeding and hyperthermia. 12 16 17 Moreover, the association of EA with certain cardiovascular anomalies may suggest an underlying neurocristopathy of the caudal pharyngeal arch. 12 18 In this context, one case of HS associated with rare cardiovascular anomalies has been described. 19 It is tempting to speculate that both conditions, EA and HS, could be a result of disturbed neural crest migration. Due to the very limited number of reported cases of HS in children and in particular in association with EA, this conclusion is only hypothetical and further research addressing the pathogenesis of HS is needed. Conclusion We report the third case of HS in a 3-year-old girl with EA after uncomplicated thoracoscopic repair. The late presentation of the HS in these cases makes a direct causal relation to the surgical procedure implausible. HS may therefore be a (primary) neurocristopathy associated with EA/TEF.

The theory of neurocristopathy describes the connection between disturbed migration of neural crest cells and maturational dysautonomia. Maldevelopment of the autonomic nervous system during embryogenesis can result in dysregulated sympathetic innervation. There is evidence for an association of EA and autonomic disturbances such as hyperhidrosis during feeding and hyperthermia. 12 16 17 Moreover, the association of EA with certain cardiovascular anomalies may suggest an underlying neurocristopathy of the caudal pharyngeal arch. 12 18 In this context, one case of HS associated with rare cardiovascular anomalies has been described. 19 It is tempting to speculate that both conditions, EA and HS, could be a result of disturbed neural crest migration. Due to the very limited number of reported cases of HS in children and in particular in association with EA, this conclusion is only hypothetical and further research addressing the pathogenesis of HS is needed. Conclusion We report the third case of HS in a 3-year-old girl with EA after uncomplicated thoracoscopic repair. The late presentation of the HS in these cases makes a direct causal relation to the surgical procedure implausible. HS may therefore be a (primary) neurocristopathy associated with EA/TEF. New Insights and the Importance for the Pediatric Surgeon Harlequin syndrome is a rare occurrence after esophageal atresia repair with unknown etiology. Conflict of Interest None.

Introduction Amyand's hernia is defined as an inguinal herniation of a normal, perforated, or acutely inflamed appendix. It was first described by Claudius Amyand in 1735, a French born surgeon working at St George's Hospital in London. On December 6, 1735, he performed the first recorded successful appendicectomy in an 11-year-old boy who had an inguinal hernia combined with an acutely inflamed appendix. 1 Amyand's hernia is a relatively rare presentation of appendicitis, with an incidence of less than 1% of all inguinal hernias according to the literature. 2 Although many presentations are described in the literature, the most common is one of an incarcerated or partially reducible inguinal hernia. We report a case of Amyand's hernia with appendicitis presenting only with inguinoscrotal edema and erythema in an almost 3-week-old baby. Case Report

Amyand's hernia is defined as an inguinal herniation of a normal, perforated, or acutely inflamed appendix. It was first described by Claudius Amyand in 1735, a French born surgeon working at St George's Hospital in London. On December 6, 1735, he performed the first recorded successful appendicectomy in an 11-year-old boy who had an inguinal hernia combined with an acutely inflamed appendix. 1 Amyand's hernia is a relatively rare presentation of appendicitis, with an incidence of less than 1% of all inguinal hernias according to the literature. 2 Although many presentations are described in the literature, the most common is one of an incarcerated or partially reducible inguinal hernia. We report a case of Amyand's hernia with appendicitis presenting only with inguinoscrotal edema and erythema in an almost 3-week-old baby. Case Report Our patient is a 19-day-old, full-term baby, born in good general condition who presented to our emergency department with 1-day history of inguinoscrotal erythema and persistent irritability. The patient was struggling with achieving full feeds, although not vomiting and managing to open bowels normally. Examination revealed a distended, soft, nontender abdomen and bilateral scrotal edema along with moderate inguinoscrotal erythema and tenderness ( Fig. 1 ). Examination of the inguinal region revealed no evidence of irreducible hernia or inguinal thickening on either side. Laboratory investigations were normal apart from increased C-reactive protein levels. Due to the unclear signs, we resorted to imaging for answers. An abdominal X-ray showed some dilated bowel loops but no evidence of intestinal obstruction or aeration in the scrotal compartments ( Fig. 2 ). An inguinoscrotal ultrasound (US) with Doppler did not show any evidence of incarcerated bowel loops or testicular torsion, although pointing to increased vascularity and echogenicity of the right testis. The patient was, therefore, admitted on the working diagnosis of epididymo-orchitis and started on intravenous antibiotics.

noscrotal ultrasound (US) with Doppler did not show any evidence of incarcerated bowel loops or testicular torsion, although pointing to increased vascularity and echogenicity of the right testis. The patient was, therefore, admitted on the working diagnosis of epididymo-orchitis and started on intravenous antibiotics. Fig. 1 Neonate presenting with inguinoscrotal erythema and edema. Fig. 2 Preoperative abdominal X-ray.

noscrotal ultrasound (US) with Doppler did not show any evidence of incarcerated bowel loops or testicular torsion, although pointing to increased vascularity and echogenicity of the right testis. The patient was, therefore, admitted on the working diagnosis of epididymo-orchitis and started on intravenous antibiotics. Fig. 1 Neonate presenting with inguinoscrotal erythema and edema. Fig. 2 Preoperative abdominal X-ray. Twelve hours later, our patient exhibited bilious vomiting and worsening inguinoscrotal edema. Abdominal examination showed signs of generalized rigidity and guarding. With no clinical evidence of an incarcerated hernia, the exclusion of testicular torsion by Doppler, and the development of an acute abdomen, we opted for an exploratory laparotomy through a right upper quadrant incision. This allowed us to be able to exclude any source of intra-abdominal sepsis while at the same time providing adequate surgical access to deal with the possibility of a missed irreducible hernia. The bowel was inspected and the appendix was found to be incarcerated in the right internal ring with the evidence of inflammation and suppuration ( Fig. 3 ). The appendix was gently reduced. Appendectomy and closure of the internal ring with a purse-string suture were done. Postoperatively, feeding was started through the nasogastric tube after 8 hours that was tolerated well and built up gradually. Antibiotics were discontinued in 5 days after improvement in inflammatory markers and resolution of the inguinoscrotal erythema. No other clinical evidence of associated comorbidities common with neonatal appendicitis was illustrated. The patient was discharged home and had an unremarkable 3-month follow-up.

gradually. Antibiotics were discontinued in 5 days after improvement in inflammatory markers and resolution of the inguinoscrotal erythema. No other clinical evidence of associated comorbidities common with neonatal appendicitis was illustrated. The patient was discharged home and had an unremarkable 3-month follow-up. Fig. 3 Intraoperative finding showing inflamed appendix. Discussion Neonatal appendicitis is a rare condition associated with comorbidities such as prematurity or other diagnoses like Hirschsprung's disease, meconium plug syndrome, and cystic fibrosis. 3 Inflamed appendix within the hernial sac, on the other hand, accounts for one-third of the neonatal appendicitis described in the literature. Variable presentations ranging from irritability with signs of irreducible hernia to systemic signs of sepsis and shock. 4 Although the exact pathophysiology of this entity is unknown, one of the theories is the presence of a congenital band extending from the appendix to the right testis going through the inguinal canal. 5 A link between Amyand's hernia and appendicitis has also been debated. Some authors suggest that it is an incidental finding, while others arguing that the appendix becomes entrapped due to the contraction of abdominal muscles, which leads to compromised blood flow with consequent inflammation and bacterial overgrowth. 6

link between Amyand's hernia and appendicitis has also been debated. Some authors suggest that it is an incidental finding, while others arguing that the appendix becomes entrapped due to the contraction of abdominal muscles, which leads to compromised blood flow with consequent inflammation and bacterial overgrowth. 6 Most authors consider diagnosis of this condition to be intricate. This is because of the nonspecific manifestations, as in this case, where no obvious signs of irreducible hernia or intestinal obstruction were found. Cankorkmaz et al described a series of 12 cases, where only one was diagnosed preoperatively by US. All patients presented with an inguinal nonreducible mass and only a few exhibited fever, vomiting, or intestinal obstruction symptoms. These patients were taken to theater with a working diagnosis of incarcerated hernia. Intraoperatively they found six cases of Amyand's hernia with inflamed appendices: two normal appendices and four of equivocal nature. 7 Another larger case series described 46 patients diagnosed with Amyand's hernia, most of which after the incidental finding of the appendix in the hernial sac. 8 The differential diagnosis for a painful mass in the inguinal area may include incarcerated hernia, inguinal lymphadenitis, epididymo-orchitis, testicular torsion, or soft-tissue infection. Making the correct diagnosis preoperatively requires awareness of this rare entity by the clinician, as well as physical examination findings consistent with a hernia mass without obstructive signs. 6 Utilizing imaging like inguinoscrotal US was helpful in achieving a preoperative diagnosis in few reported cases. 9 In one series, US scan was successful in diagnosing 9 out of 12 patients by revealing a blind ending intestinal loop. 10 At the same time, Color Doppler US can be very useful in excluding testicular torsion from the differential diagnosis with an 80 to 98% sensitivity and an accuracy of 97%. 11 Moreover, computed tomography scan was able to visualize an inflamed appendix extending into the inguinal canal of a 77-year-old female. 2 Whether or not it is worthwhile exposing a neonate to such radiation to arrive at a diagnosis will be subject to controversy. Although no mortality is reported in the literature, delayed diagnosis and surgical intervention meant that on surgical exploration, many appendices were found to be perforated. Scrotal abscess, peritonitis, and a case progressing to septic shock are other reported complications. 4

nosis will be subject to controversy. Although no mortality is reported in the literature, delayed diagnosis and surgical intervention meant that on surgical exploration, many appendices were found to be perforated. Scrotal abscess, peritonitis, and a case progressing to septic shock are other reported complications. 4 Due to the majority of cases being incidentally found on surgical exploration, treatment is mainly by primary hernia repair and appendectomy through an inguinal incision. In cases with unclear diagnosis, reports of laparotomy as a primary incision, combined abdominal and inguinal incisions, and even scrotal explorations were described. 12 Some authors advocate against removing the appendix when found incidentally without any obvious signs of inflammation. 8 Laparoscopic approach is now gaining more popularity in such cases, as it allows increased visualization of the abdominal cavity and can be diagnostic and therapeutic. 12 However, utilizing this approach would be more likely if the patient's clinical condition is stable. Diagnosing this rare entity requires a high index of suspicion by clinicians and when imaging is inconclusive, resorting to diagnostic laparoscopy should be considered to avoid laparotomy or multiple surgical incisions. While a tender, irreducible inguinal mass is the most common presentation, we report a case of Amyand's hernia with concurrent appendicitis presenting only with inguinoscrotal erythema and edema.

s inconclusive, resorting to diagnostic laparoscopy should be considered to avoid laparotomy or multiple surgical incisions. While a tender, irreducible inguinal mass is the most common presentation, we report a case of Amyand's hernia with concurrent appendicitis presenting only with inguinoscrotal erythema and edema. New Insights & the Importance for the Pediatric Surgeon Diagnosing Amyand's hernia in neonates can be difficult. While a tender, irreducible inguinal mass is the most common presentation, we report a case of Amyand's hernia with concurrent appendicitis presenting only with inguinoscrotal erythema and edema. Conflict of Interest None.

New Insights and the Importance for the Pediatric Surgeon Iatrogenic compartment syndrome can lead to severe sequelae; it is infrequent, but preventable. Small children are at higher risk due to the difficulty of noncompliance. Pediatric surgeons must be aware of its etiology to prevent harm of their patients. Introduction Acute compartment syndrome is a surgical emergency that can severely compromise the circulation, function, and even viability of an extremity. It occurs when an increase in the pressure within any closed, fixed compartment (defined by myofascial elements or bone) exceeds the perfusion pressure of the tissue, leading to ischemia and necrosis. 1 Most cases of acute extremity compartment syndrome develop after severe injuries such as fractures, but they can also occur after lesser injuries or, less frequently, they can be iatrogenic, secondary to tight dressings or tightly applied splints and casts, with the same terrible consequences. 2 We report a severe case of compartment syndrome due to a compressive burn dressing. Case Report

Acute compartment syndrome is a surgical emergency that can severely compromise the circulation, function, and even viability of an extremity. It occurs when an increase in the pressure within any closed, fixed compartment (defined by myofascial elements or bone) exceeds the perfusion pressure of the tissue, leading to ischemia and necrosis. 1 Most cases of acute extremity compartment syndrome develop after severe injuries such as fractures, but they can also occur after lesser injuries or, less frequently, they can be iatrogenic, secondary to tight dressings or tightly applied splints and casts, with the same terrible consequences. 2 We report a severe case of compartment syndrome due to a compressive burn dressing. Case Report An otherwise healthy 2-year-old girl arrived at her local health center with a contact burn with a hot iron several minutes before. She presented a noncircumferential, superficial partial-thickness burn on the dorsum of the mid phalanx of the second finger of her right hand. A compressive dressing was applied solely to the affected finger. Forty-eight hours afterward, the patient presented at the emergency room with severe pain of the finger. After removal of the dressing, a circumferential constrictive eschar was observed at the base of the finger, secondary to ischemia due to the compressive dressing, which generated a significant compartment syndrome with severe vascular compromise of the finger ( Fig. 1A ). Emergent bilateral escharotomies were performed, with immediate recovery of distal perfusion ( Fig. 1B ). One week afterward, the patient underwent surgical debridement of the burn on the dorsum of her finger and escharectomy of the ischemic eschar at the base. The lesions were covered with split-thickness skin grafts, the donor site being the ipsilateral arm ( Fig. 2A ). As soon as the graft healed, the patient was started on splinting and physical therapy. However, after 3 months, the patient presented with a palmar contracture of the digit that limited full extension of the finger ( Fig. 2B ). The contracture was surgically released and the resulting skin defect was covered with full-thickness skin graft (donor site: contralateral groin). At 8 months follow-up, and after intense splinting and physical therapy, the patient shows a normal function, achieving complete extension of the finger, with a mature, asymptomatic, flat scar ( Fig. 2C and D ).

and the resulting skin defect was covered with full-thickness skin graft (donor site: contralateral groin). At 8 months follow-up, and after intense splinting and physical therapy, the patient shows a normal function, achieving complete extension of the finger, with a mature, asymptomatic, flat scar ( Fig. 2C and D ). Fig. 1 ( A ) Ischemic circumferential constrictive eschar at the base of the finger (black arrow), secondary to compressive dressing, with severe vascular compromise of the finger. Arrowhead: burn lesion. ( B ) Immediate recovery of distal perfusion after emergent bilateral escharotomies (black arrow) were performed. Arrowhead: debrided burn. Fig. 2 Treatment of the sequelae. ( A ) Immediate postoperative result after surgical debridement of the burn on the dorsum of the finger and escharectomy of the ischemic eschar at the base and grafting with partial-thickness skin grafts. ( B ) Severe palmar contracture of the digit at 3 months follow-up. ( C ) Dorsum of hand at 8 months follow-up, after surgically releasing the contracture and grafting with full-thickness skin graft. ( D ) Palm of hand. Discussion Acute compartment syndrome is a devastating diagnosis that can lead to severe sequelae, such as the loss of an extremity or body segment. 3

Fig. 2 Treatment of the sequelae. ( A ) Immediate postoperative result after surgical debridement of the burn on the dorsum of the finger and escharectomy of the ischemic eschar at the base and grafting with partial-thickness skin grafts. ( B ) Severe palmar contracture of the digit at 3 months follow-up. ( C ) Dorsum of hand at 8 months follow-up, after surgically releasing the contracture and grafting with full-thickness skin graft. ( D ) Palm of hand. Discussion Acute compartment syndrome is a devastating diagnosis that can lead to severe sequelae, such as the loss of an extremity or body segment. 3 Any pathologic condition that increases the volume inside a closed compartment (intrinsic causes, such as bleeding or edema after a fracture) or limits the external dilation of the compartment (extrinsic causes, such as burns or tight dressings) will increase the internal pressure of the compartment, potentially leading to acute compartment syndrome, with tissue necrosis. 4 Intrinsic causes, such as fractures or severe crush injuries, are by far the most frequent cause of acute compartment syndrome. Infrequently, iatrogenic causes like compressive dressings or tight splints and casts can also produce severe damage. 5 The case of acute compartment syndrome we present in this article is distinctly of iatrogenic origin, consequence of a negligent act. The application of an excessively tight circumferential dressing around the base of the finger produced a local necrosis of the skin, which behaved like a circumferential third-degree burn, restraining the dilation of the finger and thus hindering appropriate perfusion.

atrogenic origin, consequence of a negligent act. The application of an excessively tight circumferential dressing around the base of the finger produced a local necrosis of the skin, which behaved like a circumferential third-degree burn, restraining the dilation of the finger and thus hindering appropriate perfusion. All cases of iatrogenic compartment syndrome are potentially preventable and easy to avoid if health professionals are adequately trained and provide care to their best of their knowledge, being extremely cautious in all their actions. 6 With regard to the presented case, all damage could have been avoided if a noncompressive, noncircumferential dressing had been applied and the tip of the finger had been left exposed to monitor for possible vascular compromise.

ide care to their best of their knowledge, being extremely cautious in all their actions. 6 With regard to the presented case, all damage could have been avoided if a noncompressive, noncircumferential dressing had been applied and the tip of the finger had been left exposed to monitor for possible vascular compromise. Although this case is the consequence of a neglectful act, it is nonetheless interesting to point out that the patient presented other risk factors that might have favored the development of the compartment syndrome. First, the patient sustained a thermal burn. This lesion was certainly not the cause of the compartment syndrome, for it was neither circumferential nor deep, but it must have triggered a local inflammatory response that might have contributed to the onset or aggravation of the compartment syndrome. 7 Second, small children are especially vulnerable to iatrogenic complications, for they are frequently unable to cooperate due to their immature cognitive abilities and their limited verbal capacity sometimes precludes adequate communication. 8 For a start, it can be very challenging to apply an adequate dressing on a toddler, for the patient will not collaborate. Also, some alarm symptoms (such as intense pain) might be overlooked in young children due to their inability to provide clinical information, resulting in delays in diagnosis and treatment. In any case, care providers that frequently take care of burns and/or children should be appropriately qualified and be aware of the particular idiosyncrasy of minors.

ense pain) might be overlooked in young children due to their inability to provide clinical information, resulting in delays in diagnosis and treatment. In any case, care providers that frequently take care of burns and/or children should be appropriately qualified and be aware of the particular idiosyncrasy of minors. Cases such as the one we present here should result in education and training of health personnel at all levels, with a very especial emphasis on prevention of iatrogenic complications; practitioners and nursing staff should be aware of the very severe consequences their neglectful actions may have on patients. As health-care providers, we have a responsibility to provide the best possible medical care. Conclusion Iatrogenic compartment syndrome is an infrequent but preventable cause of possible severe damages, such as the loss of an extremity or body segment. It is fundamental that health professionals are aware of its etiologies and risk factors and take utmost care in all their actions, to avoid negligent acts that could lead to severe or permanent damage to patients. Conflict of Interest None.

Introduction The adrenal gland consists of a medulla and a cortex and may give rise to various types of tumors. While adenomas and carcinomas may develop in the cortex, the medulla may be the origin of neuroblastomas, pheochromocytomas, and ganglioneuromas. All of these lesions may require surgical intervention by an open or a minimally invasive approach. The risks and benefits of pediatric laparoscopic adrenalectomy for malignant or semibenign lesions remain a matter of vivid discussion. While simple adrenal cysts or adenomas are benign lesions and a minimally invasive approach may be considered, adrenocortical carcinomas and (ganglio)neuroblastomas require careful assessment of risk factors such as serum biomarkers, hormonal status, and image-defined risk factors (IDRFs) . 1 2 3 In the present case series, distinct characteristics of technical feasibility and oncological safety of laparoscopic approaches for pediatric adrenal masses are discussed. Case Presentation Case 1

The adrenal gland consists of a medulla and a cortex and may give rise to various types of tumors. While adenomas and carcinomas may develop in the cortex, the medulla may be the origin of neuroblastomas, pheochromocytomas, and ganglioneuromas. All of these lesions may require surgical intervention by an open or a minimally invasive approach. The risks and benefits of pediatric laparoscopic adrenalectomy for malignant or semibenign lesions remain a matter of vivid discussion. While simple adrenal cysts or adenomas are benign lesions and a minimally invasive approach may be considered, adrenocortical carcinomas and (ganglio)neuroblastomas require careful assessment of risk factors such as serum biomarkers, hormonal status, and image-defined risk factors (IDRFs) . 1 2 3 In the present case series, distinct characteristics of technical feasibility and oncological safety of laparoscopic approaches for pediatric adrenal masses are discussed. Case Presentation Case 1 A 3-year-old boy presented with precocious puberty (tanner stages P2 and G2). X-rays of the extremities showed an accelerated bone age of ∼6 years. Ultrasound (US) and magnetic resonance imaging (MRI) of the abdomen revealed a right adrenal mass of approximately 4.0 × 2.5 × 4.0 cm ( Fig. 1 ). The laboratory workup of the patient is displayed in Table 1 . Laparoscopic adrenalectomy was performed. Histopathological examination confirmed complete resection of an adrenal adenoma with hamartoma components. At the 2-year follow-up, the patient presented with a normalized hormone status (cortisol, testosterone, dehydroepiandrostendionsulfate, androstenedione, 17-hydroxyprogesterone, adrenocorticotropic hormone) and without signs of recurrence in the US.

ed complete resection of an adrenal adenoma with hamartoma components. At the 2-year follow-up, the patient presented with a normalized hormone status (cortisol, testosterone, dehydroepiandrostendionsulfate, androstenedione, 17-hydroxyprogesterone, adrenocorticotropic hormone) and without signs of recurrence in the US. Fig. 1 Magnetic resonance imaging of all four patients. None of them presented with image-defined risk factors.

ed complete resection of an adrenal adenoma with hamartoma components. At the 2-year follow-up, the patient presented with a normalized hormone status (cortisol, testosterone, dehydroepiandrostendionsulfate, androstenedione, 17-hydroxyprogesterone, adrenocorticotropic hormone) and without signs of recurrence in the US. Fig. 1 Magnetic resonance imaging of all four patients. None of them presented with image-defined risk factors. Table 1 Laboratory workup of the patient Parameter Range Case 1 Case 2 Case 3 Case 4 Age at surgery 3 years 1,5 years 15 years 7 years Body weight (kg) 18 (90–97th percentile) 13 (97th percentile) 71 (80th percentile) 20 (3rd percentile) Height (cm) 103 (90th percentile) 82 (97th percentile) 182 (88th percentile) 121 (10th percentile) Serum LDH (U/L) 120–340 218 367 (+) 193 248 Serum NSE (mg/mL) 0–26 26.0 33.1 (+) 38 (+) VMA urine (µg/mg creatinine) <10 9.0 8.1 5.0 HVA urine (µg/mg creatinine) <15 15.0 11.0 10.0 Dopamine urine (µg/mg creatinine) <0.85 0.68 0.64 0.52 Bone marrow aspiration Negative N-Myc amplification Negative LH (mU/mL) 1.0–14.0 0.22 (−) <0.1 (−) 1.94 FSH (mU/mL) 0.89–11.72 0.59 (−) <0.1 (−) 1.80 Basal hGH (mU/mL) 0.5–3.0 6.9 (+) 2.1 PRL (ng/mL) 2.1–29.2 14.0 IGF-1 (mU/mL) 30.0–300.0 120.6 Basal ACTH (pg/mL) 10–51 56.6 (+) 17.1 Basal cortisol (ng/mL) 43–220 5.8 (−) Androstenedione (ng/mL) 1.0–14.0 3.3 2.58 DHEA-S (µg/mL) 0.39–4.63 8.1 (+) 1.62 Total Testosterone (ng/mL) 2.41–8.30 0.4 0.77 2.88 17OH-Progesterone (ng/mL) 0.2–0.9 0.88 0.66 Histology Adrenal adenoma Potentially malignant ACT Simple adrenal cyst Ganglioneuroblastoma Abbreviations: ACT, adrenocortical tumor(+)-elevated; (−) - decreased; ACTH, adrenocorticotropic hormone; DHEA-S, dehydroepiandrostendionsulfate; FSH, follicle stimulating hormone; hGH, human growth hormone; HVA, homovanillylic acid; IGF, insulin-like growth factor; LDH, lactate dehydrogenase; LH, luteinizing hormone; NSE, neuron specific enolase; PRL, prolactin; VMA, vanillylmandelic acid.

TH, adrenocorticotropic hormone; DHEA-S, dehydroepiandrostendionsulfate; FSH, follicle stimulating hormone; hGH, human growth hormone; HVA, homovanillylic acid; IGF, insulin-like growth factor; LDH, lactate dehydrogenase; LH, luteinizing hormone; NSE, neuron specific enolase; PRL, prolactin; VMA, vanillylmandelic acid. Case 2 A one- and half-year-old female presented with a precocious puberty (virilization with clitoris hypertrophy and pubarche P2–3). Laboratory parameters are shown in Table 1 . Abdominal US showed a hypoechogenic lesion in the right adrenal gland. MRI ( Fig. 1 ) revealed a largely solid, relatively sharply delimited, contrast-enhancing lesion of the right adrenal gland (4.0 × 3.0 × 3.5 cm) without IDRFs. Thus, a transperitoneal laparoscopic adrenalectomy was performed. Histopathology revealed a large, encapsulated adrenocortical tumor (ACT) with a diameter of 5 cm and malignant potential with tumor-free resection margins. At the 11-month follow-up, there were no signs of recurrence. Case 3

A one- and half-year-old female presented with a precocious puberty (virilization with clitoris hypertrophy and pubarche P2–3). Laboratory parameters are shown in Table 1 . Abdominal US showed a hypoechogenic lesion in the right adrenal gland. MRI ( Fig. 1 ) revealed a largely solid, relatively sharply delimited, contrast-enhancing lesion of the right adrenal gland (4.0 × 3.0 × 3.5 cm) without IDRFs. Thus, a transperitoneal laparoscopic adrenalectomy was performed. Histopathology revealed a large, encapsulated adrenocortical tumor (ACT) with a diameter of 5 cm and malignant potential with tumor-free resection margins. At the 11-month follow-up, there were no signs of recurrence. Case 3 A 15-year-old male patient presented with exercise-triggered recurrent right abdominal pain. US and MRI demonstrated an uncomplicated cystic structure in the right adrenal gland with sharp delineation to the upper renal pole and without pathological contrast enhancement ( Fig. 1 ) giving no indication for surgery. In his routine US checkups, the size of the cyst increased to 8.0 × 7.6 × 5.8 cm over 2 years indicating surgical removal. Additional information about laboratory workup is given in Table 1 . Laparoscopic adrenalectomy was done and histopathology revealed a mesothelial cyst. At the 12-month follow-up, there were no sonographic signs of recurrence of the cyst. Case 4

A 15-year-old male patient presented with exercise-triggered recurrent right abdominal pain. US and MRI demonstrated an uncomplicated cystic structure in the right adrenal gland with sharp delineation to the upper renal pole and without pathological contrast enhancement ( Fig. 1 ) giving no indication for surgery. In his routine US checkups, the size of the cyst increased to 8.0 × 7.6 × 5.8 cm over 2 years indicating surgical removal. Additional information about laboratory workup is given in Table 1 . Laparoscopic adrenalectomy was done and histopathology revealed a mesothelial cyst. At the 12-month follow-up, there were no sonographic signs of recurrence of the cyst. Case 4 A 7-year-old boy presented due to recurrent abdominal pain. The routine US revealed an inhomogeneous expansion of approximately 3 × 3 × 2 cm with calcifications in the right adrenal gland. Laboratory data are displayed in Table 1 . MRI showed a well circumscribed slightly inhomogeneous mass in the right adrenal gland of approximately 3.3 × 2.6 × 2.0 cm. Bone marrow biopsy was normal but metaiodobenzylguanidine (MIBG) scan showed pathologically increased tracer uptake. Anterior laparoscopic adrenalectomy was done with complete tumor resection. Histopathological examination revealed clinical stage I maturing ganglioneuroblastoma with free margins. No recurrence was observed at 2-year follow-up. Discussion In this case series, we present four pediatric patients with a broad spectrum of the adrenal gland masses undergoing laparoscopic resection.

A 7-year-old boy presented due to recurrent abdominal pain. The routine US revealed an inhomogeneous expansion of approximately 3 × 3 × 2 cm with calcifications in the right adrenal gland. Laboratory data are displayed in Table 1 . MRI showed a well circumscribed slightly inhomogeneous mass in the right adrenal gland of approximately 3.3 × 2.6 × 2.0 cm. Bone marrow biopsy was normal but metaiodobenzylguanidine (MIBG) scan showed pathologically increased tracer uptake. Anterior laparoscopic adrenalectomy was done with complete tumor resection. Histopathological examination revealed clinical stage I maturing ganglioneuroblastoma with free margins. No recurrence was observed at 2-year follow-up. Discussion In this case series, we present four pediatric patients with a broad spectrum of the adrenal gland masses undergoing laparoscopic resection. Generally, tumors of the adrenal gland can be malignant (with descending incidence: neuroblastoma, adrenocortical carcinoma, or sarcoma) or benign (with descending incidence: adenoma, ganglioneuroma, cysts, or adrenal hyperplasia). 3 In infants, tumors have to be discriminated from postpartum adrenal hematomas as an important differential diagnosis that has to be considered in the diagnostic workup. Regarding tumors, pheochromocytomas are rare adrenal masses that can be benignant or malignant in their behavior. When selecting patients for a minimally invasive approach, special care has to be taken to identify these children who potentially benefit from minimally invasive surgery (MIS) (better cosmetic results, less pain, and shorter hospital stay 4 5 ) and to exclude those who might be harmed by this procedure (risk of inadequate resection, tumor recurrence, trocar site metastasis, tumor rupture, and spillage as well as tumor growth or dissemination in relation to CO 2 insufflation 6 7 ).

gery (MIS) (better cosmetic results, less pain, and shorter hospital stay 4 5 ) and to exclude those who might be harmed by this procedure (risk of inadequate resection, tumor recurrence, trocar site metastasis, tumor rupture, and spillage as well as tumor growth or dissemination in relation to CO 2 insufflation 6 7 ). Guidelines for adrenalectomy poorly define the indications for MIS in the pediatric age group. The rarity of adrenal lesions in this age group, a wide pathologic spectrum, the predominance of malignant lesions, large dimensions of the masses in relation to small body size of the patients, and technical challenges with miniature instruments may be hypothesized as the underlying reasons. 8 After initial suspicion, patients with adrenal masses undergo routine diagnostic workups usually involve imaging (US, computed tomography and/or MRI) paired with laboratory analysis (routine blood chemistry, differential blood count, hormone profiles, and tumor markers). Concerning imaging special emphasis is put on the presence or absence of IDRFs. Infiltration of the renal pedicle, encasement of major vessels (inferior vena cava, aorta, superior mesenteric artery, celiac trunk, iliac, and hypogastric vessels), the porta hepatis, dumbbell tumors, muscular infiltration, and compression of kidney or ureter have been identified as IDRFs. 1 If present, IDRFs may point to malignant behavior of the tumor. Furthermore, complete resection could be achieved in only 26% of cases presenting with IDRFs in a study of Günther et al. 1

ls), the porta hepatis, dumbbell tumors, muscular infiltration, and compression of kidney or ureter have been identified as IDRFs. 1 If present, IDRFs may point to malignant behavior of the tumor. Furthermore, complete resection could be achieved in only 26% of cases presenting with IDRFs in a study of Günther et al. 1 While MIS is the mainstay in benign lesions, 3 it has to be discussed critically in malignant tumors. In selected cases of neuroblastoma (size ≤ 5 cm diameter and absence of vascular encasement), MIS led to an equal long-term oncologic outcome compared with open surgery. 9 Similarly, de Lagausie et al state that sizes greater than 6 cm, enlarged veins, and involved adjacent organs or vessels are relative contraindications for laparoscopic adrenalectomy for neuroblastomas. 10 Overall, the International Pediatric Endoscopic Group 2010 guidelines state that there are no absolute contraindications for laparoscopic adrenalectomy as long as the principles of cancer surgery are maintained. 11

nt organs or vessels are relative contraindications for laparoscopic adrenalectomy for neuroblastomas. 10 Overall, the International Pediatric Endoscopic Group 2010 guidelines state that there are no absolute contraindications for laparoscopic adrenalectomy as long as the principles of cancer surgery are maintained. 11 Special care has to be taken to identify the rare case of a malignant ACT. Suspicion should be raised in case of adrenal masses presenting with IDRFs and especially in patients with signs of precocious puberty. ACTs have a high risk of intraoperative rupture (∼20% of cases) with consecutive tumor spillage. 12 Recent recommendations opted against laparoscopic biopsy or resection of tumors suspicious for or known to be ACT even when MIS appears feasible and tempting, because complete resection of the mass is the prerequisite for recurrence-free survival. 13 14 However, there are also reports of successful MIS in ACTs. 8 15 16 These reports are supported by the current German AWMF recommendations that advocate that a MIS approach in ACTs is feasible as long as the principles of oncological surgery are maintained. 17 Furthermore, a recent publication of adult ACT cases found evidence that the open and laparoscopic approach may be comparable in terms of recurrence-free survival for patients with stage I and II ACT when the principles of surgical oncology are respected. 18

g as the principles of oncological surgery are maintained. 17 Furthermore, a recent publication of adult ACT cases found evidence that the open and laparoscopic approach may be comparable in terms of recurrence-free survival for patients with stage I and II ACT when the principles of surgical oncology are respected. 18 Taken the recent literature into account, the indication for MIS was clear in cases 1, 3, and 4 presented in this series. Histology of case 2 revealed a potentially malignant adrenocortical tumor raising the question, if MIS was the optimal therapy for this patient. As there was no evidence of IDRFs and the tumor size was < 5 cm, MIS was performed. Although an open procedure may have been beneficial, the tumor could be removed completely by MIS and the patient is free of recurrences until now (although the follow-up interval of 11 months is relatively low for an oncologic patient). In conclusion, MIS is indicated in benign lesions, but has to be discussed critically in case of potential malignancy. In these cases, a MIS approach may be considered in selected patients without IDRFs as long as the principles of oncologic surgery can be maintained. New Insights and the Importance for the Pediatric Surgeon In this series of four cases distinct characteristics of technical feasibility and oncological safety of laparoscopic approaches for pediatric adrenal masses are discussed. Conflict of Interest None declared.

Introduction Currarino triad is a rare congenital disorder characterized by the presence of three components: anorectal anomaly, sacral vertebral defect, and a presacral mass (lipomyelomeningocele and/or developmental cyst). 1 2 Cases may present with the complete form of the triad or may lack one of the three components. 3 Unlike other forms of sacral agenesis, Currarino triad is characterized by partial dysplasia sparing the first sacral vertebra. 1 4 The characteristic sacral defect (sacral scimitar) is considered the key for making the diagnosis. 5 Early reports in the literature were described by Kennedy 6 and Ashcraft and Holder. 7 However, the triad was named after an American radiologist “Guido Currarino,” who was the first to describe the triad as a unique syndrome with characteristic genetic inheritance and provided embryological explanation for its occurrence. 1 Increased awareness of this rare condition can help in achieving early detection, allowing for a more effective way of management. Case Presentation A male patient presented at birth with anorectal malformation ( Fig. 1A ); there were no signs of meconium staining in the urine or the perineum. A divided pelvic colostomy with distal mucous fistula was performed. Echocardiogram and abdominal ultrasound were negative for possible associated cardiac and renal anomalies.

A male patient presented at birth with anorectal malformation ( Fig. 1A ); there were no signs of meconium staining in the urine or the perineum. A divided pelvic colostomy with distal mucous fistula was performed. Echocardiogram and abdominal ultrasound were negative for possible associated cardiac and renal anomalies. Fig. 1 ( A ) A 3-month-old boy with anorectal anomaly underwent colostomy at birth. ( B,C ) The contrast study “distal colostogram” performed in anteroposterior and lateral views showing blind rectal termination with no evidence of fistulous communication with the urinary tract (imperforate anus without fistula). Note: a radio-opaque mark has been placed indicating the site of anal dimple on the perineum ( arrow ). ( D ) Anteroposterior view of the plain X-ray showing the characteristic sacral defect (sacral scimitar). Note: the sacral notch cannot be detected in the lateral view of sacrum ( C ) and has been masked by the contrast in the anteroposterior view ( B ). At the age of 3 months, a high-pressure distal colostogram was performed as a preparatory step for a PSARP (posterior sagittal anorectoplasty) procedure. 8 The distal colostogram demonstrated a blind rectal termination with no evidence of fistulous communication with the urinary tract (imperforate anus without fistula; Fig. 1B, C ).

3 months, a high-pressure distal colostogram was performed as a preparatory step for a PSARP (posterior sagittal anorectoplasty) procedure. 8 The distal colostogram demonstrated a blind rectal termination with no evidence of fistulous communication with the urinary tract (imperforate anus without fistula; Fig. 1B, C ). At the time of operation, the mother revealed that her elder daughter (now 6 years old) underwent a similar operation. Revising the medical records of her daughter revealed a case of Currarino triad: anal stenosis (funnel anus), vertebral defect, and presacral cyst. Because of the reported autosomal dominant inheritance in cases of Currarino triad, 1 9 we suspected that this boy might have a similar pathology. Reexamination of his X-ray studies revealed a missed sacral notch in the plain X-ray ( Fig. 1D ) in addition to widening of the retrorectal space in lateral contrast film ( Fig. 1C ). The diagnosis of familial Currarino triad was quite evident in this boy (like his sister). A decision had to be made whether to postpone the operation and wait for a preoperative pelvic magnetic resonance imaging (MRI) study (for better delineation of the anatomy) or to proceed with the PSARP procedure and explore the presacral space during operation. Influenced by the busy operative schedule and the author's previous surgical experience with similar cases, 5 we decided to operate.

preoperative pelvic magnetic resonance imaging (MRI) study (for better delineation of the anatomy) or to proceed with the PSARP procedure and explore the presacral space during operation. Influenced by the busy operative schedule and the author's previous surgical experience with similar cases, 5 we decided to operate. With the patient in the prone position, a generous posterior sagittal incision was made for mobilization of the anorectum and exploration of the presacral space. We started as usual by dissection of the fascia around the posterior and the lateral aspects of the rectal pouch. A small midline incision was made in the posterior wall of the rectum to inspect for any missed communication with the urinary tract and to guide the dissection during separation of the anterior rectal wall from the intimately adherent urinary tract. After mobilization of the anorectum, a retrorectal cyst (∼2 cm in diameter) was found and excised. Then, the steps of anorectoplasty were completed as usual by reconstruction of the levator ani and striated muscle complex around the neorectum. Postoperative recovery was uneventful. Histopathological examination of excised specimen revealed fibromuscular tissue showing multiple clefts lined by stratified squamous epithelium and entrapped transitional epithelial islands. These histopathological findings go with the diagnosis of a developmental cyst associating with Currarino triad. 10 11 12

entful. Histopathological examination of excised specimen revealed fibromuscular tissue showing multiple clefts lined by stratified squamous epithelium and entrapped transitional epithelial islands. These histopathological findings go with the diagnosis of a developmental cyst associating with Currarino triad. 10 11 12 Later, a postoperative pelvic MRI was ordered that revealed residual presacral cystic lesion ( Fig. 2A, B ), with the absence of spinal cord anomalies. The patient was scheduled for reoperation to excise the residual presacral mass while still having a diverting colostomy. This time, the patient was positioned in the supine (lithotomy) position, which provided better exposure to the presacral space. Initially, the lesion was obscured by its deep location and surrounding fat; however, deeper dissection directed toward the bony defect (sacral notch) enabled successful identification and excision of the lesion ( Fig. 2C ). Although the preoperative MRI did not identify any communication between the lesion and the thecal sac, yet careful closure of the bed of the excised lesion was followed. Again, we had uneventful postoperative recovery and histopathological analysis of the excised specimen returned similar to the previous one. Six weeks later, the patient underwent colostomy closure.

y communication between the lesion and the thecal sac, yet careful closure of the bed of the excised lesion was followed. Again, we had uneventful postoperative recovery and histopathological analysis of the excised specimen returned similar to the previous one. Six weeks later, the patient underwent colostomy closure. Fig. 2 Follow-up magnetic resonance imaging (MRI) study performed after posterior sagittal anorectoplasty showing a residual presacral mass (*) in midsagittal T2-weighted image ( A ) and axial T2-weighted image ( B ). ( C ) Reoperation in the supine lithotomy position to remove the residual presacral mass (*); note: the incision is posterior to the neoanus ( white arrow ). The patient was seen at follow-up 3 months after the closure of colostomy. He defecates spontaneously once every 1 to 2 days. He is still only 19 months old and therefore below the age of voluntary bowel control. However, the mother was informed about the possibility of constipation, problems with fecal continence, and the need for long-term follow-up. The mother has had a similar experience with her elder daughter who is now 6 years old and has voluntary bowel and urinary control. The parents (being the first-degree relatives of our case) were advised to perform screening by plain X-ray sacrum. Discussion

The patient was seen at follow-up 3 months after the closure of colostomy. He defecates spontaneously once every 1 to 2 days. He is still only 19 months old and therefore below the age of voluntary bowel control. However, the mother was informed about the possibility of constipation, problems with fecal continence, and the need for long-term follow-up. The mother has had a similar experience with her elder daughter who is now 6 years old and has voluntary bowel and urinary control. The parents (being the first-degree relatives of our case) were advised to perform screening by plain X-ray sacrum. Discussion Currarino triad is a rare syndrome that may be occasionally encountered during managing cases of anorectal anomalies. The triad consists of anorectal anomaly, sacral bony defect, and a presacral mass. 1 It has a reported female predominance and genetic inheritance. Identification of the characteristic notched sacrum (sacral scimitar) in plain X-ray (anteroposterior view) is considered the key for the diagnosis; however, not infrequently, this radiological sign is overlooked, especially with a small sacral defect and a constipated child. 5 Increased awareness about this condition among neonatologists and pediatric surgeons can help in early picking of affected cases and better planning of surgical treatment.

for the diagnosis; however, not infrequently, this radiological sign is overlooked, especially with a small sacral defect and a constipated child. 5 Increased awareness about this condition among neonatologists and pediatric surgeons can help in early picking of affected cases and better planning of surgical treatment. Currarino triad is caused by heterozygous mutation in the MNX1 (previously HLXB9 ) gene on chromosome 7q36. The disorder is an autosomal dominant genetic trait that has variable expressivity. 9 Mutations in the coding sequence of the MNX1 gene have been identified in nearly all cases of familial Currarino's syndrome and in approximately 30% of patients with sporadic Currarino's syndrome. 13 Lynch et al. reported that 4% of patients with a mutation in the gene were asymptomatic and had normal sacral X-ray imaging. 14 Cytogenetic analysis of a previously reported family with Currarino triad showed that the mother of the two affected children carried a balanced translocation between chromosome 7q36 and 12q24. Both children were monosomic for 7q36, as they had inherited the deleted chromosome 7 from their mother. 15

X-ray imaging. 14 Cytogenetic analysis of a previously reported family with Currarino triad showed that the mother of the two affected children carried a balanced translocation between chromosome 7q36 and 12q24. Both children were monosomic for 7q36, as they had inherited the deleted chromosome 7 from their mother. 15 All affected families should be offered genetic counseling, as awareness of the hereditary nature of the disorder allows identification of asymptomatic heterozygotes and patients at risk, which leads to better planning of pregnancies and appropriate management of affected patients. All first-degree relatives of patients with Currarino triad should be screened by pelvic X - ray due to a highly variable phenotype within most families. 9 Relatives with an abnormal X - ray should be referred to a surgeon for further investigations, including pelvic MRI for an occult presacral mass due to significant inter- and intrafamilial variability in expression. 9 14

iad should be screened by pelvic X - ray due to a highly variable phenotype within most families. 9 Relatives with an abnormal X - ray should be referred to a surgeon for further investigations, including pelvic MRI for an occult presacral mass due to significant inter- and intrafamilial variability in expression. 9 14 The presacral mass in Currarino triad may be a lipoma, lipomyelomeningocele, or some sort of developmental cyst. 1 2 5 The latter has been frequently described in the literature as a mature cystic teratoma, which has been considered by Weinberg to be a misnomer in cases of Currarino triad. 12 There are several observations concerning the presacral cysts associating with Currarino triad that would support the diagnosis of a developmental cyst rather than a true neoplasm (teratoma). 11 It is usually recommended to excise presacral developmental cysts as these are liable for infection and abscess formation. 5 On the other hand, presacral lipomas and meningoceles can be managed conservatively unless symptomatic. 5 Large lesions may cause obstructive symptoms (constipation) and may distort the surgical field during the repair of anorectal anomaly in addition to other related obstetric considerations. 5

nd abscess formation. 5 On the other hand, presacral lipomas and meningoceles can be managed conservatively unless symptomatic. 5 Large lesions may cause obstructive symptoms (constipation) and may distort the surgical field during the repair of anorectal anomaly in addition to other related obstetric considerations. 5 Excision of the presacral cyst is usually performed concomitantly during anorectoplasty. The prone position is the standard approach for PSARP in males; 8 however, in females, the supine position can be used as an alternative (anterior sagittal anorectoplasty). 16 In this case report, excision of the presacral cyst took place in two steps: the first excision during the PSARP procedure in the prone position, and a second operation in the supine lithotomy position to remove a residual component of the lesion that was missed during the primary operation. It was clear that the supine lithotomy position provided better access to explore the presacral space than the prone position, especially with a deeply located cyst as in our case. Other lessons learned from managing this case were the importance of proper history taking in surgical practice, possible association of Currarino triad with different types of Anorectal anomalies (not necessarily the “funnel” anus or rectoperineal anomalies), and, lastly, the necessity of performing preoperative MRI to complete the diagnosis in cases of anorectal anomalies associated with sacral defects, which might have spared our case a second operation.

arino triad with different types of Anorectal anomalies (not necessarily the “funnel” anus or rectoperineal anomalies), and, lastly, the necessity of performing preoperative MRI to complete the diagnosis in cases of anorectal anomalies associated with sacral defects, which might have spared our case a second operation. There was some delay initially in completing the diagnosis for this case; however, the management was completed with a satisfactory outcome. Lastly, the prognosis for continence may be generally good for this case owing to the absence of associated spinal cord anomalies. 5 New Insights and the Importance for the Pediatric Surgeon Currarino triad is a rare syndrome that may be occasionally encountered during managing cases of anorectal anomalies. Increased awareness of this rare condition can help in achieving early detection, allowing for a more effective way of management. Conflict of Interest None.

Introduction Laparoscopic findings in case of impalpable undescended testes have been well described in the literature. When both the vas and vessels enter the internal inguinal ring, an exploration is generally indicated. However, in the absence of blind-ending vessels in the proximity of the internal inguinal ring, laparoscopic exploration must continue in a cranial direction to locate a possible high intra-abdominal testis. We present a unique case in which vas and vessels were entering the internal inguinal ring but the testes were found to be intra-abdominal. The diagnosis turned out to be a long bilateral testicular–epididymal dissociation (TED). Case Report A newborn, term baby had been referred to the local urology department for severe penoscrotal hypospadias and bilateral impalpable testes. The genetic and endocrine investigations confirmed a 46,XY karyotype as well as a normal response to Hcg (human chorionic gonadotropin) stimulation. Diagnostic laparoscopy at 1 year of age demonstrated the vas deferens and vessels entering the internal inguinal rings bilaterally. Groin exploration revealed what was considered to be atrophic testicular nubbins, which were left in situ at parental request. Hypospadias correction was performed separately. Throughout childhood, the endocrine status revealed normal hormonal levels ( Table 1 ).

Introduction Posterior urethral valves (PUVs) are the most common cause of bladder outlet obstruction in male newborns. Initial catheter drainage alleviates the urethral obstruction prior to undergoing valve ablation. Newborns with PUVs often have small hypertonic bladders with reduced capacity and increased detrusor contractility. 1 This hypercontractile bladder can inhibit upper tract drainage despite bladder catheterization. Anticholinergic agents are commonly used to treat decreased bladder compliance and detrusor hyperactivity, with oxybutynin being most commonly used for patients with neurogenic bladder. 1 2 3 Its effectiveness is attributed to a combination of anticholinergic, antispasmodic, local anesthetic, and calcium-channel-blocking activities. 2 3 4 Long-term experience supports its safety in newborns and infants. 5 6 In children with insufficient response or significant systemic adverse side effects (ASEs) to oral oxybutynin, who would otherwise require surgical treatment, intravesical instillation of oxybutynin has emerged as an alternative in managing bladder dysfunction. This method has been found to be highly effective, reliable, and well tolerated. 3 4 7 8 We report the first case, to our knowledge, of a newborn with PUVs and ureterovesical junction obstruction caused by detrusor hypertrophy who underwent urgent intravesical instillation of oxybutynin at high doses in an attempt to avoid a diversion procedure. Case Report

essels entering the internal inguinal rings bilaterally. Groin exploration revealed what was considered to be atrophic testicular nubbins, which were left in situ at parental request. Hypospadias correction was performed separately. Throughout childhood, the endocrine status revealed normal hormonal levels ( Table 1 ). Table 1 Hcg test responses and gonadotropins levels at 1.5 and 3 years of age Testosterone (nmol/L) LH (IU/L) FSH (IU/L) 1.5 y 0.2–6.8 0.1 (<0.7) 2.4 (0–0.7) 3 y 0.3–4.2 <0.5 (<0.7) 0.8 (0–0.7) Abbreviations: FSH, follicle-stimulating hormone; Hcg, human chorionic gonadotropin; LH, luteinizing hormone. Spontaneous puberty and inability to locate palpable gonads triggered further investigations. Endocrine profiling at this point showed normal testosterone levels sustained by high gonadotropins. The anti-Müllerian hormone (AMH) and Inhibin-B levels were at the lower end of the reference range, suggesting functioning testicular tissue ( Tables 2 and 3 ).

Anticholinergic agents are commonly used to treat decreased bladder compliance and detrusor hyperactivity, with oxybutynin being most commonly used for patients with neurogenic bladder. 1 2 3 Its effectiveness is attributed to a combination of anticholinergic, antispasmodic, local anesthetic, and calcium-channel-blocking activities. 2 3 4 Long-term experience supports its safety in newborns and infants. 5 6 In children with insufficient response or significant systemic adverse side effects (ASEs) to oral oxybutynin, who would otherwise require surgical treatment, intravesical instillation of oxybutynin has emerged as an alternative in managing bladder dysfunction. This method has been found to be highly effective, reliable, and well tolerated. 3 4 7 8 We report the first case, to our knowledge, of a newborn with PUVs and ureterovesical junction obstruction caused by detrusor hypertrophy who underwent urgent intravesical instillation of oxybutynin at high doses in an attempt to avoid a diversion procedure. Case Report The patient was a male newborn (birth weight: 2,270 g) with PUVs, bilateral ureterohydronephrosis, and significant bladder wall thickening. A Foley catheter placed at birth stopped producing urine at 12 hours of life. During the following 3 days, bilateral grade V ureterohydronephrosis and renal insufficiency with oligoanuria and severe electrolyte disturbances (hyponatremia, hyperkalemia, and a 3-mg/dL rise in creatinine) developed despite attempts to correct water and electrolyte imbalances by intravenous fluid therapy. On ultrasound, the bladder was empty, with the hypertrophied wall closely clasping the balloon of the Foley catheter ( Fig. 1 ). Prior to performing a necessary diversion procedure, we decided to try empirical treatment with intravesical instillation of oxybutynin (solution: 5 mg of oxybutynin hydrochloride and 20 mL of saline, instilling 4 mL every 4 hours = 2.6 mg/kg/day). With each instillation, the drug remained in the bladder for 15 minutes. Twelve hours following the initiation of treatment, urine output was 10 mL/kg/hour, with complete correction of electrolyte disorders within 3 days ( Fig. 2 ). No local or systemic ASEs were noted.

, instilling 4 mL every 4 hours = 2.6 mg/kg/day). With each instillation, the drug remained in the bladder for 15 minutes. Twelve hours following the initiation of treatment, urine output was 10 mL/kg/hour, with complete correction of electrolyte disorders within 3 days ( Fig. 2 ). No local or systemic ASEs were noted. Fig. 1 Ultrasound scan showing bilateral ureterohydronephrosis (A) and an empty bladder with the hypertrophied wall (asterisk) closely clasping the balloon of the vesical catheter (B). Fig. 2 Graph showing increased urine output following the initiation of intravesical treatment with oxybutynin (red arrow), with complete correction of electrolyte disorders following 3 days of therapy. On the 15th day of life, endoscopic valve ablation was performed and we transitioned to oral oxybutynin (0.3 mg/kg/day). Oxybutynin was administrated for 18 months, with urodynamic studies showing an increased bladder capacity with low maximum filling pressures. Discussion

Fig. 2 Graph showing increased urine output following the initiation of intravesical treatment with oxybutynin (red arrow), with complete correction of electrolyte disorders following 3 days of therapy. On the 15th day of life, endoscopic valve ablation was performed and we transitioned to oral oxybutynin (0.3 mg/kg/day). Oxybutynin was administrated for 18 months, with urodynamic studies showing an increased bladder capacity with low maximum filling pressures. Discussion In 1989, Brendler et al first reported the use of intravesical oxybutynin (IVO) for the treatment of neurogenic bladder. 9 Since then, multiple reports of the efficacy of IVO have been published. 3 4 8 10 11 Treatment with oral oxybutynin has a high incidence of systemic anticholinergic ASEs. In some cases, adequate suppression of the detrusor can only be achieved when elevating the dosage, which will also increase the side effects. 2 IVO reduces first-pass metabolism in the liver, resulting in decreased generation of the N-desethyl metabolite, which is associated with the anticholinergic effects. 2 3 12 In addition, a pharmacokinetic study by Di Stasi et al reported a direct local effect of IVO within the bladder wall, probably through urothelial accumulation, suppression of muscarinic receptor-mediated detrusor muscle contractions, and blocking of muscarinic receptors in bladder-afferent pathways. 13 These pharmacological properties allowed us to delivered high doses of the drug to the target tissue with maximum pharmacological response and no ASEs.

urothelial accumulation, suppression of muscarinic receptor-mediated detrusor muscle contractions, and blocking of muscarinic receptors in bladder-afferent pathways. 13 These pharmacological properties allowed us to delivered high doses of the drug to the target tissue with maximum pharmacological response and no ASEs. Although the optimum dose for IVO has not been determined, a systematic review by Guerra et al states that most published studies used a dose of 0.2 mg/kg/day (average 10 mg daily). 3 Given the better tolerability and more rapid onset of action of IVO compared with oral treatment noticed in our experience with neurogenic bladder, we decided to further increase the dose in our patient. It seems that despite early relief of the bladder outlet obstruction, pathological changes in the detrusor from in utero obstruction resulted in an ureterovesical junction entrapment caused by an overactive hypertrophied detrusor. This was sufficiently intense to induce renal failure. Therefore, we needed a strong and fast detrusor-suppressing effect. As myogenic failure is a legitimate concern when initiating any anticholinergic therapy, urodynamic monitoring is mandatory during treatment. However, drug-induced myogenic failure is reversible upon stopping the medication. 1 5 In addition, although our patient did not develop any ASEs with intravesical therapy, adverse effects are still possible, likely secondary to drug absorption through the bladder mucosa. 3

mic monitoring is mandatory during treatment. However, drug-induced myogenic failure is reversible upon stopping the medication. 1 5 In addition, although our patient did not develop any ASEs with intravesical therapy, adverse effects are still possible, likely secondary to drug absorption through the bladder mucosa. 3 Conclusion This case suggests that an overactive hypertrophied detrusor contributed to the ureterovesical junction obstruction. It was reasonable to attempt to use an anticholinergic to adequately suppress detrusor activity. It was only the intravesical therapy that allowed high doses of oxybutynin to be administered with no side effects. This type of treatment should be considered as an alternative to an immediate diversion procedure in patients with PUVs. Nevertheless, the perceived positive effect of this treatment modality requires validation in controlled trials. New Insights & the Importance for the Pediatric Surgeon First reported use of intravesical oxybutynin as a measure to avoid a diversion procedure in a newborn with posterior urethral valves and ureterovesical junction obstruction caused by detrusor hypertrophy. Conflict of Interest None.

Introduction Lipoblastomas are rare benign mesenchymal tumors that arise from embryonal fat cells. They are usually discovered in infants and children under 3 years of age, and mostly occur in the trunk (from 10 to 60%) and extremities (from 40 to 45%), while head and neck localizations are rare, with only five cases described to date. 1 They are classified as benign adipocytic tumors, along with lipomas, lipomatosis, lipoblastomatosis, angiolipomas, and myolipomas. Intermediate (locally aggressive) adipocytic tumors are atypical lipomatous tumors and well-differentiated liposarcoma, while malignant tumors are dedifferentiated liposarcoma, myxoid liposarcoma, pleomorphic liposarcoma, and mixed-type liposarcoma. So far, 85 cases of lipoblastoma have been described in childhood, of which 12 presented with an abdominal mass. 1 The treatment of choice is complete surgical excision. Only five cases of liposarcoma have been reported in childhood, of which two had arisen from the porta hepatis. In this report, we describe three cases of lipoblastoma in infants below 4 years of age. Case Presentation Case 1

So far, 85 cases of lipoblastoma have been described in childhood, of which 12 presented with an abdominal mass. 1 The treatment of choice is complete surgical excision. Only five cases of liposarcoma have been reported in childhood, of which two had arisen from the porta hepatis. In this report, we describe three cases of lipoblastoma in infants below 4 years of age. Case Presentation Case 1 A 4-year-old female was hospitalized for a suspected ovarian mass. The child complained of recurrent abdominal pain and dysuria for 1 month; an abdominal ultrasound and magnetic resonance imaging (MRI) showed a 6 cm right-sided ovarian mass that was suspected to be a teratoma ( Fig. 1A–C ). The girl underwent laparoscopy with 5 mm instruments. However, no ovarian mass was found, but an 8-cm exophytic lesion was discovered, arising from the retroperitoneum, on the right side of the pelvis, adjacent to the bladder with multiple adhesions with the ascending colon and the uterus ( Fig. 1D ). The tumor was completely resected by laparoscopy with no residual lesions, and the postoperative course was uneventful. Fig. 1 Abdominal ultrasound showing a 6 cm right-sided ovarian mass that was consistent with teratoma ( A ). To better characterize the mass, an abdominal magnetic resonance imaging was performed and the initial diagnosis was confirmed ( B, C ). Laparoscopic view of the mass ( D ). Pathological examination revealed a solid mass composed of small lobules of mature and immature fat cells separated by fibrous septa, and containing small and dilated blood vessels (lipoblastoma) ( Fig. 2A, B ).

Fig. 1 Abdominal ultrasound showing a 6 cm right-sided ovarian mass that was consistent with teratoma ( A ). To better characterize the mass, an abdominal magnetic resonance imaging was performed and the initial diagnosis was confirmed ( B, C ). Laparoscopic view of the mass ( D ). Pathological examination revealed a solid mass composed of small lobules of mature and immature fat cells separated by fibrous septa, and containing small and dilated blood vessels (lipoblastoma) ( Fig. 2A, B ). Fig. 2 Lipoblastoma histology: multilobulated soft tissue lesion comprising groups of peripherally lipoblast that are separated by fibrous septa and a distinct peripheral pseudocapsule was typical of all cases. Hemosiderin staining and chronic inflammation with extensive liponecrosis were present in patient 1( A , B ) and signs of vascular congestion were present in patient 2 ( C , D ). Hemosiderin staining and chronic inflammation were particularly represented in patient 3 ( E , F ). Case 2 A 2-year-old otherwise healthy female presented with acute abdominal pain and reddish jelly-like stool.

Fig. 2 Lipoblastoma histology: multilobulated soft tissue lesion comprising groups of peripherally lipoblast that are separated by fibrous septa and a distinct peripheral pseudocapsule was typical of all cases. Hemosiderin staining and chronic inflammation with extensive liponecrosis were present in patient 1( A , B ) and signs of vascular congestion were present in patient 2 ( C , D ). Hemosiderin staining and chronic inflammation were particularly represented in patient 3 ( E , F ). Case 2 A 2-year-old otherwise healthy female presented with acute abdominal pain and reddish jelly-like stool. On abdominal ultrasound, an ileocecal intussusception was confirmed ( Fig. 3A, B ). Water-soluble contrast enema was performed but was ineffective ( Fig. 3C ); hence, the patient underwent surgical exploration via transverse right-sided supraumbilical laparotomy ( Fig. 3D ). An ileo-appendico-ceco-colic intussusception was found, along with a 4 cm mass as a lead point, located at 1 cm to the ileocecal valve. The mass was firm, round, yellow, similar to a lipoma, with adhesions to the ilocecal angle. Enlargement of mesenteric lymph nodes was also found. Due to the impossibility of manually reducing the intussusception, we opted for an en bloc ileocecal resection including the above-mentioned mass. Finally, lymph node sampling and an end-to-end ileocolic anastomosis were performed. Fig. 3 Abdominal ultrasound confirming the intussusception ( A, B ). Water-soluble contrast enema was performed but the result was ineffective ( C ); hence, the patient underwent a surgical exploration ( D ).

On abdominal ultrasound, an ileocecal intussusception was confirmed ( Fig. 3A, B ). Water-soluble contrast enema was performed but was ineffective ( Fig. 3C ); hence, the patient underwent surgical exploration via transverse right-sided supraumbilical laparotomy ( Fig. 3D ). An ileo-appendico-ceco-colic intussusception was found, along with a 4 cm mass as a lead point, located at 1 cm to the ileocecal valve. The mass was firm, round, yellow, similar to a lipoma, with adhesions to the ilocecal angle. Enlargement of mesenteric lymph nodes was also found. Due to the impossibility of manually reducing the intussusception, we opted for an en bloc ileocecal resection including the above-mentioned mass. Finally, lymph node sampling and an end-to-end ileocolic anastomosis were performed. Fig. 3 Abdominal ultrasound confirming the intussusception ( A, B ). Water-soluble contrast enema was performed but the result was ineffective ( C ); hence, the patient underwent a surgical exploration ( D ). The histology report described a lipoblastoma with signs of vascular congestion and infiltration of the bowel wall ( Fig. 2C, D ); the removed lymph node was found to be normal. Case 3

Fig. 3 Abdominal ultrasound confirming the intussusception ( A, B ). Water-soluble contrast enema was performed but the result was ineffective ( C ); hence, the patient underwent a surgical exploration ( D ). The histology report described a lipoblastoma with signs of vascular congestion and infiltration of the bowel wall ( Fig. 2C, D ); the removed lymph node was found to be normal. Case 3 A 2-year-old female presented with a gluteal mass with slow and continuous growth. MRI showed a multilobulated epifascial lesion measuring 8 × 6 cm, in the context of the gluteal muscle extending to the pelvis ( Fig. 4A–C ). The child underwent complete surgical excision of the tumor including the overlying skin. Intraoperatively, an encapsulated multilobulated subcutaneous fatty tumor was discovered ( Fig. 4D ). Histology revealed a lipoblastoma with chronic inflammation ( Fig. 2E, F ). Fig. 4 Magnetic resonance imaging showed a multilobulated epifascial lesion measuring 8 × 6 cm in the context of the gluteal muscle that deepened in the pelvis ( A – C ). Complete surgical excision was achieved via a posterior–sagittal approach ( D ). In all cases, immunohistochemistry showed primitive mesenchymal cells reactive for Desmin, adipocytes, and vascular endothelial cells positive for CD34, and adipocytes also positive for S100 protein. A follow-up at 12 (Case 3), 14 (Case 2), and 16 (Case 1) months showed all three children doing well. There is no evidence of recurrence, either clinically or on ultrasound. Discussion

In all cases, immunohistochemistry showed primitive mesenchymal cells reactive for Desmin, adipocytes, and vascular endothelial cells positive for CD34, and adipocytes also positive for S100 protein. A follow-up at 12 (Case 3), 14 (Case 2), and 16 (Case 1) months showed all three children doing well. There is no evidence of recurrence, either clinically or on ultrasound. Discussion Lipoblastomas are soft tissue tumors composed of embryonal/fetal fat and characterized by a benign nature, early presentation, male predominance, and rapid growth. 2 It mostly occurs in infants and children under 3 years of age, and the most common locations are extremities and trunk, followed by head and neck. 3 The age at presentation ranges from 1 month to 14 years (median: 2 years). Sometimes lipoblastoma arises as an abdominal mass from the retroperitoneum, as in our first case, involving the mesentery or the omentum, or acutely as a lead point of an intussusception, as in our second case. In 1926, Jeff et al 1 first described lipoblastoma as an atypical lipoma that consists of cells resembling embryonic fat (lipoblasts). Two variants are described: lipoblastoma (a well circumcised and capsulated tumor) and lipoblastomatosis (a noncapsulated multicentric and infiltrative tumor). Both are rare and benign mesenchymal tumors . Lipoblastomatosis has the same demographic characteristics as lipoblastoma but is more common in the head-and-neck area. Myxoid liposarcoma is the most common liposarcoma subtype, usually occurring in extremities (thigh), mostly in adolescent males, at a median age of 16 years.

Two variants are described: lipoblastoma (a well circumcised and capsulated tumor) and lipoblastomatosis (a noncapsulated multicentric and infiltrative tumor). Both are rare and benign mesenchymal tumors . Lipoblastomatosis has the same demographic characteristics as lipoblastoma but is more common in the head-and-neck area. Myxoid liposarcoma is the most common liposarcoma subtype, usually occurring in extremities (thigh), mostly in adolescent males, at a median age of 16 years. It has an excellent prognosis after surgery, differing from the pleomorphic subtype, more aggressive and with poor prognosis. Its cytological findings are adipocytes, lipoblasts, and spindle cells that show moderate cytological atypia immersed in a vascularized myxoid stroma. 5 Of the five cases of liposarcoma reported in childhood, three children died, one of them due to a recurrence 12 years after an initial response to surgical excision combined with irradiation and chemotherapy. 2 In all studies reported in the literature, MRI is the diagnostic tool of choice for the evaluation of tumor extension and to distinguish lipoblastoma from normal subcutaneous fat. Lipoblastomas appear in fact as lobulated lesions characterized by high signal intensities. Tumor size may vary from few centimeters to 20 to 25 cm. Treatment of choice is complete surgical resection with preservation of vital organs.

In all studies reported in the literature, MRI is the diagnostic tool of choice for the evaluation of tumor extension and to distinguish lipoblastoma from normal subcutaneous fat. Lipoblastomas appear in fact as lobulated lesions characterized by high signal intensities. Tumor size may vary from few centimeters to 20 to 25 cm. Treatment of choice is complete surgical resection with preservation of vital organs. All tumors described so far, presented with lobulated appearance, were soft in consistency and had a yellowish surface. The histology consists of multilobulated soft tissue lesion comprising groups of peripherally arranged immature lipoblast that is separated by thin fibrous septa and a distinct peripheral pseudocapsule. Diagnosis is typically made after pathological examination of the operative specimen, as in our cases. Immunohistochemistry (CD34, S100 protein, and Desmin) is usually performed in all studies. Primitive mesenchymal cells are reactive for Desmin, adipocytes and vascular endothelial cells are positive for CD34, and adipocytes are also positive for S100 protein. The recent use of fine-needle aspiration cytology has proven to be helpful for the diagnosis of lipoblastomas, as mutations involving chromosome 8 (8q11–13 rearrangement involving the PLAG1 gene) have been found in 70% of patients. 5 This examination could be helpful in primary or recurrent tumors with diagnostic dilemmas.

The recent use of fine-needle aspiration cytology has proven to be helpful for the diagnosis of lipoblastomas, as mutations involving chromosome 8 (8q11–13 rearrangement involving the PLAG1 gene) have been found in 70% of patients. 5 This examination could be helpful in primary or recurrent tumors with diagnostic dilemmas. The long-term prognosis for lipoblastoma is good; metastases have never been reported and the potential recurrence (ranging from 9 to 22%, depending on the study) is due to incomplete initial excision of the tumor. 4 For example, in the study of Abdul-Ghafar, 6 a 14-year-old girl with a mediastinal lipoblastoma with infiltrative margins developed four separate recurrences and underwent repeated resections. In lipoblastomatosis the resection is more demanding, due to the multicentric type of lesions and irregular margins, and a higher rate of recurrence is noted. 6 Furukawa et al described a successful laparoscopic removal of a giant omental lipoblastoma in a 4-year-old girl. They concluded that for large abdominal tumors in children also, the laparoscopic approach could be recommended as the first procedure when the tumor is preoperatively considered to be benign and resectable. 7 Reported surgical complications associated with tumor resection were chronic bilateral venostasis after excision, internal iliac artery, and vein reconstruction at resection, and one patient that developed septic shock died; all tumors were retroperitoneal lipoblastomas. 4 All authors agree that a follow-up of at least 5 years is essential.

Reported surgical complications associated with tumor resection were chronic bilateral venostasis after excision, internal iliac artery, and vein reconstruction at resection, and one patient that developed septic shock died; all tumors were retroperitoneal lipoblastomas. 4 All authors agree that a follow-up of at least 5 years is essential. Most of the studies did not report any recurrence during the follow-up period. Conclusions Lipoblastoma is an uncommon benign soft tissue tumor, sometimes arising from the retroperitoneum. Lipoblastoma usually manifests with local swelling but may also be an incidental diagnosis after surgical resection of a mass or intussusception. Prognosis is generally good and local recurrence is rare, with no risk of metastatic dissemination. However, prolonged ultrasonographic follow-up is recommended. Radical surgical excision is the current standard of care. Surgery should be accurate and complete to avoid potential recurrence. New Insights and the Importance for the Pediatric Surgeon Case gallery, tretament option, and literature review of a very rare lesion in children. Conflict of Interest None declared.

Introduction Fibrous hamartoma of infancy (FHI) is a solid benign tumor of the subcutis, which usually occurs within the first 2 years of life. It was first described by Reye in 1956, as the dermal fibromatous tumor of infancy, and later, within a larger study, Enzinger in 1965 renamed it into fibrous hamartoma of infancy. It accounts for 0.02% of all benign soft tissue tumors. 1 As a congenital entity, it occurs in 15 to 20% of all cases. 2 3 It predominantly occurs in males in the ratio of 2:1. 1 3 4 Clinically, it is presented as a solid, painless, well-limited subcutaneous formation, tending to grow. 5 In most cases, it has no symptoms, so patients report to a doctor only when parents notice a palpable formation. It occurs in various locations in the body, most frequently in the upper arm and the axillary region 2 5 6 ( Fig. 1 ). We report on a child with an FHI of the scrotum. Fig. 1 Fibrous hamartoma appears in various places. The scheme presents the most frequent locations. Case Report

Fibrous hamartoma of infancy (FHI) is a solid benign tumor of the subcutis, which usually occurs within the first 2 years of life. It was first described by Reye in 1956, as the dermal fibromatous tumor of infancy, and later, within a larger study, Enzinger in 1965 renamed it into fibrous hamartoma of infancy. It accounts for 0.02% of all benign soft tissue tumors. 1 As a congenital entity, it occurs in 15 to 20% of all cases. 2 3 It predominantly occurs in males in the ratio of 2:1. 1 3 4 Clinically, it is presented as a solid, painless, well-limited subcutaneous formation, tending to grow. 5 In most cases, it has no symptoms, so patients report to a doctor only when parents notice a palpable formation. It occurs in various locations in the body, most frequently in the upper arm and the axillary region 2 5 6 ( Fig. 1 ). We report on a child with an FHI of the scrotum. Fig. 1 Fibrous hamartoma appears in various places. The scheme presents the most frequent locations. Case Report The first and healthy term child was born to a mother with an unremarkable family history. The mother noticed a formation in the right hemiscrotum when the child was 8 months old. She was not sure if formation changed its size according to daily activities of the child. On examination, a solid, movable, painless formation was verified, 1 cm in diameter, located in the upper half of the right hemiscrotum. Both testicles were descended in the scrotum. The right testicle did not seem connected to the formation. No clear signs of inguinal hernia or testicular hydrocele were found. Regional lymph nodes were not increased. The skin was intact. The child seemed clinically unaffected and the working diagnosis was hydrocele funiculi. Specific laboratory and radiological tests such as ultrasonography, computed tomography (CT) scan, magnetic resonance imaging (MRI), and tumor markers were not done. According to the guidelines of our hospital, the follow-up was after 3 months. However, during a follow-up examination 3 months later, progression was verified. A solid formation was palpated, still located in the upper half of the right hemiscrotum, painless, without symptoms, not connected to the testicle, now ∼2 × 2.5 cm in diameter ( Fig. 2 ). After 3 months, mother was still unsure if the formation changed its size. Working diagnosis was still hydrocele funiculi. Because of the growing mass, the location of the tumor, age of the child, and concern of the parents, the indication for surgery was made. We used scrotal approach. Horizontal incision was made in upper half of right hemiscrotum. During surgery, a well-limited solid formation was found on the external inguinal ring, 3 × 2 cm in diameter, not infiltrating the surrounding structures ( Fig. 3 ). Considering the location of the formation, we decided to use an additional inguinal approach to examine the communication between the formation and the structures of the inguinal canal. The formation was not connected to the elements of the inguinal canal, the spermatic cord, and the testicle.

ng structures ( Fig. 3 ). Considering the location of the formation, we decided to use an additional inguinal approach to examine the communication between the formation and the structures of the inguinal canal. The formation was not connected to the elements of the inguinal canal, the spermatic cord, and the testicle. Malignant formation was not verified, enlarged regional lymph nodes were also not verified, and for that reason, open biopsy or urgent histopathological analysis was not done. The tumor was completely removed preserving the surrounding structures ( Fig. 4 ). Histologically characteristic components included well-differentiated fibrous connective tissue, primitive mesenchymal stroma, and islands of mature fat cells. Immunohistochemical analyses (smooth muscle actin, h-caldesmon, desmin, S-100, epithelial membrane antigen, b-catherin, K i -67, Bcl-2, CD 34) confirmed the diagnosis of an FHI ( Figs. 5 and 6 ). Macroscopic resection margins were tumor free, which was later also confirmed by histopathological analysis. Fig. 2 Tumor in the upper half of the right hemiscrotum marked by an arrow. Fig. 3 Solid tumor of 3 × 2 cm in size marked by an arrow. Fig. 4 Extirpated tumor of 3 × 2 cm in size. Fig. 5 Electron microscope increased ×20 hematoxylin and eosin stain; characteristic components—fibrous connective tissue ( A ), islands of mature fat cells ( B ), and mesenchymal stroma ( C ). Fig. 6 Immunohistochemistry increased ×20; CD34 positive cells inside hyaline stroma. Discussion

Fig. 4 Extirpated tumor of 3 × 2 cm in size. Fig. 5 Electron microscope increased ×20 hematoxylin and eosin stain; characteristic components—fibrous connective tissue ( A ), islands of mature fat cells ( B ), and mesenchymal stroma ( C ). Fig. 6 Immunohistochemistry increased ×20; CD34 positive cells inside hyaline stroma. Discussion This case demonstrates a rare tumor of the scrotal region which, due to its nonspecific clinical picture, presents a diagnostic and therapeutic challenge in clinical practice. Considering the nonspecific clinical picture, the consistency of the formation, the area it affects, and its tendency to grow, in terms of differential diagnosis, several conditions can be taken into account 7 8 9 10 ( Table 1 ). Out of all diseases, malignant ones deserve special attention. For this reason, according to the literature, every solid formation in the scrotal region which tends to grow is considered as malignant until the opposite is proven. 8 It has been shown that 75% of tumors of the scrotum, including malignant tumors, occur before the second year of life of a child, which correlates with period of occurrence of FHI. 7 Our patient had unspecific clinical presentation and medical history. Guided by this information, during preoperative follow-up period, working diagnosis was hydrocele funiculi. As such, it became indication for operation. In retrospective, after finished treatment, we should have done more extensive tests which would have given us wider perspective, especially on potential malignity. FHI is usually diagnosed up to second year of child's life and in that period, the most frequent tumors of scrotal region are: yolk sac tumors, teratomas, epidermoid cysts, choriocarcinomas, and sarcomas. 9 10 11 12 Ultrasonography is the diagnostic method for scrotal formations. 11 Depending on the ultrasonography findings, it is advisable to extend tests to X-ray, CT scan, and MRI for staging and exclusion of metastasis. 9 11 In laboratory tests, serum for tumor markers, alpha-fetoprotein (AFP), and beta human chorionic gonadotropin are examined. 9 10 11 12 It should be taken into account that AFP is physiologically elevated from 6 to 8 months after birth. 11 12 Since clinically and radiologically, it is not possible to establish the diagnosis of FHI and exclude the possibility of a malignant formation which would require a different approach and treatment, the method of choice is total removal of the formation and definite histopathological analysis. 3 5

hs after birth. 11 12 Since clinically and radiologically, it is not possible to establish the diagnosis of FHI and exclude the possibility of a malignant formation which would require a different approach and treatment, the method of choice is total removal of the formation and definite histopathological analysis. 3 5 Table 1 Differential diagnosis of FHI Neurofibromatosis Fibrolipoma Angiolipoma Juvenile hyaline fibromatosis Hemangioma Lymphangioma Dermoid cyst Germ cell tumors Teratoma Seminoma Endodermal sinus tumor Gonadal stromal tumors Leydig cell Sertoli cell Juvenile Granulosa cell Gonadoblastoma Rhabdomyosarcoma Leiomyosarcoma

Table 1 Differential diagnosis of FHI Neurofibromatosis Fibrolipoma Angiolipoma Juvenile hyaline fibromatosis Hemangioma Lymphangioma Dermoid cyst Germ cell tumors Teratoma Seminoma Endodermal sinus tumor Gonadal stromal tumors Leydig cell Sertoli cell Juvenile Granulosa cell Gonadoblastoma Rhabdomyosarcoma Leiomyosarcoma In most cases, the microscopic finding has three characteristic components: well-differentiated fibrous connective tissue, primitive mesenchymal stroma, and islands of mature fat cells. 2 According to some authors, it is exactly the primitive mesenchymal cells that can be misinterpreted as rhabdomyosarcoma, infantile fibrosarcoma, or infantile myofibromatosis. 2 9 Therefore, establishing the diagnosis is a challenge for a histopathologist as well. It is pointed out in the literature that there is no actual proof of remission of FHI, its metaplasia or malignant dysplasia. 5 In case of incomplete removal, there is a possibility of a relapse of up to 15% with median rate of occurrence in the fifth month from the operation. 1 2 3 5 6 13 In our case, complete removal was done with tumor-free margins. Follow-up was in 6th and 12th months from the procedure using ultrasonography. One year after the procedure, a control ultrasonography examination showed no relapse. Considering that clinical findings and radiology tests were normal, it was concluded that the treatment was finished.

al was done with tumor-free margins. Follow-up was in 6th and 12th months from the procedure using ultrasonography. One year after the procedure, a control ultrasonography examination showed no relapse. Considering that clinical findings and radiology tests were normal, it was concluded that the treatment was finished. Conclusion FHI is a benign and rare formation in the subcutis with a good prognosis. Considering the nonspecific clinical picture, this formation deserves wider diagnostic tests to exclude malignant disease. The method of treatment is complete excision. The final diagnosis is confirmed by histopathological analysis. New Insights and the Importance for the Pediatric Surgeon This case report is significant for the pediatric surgeon because it presents a rare benign tumor which is presented by a nonspecific clinical picture and diagnostic findings according to which it is not possible to exclude the possibility of a malignant formation. For that reason, we believe it is important to present this work, to demonstrate the treatment approach and the outcome, which is significantly different from those related to a malignant formation. Conflict of Interest None.

Introduction Esophageal lung is a rare bronchopulmonary foregut congenital malformation (BPFM). It is characterized by a communication between a lung segment, lung tissue, and/or the main bronchus with the esophagus. 1 The clinical findings vary according to the severity of the malformation; the patients may present with a chronic cough, feeding difficulty, repeated pulmonary infections, and nonspecific dyspnea, as described by Sugandhi et al and Katz et al, 2 3 some patients might even develop severe respiratory distress leading to death. In most cases worldwide, the diagnosis is suspected by the presence of hemoptysis, dysphagia, feeding-related respiratory distress, and recurrent respiratory infections. 4 Currently, there are no guidelines established for treatment. The management is determined by the aberrant anatomy presentation, knowledge, and expertise of the attending physicians. Based on literature findings, the surgical objective is aimed at separating the existing communication between the esophagus and the bronchial structure in question. We report a case of a preterm newborn male with a diagnosis of esophageal lung type III by Srikanth et al classification, and describe the initial experience in the management of this pathology with thoracotomy. Case Report

Currently, there are no guidelines established for treatment. The management is determined by the aberrant anatomy presentation, knowledge, and expertise of the attending physicians. Based on literature findings, the surgical objective is aimed at separating the existing communication between the esophagus and the bronchial structure in question. We report a case of a preterm newborn male with a diagnosis of esophageal lung type III by Srikanth et al classification, and describe the initial experience in the management of this pathology with thoracotomy. Case Report The child was a preterm born at 31 weeks of gestational age, via emergency cesarean section due to polyhydramnios. He was a twin product of a dichorionic diamniotic pregnancy, APGAR scores were 8 at 1 minute and 10 at 5 minutes, birth weight was 1,635 g, and height 46 cm. Referred from Venezuela to our institution in Cúcuta, Colombia, the child was admitted to the pediatric intensive care unit because of high risk for septicemia and the presence of respiratory distress. The patient was intubated, and placed on mechanical ventilation due to his clinical condition. Several blood test and imaging studies were performed during hospitalization: chest X-ray showed an opacity with air bronchogram, as well as a markedly hyperinsufflated lung in the lower two-thirds of the right lung ( Fig. 1 ). Antibiotic treatment was initiated due to clinical suspicion of in utero pneumonia. Fig. 1 Full body X-rays showing opacities in the right hemithorax and air bronchogram.

o locate palpable gonads triggered further investigations. Endocrine profiling at this point showed normal testosterone levels sustained by high gonadotropins. The anti-Müllerian hormone (AMH) and Inhibin-B levels were at the lower end of the reference range, suggesting functioning testicular tissue ( Tables 2 and 3 ). Table 2 GnRH test at age 9.5 years and basal gonadotropins throughout puberty (9.5–17.5 years) LH (IU/L) FSH (IU/L) Testosterone (nmol/L) 9.5 y GnRH at 20'-60' <0.2 6.9–4.7 3.1 9.1–13 < 0.69 10 y (normal ranges) 0.8 (0.3–1.4) 5.8 (0.5–6.4) < 0.69 11.7 y (normal ranges) 7.9 (0.3–1.4) 21.2 (0.5–6.4) 7.97 13.2 y (normal ranges) 10.2 (0.4–0.6) 24.4 (0.7–6.9) 15.6 15.7 y (normal ranges) 25 (1.7–8.6) 35.9 (1.5–12.4) 16.8 (7.6–31.4) 17.5 y (normal ranges) 30.8 (1.7–8.6) 46.9 (1.5–12.4) 14.3 (7.6–31.4) Abbreviations: FSH, follicle-stimulating hormone; GnRH, gonadotropin-releasing hormone; Hcg, human chorionic gonadotropin; LH, luteinizing hormone. Table 3 Three-week Hcg test at age 9.5 years Time 0 3 wk DHEA-S (μmol/L) (0.4–2) 0.866 1.66 Androstenedione (nmol/L) <1.05 3.5 Testosterone (nmol/L) <0.69 15.5 DHT (nmol/L) <0.1 0.27 Inhibin B (pg/mL) 74.2 (50–310) AMH (pmol/L) 116.2 (134–184) Abbreviations: AMH, anti-Müllerian hormone; DHEA-S, dehydroepiandrosterone sulfate; DHT, dihydrotestosterone; Hcg, human chorionic gonadotropin.

The child was a preterm born at 31 weeks of gestational age, via emergency cesarean section due to polyhydramnios. He was a twin product of a dichorionic diamniotic pregnancy, APGAR scores were 8 at 1 minute and 10 at 5 minutes, birth weight was 1,635 g, and height 46 cm. Referred from Venezuela to our institution in Cúcuta, Colombia, the child was admitted to the pediatric intensive care unit because of high risk for septicemia and the presence of respiratory distress. The patient was intubated, and placed on mechanical ventilation due to his clinical condition. Several blood test and imaging studies were performed during hospitalization: chest X-ray showed an opacity with air bronchogram, as well as a markedly hyperinsufflated lung in the lower two-thirds of the right lung ( Fig. 1 ). Antibiotic treatment was initiated due to clinical suspicion of in utero pneumonia. Fig. 1 Full body X-rays showing opacities in the right hemithorax and air bronchogram. Upon arrival to our institution, a multidisciplinary group, that included members of pediatric surgery, neonatology, cardiology, nutrition(ist), social work, and psychology departments, was created for the assessment and management of the patient and support to his family. He was in respiratory distress requiring supplemental oxygen and nutritionally compromised in the need of nutritional support. During hospitalization, the patient developed oral intake intolerance, the multidisciplinary group recommended an upper gastrointestinal (GI) X-ray to rule out possibility of a tracheoesophageal fistula (TEF) ( Fig. 2 ). A partial obstruction of the distal esophagus with contrast filling of the right bronchial tree and connection to the site of the obstruction was observed.

e intolerance, the multidisciplinary group recommended an upper gastrointestinal (GI) X-ray to rule out possibility of a tracheoesophageal fistula (TEF) ( Fig. 2 ). A partial obstruction of the distal esophagus with contrast filling of the right bronchial tree and connection to the site of the obstruction was observed. Fig. 2 Upper digestive tract X-rays: Partial obstruction of the distal esophagus with right bronchial tree image stemming from the site of the obstruction. On the ninth day of life in the multidisciplinary assessment and suspicion of BPFM, it was decided to perform a computed tomographic (CT) scan which showed a solidification of right basal lung tissue. An echocardiogram showed no vascular anomalies. Because of these findings, a right thoracotomy and right inferior lobectomy were performed, resecting an anomalous pulmonary tissue from its stemming point on the esophagus as well as an esophageal mass ( Fig. 3 ), resulting in resolution of the obstructive esophageal stenosis, and regaining of the oral intake tolerance. Fig. 3 Surgical findings of bronchopulmonary esophageal fistula indicated with white asterix. The histopathology showed a hypoplastic lung with bronchus lined by respiratory epithelium and a bronchogenic cyst, lymphocytic infiltrate, presence of lymphoid follicles, and well-formed germinal centers.

On the ninth day of life in the multidisciplinary assessment and suspicion of BPFM, it was decided to perform a computed tomographic (CT) scan which showed a solidification of right basal lung tissue. An echocardiogram showed no vascular anomalies. Because of these findings, a right thoracotomy and right inferior lobectomy were performed, resecting an anomalous pulmonary tissue from its stemming point on the esophagus as well as an esophageal mass ( Fig. 3 ), resulting in resolution of the obstructive esophageal stenosis, and regaining of the oral intake tolerance. Fig. 3 Surgical findings of bronchopulmonary esophageal fistula indicated with white asterix. The histopathology showed a hypoplastic lung with bronchus lined by respiratory epithelium and a bronchogenic cyst, lymphocytic infiltrate, presence of lymphoid follicles, and well-formed germinal centers. VACTERL sequence was ruled out. Postoperative course on day 1 was uneventful, and enteral nutrition via nasogastric tube was initiated on postoperative day 2. On postoperative day 5, an upper GI contrast study confirmed no leak from the repaired esophagus; the mid-esophageal dilatation had also subsided significantly with normal dye passage distally into the stomach, then oral intake was initiated. The patient had difficulty tolerating oral intake, and required to be hospitalized for 1 month to improve his nutritional status. The child was then discharged on oral feeds. The patient presented recurrent pulmonary infection after the surgery up to the age of 10 months. The patient was discharged after adequate postoperative care, and was asymptomatic during 1-year follow-up.

red to be hospitalized for 1 month to improve his nutritional status. The child was then discharged on oral feeds. The patient presented recurrent pulmonary infection after the surgery up to the age of 10 months. The patient was discharged after adequate postoperative care, and was asymptomatic during 1-year follow-up. Discussion Esophageal lung is an extremely rare type of BPFM, the incidence is described mainly in sporadic case reports throughout the world, without any predominant geographical area. This anomaly is described by some as being a part of the spectrum of the more severe malformation of tracheal agenesis, with the distal trachea, the bronchi, or both arising separately from the esophagus. The first case was described by Klebs in 1874, 4 5 then in 1968, Gerle et al raised the use of the terminology of communicating malformations of the anterior bowel. 6 However, it was not until 1996 when Floyd et al made the last characterization as part of tracheal and esophageal malformations. 5 Possible etiological causes on the formation of the communicating epithelium from the anterior intestine between the squamous and the respiratory epithelium, even though a clear, established etiology, has not been described. 7 8

yd et al made the last characterization as part of tracheal and esophageal malformations. 5 Possible etiological causes on the formation of the communicating epithelium from the anterior intestine between the squamous and the respiratory epithelium, even though a clear, established etiology, has not been described. 7 8 The respiratory primordium grows caudally while the esophageal primordium grows cephalically. At the crossing of these structures during growth, it is believed that the formation of this communication is formed by small early structures of the previously mentioned tissues. Srikanth et al show that the esophageal lung entity is predominant on the right lung in almost 94% of cases. 6 9 Our patient presented an esophageal lung type III as Srikanth et al described ( Fig. 4 ). Fig. 4 Three-dimensional (3D) model of esophageal lung type III by Srikanth et al classification. Patients with this condition are usually diagnosed during the neonatal period who develop lower respiratory tract infections, or where a genetic anomaly is suspected as were presented in our patient. The main features of esophageal lung are the complete involvement of the affected lung and its circulation is provided mainly by the pulmonary artery. These features allow the differentiation from pulmonary sequestration, according to the description by Sugandhi et al. 2

y is suspected as were presented in our patient. The main features of esophageal lung are the complete involvement of the affected lung and its circulation is provided mainly by the pulmonary artery. These features allow the differentiation from pulmonary sequestration, according to the description by Sugandhi et al. 2 X-ray, barium contrast studies, CT scan angiography, and magnetic resonance imaging (MRI) angiography could be used in the diagnostic approach, and could also provide an approximation on differential diagnoses, such as bronchopulmonary sequestration and enteric duplication cysts. 10 11 Colleran et al 12 conclude that lung opacification, ipsilateral mediastinal shift, an abnormal carina, and anomalous vascular anatomy suggest an esophageal bronchus or an esophageal lung on CT, findings compatible with what was presented in the case. Nevertheless, MRI and fibrobronchoscopy should be used as a complementary study that shows the malformation added to vascular studies and the respiratory tree anatomy. 13 Early diagnosis of esophageal bronchus might prevent complications, such as aspiration and infection, which can allow for parenchymal sparing surgery as opposed to pneumonectomy. 12

hoscopy should be used as a complementary study that shows the malformation added to vascular studies and the respiratory tree anatomy. 13 Early diagnosis of esophageal bronchus might prevent complications, such as aspiration and infection, which can allow for parenchymal sparing surgery as opposed to pneumonectomy. 12 Multiple malformations in association with this pathology, among them, esophageal atresia, stenosis or duodenal atresia, congenital heart disease, costovertebral malformations, and “pulmonary artery sling,” 14 15 are present and so the VACTERL (vertebral defects, anal atresia, cardiac defects, TEF, renal anomalies, and limb abnormalities) association should be considered, as it was ruled out in our patient. 4 11 12 16 Initially, resection has been described for management of abnormal communicating tissue in addition to removal of the hypoplastic lung that has been destroyed by recurrent infections; with repairing of the esophageal communication. 16 17 Lallemand et al reported on successful bronchus implantation in neonatal patients, which has shown a good clinical course and postoperative function, mainly in patients diagnosed early in the neonatal period. 1 11 Traditional management consists of surgical excision of this abnormal communication. 2 Molina et al showed that management through thoracoscopy in a case report from their experience has been followed and is feasible when there are no vascular anomalies. However, in the reviewed literature worldwide there are few such cases. 8

nal management consists of surgical excision of this abnormal communication. 2 Molina et al showed that management through thoracoscopy in a case report from their experience has been followed and is feasible when there are no vascular anomalies. However, in the reviewed literature worldwide there are few such cases. 8 Compared with conventional open repair, the main advantages of thoracoscopy are the superior visualization and avoidance of the thoracotomy incision. However, the hemodynamic and respiratory status of our patients do not allow a minimally invasive approach, so it must be taken into account when deciding the type of approach. 18 19 20 In conclusion, the management of esophageal lung is challenging. We believe that, if feasible, however, all clinical and surgical variables should be taken into account. Different types of management have been described as tracheobronchial anastomosis with the preservation of the lung to allow the best quality of life to the affected child as were reported by Ichino et al. 21 However, if this approach is not possible for anatomical reasons, a reasonable treatment plan is an early closure and division of the esophageal bronchus, followed by delayed pneumonectomy of the esophageal lung. Ichino et al explain that this type of management allows a slow adaptation of thoracic anatomy to reduce the risk of postpneumonectomy syndrome. 21

e for anatomical reasons, a reasonable treatment plan is an early closure and division of the esophageal bronchus, followed by delayed pneumonectomy of the esophageal lung. Ichino et al explain that this type of management allows a slow adaptation of thoracic anatomy to reduce the risk of postpneumonectomy syndrome. 21 Conclusion Esophageal lung is a rare entity, part of the spectrum of BPFM, presented in the neonatal period. The clinical presentation of this entity is a respiratory distress secondary to recurrent respiratory infections and/or swallowing difficulty. In the majority of the cases, the management is directed to the excision of the abnormal lung, nevertheless proper anatomy identification is required to develop an adequate plan for a surgical approach. This type of pathology requires a multidisciplinary approach and a careful follow-up. New Insights and the Importance for the Pediatric Surgeon Esophageal lung is uncommon in children and is extremely rare with few cases reported in literature. The management of esophageal lung is a challenging subject. It represents a diagnostic challenge and should be included in the differential diagnosis of nonspecific respiratory symptoms in newborn. Conflict of Interest None declared. Informed Consent The family of the newborn agrees to their personal information not being disclosed. Ethical Approval None.

l/L) (0.4–2) 0.866 1.66 Androstenedione (nmol/L) <1.05 3.5 Testosterone (nmol/L) <0.69 15.5 DHT (nmol/L) <0.1 0.27 Inhibin B (pg/mL) 74.2 (50–310) AMH (pmol/L) 116.2 (134–184) Abbreviations: AMH, anti-Müllerian hormone; DHEA-S, dehydroepiandrosterone sulfate; DHT, dihydrotestosterone; Hcg, human chorionic gonadotropin. The discrepancy between good hormonal response at a repeat Hcg test and the ongoing impalpable nature of the gonads led to the question of possibly missed intra-abdominal testes. Ultrasound scan failed to detect any gonads; however, subsequent abdominal magnetic resonance imaging (MRI) suggested intra-abdominal testes close to the lower pole of the respective kidneys ( Fig. 1 ). Family and patient consented to a diagnostic laparoscopy but no intervention: this confirmed vas and vessels entering the deep inguinal rings ( Fig. 2 ); however, further mobilization of colon medially allowed the vessels to be traced from the internal ring cranially to finally reveal the testis on the psoas muscle abutting the lower pole of each kidney ( Fig. 3 ). The left testis has been considered suitable for a Fowler–Stephen two-stage laparoscopic orchidopexy, whereas the right testis showed a poor vascular pedicle, and orchidectomy has been recommended. Fig. 1 Magnetic resonance imaging (MRI). Fig. 2 Laparoscopy: vas and vessels entering deep inguinal rings. Fig. 3 Laparoscopy: testis on the psoas muscle abutting the lower pole of the kidney. Discussion

The discrepancy between good hormonal response at a repeat Hcg test and the ongoing impalpable nature of the gonads led to the question of possibly missed intra-abdominal testes. Ultrasound scan failed to detect any gonads; however, subsequent abdominal magnetic resonance imaging (MRI) suggested intra-abdominal testes close to the lower pole of the respective kidneys ( Fig. 1 ). Family and patient consented to a diagnostic laparoscopy but no intervention: this confirmed vas and vessels entering the deep inguinal rings ( Fig. 2 ); however, further mobilization of colon medially allowed the vessels to be traced from the internal ring cranially to finally reveal the testis on the psoas muscle abutting the lower pole of each kidney ( Fig. 3 ). The left testis has been considered suitable for a Fowler–Stephen two-stage laparoscopic orchidopexy, whereas the right testis showed a poor vascular pedicle, and orchidectomy has been recommended. Fig. 1 Magnetic resonance imaging (MRI). Fig. 2 Laparoscopy: vas and vessels entering deep inguinal rings. Fig. 3 Laparoscopy: testis on the psoas muscle abutting the lower pole of the kidney. Discussion Testis and epididymis follow differing embryological pathways: the former originates from the urogenital crest, whereas the latter derives from the mesonephric duct. The SRY (Sex-determining Region of Y-chromosome) gene expression guides the undifferentiated sex cords to transform into seminiferous cords first and rete testes later. 1 At 6 months of gestation, rete testes and the mesonephric tubules share a common lumen. 2 The epididymis always precedes the testicle in the descent through the groin into the scrotum and is constantly anatomically distal to the gonad. 3 Fusion defects between epididymis and testicle can occur at different levels of the descent pathway and in different portions of the epididymis. Turek et al in 1994 defined the normal epididymal anatomy: the “looped” body epididymis connected with the testis by head and tail (type-1) is the most common configuration (83.9%) followed by the complete attachment of the epididymis to the testis (12.5%). Head or tail attachment only and complete nonfusion were the rarest varieties. 4 The incidence of fusion defects is higher in an undescended testicle. 5 Windholz classified fusion abnormalities with undescended testis into four categories: (1) absent testis with epididymis/vas deferens within the scrotum, (2) undescended testis with epididymis/vas deferens within the scrotum, (3) undescended testis and epididymis with only the vas deferens within the scrotum, and (4) both testis and epididymis descended within the scrotum but nonfused. 6

four categories: (1) absent testis with epididymis/vas deferens within the scrotum, (2) undescended testis with epididymis/vas deferens within the scrotum, (3) undescended testis and epididymis with only the vas deferens within the scrotum, and (4) both testis and epididymis descended within the scrotum but nonfused. 6 The classifications proposed by Turek et al and Windholz focused on features of aberrant fusion but lacked a qualitative description of the epididymis in its different portions in case of fusion defects. The epididymis can, in fact, be also maldeveloped. Simon included in their classification qualitative aspects of the fusion defect. They categorized cases of head attachment achieved by elongated efferent ducts (type b), as well as direct testis-vas deferens fusion with absent epididymis (type c), absent epididymal body (type d), and absent epididymal head and body (type e). 7 8 Variations among classifications account for many possible anatomical variants in terms of both fusion defect and segmental maldevelopment. TEDs can subsequently be represented by a pure fusion defect, a segmental structural maldevelopment, or their association. Our case is probably a fusion defect of intra-abdominal high testis characterized by absent epididymal head and body (type-e, Simon's classification), replaced by fibrotic structures, with descended epididymal tail and vas deferens, where the tail was probably originally misdiagnosed as testicular remnant bilaterally. 9 However, the lack of the original laparoscopic appearances makes accommodation of our case into a particular classification debatable. In retrospect, an MRI scan after the first surgery at the first year of age, in light of the positive Hcg test, as well as the AMH and inhibin results, would have been helpful. However, we are describing historical data, over which we could have no control.

dation of our case into a particular classification debatable. In retrospect, an MRI scan after the first surgery at the first year of age, in light of the positive Hcg test, as well as the AMH and inhibin results, would have been helpful. However, we are describing historical data, over which we could have no control. Laparoscopy is considered the gold standard for the assessment of intra-abdominal testes. Once both vas and vessels are seen entering the internal inguinal ring, an inguinal exploration is generally advised. On the contrary, if both vas and vessels are blind ending, an inguinal exploration should be theoretically unnecessary. 10 Snodgrass et al demonstrated scrotal nubbins associated with blind ending vas and vessels. 11 When a normal vas without vessels in its proximity is seen entering the internal inguinal ring, a strong recommendation is to look for high intra-abdominal testes. In our case, the vessels entering the internal inguinal ring bilaterally could be the epididymal vessels. These arteries are usually branches of the internal spermatic artery: three epididymal arteries usually arise from the epididymis to serve the head, body, and tail independently. The epididymal venous drainage is through the pampiniform plexus, and varicosity of the vessels on the left side of this case could represent that contribution. 12

ally branches of the internal spermatic artery: three epididymal arteries usually arise from the epididymis to serve the head, body, and tail independently. The epididymal venous drainage is through the pampiniform plexus, and varicosity of the vessels on the left side of this case could represent that contribution. 12 As the appearance of the nubbins was initially strongly indicative of gonadal remnants, we have also retrospectively postulated the polyorchidism variant. This is more common on the left side and can be associated with cryptorchidism. 13 Leung described four types of polyorchidism based on the different relations that the supernumerary testis can have with the epididymis and vas. 14 In type 3 of Leung's classification, in fact, the supernumerary, proximal testis has its own epididymis but shares a common vas with the distal testis. However, the absence of two independent testicular vascular systems excludes this hypothesis. Polyorchidism is also difficult to correlate with the findings on the right side, where the vas deferens remnant is clearly following a normal pathway. A two-stage Fowler–Stephen procedure has been offered for the left testicle as TEDs do not correlate with significant histological gonadal abnormalities; therefore, orchidopexy remains an option if the testicle is eligible for it. 15

As the appearance of the nubbins was initially strongly indicative of gonadal remnants, we have also retrospectively postulated the polyorchidism variant. This is more common on the left side and can be associated with cryptorchidism. 13 Leung described four types of polyorchidism based on the different relations that the supernumerary testis can have with the epididymis and vas. 14 In type 3 of Leung's classification, in fact, the supernumerary, proximal testis has its own epididymis but shares a common vas with the distal testis. However, the absence of two independent testicular vascular systems excludes this hypothesis. Polyorchidism is also difficult to correlate with the findings on the right side, where the vas deferens remnant is clearly following a normal pathway. A two-stage Fowler–Stephen procedure has been offered for the left testicle as TEDs do not correlate with significant histological gonadal abnormalities; therefore, orchidopexy remains an option if the testicle is eligible for it. 15 Conclusions The incidence of testicular–epididymal fusion defects is high for undescended intra-abdominal testes, and, at laparoscopy, vessels deriving from an aberrant fusion attempt might be seen. Those vessels, if examined at the level of the internal inguinal ring only, can be indistinguishable from normal retroperitoneal spermatic artery and veins. If only a nubbin is found at exploration and the preoperative Hcg test is positive, suggesting functioning testicular tissue, a careful search along the pathway of testicular descent, with colonic mobilization, looking for high intra-abdominal gonads, is therefore strongly recommended to avoid missing the anatomical variant presented.

is found at exploration and the preoperative Hcg test is positive, suggesting functioning testicular tissue, a careful search along the pathway of testicular descent, with colonic mobilization, looking for high intra-abdominal gonads, is therefore strongly recommended to avoid missing the anatomical variant presented. New Insights and the Importance for the Pediatric Surgeon A careful search along the pathway of testicular descent is recommended to avoid missing the anatomical variant of testicular–epididymal fusion defects. Conflict of Interest None. Supplementary Material Supplementary Material Supplementary Material

Introduction A gastrosplenic fistula is a very rare pathological condition. It was first described in two patients with splenic lymphoma by de Scoville et al in 1962. 1 “Aerosplenomegaly” is the radiological term used to describe an enlarged spleen, which appeared to be filled with air, which would suggest an enterosplenic fistula. A gastrosplenic fistula can form as a complication of a variety of conditions, although splenic lymphoma has most frequently been reported in previous literature. 2 We discuss a nonmalignant pediatric case of a gastrosplenic fistula which was treated successfully by surgical intervention.

gest an enterosplenic fistula. A gastrosplenic fistula can form as a complication of a variety of conditions, although splenic lymphoma has most frequently been reported in previous literature. 2 We discuss a nonmalignant pediatric case of a gastrosplenic fistula which was treated successfully by surgical intervention. Case Report Our patient is a 16-year-old male with a history of type 1 diabetes mellitus that presented to the emergency department with diabetic ketoacidosis and shock. His blood glucose was 1,037 mg/dL with a pH of 6.95. He was resuscitated, started on an insulin drip, given vasopressors, and admitted to the pediatric intensive care unit (PICU) where he was intubated. During his stay in the PICU, he abruptly had 1.5 L of dark brown to bloody drainage from his nasogastric tube. The dark blown to bloody fluid did not persist and the patient never had a significant drop in his hemoglobin. Had either of these factors developed, an earlier endoscopy would have been warranted. Because of an elevated creatinine (3.4 mg/dL), an abdominal magnetic resonance imaging (MRI) was obtained which showed a small amount of perisplenic fluid and evidence of a splenic infarction. Intravenous antibiotic therapy was initiated with clindamycin. The patient recovered and was discharged from the hospital on day 9.

use of an elevated creatinine (3.4 mg/dL), an abdominal magnetic resonance imaging (MRI) was obtained which showed a small amount of perisplenic fluid and evidence of a splenic infarction. Intravenous antibiotic therapy was initiated with clindamycin. The patient recovered and was discharged from the hospital on day 9. The patient presented to the emergency department 2 weeks later with severe epigastric pain, poor appetite, and weight loss of 20 pounds since discharge. He also presented with labored breathing and a chest X-ray showed a left pleural effusion. Initial laboratories were as follows: C reactive protein, 5.5 mg/dL; white blood cell count, 16.10 3 /µL; platelet count, 1,206 × 10 3 /µL; hemoglobin, 9.1 g/dL; and serum lipase 890 U/L. On examination, the patient was ill-appearing and pale with tenderness in the epigastric region. During his hospital stay, the patient's oral intake decreased, and persistent abdominal pain prompted a CT of chest, abdomen, and pelvis with intravenous contrast. The results showed bilateral pleural effusions, left pelvic abscess, pancreatic pseudocyst, and a splenic infarction. Additionally, the computed tomography (CT) was suspicious for a gastrosplenic fistula, as the coil of the patient's transpyloric feeding tube appeared to lie within the upper pole of the spleen ( Fig. 1 ). To further evaluate the possibility of a gastrosplenic fistula, an esophagogastroduodenoscopy was performed, which noted multiple dispersed nonbleeding erosions of the gastric wall with two visualized openings of the posterior gastric wall ( Fig. 2 ). The time between the first and second endoscopy was 3 weeks. There is a growing body of literature to support the use of endoscopic closure of gastric perforations; however, this was not initially considered because of the degree of inflammation and friability of the gastric mucosa.

e posterior gastric wall ( Fig. 2 ). The time between the first and second endoscopy was 3 weeks. There is a growing body of literature to support the use of endoscopic closure of gastric perforations; however, this was not initially considered because of the degree of inflammation and friability of the gastric mucosa. Fig. 1 CT scan images demonstrating gastrosplenic fistula. (*) shows fistulous communication between stomach and spleen. Arrowhead represents coiled feeding tube within the capsule of the spleen. CT, computed tomography. Fig. 2 Endoscopic images of gastric inflammation and gastrosplenic fistula. ( A and B ) Images show pictures of two separate ulcers within there gastric wall. ( C ) Image shows generalized immflamation and mucosal erosions of the gastric wall. Due to concern for malignancy, tumor markers were checked and were within normal limits. A nonoperative approach would have been to support this patient with adequate nutrition and allow for time for the inflammatory process to subside; however, the patient had persistent epigastric pain and feeding intolerance.

Fig. 2 Endoscopic images of gastric inflammation and gastrosplenic fistula. ( A and B ) Images show pictures of two separate ulcers within there gastric wall. ( C ) Image shows generalized immflamation and mucosal erosions of the gastric wall. Due to concern for malignancy, tumor markers were checked and were within normal limits. A nonoperative approach would have been to support this patient with adequate nutrition and allow for time for the inflammatory process to subside; however, the patient had persistent epigastric pain and feeding intolerance. The exact etiology of the gastroplenic fistula was not clear and malignancy had not been completely excluded. The history and workup for other causes of a gastrosplenic fistula did not support another etiology. The patient denied a toxic ingestion. Also, none of his imaging on CT scan or MRI showed any evidence of inflammatory bowel disease. He did have an elevated lipase but there were no signs of acute or chronic pancreatitis on his imaging. A positron emission tomography (PET) scan could not be obtained because of the patient's brittle diabetes and insulin requirements. Because of the patient's failure to improve with nonoperative management, surgical exploration was recommended. The operation began with a repeat upper endoscopy to assess the condition of the gastric lumen. However, the scope could not be advanced much beyond the gastroesophageal (GE) junction as the stomach had become very inflamed at this location. Biopsies were not taken at the time of the first endoscopy because of the degree of inflammation and friability of the gastric wall. The changes between the two endoscopies were presumed to be due to progression of the inflammatory process. A left subcostal incision was made and extended across the midline partially to the right subcostal region and extended superiorly to the level of the xiphoid process. Adhesiolysis was performed to take down very dense adhesions between the omentum and left upper quadrant. The anterior gastric wall was intimately attached to the anterior abdominal wall, liver, and spleen. We encountered a very dense inflammatory process involving the upper pole of the spleen. The inflammatory process involving the upper pole of the spleen was able to be divided from the remaining portion of the spleen living the majority of the splenic parenchyma intact. The body of the stomach was completely fibrotic and necrotic. After separating these structures, 4 to 5 cm of proximal stomach beyond the GE junction was found to be viable as was 2 to 3 cm of antrum proximal to the pylorus.

from the remaining portion of the spleen living the majority of the splenic parenchyma intact. The body of the stomach was completely fibrotic and necrotic. After separating these structures, 4 to 5 cm of proximal stomach beyond the GE junction was found to be viable as was 2 to 3 cm of antrum proximal to the pylorus. Thus, a subtotal gastrectomy was completed by transecting the proximal stomach at the level of viability and transecting the distal stomach at the level of the antrum. A “Roux-en-Y” reconstruction was performed as well as placement of a feeding jejunostomy. Total operative time was 5 hours. The patient initially did well but developed an intra-abdominal abscess in the left upper quadrant seen on a CT that was obtained secondary to fever. The abscess was drained by interventional radiology and found to be from a leak from his gastrojejunal anastomosis. This was managed with external drainage and resolved in 4 weeks. Currently the patient is doing well, has no epigastric pain, and is maintaining his weight on a combination of oral and jejunostomy feeds. Pathological evaluation of the resected stomach revealed marked edema with necrotizing inflammation. There was no evidence of malignancy. A widely patent fistulous tract was seen extending into the congested and hemorrhagic omental tissue. Discussion

Thus, a subtotal gastrectomy was completed by transecting the proximal stomach at the level of viability and transecting the distal stomach at the level of the antrum. A “Roux-en-Y” reconstruction was performed as well as placement of a feeding jejunostomy. Total operative time was 5 hours. The patient initially did well but developed an intra-abdominal abscess in the left upper quadrant seen on a CT that was obtained secondary to fever. The abscess was drained by interventional radiology and found to be from a leak from his gastrojejunal anastomosis. This was managed with external drainage and resolved in 4 weeks. Currently the patient is doing well, has no epigastric pain, and is maintaining his weight on a combination of oral and jejunostomy feeds. Pathological evaluation of the resected stomach revealed marked edema with necrotizing inflammation. There was no evidence of malignancy. A widely patent fistulous tract was seen extending into the congested and hemorrhagic omental tissue. Discussion The etiology of the case is unclear. Possible etiologies include an infarct of the upper pole of the spleen and stomach from a low flow state, complications of pancreatitis, or a toxic ingestion causing necrosis of the stomach. Another cause of this fistula is nasogastric tube trauma; however, this is less likely due to multiple perforations seen on endoscopy. Gastrosplenic fistula can arise as a complication of a primary splenic or gastric malignancy, most commonly diffuse large B cell lymphoma (DLBCL). 2 This may be attributed to this tumor's nature to widely infiltrate the serosa and failure to initiate a desmoplastic reaction. This fistula may occur spontaneously or after recent chemotherapy treatment as a possible consequence of tumor lysis syndrome. 3 Although less frequent benign conditions, such as a benign gastric ulcer, splenic abscess, and Crohn's disease, have also been implicated as a cause. 4 5 6 More recently, a study reported three cases of gastrosplenic fistula as a complication of sleeve gastrectomy. 7 A literature review identified a total of 28 cases reports; the etiology in 75% of cases was determined to be a lymphoma. 8 The most common presenting complaints included abdominal pain (32%), weakness (21%), and upper gastrointestinal bleeding (14%). 8 Abdominal CT is the most useful radiological test for reaching a definitive diagnosis. 9 In a noncontrast CT, an air-fluid level or free air in the spleen may indicate a gastrosplenic fistula, although splenic abscess must be ruled out. Contrasted CT may allow one to visualize the anomalous tract and orally administered contrast may be seen flowing from the stomach to the spleen. Other tests that may be performed include upper GI series as well as upper endoscopy to visualize the opening of the fistula and for tissue sampling.

scess must be ruled out. Contrasted CT may allow one to visualize the anomalous tract and orally administered contrast may be seen flowing from the stomach to the spleen. Other tests that may be performed include upper GI series as well as upper endoscopy to visualize the opening of the fistula and for tissue sampling. Surgical resection of the fistula is usually indicated. 9 This is done to prevent leaking of gastric contents that may erode the splenic vessels resulting in life-threatening hematemesis. The operation may include of partial gastrectomy, splenectomy, and/or distal pancreatectomy depending on the underlying etiology. A feeding jejunostomy should also be considered. It is generally done as an open procedure, although a laparoscopic approach has previously been described. 10 Conclusion Gastrosplenic fistulas have most commonly been reported in association with splenic and gastric malignancies. Abdominal CT is the most helpful radiological test for diagnosing this because of the ability to show thin cuts and demonstrate very detailed anatomy in this region. Surgical intervention is usually indicated. We have presented a case of gastrosplenic fistula in the pediatric population of unknown etiology that required surgical treatment. New Insights and the Importance for the Pediatric Surgeon

Conclusion Gastrosplenic fistulas have most commonly been reported in association with splenic and gastric malignancies. Abdominal CT is the most helpful radiological test for diagnosing this because of the ability to show thin cuts and demonstrate very detailed anatomy in this region. Surgical intervention is usually indicated. We have presented a case of gastrosplenic fistula in the pediatric population of unknown etiology that required surgical treatment. New Insights and the Importance for the Pediatric Surgeon Gastrosplenic fistula is a very rare entity most commonly occurring as a distinctive complication of splenic or gastric malignancies, most notably diffuse large B cell lymphoma (DLBCL). We report a nonmalignant pediatric case of a gastrosplenic fistula of unclear etiology and the surgical management. Conflict of Interest None.

Introduction Juvenile-type granulosa cell tumor (JGCT) of the testis, although rare, is the most common testicular neoplasm in the first 6 months of life. 1 It is frequently diagnosed in the neonatal period; it is uncommon in older children and exceptional in adults. 2 Typical presentation is a painless neonatal scrotal mass 3 ; occasionally it occurs in cryptorchidic testes 3 4 5 or in infants with abnormal karyotypes and ambiguous genitalia 3 ; all cases reported have had a benign outcome. 3 6 Inguinal orchiectomy was invariably considered the treatment of choice but new treatment trends advocate a trans-scrotal approach 7 and testis-sparing surgery where preoperative staging determines that this is safe. 6 8 We report a case of JGCT of the testis prenatally diagnosed, an event described only twice in the literature so far, 7 9 followed by inguinal orchiectomy. Case Report A healthy 2-day-old newborn was admitted to our department for a left scrotal mass. He had undergone a prenatal ultrasound a week before delivery (38th week of gestation) for suspected nonvertex presentation. On that occasion, a left testicular cystic mass (2 × 2 cm), suspected to be a JGCT due to its multicystic aspect, was identified. At clinical presentation, the left testicle appeared in situ, with increased size and consistency, while the right testicle and the penis were normal.

) for suspected nonvertex presentation. On that occasion, a left testicular cystic mass (2 × 2 cm), suspected to be a JGCT due to its multicystic aspect, was identified. At clinical presentation, the left testicle appeared in situ, with increased size and consistency, while the right testicle and the penis were normal. Ultrasound revaluation after birth excluded torsion of the testis and confirmed the presence of a voluminous multicystic left testicular mass without normal-appearing parenchyma. Serum α-fetoprotein (AFP) and β-human chorionic gonadotropin (β-HCG) were normal for age. Karyotype was normal. Abdominal ultrasound did not show any anomalies. Following surgical oncological criteria, we opted for an inguinal approach: after groin incision, the spermatic chord was identified and clamped at the level of the deep inguinal orifice. Testis examination revealed a cystic mass beneath the tunica albuginea replacing all normal parenchyma. We performed funiculo-orchiectomy, since organ-sparing excision of the mass was considered not possible. There was no evidence of enlarged inguinal lymph nodes. Gross examination of the surgical specimen revealed a well-circumscribed 2 × 1.5 cm white mass. The cut surface showed multiple, thin-walled cysts containing clear fluid ( Fig. 1 ). Fig. 1 Juvenile granulosa cell tumor gross specimen showing cystic mass replacing all normal testicular parenchyma.

Ultrasound revaluation after birth excluded torsion of the testis and confirmed the presence of a voluminous multicystic left testicular mass without normal-appearing parenchyma. Serum α-fetoprotein (AFP) and β-human chorionic gonadotropin (β-HCG) were normal for age. Karyotype was normal. Abdominal ultrasound did not show any anomalies. Following surgical oncological criteria, we opted for an inguinal approach: after groin incision, the spermatic chord was identified and clamped at the level of the deep inguinal orifice. Testis examination revealed a cystic mass beneath the tunica albuginea replacing all normal parenchyma. We performed funiculo-orchiectomy, since organ-sparing excision of the mass was considered not possible. There was no evidence of enlarged inguinal lymph nodes. Gross examination of the surgical specimen revealed a well-circumscribed 2 × 1.5 cm white mass. The cut surface showed multiple, thin-walled cysts containing clear fluid ( Fig. 1 ). Fig. 1 Juvenile granulosa cell tumor gross specimen showing cystic mass replacing all normal testicular parenchyma. Microscopic examination showed multiple follicle-like structures of varying size, round to oval, filled with basophilic fluid stained by mucicarmine. The follicles were lined by variable layers of cells with round hyperchromatic nuclei and scant pale cytoplasm ( Fig. 2 ). Nucleoli were not prominent but were occasionally visible, and nuclear grooves were absent. Mitotic activity was low. The stroma was composed of edematous fibrovascular tissue that formed a dense layer of spindle cells around the follicles. The neoplastic cells were immunoreactive for α-inhibin, CD99, and calretinin antibodies. Focal expression of cytokeratin was also observed. Immunoreactions for FLAP, AFP, CD30, vimentin, and β-HCG were negative. Immunostaining for Ki67 showed a low proliferative index (1% of the tumor cells).

ells around the follicles. The neoplastic cells were immunoreactive for α-inhibin, CD99, and calretinin antibodies. Focal expression of cytokeratin was also observed. Immunoreactions for FLAP, AFP, CD30, vimentin, and β-HCG were negative. Immunostaining for Ki67 showed a low proliferative index (1% of the tumor cells). Fig. 2 Multiple variably sized follicles containing basophilic material and lined by one to several layers of cells with pale cytoplasm. Based on morphologic and immunohistochemical findings, a diagnosis of JGCT was formulated. The tumor did not extend into the spermatic cord, epididymis, or tunica vaginalis. Nevertheless, only a small rim of residual testis was present. The baby was discharged 2 days after the surgical procedure. Seven-year-follow-up was uneventful. Discussion Prepubertal primary testicular tumors account for ∼1% of all solid pediatric tumors. Gonadal stromal tumors, including Leydig cell, Sertoli cell, and granulosa cell tumors, account for ∼8% of these neoplasms and are therefore extremely rare. JGCT accounts for only 1.2% of all prepubertal testis tumors recorded in the Pre-pubertal Testis Tumor Registry. 10 Nevertheless, JGCT is the most common stromal cord neoplasm of the testis in the first 6 months of life. 3 This tumor may be associated with anomalies of the genitalia or sexual chromosome abnormalities. JGCT is considered a benign tumor since no reports of metastatic disease are described in literature. 3 6

gistry. 10 Nevertheless, JGCT is the most common stromal cord neoplasm of the testis in the first 6 months of life. 3 This tumor may be associated with anomalies of the genitalia or sexual chromosome abnormalities. JGCT is considered a benign tumor since no reports of metastatic disease are described in literature. 3 6 Differential diagnosis for JGCT includes evaluation for yolk-sac and other sex cord-stromal tumors. 3 11 The juvenile form can be distinguished from the adult one by the lack of nuclear grooves and Call–Exner bodies, and the greater degree of irregularity in size and shape of the follicles, which show intraluminal basophilic fluid. Overall, the typically very young age of patients is helpful in diagnosis 11 12 : yolk-sac tumor, also relatively frequent in infants, is usually seen beyond 6 months of age. 3 The presence of follicular structures, the absence of the various characteristic patterns of yolk-sac tumor, a lack of reactivity for AFP, and positivity for α-inhibin favor a diagnosis of JGCT. 6 12 13 Sertoli cell tumors usually exhibit a prominent tubular differentiation with bland cytologic features and little mitotic activity, in contrast with the follicular pattern and more immature-appearing nuclei of JGCT. Furthermore, in the largest series of Sertoli cell tumors, no patient was under 15 years of age. 11 12 13 14

toli cell tumors usually exhibit a prominent tubular differentiation with bland cytologic features and little mitotic activity, in contrast with the follicular pattern and more immature-appearing nuclei of JGCT. Furthermore, in the largest series of Sertoli cell tumors, no patient was under 15 years of age. 11 12 13 14 In one case reported in the literature, retrospectively reviewed prenatal ultrasounds of a newborn treated for JGCT showed a testicular mass. 15 To our knowledge, we report only the third case where JGCT was suspected prenatally. 7 9 15 As in our experience, prenatal diagnosis in the two previously reported cases was accidental and made at 36 and 38 weeks of gestation, respectively. In our case, the mass was not observed at the previous routine ultrasound examination. So, suggesting that in utero diagnosis of this rare tumor is possible only in the last weeks of pregnancy.

prenatal diagnosis in the two previously reported cases was accidental and made at 36 and 38 weeks of gestation, respectively. In our case, the mass was not observed at the previous routine ultrasound examination. So, suggesting that in utero diagnosis of this rare tumor is possible only in the last weeks of pregnancy. Due to their benignancy, JGCTs have been treated with testis-sparing surgery 6 8 16 where it is possible to identify a rim of normal parenchyma; in these cases, normal preoperative levels of AFP and intraoperative biopsies and frozen section analysis are essential to exclude malignant lesions. 6 Likewise, the less invasive trans-scrotal approach used in a case reported in 2012 may be safe and feasible where AFP levels are normal and where ultrasound strongly suggests the presence of a JGCT, 7 although the oncological outcome for this patient is unknown due to lack of long-term follow-up. However, if this procedure is not possible due to a tumor completely replacing the testis as in our case, orchiectomy is the only treatment option. Conclusion Our case shows that JGCT can be diagnosed prenatally in the last weeks of gestation. In our case, diagnosis in the 38th weeks of pregnancy after unremarkable previous routine ultrasounds indicates late fetal development of the tumor. Although successful trans-scrotal removal of JGCT has been reported, inguinal orchiectomy remains the safest way in terms of oncological criteria.

last weeks of gestation. In our case, diagnosis in the 38th weeks of pregnancy after unremarkable previous routine ultrasounds indicates late fetal development of the tumor. Although successful trans-scrotal removal of JGCT has been reported, inguinal orchiectomy remains the safest way in terms of oncological criteria. New Insights and the Importance for the Pediatric Surgeon Juvenile granulosa cell tumor is a benign tumor of the testis that should be treated, when possible, with a conservative approach. Conflict of Interest None.

Introduction Meconium peritonitis is a rare type of sterile peritonitis caused by antenatal bowel perforation with spillage of meconium into the peritoneal cavity. 1 The estimated prevalence is 1 per 30,000 live births, and mortality ranges from 11 to 50%. 2 The bowel perforation may have different causes including atresia, stricture, meconium ileus, internal hernia, volvulus, intussusception, duplication, or Meckel's diverticulum. 2 3 Meconium peritonitis leads to inflammation and fibrosis (fibroadhesive-type) and can be complicated by the presence of pseudocysts (cystic-type). 4 The typically rim-calcified cysts lack an intestinal epithelial layer due to inflammation by digestive enzymes. Usually a small muscle sheath connects the cyst to the rest of the bowel. 2 Case Report A 34-year-old woman, gravida 2, para 1, was referred from an outside hospital with premature rupture of membranes and contractions. Two weeks before an abdominal mass of unknown origin was detected on routine ultrasound. After 36 + 0 weeks of gestation, a male preterm newborn of 3,410 g (P 89 ) was spontaneously delivered.

-year-old woman, gravida 2, para 1, was referred from an outside hospital with premature rupture of membranes and contractions. Two weeks before an abdominal mass of unknown origin was detected on routine ultrasound. After 36 + 0 weeks of gestation, a male preterm newborn of 3,410 g (P 89 ) was spontaneously delivered. Despite the palpable abdominal mass, the boy seemed not be affected. At 2 hours of life, an abdominal plain X-ray showed dilated intestinal loops in the upper abdomen and an “eggshell” oval calcification suggestive of a meconium pseudocyst ( Fig. 1A ). On ultrasound ( Fig. 2A and B ), the mass (6 × 5 × 3 cm) had an echogenic wall without increased perfusion and a heterogeneous content. In the left upper abdomen were grossly distended intestinal loops with a to-and-fro peristalsis, the colon appears empty. On contrast enema the colon appeared small and unused without any stricture or atresia ( Fig. 2C ). A nasogastric tube was placed, and the patient was scheduled for surgery. Fig. 1 Preoperative plain abdominal film (A) and intraoperative findings (B). Meconium pseudocyst, the dilated prestenotic jejunum (*), and unused small poststenotic jejunum (+) at the base of the pseudocyst. Note the substantial caliber difference. Fig. 2 Ultrasound and contrast enema. ( A ) Ultrasound of the meconium pseudocyst with an echogenic rim and heterogenous content. ( B ) In the left abdomen were grossly distended intestinal loops, the descending colon appears empty. ( C ) Contrast enema shows an unused microcolon.

Fig. 1 Preoperative plain abdominal film (A) and intraoperative findings (B). Meconium pseudocyst, the dilated prestenotic jejunum (*), and unused small poststenotic jejunum (+) at the base of the pseudocyst. Note the substantial caliber difference. Fig. 2 Ultrasound and contrast enema. ( A ) Ultrasound of the meconium pseudocyst with an echogenic rim and heterogenous content. ( B ) In the left abdomen were grossly distended intestinal loops, the descending colon appears empty. ( C ) Contrast enema shows an unused microcolon. On the 4th day of life, the patient underwent explorative laparotomy: A large meconium pseudocyst and a small part of obstructed bowel were removed ( Fig. 1B ). With a size discrepancy of 7:1, a single layer end-to-back anastomosis between the two ends of the jejunum was performed. In addition, a large percutaneous central venous catheter was placed to facilitate parenteral nutrition. Further laboratory workup did not confirm cystic fibrosis as a potential cause of meconium peritonitis. Enteral feeds were started on the fifth postoperative day and advanced slowly until full feeds on the 28th day of life. The patient was discharged on breastfeeding with a weight of 3,750 g. On follow-up after 3 months, the child was thriving. Discussion Meconium pseudocyst is the result of meconium peritonitis where adjacent intestinal loops adhere to the meconium leak. 2 Like in our patient, the bowel distal to the pseudocyst is usually unused and of small caliber. 3 4

On the 4th day of life, the patient underwent explorative laparotomy: A large meconium pseudocyst and a small part of obstructed bowel were removed ( Fig. 1B ). With a size discrepancy of 7:1, a single layer end-to-back anastomosis between the two ends of the jejunum was performed. In addition, a large percutaneous central venous catheter was placed to facilitate parenteral nutrition. Further laboratory workup did not confirm cystic fibrosis as a potential cause of meconium peritonitis. Enteral feeds were started on the fifth postoperative day and advanced slowly until full feeds on the 28th day of life. The patient was discharged on breastfeeding with a weight of 3,750 g. On follow-up after 3 months, the child was thriving. Discussion Meconium pseudocyst is the result of meconium peritonitis where adjacent intestinal loops adhere to the meconium leak. 2 Like in our patient, the bowel distal to the pseudocyst is usually unused and of small caliber. 3 4 There are many differential diagnoses for intraabdominal calcifications in the newborn period, most of them neoplastic. 5 However, “eggshell calcifications” are pathognomonic for meconium pseudocysts. These calcifications are thought to be caused by a chemical reaction of pancreatic enzymes within the extravasated meconium. Their presence speaks against (but does not rule out) cystic fibrosis, which is present in 15 to 40% of children with meconium peritonitis in Caucasians. 6 Resection of the pseudocyst as well as the segment of small bowel followed by an enteral anastomosis is the treatment of choice. 3 4

thin the extravasated meconium. Their presence speaks against (but does not rule out) cystic fibrosis, which is present in 15 to 40% of children with meconium peritonitis in Caucasians. 6 Resection of the pseudocyst as well as the segment of small bowel followed by an enteral anastomosis is the treatment of choice. 3 4 Conclusion Meconium pseudocysts are easy to recognize and an important radiologic spot diagnosis that every pediatrician and pediatric surgeon should be aware of. Resection of the pseudocyst is the therapy of choice and can be undertaken electively in the first days of life. New Insights & the Importance for the Pediatric Surgeon Meconium pseudocyst is a rare entity that can be immediately diagnosed with an abdominal X-ray. Every doctor dealing with newborns should be able to recognize it. Acknowledgments We acknowledge support from the German Research Foundation (DFG) and Leipzig University within the program of Open Access Publishing. Conflict of Interest None declared.

Introduction Multiple duodenal atresia is an extremely rare condition that may be either double or triple. To our knowledge, only 24 papers in the English literature reported this anomaly. Of these, two patients had triple duodenal atresia and the remainder had a double duodenal atresia. 1 Duodenal atresia is thought to occur by failure of embryological recanalization. However, a multiple atresia is difficult to explain with this theory. Yoshida and Migita described it as a result of a malrotation that causes a twist and results in pressure necrosis and atresia in more than one point. 2 This rare deformity has different presentations ranging from neonatal intestinal obstruction in case of complete atresia to infantile presentation with incomplete intestinal obstruction. This is associated with or without a palpable abdominal mass. 3 This anomaly can be associated with other conditions as Zamfir et al reported a case of double duodenal atresia associated with Cri du Chat (cat cry) syndrome. 4 Also this anomaly may be associated with peptic ulcer. 3

th incomplete intestinal obstruction. This is associated with or without a palpable abdominal mass. 3 This anomaly can be associated with other conditions as Zamfir et al reported a case of double duodenal atresia associated with Cri du Chat (cat cry) syndrome. 4 Also this anomaly may be associated with peptic ulcer. 3 Case Report A male newborn (37 weeks of gestation; 2.43 kg) presented with bilious vomiting. Clinical examination revealed mild epigastric distension with the passage of mucous on digital rectal examination. A palpable epigastric firm mass measuring 1 × 1 cm was found. General examination revealed severe respiratory distress due to meconium aspiration pneumonia with associated signs of sepsis; however, there was no sign of peritonitis. Meconium aspiration pneumonia was diagnosed after reviewing the records of delivery that revealed meconium-stained amniotic fluid in association with immediate postnatal respiratory distress. Laboratory investigations revealed hypochromic microcytic anemia with moderate leukocytosis in association with mild hypokalemia and metabolic alkalosis. Hanging abdominal X-ray showed a hugely distended stomach with a faint double bubble sign and absent gas distribution in the remaining abdomen as shown in Fig. 1 . Also, plain X-ray (PXR) chest revealed patches of consolidation and areas of hyperinflation that raised the suspicion of meconium aspiration pneumonia. Fig. 1 PXR abdomen standing showing hugely dilated stomach and first part of the duodenum (double bubble sign).

Laboratory investigations revealed hypochromic microcytic anemia with moderate leukocytosis in association with mild hypokalemia and metabolic alkalosis. Hanging abdominal X-ray showed a hugely distended stomach with a faint double bubble sign and absent gas distribution in the remaining abdomen as shown in Fig. 1 . Also, plain X-ray (PXR) chest revealed patches of consolidation and areas of hyperinflation that raised the suspicion of meconium aspiration pneumonia. Fig. 1 PXR abdomen standing showing hugely dilated stomach and first part of the duodenum (double bubble sign). The mother had an antenatal ultrasound that revealed full stomach in association with polyhydramnios. After stabilization of the septic patient, a right supraumbilical explorative laparotomy was performed. We performed a Kocher maneuver and found a cyst between the first and the second part of the duodenum as shown in Figs. 2 and 3 . Fig. 2 Intraoperative double duodenal atresia with duodenal cyst in between. Fig. 3 Illustration showing the duodenal pathology.

After stabilization of the septic patient, a right supraumbilical explorative laparotomy was performed. We performed a Kocher maneuver and found a cyst between the first and the second part of the duodenum as shown in Figs. 2 and 3 . Fig. 2 Intraoperative double duodenal atresia with duodenal cyst in between. Fig. 3 Illustration showing the duodenal pathology. A longitudinal duodenotomy distal to the cyst was performed and the ampulla of Vater was identified. We advanced a 6-inch French tube through this incision into the distal intestine with injection of warm saline to exclude further distal intestinal obstructions. Then transverse duodenotomy proximal to the cyst was applied and we found a duodenal diaphragm at the proximal end of the cyst causing partial duodenal obstruction; this diaphragm was resected. Another complete duodenal atresia was found at the distal end of the cyst that explained the development of this cyst between a proximal partial obstruction and a distal complete obstruction. This cyst was bypassed by a diamond-shaped duodeno-duodenal anastomosis between the transverse duodenotomy proximal to it and the longitudinal duodenotomy distal to it. Stool was passed on the 5th postoperative day; however, the child still had bilious reflux, so we performed a contrast passage study, which showed that the contrast passed to the distal intestine freely. However, with a remaining proximal dilatation the decision to delay oral intake was taken ( Fig. 4 ). Fig. 4 Postoperative contrast study with the dye passing into the small intestine.

Stool was passed on the 5th postoperative day; however, the child still had bilious reflux, so we performed a contrast passage study, which showed that the contrast passed to the distal intestine freely. However, with a remaining proximal dilatation the decision to delay oral intake was taken ( Fig. 4 ). Fig. 4 Postoperative contrast study with the dye passing into the small intestine. Unfortunately, the baby progressively deteriorated due to the meconium aspiration pneumonia that was resistant to antibiotic treatment. The condition was associated with severe uncontrollable sepsis that was progressed to septic shock, disseminated intravascular coagulation (DIC), and multiorgan failure that ended with death of the child 15 days postoperatively Discussion Double duodenal atresia is an extremely rare condition occurring in 1:5,000 to 1:10,000 live births; Stringer et al published a series of four patients and the postoperative survival was reported in three cases and only one case died due to the associated congenital heart disease. 5 Also, along his 20-year experience, Grosfeld and Rescorla described only three patients with double duodenal atresia. 6 It usually occurs as a sporadic congenital anomaly and only two reports documented its presence in siblings. 7 Besides the previously mentioned presentations, double duodenal atresia may present postoperatively after the treatment of a first point duodenal obstruction by intestinal obstruction, perforation, and/or abdominal cyst. This usually develops if the patency of the distal intestine was not ensured during the first operation. 5 8

he previously mentioned presentations, double duodenal atresia may present postoperatively after the treatment of a first point duodenal obstruction by intestinal obstruction, perforation, and/or abdominal cyst. This usually develops if the patency of the distal intestine was not ensured during the first operation. 5 8 In our case, a cystic dilatation between the first and the second part of the duodenum was found during operative exploration. This is consistent with other similar studies discussing the same topic like that one belonging to Stringer et al, which was published in 1992. 5 Endoscopic incision of double duodenal webs has been advocated by some surgeons as a new treatment modality in infants presenting with partial double duodenal diaphragms. However, it is not feasible in newborns because a minimum weight of ∼8 kg is mandatory for endoscopic intervention as reported by Barabino et al. These authors recorded a successful endoscopic release of duodenal web in a 11-month-old baby (8 kg). 9 Another study was conducted by Bittencourt et al who documented 9 to 12 months as a minimum age for endoscopic intervention of infantile duodenal membranes. 10 Finally, an endoscopic dilatation and partial resection of a duodenal web were reported by Beeks et al in a 15-month-old patient (8 kg). 11

h-old baby (8 kg). 9 Another study was conducted by Bittencourt et al who documented 9 to 12 months as a minimum age for endoscopic intervention of infantile duodenal membranes. 10 Finally, an endoscopic dilatation and partial resection of a duodenal web were reported by Beeks et al in a 15-month-old patient (8 kg). 11 Reviewing the records of our patient during delivery revealed that delayed cord clamping was not done in this condition; this may be the cause of hypochromic microcytic anemia. Our patient died 2 weeks postoperatively from meconium aspiration pneumonia that was resistant to antibiotic treatment and associated with uncontrollable sepsis. The condition progressed to septic shock, DIC, and multiorgan failure that ended with death of the child. Surgical exploration and anastomosis are usually curative in all cases. Mortality is only known due to other causes, such as associated cardiac malformations or pneumonia as in our patient. 6 Conclusion Double duodenal atresia is a rare condition. Operative detection of one atresia does not preclude the presence of further distal one, so thorough examination of the distal intestine with a tube and warm saline injection in case of intestinal atresia is mandatory to exclude other distal obstructing lesions. New Insights and the Importance for the Pediatric Surgeon Double duodenal atresia is very important to be known. Conflict of Interest None.

Introduction Endometriosis is defined as the presence of endometrial glands and stroma outside the uterus. This ectopic finding affects 7 to 10% of women of reproductive age. It commonly occurs in the pelvic organs, presenting with dysmenorrhea, menorrhagia, pelvic pain, and infertility. 1 Ectopic endometrium can occur in the abdominal wall in women as a result of obstetric or gynecologic procedures and also following laparoscopic or other surgical procedures involving the umbilicus (secondary umbilical endometriosis). Primary umbilical endometriosis (PUE) is the presence of ectopic endometrial tissue in the umbilicus in the absence of previous surgical procedure in that area. PUE is rare and its pathogenesis is unclear. 2 3 4 This is a report of a rare case of PUE in a 16-year-old female that presented as a painful, dark-colored nodule in the umbilicus. Case Report A 16-year-old female patient presented with a painful umbilical lump for a period of 3 months. The lump had been gradually enlarging and was very painful and itchy. It did not respond to two courses of antibiotics, topical steroids, and topical antifungal treatment prescribed by her general practitioner.

Primary umbilical endometriosis (PUE) is the presence of ectopic endometrial tissue in the umbilicus in the absence of previous surgical procedure in that area. PUE is rare and its pathogenesis is unclear. 2 3 4 This is a report of a rare case of PUE in a 16-year-old female that presented as a painful, dark-colored nodule in the umbilicus. Case Report A 16-year-old female patient presented with a painful umbilical lump for a period of 3 months. The lump had been gradually enlarging and was very painful and itchy. It did not respond to two courses of antibiotics, topical steroids, and topical antifungal treatment prescribed by her general practitioner. She had many medical problems including learning difficulties with autistic spectrum disorder due to a mutation in the TBL1XR1 gene, precocious puberty, obesity (body mass index of 35.4 kg/m 2 ), constipation, menorrhagia, and night time bedwetting due to presumed bladder overactivity. She was under endocrinology and urology team review and was treated with Depo-Provera for her menorrhagia. Due to excessive weight gain, Depo-Provera was discontinued and replaced with tranexamic acid. On physical examination, she had a firm, dark, painful irreducible swelling, 2 cm in diameter, in the umbilicus.

She had many medical problems including learning difficulties with autistic spectrum disorder due to a mutation in the TBL1XR1 gene, precocious puberty, obesity (body mass index of 35.4 kg/m 2 ), constipation, menorrhagia, and night time bedwetting due to presumed bladder overactivity. She was under endocrinology and urology team review and was treated with Depo-Provera for her menorrhagia. Due to excessive weight gain, Depo-Provera was discontinued and replaced with tranexamic acid. On physical examination, she had a firm, dark, painful irreducible swelling, 2 cm in diameter, in the umbilicus. An abdominal X-ray showed a nonspecific bowel gas pattern in the large and small bowel with no features of bowel obstruction. Ultrasonography showed a 2 × 1.5 cm heterogeneous echogenic material within the umbilicus. There was no visible bowel loop or peristalsis within the mass. The appearances were thought to be consistent with an umbilical hernia with narrow neck, possibly containing mesentery or intra-abdominal fat. The lesion was not reducible on examination ( Fig. 1). Fig. 1 Ultrasonography of the umbilicus demonstrated a 2 × 1.5 cm heterogeneous echogenic material within the umbilicus. The provisional clinical diagnosis was irreducible umbilical hernia with possible strangulated fatty tissue within the hernia sac, and therefore surgical exploration of the umbilicus was performed under general anesthesia with a subumbilical incision.

Fig. 1 Ultrasonography of the umbilicus demonstrated a 2 × 1.5 cm heterogeneous echogenic material within the umbilicus. The provisional clinical diagnosis was irreducible umbilical hernia with possible strangulated fatty tissue within the hernia sac, and therefore surgical exploration of the umbilicus was performed under general anesthesia with a subumbilical incision. During exploration, a dark, firm nodule was found within the umbilicus. The nodule was excised with its surrounding tissues consisting of skin, fat, and fascia. Repair of the underlying fascia and peritoneum was performed. Histopathological examination revealed skin and fatty tissue with dense fibrosis. There were several glandular structures lined by simple columnar epithelium and surrounded by stroma, resembling endometrial stroma. Mild inflammatory changes were found in the surrounding tissue. No granulomas were seen ( Fig. 2 ). Fig. 2 ( A , B ) Histology of the umbilical lesion showing dense fibrosis and glandular structures lined by simple columnar epithelium together with endometrial stroma. CD10 immunostaining confirmed the presence of endometrial stroma, therefore confirming the diagnosis of endometriosis ( Fig. 3 ). Perl's staining showed few hemosiderin laden macrophages. Fig. 3 Positive CD10 immunostaining confirmed the presence of endometrial stroma. At 6 months follow-up after the surgery, the patient was asymptomatic with complete healing of the umbilical wound with good cosmetic outcome. She continues to be followed up by the endocrinology team for her weight and menorrhagia. Discussion

Fig. 3 Positive CD10 immunostaining confirmed the presence of endometrial stroma. At 6 months follow-up after the surgery, the patient was asymptomatic with complete healing of the umbilical wound with good cosmetic outcome. She continues to be followed up by the endocrinology team for her weight and menorrhagia. Discussion Endometriosis is defined as the presence of endometrial tissue outside the uterus. The pathogenesis of endometriosis is not known. Possible causes for the development of endometriosis include direct spread, retrograde menstruation, coelomic metaplasia, embryonal rest, and lymphatic or hematogenous spread. Umbilical endometriosis is a rare entity with an estimated incidence of ∼0.5 to 1% of all cases of extragenital endometriosis. 4 However, PUE is an even rarer disorder with very few reported cases in the literature. It has not been reported in a patient as young as our patient (16 years). The pathogenesis of PUE is not clear. Possible mechanisms include the migration of endometrial cells to the umbilicus through the abdominal cavity, through the lymphatic system, or through the embryonic remnants in the umbilical fold such as the urachus and the umbilical vessels. 5 6 Another possibility is the phenomenon of “coelomic metaplasia.”

ot clear. Possible mechanisms include the migration of endometrial cells to the umbilicus through the abdominal cavity, through the lymphatic system, or through the embryonic remnants in the umbilical fold such as the urachus and the umbilical vessels. 5 6 Another possibility is the phenomenon of “coelomic metaplasia.” Secondary umbilical endometriosis following laparoscopic surgery could result from direct seeding of the endometrium into an umbilical port scar. 7 8 Our patient had no history of laparoscopic surgery in the past, but she had a history of menorrhagia. Whether or not this had any relation to the umbilical endometriosis is unknown. The menorrhagia was treated with Depo-Provera and tranexamic acid. We are not aware of any relationship between the use of these drugs and the development of endometriosis. Women of reproductive age with umbilical endometriosis present with painful, firm pigmented umbilical nodules associated with cyclic bleeding or discharge during menstruation. The nodule may have a brown, blue, or dark discoloration. 4 There are reported cases where pain may not be associated with menstruation or “cyclic” but “constant” as in our patient. Umbilical endometriosis should be considered in differential diagnosis of a painful umbilical lesion. Other possibilities include incarcerated hernia, umbilical granuloma, abscess, omphalomesenteric or urachal remnant, and a metastatic malignant lesion.

Women of reproductive age with umbilical endometriosis present with painful, firm pigmented umbilical nodules associated with cyclic bleeding or discharge during menstruation. The nodule may have a brown, blue, or dark discoloration. 4 There are reported cases where pain may not be associated with menstruation or “cyclic” but “constant” as in our patient. Umbilical endometriosis should be considered in differential diagnosis of a painful umbilical lesion. Other possibilities include incarcerated hernia, umbilical granuloma, abscess, omphalomesenteric or urachal remnant, and a metastatic malignant lesion. Imaging modalities such as ultrasonography, computed tomography scan, and magnetic resonance imaging (MRI) are not helpful in establishing a definitive diagnosis of umbilical endometriosis. Ultrasonography can provide some information about the size of the nodule and its adherence to the surrounding tissues. 6 9 For preoperative planning for the excision of the umbilical nodule, MRI is the most accurate method used to assess the depth, especially in relation to the fascial sheath and the peritoneum. This was not performed in this case as the working diagnosis was an umbilical hernia, and umbilical endometriosis was not suspected. Management of PUE with hormonal therapy using progesterone, danazol, norethisterone, and gonadotropin-releasing hormone analogues has not shown reliable results. However, there are reports of success in relieving symptoms and reducing the size of the endometrial nodule using medical hormonal treatment. 10

Imaging modalities such as ultrasonography, computed tomography scan, and magnetic resonance imaging (MRI) are not helpful in establishing a definitive diagnosis of umbilical endometriosis. Ultrasonography can provide some information about the size of the nodule and its adherence to the surrounding tissues. 6 9 For preoperative planning for the excision of the umbilical nodule, MRI is the most accurate method used to assess the depth, especially in relation to the fascial sheath and the peritoneum. This was not performed in this case as the working diagnosis was an umbilical hernia, and umbilical endometriosis was not suspected. Management of PUE with hormonal therapy using progesterone, danazol, norethisterone, and gonadotropin-releasing hormone analogues has not shown reliable results. However, there are reports of success in relieving symptoms and reducing the size of the endometrial nodule using medical hormonal treatment. 10 Surgical exploration and excision of the nodule should be considered in patients with a symptomatic PUE. The surgical technique should include total excision of the endometriosis lesion, with an adequate rim of normal tissue to avoid local recurrence. It may be necessary to repair the underlying fascia and peritoneum as in our patient. In a patient with PUE, laparoscopy to exclude pelvic endometriosis is a matter of debate. Some authors say it should be avoided in asymptomatic patients as there is a potential risk of introducing endometriosis into the pelvic cavity, 11 while others are in favor of simultaneous laparoscopic exploration to exclude possible further foci of intra-abdominal endometriosis. They argue that pelvic endometriosis cannot be definitively excluded on transvaginal ultrasound or clinical examination. 12 13

isk of introducing endometriosis into the pelvic cavity, 11 while others are in favor of simultaneous laparoscopic exploration to exclude possible further foci of intra-abdominal endometriosis. They argue that pelvic endometriosis cannot be definitively excluded on transvaginal ultrasound or clinical examination. 12 13 Malignant transformation is estimated to occur in 0.6 to 0.8% of cases of ovarian endometriosis. 14 Malignant transformation of umbilical endometriosis has only rarely been reported in the literature. The first reported case of adenocarcinoma of umbilical endometriosis was in 1972. 15 Subsequently in 2013, Obata et al reported a case of clear cell adenocarcinoma arising from umbilical endometriosis in a 60-year-old lady. 16 Koguchi-Yoshioka et al reported a primary adenocarcinoma arising from umbilical endometriosis in a 45-year-old lady in 2016. 14 Conclusion PUE is a rare presentation of endometriosis. Complete excision and histology are highly recommended to obtain a definitive diagnosis, exclude malignancy, and prevent recurrence. Laparoscopy to exclude pelvic endometriosis in patients with PUE is debatable. New Insights & the Importance for the Pediatric Surgeon Primary umbilical endometriosis should be considered in differential diagnosis in adolescent girls presented with painful umbilical lesion. Complete excision is prerequisite to prevent recurrence. Conflict of Interest None.

Introduction African traditional medicine often includes the use of herbal or home-made enemas with a reported use of 89% in babies under 3 months of age in a study in Kwazulu-Natal. 1 The motivations for enema use vary in different cultures—from giving newborns enemas to “cleanse their meconium,” to treating constipation and administering herbal remedies that are too bitter to take orally. 1 At-home enema use is still very popular, even though serious health risks have been described, such as infections, dehydration, and injury to the rectal wall. 1 We present two cases that illustrate how extensive and potentially incapacitating these injuries can be. The aim of the study is to describe two procedures, derived from the posterior sagittal anorectoplasty (PSARP) for anorectal malformations and the pull-through for Hirschsprung's disease to address the long-term sequelae of traditional enema injuries. Case Report 1: Caustic Enema Injury A 2-year-old girl presented with a 2-day history of perianal swelling and foul-smelling diarrhea post enema administration. The enema administration was initially denied and the ingredients of the herbal enema are unknown. Clinical examination revealed an acute abdomen and perianal discoloration with pus discharging from the anus. The white cell count was 9.89 × 10 9 /L and the C reactive protein was 3; and the child had a normal renal function.

nema administration was initially denied and the ingredients of the herbal enema are unknown. Clinical examination revealed an acute abdomen and perianal discoloration with pus discharging from the anus. The white cell count was 9.89 × 10 9 /L and the C reactive protein was 3; and the child had a normal renal function. The child was taken to theater where examination of the perineum and anus revealed necrotic perianal skin and dentate line. At laparotomy, a necrotic rectum and sigmoid were identified without any signs of perforation ( Fig. 1 ). An end colostomy was brought out at the descending colon and the necrotic colon and rectum removed transanally. Packs were left in situ and the patient had a planned relook in 48 hours. At relook, further debridement of slough and necrotic muscle was performed ( Fig. 2 ). Histology showed transmural necrosis and serositis; no infection and ganglion cells were present throughout. Eight months post the diverting colostomy, the child had a completely closed anal opening with perineal scarring. The decision was made to perform a pull-through of the descending colostomy using a PSARP approach. A total body preparation was performed. Using a lower midline laparotomy, the stoma was taken down and the transverse colon was mobilized. Due to the extensive inflammatory reaction in the pelvic area, an iatrogenic posterior wall vaginal tear was caused during the dissection. The vaginal tear was located and repaired. After completing the pelvic dissection, the patient was positioned prone. Good muscular contractions were noted with stimulation (areas of sphincters marked) and a limited posterior sagittal incision was performed and deepened. The patient was subsequently moved back into supine position and the stoma end was pulled through the posterior sagittal incision. The anoplasty was performed after reconstruction of the muscle complex and perineal body. A Malone Antegrade Continence Enema (MACE) was fashioned in the right iliac fossa. A protective loop ileostomy was also brought out and reversed a month later, at the age of 3 years. The patient recovered uneventfully postoperatively and was discharged home to be followed up at the anorectal and stoma clinic. She was temporarily lost to follow-up and later represented with a complete stricture of the MACE. She was transitioned to rectal washout and is currently clean.

later, at the age of 3 years. The patient recovered uneventfully postoperatively and was discharged home to be followed up at the anorectal and stoma clinic. She was temporarily lost to follow-up and later represented with a complete stricture of the MACE. She was transitioned to rectal washout and is currently clean. Fig. 1 A 2-year-old during the first laparotomy post herbal enema administration—a necrotic rectum and sigmoid identified without any signs of perforation. Fig. 2 A 2-year-old at relook of the perineum for removal of packs 48 hours post laparotomy, resection of necrotic rectum, and sigmoid colon and end colostomy. Case Report 2: Hot-Water Enema Injury

Fig. 1 A 2-year-old during the first laparotomy post herbal enema administration—a necrotic rectum and sigmoid identified without any signs of perforation. Fig. 2 A 2-year-old at relook of the perineum for removal of packs 48 hours post laparotomy, resection of necrotic rectum, and sigmoid colon and end colostomy. Case Report 2: Hot-Water Enema Injury A 21-month-old male presented to another institution after a hot-water enema was administered for constipation. The child was fluid resuscitated and taken to theater the following day for an examination under anesthesia (EUA). He was found to have a 2 cm circumferential full-thickness burn, proximal to the dentate line, but involving the sphincter muscles and a left-sided abdominal mass was palpated. A laparotomy was therefore performed. No contamination in the peritoneal cavity was noted but there was severe inflammation of the rectum (up to the rectosigmoid junction) with areas of patchy necrosis. The mass palpated was found to be a pelvic kidney. An end colostomy was brought out. The patient was discharged 9 days post colostomy and referred to our center. At EUA, a rectal stricture was identified 4 cm proximal to the dentate line and a long colonic/rectal stricture was later confirmed on a contrast enema ( Fig. 3 ). The patient underwent a transanal pull-through 7 months after the diverting colostomy. A full body preparation was performed and the child was positioned prone in jack knife decubitus. A lone star was positioned and circumferential stay sutures were positioned 1 cm proximal to the dentate line. A Swenson-type dissection of the rectum and distal colon was performed transanally. The child was then positioned prone and the abdomen was entered through the old laparotomy wound. The Hartmann colostomy was taken down and the left colic vessels ligated (relying on the middle colic to provide blood supply) to achieve sufficient colonic length for the pull-through procedure. The distal bowel was mobilized and resected, the stoma was pulled through on the left, and a double layer coloanal anastomosis was fashioned. He recovered well after surgery and is now growing well and fully continent to stools.

provide blood supply) to achieve sufficient colonic length for the pull-through procedure. The distal bowel was mobilized and resected, the stoma was pulled through on the left, and a double layer coloanal anastomosis was fashioned. He recovered well after surgery and is now growing well and fully continent to stools. Fig. 3 Contrast enema post hot-water enema administration showing a rectal stricture with total cutoff at the sacral promontory. Discussion In this report, we present two cases of unusual and devastating injuries caused by home enema administration. Both cases required emergency surgical intervention with local debridement and stool diversion. Ultimately, they underwent repair applying the principles used for the Swenson endoanal pull-through and the PSARP.

n this report, we present two cases of unusual and devastating injuries caused by home enema administration. Both cases required emergency surgical intervention with local debridement and stool diversion. Ultimately, they underwent repair applying the principles used for the Swenson endoanal pull-through and the PSARP. There are a few peculiar challenges that have been identified when operating on these cases. Acutely, a crucial step in the management of these children is fecal diversion. This needs to be performed as an emergency, to avoid peritoneal soiling secondary to possible perforation of the necrotic rectosigmoid and to avoid perineal necrotizing fasciitis. 2 3 The colostomy allows for the resolution of any sepsis before the definitive procedure can be performed. Following our experience with these two cases, in which severe or complete strictures of the anorectum have been observed, we advocate for a diverting colostomy, with a distal mucus fistula (if the bowel is not completely resected). A mucous fistula prevents a formation of a blind loop, should a stricture develop and it enables to later perform a distal colostogram to assess the condition and length of the distal bowel, if required. Once the perineal and pelvic inflammatory reaction has settled, the patient might be amenable for definitive repair. Our patients had definitive repair at 7- and 8-month post diversion, but it is reasonable to think that 3 to 6 months post trauma could be an adequate time for the inflammation to resolve. A preoperative EUA could help assess the timing of the definitive repair.

led, the patient might be amenable for definitive repair. Our patients had definitive repair at 7- and 8-month post diversion, but it is reasonable to think that 3 to 6 months post trauma could be an adequate time for the inflammation to resolve. A preoperative EUA could help assess the timing of the definitive repair. We believe that counseling the parents extensively plays a crucial role before the reconstructive surgery. The families need to be aware of the risks of fecal incontinence to adjust their expectations and to be prepared for the need for daily washouts to keep the children mechanically clean. Regular follow-up is also very important after reconstructive surgery but it can be quite challenging in our setting mainly due to socioeconomic reasons such as lack of money for transport. Both our patients had poor prognostic factors for achieving fecal continence because of the involvement of their anal sphincters, the dentate line, and the extensive fibrosis in the area. Due to the significantly high risk of incontinence in the first case, compounded by absence of a rectum as a reservoir, a decision was made to fashion a MACE at the time of reconstruction.

ing fecal continence because of the involvement of their anal sphincters, the dentate line, and the extensive fibrosis in the area. Due to the significantly high risk of incontinence in the first case, compounded by absence of a rectum as a reservoir, a decision was made to fashion a MACE at the time of reconstruction. The two techniques we used were derived from the Swenson type endoanal pull-through in the one case and a PSARP in the other case. Both these procedures are usually done in the neonatal period or in infancy. Operating on an older child has the disadvantage of a more rigid pelvis and therefore more difficult mobilization. 4 A thickened mesentery, inflamed mucosa, and a long-standing dilated colon have also been described as causes of some difficulties. 5 In our cases, the difficulty was further increased as we had to operate in a field with an enormous amount of scar tissue secondary to the extensive inflammatory reaction. This challenge has been reported by other authors when dealing with redo pull-through surgery. 6 Both procedures were successfully completed and both patients recovered uneventfully. It is important to highlight the fact that these injuries are completely preventable and unacceptable. Public awareness with regard to the dangers and complications of at-home-enema use needs to be prioritized. This can be done tactfully, without disrespecting any cultural beliefs. Engaging and understanding the motivations for the continued use of these enemas could help in offering acceptable alternative solutions.

e. Public awareness with regard to the dangers and complications of at-home-enema use needs to be prioritized. This can be done tactfully, without disrespecting any cultural beliefs. Engaging and understanding the motivations for the continued use of these enemas could help in offering acceptable alternative solutions. Conclusion The potential complications associated with the practice of administering at-home enemas are devastating. The first step would be to prevent these injuries by creating public awareness with regard to the potential dangers. When extensive rectal and perineal disruption is encountered, a diverting stoma needs to be performed. Fibrosis and severe scarring can be expected secondary to the severe inflammation. Although challenging, a full-thickness transanal pull-through and a PSARP have been proven to be successful techniques in patients who have suffered rectal burns due to traditional enemas. Fecal incontinence needs to be expected and addressed properly in these patients. New Insights & the Importance for the Pediatric Surgeon It is important to know the different surgical options that are available when children present with extensive injuries to the perineum and rectum. Conflict of Interest None.

Introduction Male circumcision is one of the commonest surgical procedures practiced in children worldwide. According to several studies, almost a third of the world male population is circumcised. 1 2 A complication rate of up to 16% has been reported. 1 Bladder rupture is a very rare and life-threatening complication of such a procedure. In a review of literature, only three such cases were reported with this being the fourth. 3 4 5 We would like to report on a 7-day-old patient that suffered from an intraperitoneal bladder rupture following neonatal male circumcision. Case Report Our patient presented to our emergency department on the seventh day of life with severe abdominal distension and vomiting. A thorough history revealed that the baby was circumcised on the second day of life following an uncomplicated cesarian section delivery. The exact mode of circumcision was unknown to the parents. After this the baby was found to be fussy and constantly crying and was no longer tolerating his feeds. He then started to become lethargic and show progressive abdominal distension. The baby was transferred to a neonatal unit with a provisional diagnosis of neonatal sepsis. Three days later he was referred to our center to assess and manage his ongoing abdominal distension.

crying and was no longer tolerating his feeds. He then started to become lethargic and show progressive abdominal distension. The baby was transferred to a neonatal unit with a provisional diagnosis of neonatal sepsis. Three days later he was referred to our center to assess and manage his ongoing abdominal distension. General examination revealed an elevated temperature of 38°C, pulse of 155, and a blood pressure of 70/38. The abdomen was diffusely enlarged with prominent dilated veins, a large protuberant umbilical hernia, and mild erythema. There was no tenderness on palpation. It was dull all over on percussion with the evidence of a transmitted thrill. The baby was passing stool normally and there were no bilious aspirates when an nasogastric tube was placed. Careful examination of the site of circumcision revealed that the glans penis was ischemic with signs of inflammation at the shaft ( Fig. 1 ). Laboratory investigations revealed an elevated serum creatinine level of 2.3 mg/dL, C-reactive protein of 96 mg/L, hyperkalemia (5.7 mMol/L), hyponatremia (122 mMol/L), and an elevated leucocyte count of 15,000/mm 3 . Fig. 1 Clinical picture of the baby at first presentation. Notice the gross distended abdomen, dilated abdominal veins, mild erythema of the abdominal wall and dry gangrene of the distal glans of the penis.

General examination revealed an elevated temperature of 38°C, pulse of 155, and a blood pressure of 70/38. The abdomen was diffusely enlarged with prominent dilated veins, a large protuberant umbilical hernia, and mild erythema. There was no tenderness on palpation. It was dull all over on percussion with the evidence of a transmitted thrill. The baby was passing stool normally and there were no bilious aspirates when an nasogastric tube was placed. Careful examination of the site of circumcision revealed that the glans penis was ischemic with signs of inflammation at the shaft ( Fig. 1 ). Laboratory investigations revealed an elevated serum creatinine level of 2.3 mg/dL, C-reactive protein of 96 mg/L, hyperkalemia (5.7 mMol/L), hyponatremia (122 mMol/L), and an elevated leucocyte count of 15,000/mm 3 . Fig. 1 Clinical picture of the baby at first presentation. Notice the gross distended abdomen, dilated abdominal veins, mild erythema of the abdominal wall and dry gangrene of the distal glans of the penis. The baby was immediately admitted and resuscitation was performed. An abdominal X-ray failed to show any free air in the abdomen and a concomitant abdominal ultrasound showed marked free fluid with internal echoes and bilateral moderate hydronephrosis. A provisional diagnosis of intraperitoneal bladder rupture was made based on this data and a conservative approach was decided. An abdominal drain was placed in the operating room under general anesthesia yielding >500ccs of yellowish turbid fluid. A urinary catheter was placed to drain the bladder; however, catheterization was difficult due to involvement of the distal part of the penile urethra.

s data and a conservative approach was decided. An abdominal drain was placed in the operating room under general anesthesia yielding >500ccs of yellowish turbid fluid. A urinary catheter was placed to drain the bladder; however, catheterization was difficult due to involvement of the distal part of the penile urethra. The baby remained under conservation for 2 days after which a retrograde cystogram was performed through the catheter revealing leakage of contrast from the bladder near the trigone into the peritoneum ( Fig. 2 ). An open exploration was decided upon through a Pfannenstiel incision. Due to the close proximity of the perforation to the ureters, we decided to approach from within the bladder to allow us to cannulate the ureters and avoid injuring them during the repair ( Fig. 3 ). Fig. 2 Ascending cytogram (lateral view) showing an intraperitoneal leakage of contrast from the dome of the bladder posteriorly close to the trigone. Fig. 3 Intraoperative view. The bladder is bivalved (opened), the ureters are cannulated and the perforation is evident.

The baby remained under conservation for 2 days after which a retrograde cystogram was performed through the catheter revealing leakage of contrast from the bladder near the trigone into the peritoneum ( Fig. 2 ). An open exploration was decided upon through a Pfannenstiel incision. Due to the close proximity of the perforation to the ureters, we decided to approach from within the bladder to allow us to cannulate the ureters and avoid injuring them during the repair ( Fig. 3 ). Fig. 2 Ascending cytogram (lateral view) showing an intraperitoneal leakage of contrast from the dome of the bladder posteriorly close to the trigone. Fig. 3 Intraoperative view. The bladder is bivalved (opened), the ureters are cannulated and the perforation is evident. Two pathological perforations were appreciated near the trigone between the ureteric orifices. These were debrided and closed in two layers with 4/0 polygalactine suture. The bladder was then closed over a suprapubic self-retaining catheter. An abdominal toilet was done, a drain was placed, and the incision was closed. A clear line of demarcation was visible at the time of surgery between the gangrenous and healthy penile tissue as almost a week had passed since the initial injury. This prompted us to perform debridement of the dead tissue to avoid sending the patient for a second general anesthetic.

ced, and the incision was closed. A clear line of demarcation was visible at the time of surgery between the gangrenous and healthy penile tissue as almost a week had passed since the initial injury. This prompted us to perform debridement of the dead tissue to avoid sending the patient for a second general anesthetic. The baby was transferred to the surgical neonatal intensive care unit where he remained on broad-spectrum antibiotics for 3 days. Oral feeds were commenced on day 2. On day 5 postoperatively, a contrast study was done through the suprapubic catheter revealing absence of any leakage. The suprapubic catheter was then clamped. When the baby was voiding normally the catheter was removed. The baby was discharged on the seventh postoperative day. Discussion

The baby was transferred to the surgical neonatal intensive care unit where he remained on broad-spectrum antibiotics for 3 days. Oral feeds were commenced on day 2. On day 5 postoperatively, a contrast study was done through the suprapubic catheter revealing absence of any leakage. The suprapubic catheter was then clamped. When the baby was voiding normally the catheter was removed. The baby was discharged on the seventh postoperative day. Discussion Neonatal bladder rupture is a rare problem that has been reported secondary to several different pathologies. The most common of these is urinary tract obstruction due to a posterior urethral valve or anterior urethral valve. 6 7 It has also been reported on as a possible complication of umbilical catheterization 8 and severe neonatal urinary tract infection (UTI). 9 Only three cases of bladder rupture following circumcision have been reported in literature till today. Two of these were due to circumcision with a Plastibell device where the urinary tract had become obstructed due to migration and trapping of the inner perpetual skin beneath it. 3 4 The third case was in a 2-year-old child and had occurred when deep hemostasis sutures were taken following circumcision to control bleeding and the surgeon had inadvertently damaged the distal subcoronal urethral causing urinary tract obstruction. 5

to migration and trapping of the inner perpetual skin beneath it. 3 4 The third case was in a 2-year-old child and had occurred when deep hemostasis sutures were taken following circumcision to control bleeding and the surgeon had inadvertently damaged the distal subcoronal urethral causing urinary tract obstruction. 5 In our patient, the situation was a little different. The circumcision was performed by a traditional practitioner employing mostly a bone cutter. Our hypothesis is that the distal end of the glans had become caught in the device causing a crush injury to it and the most distal part of the urethra and this wasn't managed correctly unfortunately. This caused an acute urinary retention that was missed on initial examination and ultimately led to pressure build up and bladder rupture.

hat the distal end of the glans had become caught in the device causing a crush injury to it and the most distal part of the urethra and this wasn't managed correctly unfortunately. This caused an acute urinary retention that was missed on initial examination and ultimately led to pressure build up and bladder rupture. Our initial management was conservative through catheterization of the bladder per urethra and drainage of the urinary ascites after proper patient resuscitation. The decision was made for exploration due to a worsening general condition and the retrograde cytogram that demonstrated an intraperitoneal leak. Both modalities of treatment have been proposed to manage intraperitoneal bladder rupture; however, the inclination is usually toward a formal surgical repair especially when there is a deterioration of the patient's general condition. Our management was in accordance with published guidelines from the consensus on genitourinary trauma which underlined that neonatal bladder trauma usually requires surgical management. 10 In addition, published case reports on similar conditions ultimately resorted to surgical intervention to as a course of management. 2 3 4 5 6 7 8 9

s in accordance with published guidelines from the consensus on genitourinary trauma which underlined that neonatal bladder trauma usually requires surgical management. 10 In addition, published case reports on similar conditions ultimately resorted to surgical intervention to as a course of management. 2 3 4 5 6 7 8 9 Most authors report that an open approach but some have advocated for a laparoscopic approach to repair bladder ruptures. 6 In any case, the repair is performed from outside the bladder. In our patient, we opted to bivalve the bladder and perform the repair from inside due to the close proximity of the perforation to the ureters and fear of the repair leading to ureteric obstruction. This approach allowed us to identify and cannulate both ureters in addition to performing a mucosal and seromuscular repair of the bladder. Long-term monitoring of patients where suturing in done near the trigone is vital as such patients are at a higher risk of long-term bladder dysfunction. 11 We were unable to do so in this patient unfortunately as he was lost to follow-up.

Most authors report that an open approach but some have advocated for a laparoscopic approach to repair bladder ruptures. 6 In any case, the repair is performed from outside the bladder. In our patient, we opted to bivalve the bladder and perform the repair from inside due to the close proximity of the perforation to the ureters and fear of the repair leading to ureteric obstruction. This approach allowed us to identify and cannulate both ureters in addition to performing a mucosal and seromuscular repair of the bladder. Long-term monitoring of patients where suturing in done near the trigone is vital as such patients are at a higher risk of long-term bladder dysfunction. 11 We were unable to do so in this patient unfortunately as he was lost to follow-up. Neonatal circumcision remains a controversial subject to this day. Many studies have been designed to assess the advantages and disadvantages of this practice. Recently the role of neonatal male circumcision in the reduction in the risk of UTIs has been emphasized. 12 Furthermore, it has been shown that neonatal circumcision if performed under the appropriate controlled conditions has a much lower risk of short-term complications than if performed at an older age. The risk of additional surgery is in fact almost 1% if circumcision was initially performed after the neonatal period. 13

urthermore, it has been shown that neonatal circumcision if performed under the appropriate controlled conditions has a much lower risk of short-term complications than if performed at an older age. The risk of additional surgery is in fact almost 1% if circumcision was initially performed after the neonatal period. 13 However, this statement isn't supported by many. The relationship between circumcision and the development of UTIs according to several authors is only established in those with a predisposition to the condition such as those boys with PUJ (pelviureteric junction) obstruction, high-grade vesicoureteral reflux, and hydronephrosis. 14 This is why they feel that routine nontherapeutic practice of circumcision should be restricted to those subgroups of patients. To add to this point, it was found that the risk rate for the development of meatal stenosis and other urethral stricture diseases was higher in religious and cultural ethnicities that practiced nontherapeutic circumcision. This greatly adds to the health service burden. 15 It is our message that although circumcision is performed routinely in many cultures, it can still lead to catastrophic and life-threatening problems adding a considerable cost to the already taxed health services especially in low- and middle-income countries. It cannot be stressed enough that this should only be performed by a trained professional under safe, sterile circumstances and where a proper follow-up can be provided to detect and manage any complications as early as possible should they occur.

dy taxed health services especially in low- and middle-income countries. It cannot be stressed enough that this should only be performed by a trained professional under safe, sterile circumstances and where a proper follow-up can be provided to detect and manage any complications as early as possible should they occur. New Insights & the Importance for the Pediatric Surgeon Circumcision is one of the most practiced pediatric surgery procedures. This is the fourth reported case of bladder rupture as a complication to this procedure worldwide. Conflict of Interest None.

Introduction Congenital posterolateral diaphragmatic hernia (CDH) or Bochdalek hernia (BH) is a life-threatening malformation characterized by an anomaly of the posterolateral foramina of Bochdalek, through which the abdominal viscera migrate into the chest during fetal development.1 The incidence of CDH is 1 in 2,500 to 3,000 newborns.2 3 Vertebrates exhibit evolutionarily conserved asymmetries in the pattern of internal organ placement, which are essential for normal physiological function. Left–right asymmetries in organ situs are dependent on the formation of an intact left–right axis during embryogenesis. Alterations in the classic asymmetries (situs solitus) may occur as a complete mirror image reversal of all of the organs (situs inversus totalis), or an incomplete reversal resulting in a wide spectrum of intermediate defects (situs ambiguus), including heterotaxy (reversal of individual organs along the left–right axis) and isomerism (duplication or changes in normal symmetry).

ur as a complete mirror image reversal of all of the organs (situs inversus totalis), or an incomplete reversal resulting in a wide spectrum of intermediate defects (situs ambiguus), including heterotaxy (reversal of individual organs along the left–right axis) and isomerism (duplication or changes in normal symmetry). In situs inversus totalis, the heart is located on the right side of the chest, the left lung is trilobed and the right lung is bilobed, and the stomach and spleen occupy the right side, while the liver and gallbladder are present on the left side of the abdomen and the intestine is transposed.3 The situs inversus (SI) condition has an estimated occurrence of 1 in 5,000 to 20,000 births and is either undiagnosed or is diagnosed incidentally during the investigation of another associated aliment.4 5 One-fourth to one-fifth of patients with SI have a genetically heterogeneous, autosomal-recessive disorder known as primary ciliary dyskinesia (PCD), a dysfunction of the cilia that manifests during embryologic development.3 6 Normal functioning cilia are critical to the embryological development of proper organ laterality, and approximately 50% of PCD patients present with abnormalities of thoracoabdominal symmetry.6 These patients have Kartagener syndrome (KS), initially reported in the early 1900s, which is characterized by the triad of SI, chronic sinusitis, and bronchiectasis.7 To the best of our knowledge, this is the first report documenting a case of a right-sided BH in a patient with KS.

ities of thoracoabdominal symmetry.6 These patients have Kartagener syndrome (KS), initially reported in the early 1900s, which is characterized by the triad of SI, chronic sinusitis, and bronchiectasis.7 To the best of our knowledge, this is the first report documenting a case of a right-sided BH in a patient with KS. Case Report The report focuses on a 4.180-kg baby boy born by cesarian section from an epileptic mother, with the end of a gestation complicated by threatened abortion and polyhydramnios. Soon after birth, the boy developed acute respiratory distress and cyanosis. Physical examination revealed a distended thorax, intercostal retractions with decreased breathing sounds on the right side of the thorax, and a scaphoid abdomen. A chest radiograph revealed the presence of intestinal loops in the right pleural cavity with left displacement of the mediastinum (Fig. 1). The baby was initially stabilized by emergency endotracheal intubation and ventilated with synchronized intermittent positive pressure ventilation with the following parameters: Ti 0.3, Te 1, PEEP 4, and Fio 2 35%. After 48 hours of stabilization, the baby underwent a laparotomy through the right abdomen. The right diaphragmatic defect was exposed and was sutured with interrupted nonabsorbable sutures. During laparotomy, the liver and the gallbladder appeared in the left and the spleen in the right hypochondrium. Therefore, SI was suspected. The postoperative course was complicated by drainage of the right pneumothorax. The baby was weaned from ventilation on the 7th postoperative day. A chest X-ray (Fig. 2), echocardiography, and electrocardiograms revealed a right-sided heart; the barium meal documented greater curvature of the right-oriented stomach and the barium enema showed a mirror image of intestinal tube, thereby confirming SI. The baby was discharged on the 15th postoperative day with medical therapy for gastroesophageal reflux. During follow-up, the baby had several episodes of bronchitis and otitis. He underwent examinations to exclude cystic fibrosis, which included a sweat test and a genetic test that revealed the lack of relevant mutations. In vitro and electron microscopic ultrastructural analyses of nasal scraping respiratory cilia confirmed KS. A tympanogram revealed a flat curve, and parental history of SI was negative.

examinations to exclude cystic fibrosis, which included a sweat test and a genetic test that revealed the lack of relevant mutations. In vitro and electron microscopic ultrastructural analyses of nasal scraping respiratory cilia confirmed KS. A tympanogram revealed a flat curve, and parental history of SI was negative. Fig. 1 Chest radiograph documenting right diaphragmatic hernia. Fig. 2 Postoperative chest radiograph documenting dextrocardia.

examinations to exclude cystic fibrosis, which included a sweat test and a genetic test that revealed the lack of relevant mutations. In vitro and electron microscopic ultrastructural analyses of nasal scraping respiratory cilia confirmed KS. A tympanogram revealed a flat curve, and parental history of SI was negative. Fig. 1 Chest radiograph documenting right diaphragmatic hernia. Fig. 2 Postoperative chest radiograph documenting dextrocardia. Discussion Right-sided BH and KS are two rare disorders, with no reported association. Embryogenesis of BH has been described as a failure in the closure of the pericardioperitoneal canal by the pleuroperitoneal membranes, which occurs during gestational week 8th.8 For this reason, it is hypothesized that an early return in the coelomic cavity of the foregut results in wide posterolateral spaces and dysfunctional pleuroperitoneal membranes. Consequently, the abdominal viscera herniate into the thorax, which is thought to cause pulmonary hypoplasia by compression of the growing lung.8 The left BH is the most frequent CDH, and its preponderance with respect to the right side may be related to the early closure of the right pleuroperitoneal opening, although no theories have been postulated to explain this.9 CDH can be associated with different syndromes, such as Fryns, Donnai-Barrow, Beckwith-Wiedemann, Sympson-Golabi-Behmel, Coffin-Siris, and Denys-Drash syndromes. However, the association with KS has never been described.10 KS is characterized by the triad of SI, chronic sinusitis, and bronchiectasis and is a subset of one of the most widely recognized ciliopathies, PCD, in which ciliary motility defects occur. Particularly during embryogenesis, KS could alter normal left–right axis formation, which determines the SI. Sutherland and Ware have recently offered a detailed view of the five steps necessary to establish asymmetry in the vertebrate. SI, caused by PCD, usually results from defects in signaling at the node involving cilia structure and function.10 Three previous reports have described single laterality abnormalities in combination with a right-sided diaphragmatic defect; only one has shown an abdominal SI associated with a right-sided BH in which the karyotype was normal and the family history mentioned no other cases of laterality defects.3 Retinoic acid has been implicated in both diaphragmatic development and situs anomalies. However, no evidence exists in the literature regarding the role of retinoic acid in KS. Thus, the two malformations in the present case are considered an occasional occurrence.

tory mentioned no other cases of laterality defects.3 Retinoic acid has been implicated in both diaphragmatic development and situs anomalies. However, no evidence exists in the literature regarding the role of retinoic acid in KS. Thus, the two malformations in the present case are considered an occasional occurrence. Conclusions This is the first case of the association of right-sided BH and KS in a live patient. When dealing with an association of diaphragmatic hernia with situs inversus totalis, physicians should be mindful of the potential contribution of ciliary dyskinesia to chronic respiratory disease (particularly bronchiectasis), which might otherwise be attributed to gastroesophageal reflux disease. Multidisciplinary clinical management of both malformations with early identification and treatment is warranted. Conflict of Interest None

Introduction Congenital esophageal stenosis (CES) is a rare, yet significant entity because of its clinical manifestation and its association with tracheoesophageal fistula (TEF) or esophageal atresia (EA).1 The incidence of CES is estimated at 1 in 25,000 to 50,000 live births.2 There is associated EA in one-third of the cases; the remainder are classified as isolated CES.2 Three histopathologic types of CES have been described in the literature. Management options include dilatation, endoscopic excision, or segmental resection. Our case, a 2-day old preterm female neonate weighing 1.6 kg, underwent primary excision of the stenotic segment and esophageal reanastomosis with ultimate healing of the anastomosis. High index of suspicion of mid/lower EA on the basis of X-ray findings led us decide upon thoracotomy rather than performing stomas and a staged surgery in this preterm low birth weight neonate.

kg, underwent primary excision of the stenotic segment and esophageal reanastomosis with ultimate healing of the anastomosis. High index of suspicion of mid/lower EA on the basis of X-ray findings led us decide upon thoracotomy rather than performing stomas and a staged surgery in this preterm low birth weight neonate. Case History A 2-day-old female neonate, weighing 1.6 kg was brought to us with history of excessive frothing from the mouth. She was second born, vaginally delivered at a gestational age of 35 weeks. The pregnancy and delivery were uncomplicated. The antenatal ultrasonography was reported to be normal. On examination, thick mucoid secretions were emanating from the mouth. The baby had moderate respiratory distress, with respiratory rate of 80/min. Her cry, tone, and activity were normal. The abdomen was scaphoid. Anal opening, external genitals, and the spine were normal. Baby had passed meconium. There were no stigmata of any other congenital anomaly. X-ray chest with gentle pressure on the stiff (10 Fr) red rubber catheter inserted through mouth showed upper pouch almost upto T8 level (Fig. 1). There was no gas visible in the abdomen and pelvis (gasless abdomen). Ultrasonography of the abdomen was normal. Fig. 1 Chest X-ray showing upper pouch ending at T8. Also note the absence of gas in the abdomen and pelvis.

Case History A 2-day-old female neonate, weighing 1.6 kg was brought to us with history of excessive frothing from the mouth. She was second born, vaginally delivered at a gestational age of 35 weeks. The pregnancy and delivery were uncomplicated. The antenatal ultrasonography was reported to be normal. On examination, thick mucoid secretions were emanating from the mouth. The baby had moderate respiratory distress, with respiratory rate of 80/min. Her cry, tone, and activity were normal. The abdomen was scaphoid. Anal opening, external genitals, and the spine were normal. Baby had passed meconium. There were no stigmata of any other congenital anomaly. X-ray chest with gentle pressure on the stiff (10 Fr) red rubber catheter inserted through mouth showed upper pouch almost upto T8 level (Fig. 1). There was no gas visible in the abdomen and pelvis (gasless abdomen). Ultrasonography of the abdomen was normal. Fig. 1 Chest X-ray showing upper pouch ending at T8. Also note the absence of gas in the abdomen and pelvis. Suspecting an unusual type of atresia of the esophagus, we chose to surgically intervene. A right thoracotomy was done through sixth intercostal space (transpleural approach). The upper pouch was dilated to 1.5-cm diameter and in-continuity with the lower pouch (Fig. 2). The lower pouch was grossly thickened with wall thickness of 4 to 5 mm. There was stenotic segment of approximately 4 to 5 mm between the two pouches, which was excised. No fistula was identified. Spatulation of the lower pouch was done to match the diameter of the upper pouch. A single layer, end to oblique esophago–esophageal anastomosis was done using 5–0 Polyglactin over a nasally placed 6 Fr infant feeding tube. There was no tension at the suture line. The thoracic cavity was drained by intercostal tube drainage, and the incision was closed in layers.

tch the diameter of the upper pouch. A single layer, end to oblique esophago–esophageal anastomosis was done using 5–0 Polyglactin over a nasally placed 6 Fr infant feeding tube. There was no tension at the suture line. The thoracic cavity was drained by intercostal tube drainage, and the incision was closed in layers. Fig. 2 Operative photograph showing dilated upper pouch, in-continuity with the thickened lower pouch. No fistula is noted. Postoperatively, intravenous antibiotics, regular chest physiotherapy, and normal saline nebulization were given. A radiocontrast study using water-soluble dye was done on the 7th postoperative day. There was no anastomotic leak and the contrast flowed freely across the anastomosis into the stomach within seconds (Fig. 3). Feeds were started through the feeding tube initially and gradually advanced to full orals. The infant feeding tube was removed on 9th postoperative day. Intercostal drain was taken out on the 10th postoperative day. Overall, there was no complication. The histopathology of the excised stenotic segment showed smooth muscle hypertrophy. Presently, at a follow-up age of 10 months, the baby is thriving well and has normal milestones for her age. Barium study at 10 months shows a widely patent anastomosis and absence of any gastroesophageal reflux. Fig. 3 Contrast study showing a widely patent anastomosis and no anastomotic leak.

The histopathology of the excised stenotic segment showed smooth muscle hypertrophy. Presently, at a follow-up age of 10 months, the baby is thriving well and has normal milestones for her age. Barium study at 10 months shows a widely patent anastomosis and absence of any gastroesophageal reflux. Fig. 3 Contrast study showing a widely patent anastomosis and no anastomotic leak. Discussion CES is a rare, yet significant entity because of its clinical manifestation and its association with TEF or EA (EA).1 In stenosis, there is obliteration of the esophageal lumen. Stenosis results from abnormal rests of respiratory tissue in the esophageal wall or fibromuscular hypertrophy. As per the popularly used Gross classification, esophageal stenosis, that is, Type F of EA along with Types B, D, and G that are rarer varieties, together constitutes 3 to 5% of all EAs, thus defining the rarity of the condition.3 4 The clinical presentation of CES is varied, and a high index of suspicion is essential to arrive at an early diagnosis.5 Management depends on the type and severity of the stenosis. Stenosis due to tracheobronchial remnants requires segmental resection. Fibromuscular stenosis may respond to dilatation, but severe degrees of stenosis require segmental excision. Membranous diaphragms usually respond to dilatation or may require endoscopic excision.5 Three cases of CES of different anatomical varieties involving the lower third of esophagus have been described by Ramesh et al.5 They have done segmental resection anastomosis in two patients and dilatation for one child.

The clinical presentation of CES is varied, and a high index of suspicion is essential to arrive at an early diagnosis.5 Management depends on the type and severity of the stenosis. Stenosis due to tracheobronchial remnants requires segmental resection. Fibromuscular stenosis may respond to dilatation, but severe degrees of stenosis require segmental excision. Membranous diaphragms usually respond to dilatation or may require endoscopic excision.5 Three cases of CES of different anatomical varieties involving the lower third of esophagus have been described by Ramesh et al.5 They have done segmental resection anastomosis in two patients and dilatation for one child. In this case, the history of excessive frothing from the mouth as well as radiological findings of the upper pouch extending upto T8 level alongwith the absence of gas in the abdomen and pelvis led to suspicion of lesion in the lower third of esophagus. Suspecting underlying esophageal stenosis, decision was taken to perform a right thoracotomy. Intraoperatively, in case the lower pouch was found undeveloped/far off for a safe anastomosis, we had planned to pull up the stomach (primary pull through). Hence, we purposefully entered the thoracic cavity through the sixth intercostal space despite the usual entry for TEF through the fourth intercostal space. The upper and lower pouches were in-continuity. However, the real challenge was anastomosing the grossly thickened lower pouch to the grossly dilated (1.5 cm) upper pouch. Excision of the stenotic segment and adequate spatulation of the lower pouch to match the diameter of the upper pouch was done. As a result, almost an end-to-oblique, widely patent, tension-free, primary esophageal anastomosis could be achieved. Antireflux procedure was not considered necessary because we did not have to mobilize the lower pouch at all and its length was adequate.

e lower pouch to match the diameter of the upper pouch was done. As a result, almost an end-to-oblique, widely patent, tension-free, primary esophageal anastomosis could be achieved. Antireflux procedure was not considered necessary because we did not have to mobilize the lower pouch at all and its length was adequate. Few other authors have previously described the management of CES by primary resection anastomosis. Murphy et al2 have described successful management of three patients with isolated CES, using limited resection anastomosis via a left thoracotomy. Their patients presented around 6 to 12 months of age, with feeding difficulty upon introduction of solids. Initial hydrostatic dilatation was tried, but later on they had to resort to resection anastomosis because of failure of dilatation. Similar to our case, the stenoses were located in the distal third of esophagus, near its junction with the middle third. Amae et al also had done resection anastomosis in 11 of 14 patients who had initially undergone dilatation.6

ut later on they had to resort to resection anastomosis because of failure of dilatation. Similar to our case, the stenoses were located in the distal third of esophagus, near its junction with the middle third. Amae et al also had done resection anastomosis in 11 of 14 patients who had initially undergone dilatation.6 The crux of management of our case was the decision making to plan thoracotomy and primary repair in the neonatal period. In the normal course of events, one would do esophagostomy and feeding gastrostomy in a neonate with (long gap) pure EA. In our case, we were sure that the upper pouch was pretty long, and hence we decided to take the risk and open the thoracic cavity. Here, the decision of performing thoracotomy was more crucial rather than the actual technical (operative) exercise. This case also highlights the importance of accurate interpretation of a well-taken chest X-ray with a stiff (10 Fr) rubber catheter in situ for diagnosis and management of a very rare variety of EA. Conflict of Interest None

Introduction Neutropenic patients are at high risk of developing invasive fungal infections. In this patient population, invasive aspergillosis is the most common systemic fungal infection. The primary therapy for invasive aspergillosis is based on drug treatment. However, surgical resection is indicated in hemoptysis or progressive radiological findings.1 Video-assisted thoracic surgery (VATS) and anatomic lobectomy were initially described in 1993 mainly for the treatment of lung cancer. By the mid-1990s, VATS became an important approach for the treatment of lung and mediastinal lesions in adults and some specific cases in children. No significant experience in children with thoracoscopic lobectomy has been previously reported. VATS surgery is usually performed using three incisions; however, some surgeons employ just two ports or even only one in a superior surgical approach.2

and mediastinal lesions in adults and some specific cases in children. No significant experience in children with thoracoscopic lobectomy has been previously reported. VATS surgery is usually performed using three incisions; however, some surgeons employ just two ports or even only one in a superior surgical approach.2 Case Report An 11-year-old girl was recently diagnosed with acute lymphoblastic leukemia. After a course of the chemotherapy, she developed persistent fever. A computed tomography (CT) scan of the lungs showed new right interstitial infiltrates. Antifungal prophylaxis was given, but because of the patient's persistent fever, the treatment was changed to broad-spectrum antibiotics. Five days from the beginning of the new treatment, a second CT scan showed a right hydropneumothorax. A chest tube was placed to drain fluid. No viral, bacterial, or fungal agents were isolated from the fluid. Two weeks later, the fever persisted and the CT scan of the lungs showed a new localized lesion in the middle pulmonary lobe in direct contact with the lower right lobe fissure and the parietal pleura (Fig. 1). These findings suggested the diagnosis of pulmonary aspergilloma and antifungal treatment with voriconazole was added to medical treatment. As part of the treatment for the hematologic disease, the patient was waiting for stem cell transplantation; however, it is well known that these therapies increase the risk of mortality for invasive aspergillosis. Therefore, a multidisciplinary team (a hematologist, a pneumologist, pediatricians, and thoracic surgeons) decided a therapeutic approach by combining the most conservative pulmonary resection using the less aggressive approach and systemic antifungal treatment. Accordingly, the patient underwent a surgical resection of the pulmonary lesion with a minimal approach for VATS single port. A middle lobectomy and wedge right lower lobe resection were performed. Histopathological examination confirmed the diagnosis of pulmonary aspergilloma. No surgical complications were observed. Postoperative course was successful. After 2 days of operation, the chest drain was removed and pain was managed just with acetaminophen.

bectomy and wedge right lower lobe resection were performed. Histopathological examination confirmed the diagnosis of pulmonary aspergilloma. No surgical complications were observed. Postoperative course was successful. After 2 days of operation, the chest drain was removed and pain was managed just with acetaminophen. Fig. 1 (a) The middle pulmonary artery. (b) The superior and middle pulmonary vein. (c) The middle lobe bronchus.

bectomy and wedge right lower lobe resection were performed. Histopathological examination confirmed the diagnosis of pulmonary aspergilloma. No surgical complications were observed. Postoperative course was successful. After 2 days of operation, the chest drain was removed and pain was managed just with acetaminophen. Fig. 1 (a) The middle pulmonary artery. (b) The superior and middle pulmonary vein. (c) The middle lobe bronchus. Surgical Technique The unilateral lung ventilation was achieved with a double-lumen Robertshaw 26 endotracheal left tube (P3 Medical Ltd.). The patient was positioned in usual lateral decubitus position. A 4 cm single incision was made in the fifth intercostal space (Fig. 2b). A 5 mm/30° camera was used to get an enhanced view. Multiple pulmonary adhesions were found and they were isolated by blunt dissection using Endopath (Ethicon) and LigaSure (Covidien) devices. The lesion was visualized in the middle lobe, across the major fissure affecting the lower and middle lobes. The pulmonary artery and its branches were identified within the fissure (Fig. 1a). Then, the lung was rotated to expose the superior and middle pulmonary vein (Fig. 1b). The middle branch was divided and ligated with a vascular stapler; afterward the middle bronchus was carefully identified, dissected, and sectioned with a bronchial stapler (Fig. 1c). Once the middle bronchus was transected, the middle lobe artery was exposed and clipped proximally and thermo-sealed distally. The minor fissure was completely opened through serial application of GIA stapler. Finally, the inferior pulmonary vein was identified. The middle lobectomy was completed including a wedge resection of the lower lobe by stapling this lobe from the dissected inferior vein to the stapled line of the minor fissure (Fig. 2a).

minor fissure was completely opened through serial application of GIA stapler. Finally, the inferior pulmonary vein was identified. The middle lobectomy was completed including a wedge resection of the lower lobe by stapling this lobe from the dissected inferior vein to the stapled line of the minor fissure (Fig. 2a). Fig. 2 (a) Surgical piece consisting of the middle and lower lobe right pulmonary segments. (b) 4-cm single port incision. The chest drain was extracted after 3 days from surgery, and the pain was controlled with oral acetaminophen for 6 days. No postoperative complications were observed. Discussion Pulmonary aspergilloma is a severe complication in immunosuppressed patients. Standard treatment, combining early surgery and antifungal therapy, has the best prognosis in these cases. Surgical resection can be curative in certain patients after antifungal treatment. Furthermore, in patients who would need immunosuppressive treatment, aggressive surgical management has been suggested.3 The surgery for pulmonary aspergilloma can be technically challenging because multiple adhesions can be usually found between the main lesion and adjacent normal anatomical structures. Despite the potential benefits of a minimally invasive procedure in immunosuppressed patients, the VATS approach to perform lobectomy for pulmonary aspergilloma is anecdotal. We have not found studies of pulmonary aspergilloma treated by VATS single port in adults, and no significant experience with thoracoscopic lobectomy in children has been previously reported.

invasive procedure in immunosuppressed patients, the VATS approach to perform lobectomy for pulmonary aspergilloma is anecdotal. We have not found studies of pulmonary aspergilloma treated by VATS single port in adults, and no significant experience with thoracoscopic lobectomy in children has been previously reported. Acknowledgments We especially thank our anesthesiology and pediatrics service for their efficient coordination and cooperation. Conflict of Interest None

Introduction Pulmonary lymphangiectasia (PL) is a rare disease characterized by dilated but otherwise normal lymphatics typical of infancy and early childhood. The postneonatal form of PL has a better prognosis,1 2 but it can be misdiagnosed with other respiratory diseases. We report a rare case of PL presenting in a 7-year-old healthy boy with chylothorax, effectively treated with mini-invasive surgery. Case Report A 7-year-old boy came to our attention for suspected chylothorax. Up to 2 months before when he was treated for Streptococcus pyogenes septic shock at another hospital, the boy had always been well. The shock was probably due to bilateral pneumonia as shown by radiological investigations. In that occasion, he needed respiratory support (orotracheal ventilation for 10 days). After the positioning of a pleural drainage together with the administration of antibiotic therapy, there was a progressive improvement of clinics, laboratory tests, and radiological examinations so he was discharged. During radiological follow-up (2 months later), an organized left pleural effusion was shown by thorax ultrasound (US). Another chest drain was placed and the patient was transferred to our pediatric surgery unit. The milky leakage had the chemical features of chilo: triglycerides > 110 mg/dL, protein = 5.7 g/dL, lactate dehydrogenase = 290 U/L, glucose = 77 mg/dL, amylase = 37 U/L, albumin = 3.7 g/dL, cellularity > 1,000 cell/L (lymphocytes 90%, neutrophils 2%, and macrophages 8%).

was transferred to our pediatric surgery unit. The milky leakage had the chemical features of chilo: triglycerides > 110 mg/dL, protein = 5.7 g/dL, lactate dehydrogenase = 290 U/L, glucose = 77 mg/dL, amylase = 37 U/L, albumin = 3.7 g/dL, cellularity > 1,000 cell/L (lymphocytes 90%, neutrophils 2%, and macrophages 8%). We performed thorax X-ray and computed tomography (CT) with the evidence of left pleural effusion, small left apical and medial pneumothorax, thickening of interlobular septa and bronchial wall on both sides (left > right), and areas of ground-glass attenuation (Fig. 1). Fig. 1 Thorax computed tomographic scans showing left pleural effusion, ground–glass phenomenon, and thickening of bronchi and interlobular septa.

We performed thorax X-ray and computed tomography (CT) with the evidence of left pleural effusion, small left apical and medial pneumothorax, thickening of interlobular septa and bronchial wall on both sides (left > right), and areas of ground-glass attenuation (Fig. 1). Fig. 1 Thorax computed tomographic scans showing left pleural effusion, ground–glass phenomenon, and thickening of bronchi and interlobular septa. Initial therapy was conservative. The chest tube was maintained for the drainage of effusions, a central line was placed for total parenteral nutrition, antibiotic (ceftriaxone) and somatostatin synthetic analogues (octreotide, 1 μg/kg/h = 20 μg/h gradually increased up to 5 μg/kg/h = 100 μg/h) were used as pharmacological agents. Overall, this management was well-tolerated except for a few presyncope. Differential diagnoses included traumatic events (no attributable anamnesis), neoplasm (bronchoalveolar lavage negative for malignant tumor cells and CT negative for suspected masses), heart diseases (normal echocardiogram), alterations of large vessels (a Doppler US was used to rule out occlusive processes), storage diseases (negative US and laboratory tests), and Gorham–Stout syndrome (normal plain radiographs). Blood tests never showed an alteration of inflammatory indexes. Plasma transfusions and administration of albumin were required for progressive loss of protein. After 1 month, we decided to refer the patient for surgery because there was no significant reduction of the effusion (mean fluid drained: 818 mL/d). Before surgery, the patient was given 200 cc of cream to highlight the origin of the leak more easily. An endobronchial blocker was used to exclude the left lung. We used three operative trocars and a 5-mm optic with the patient lying in the standard right lateral decubitus with slightly pronation. The pleural space was occupied by some adhesions that were detached. Visceral pleura appeared suffering and macerated (Fig. 2).

ndobronchial blocker was used to exclude the left lung. We used three operative trocars and a 5-mm optic with the patient lying in the standard right lateral decubitus with slightly pronation. The pleural space was occupied by some adhesions that were detached. Visceral pleura appeared suffering and macerated (Fig. 2). Fig. 2 Thoracoscopy permits the visualization of suffering visceral pleura (left) and a secreting area near the mediastinum (center). A massive ligation of lymphatic vessels with metal clips reduces the effusion (right). We found a poorly defined secreting area near the posterior mediastinum. We performed a massive ligation of the thoracic ducts collaterals applying metal clips. After obtaining a sharp reduction of the leak, we took a lung biopsy and we left two pleural tubes. Histological examination revealed type 1 congenital pulmonary lymphangiectasia (Fig. 3). Fig. 3 Lung biopsy. The microscopic examination shows lymphatic cystic dilatations in the visceral pleura (left), surrounding the alveoli. In the lung parenchyma, cystic lymphatic areas are identified with CD31 staining, specific for endothelial cells (right).

We found a poorly defined secreting area near the posterior mediastinum. We performed a massive ligation of the thoracic ducts collaterals applying metal clips. After obtaining a sharp reduction of the leak, we took a lung biopsy and we left two pleural tubes. Histological examination revealed type 1 congenital pulmonary lymphangiectasia (Fig. 3). Fig. 3 Lung biopsy. The microscopic examination shows lymphatic cystic dilatations in the visceral pleura (left), surrounding the alveoli. In the lung parenchyma, cystic lymphatic areas are identified with CD31 staining, specific for endothelial cells (right). Postoperatively, the boy was doing well. After 6 days, he was discharged with pharmacological decalage. Three weeks later, there was an increment of pleural effusion on a chest X-ray and so the boy was readmitted. We performed thoracoscopic mechanical and chemical pleurodesis with scissors, hook coagulator, and talc. At the end of the maneuver, we left two pleural tubes. The boy required intensive postoperative care for 9 days and respiratory support for the onset of a left pneumothorax. The pleural tubes were removed respectively 7 and 14 days after surgery (mean fluid drained = 5 mL/d). Enteral nutrition was gradually reintroduced and the overall hospital stay after surgery was 25 days. During the follow-up, we performed a thorax X-ray every month for 5 months and then every 2 months. Radiological investigations showed great improvement of both the pleural and parenchymal components. The boy was doing well 1 year after surgery.

ally reintroduced and the overall hospital stay after surgery was 25 days. During the follow-up, we performed a thorax X-ray every month for 5 months and then every 2 months. Radiological investigations showed great improvement of both the pleural and parenchymal components. The boy was doing well 1 year after surgery. Discussion and Conclusions Pulmonary lymphangiectasia (PL) is a rare developmental disorder with diffuse dilatation of the pulmonary lymphatics of unknown origin that led to fluid accumulation. Noonan et al3 classified the disease into the primary form (congenital), that can be generalized, localized (pulmonary), or associated with syndromes (syndromal–Noonan, Ullrich/Turner, Down) and the secondary form (due to pulmonary hypertension, cardiovascular problems, or lymphatic obstruction). The overall incidence is unknown with men being affected more than women in the primary form.4 Symptoms can arise prenatally (hydrops fetalis and polyhydramnios), at birth (cyanosis, tachypnea, respiratory distress) or, rarely, after a period of wellness in stable patients with localized forms. Postneonatal congenital PL is commonly associated with pleural effusions and chylothorax.5 6 Chylothorax is associated with high mortality and morbidity if not treated. It is characterized by specific chemical features of the pleural effusion that has a high triglyceride content with elevated protein and albumin concentration and a cell count of more than 1,000 cells/mcL with a predominance of lymphocytes ( > 80%).7 Chylothorax can be the only sign of PL. Radiological examinations are useful to point out elements important for diagnosis8 and to verify improvements or to detect relapses during the follow-up. In our case, CT scans showed inhomogeneity of the lung parenchyma with a diffuse thickening of the septa surrounding the lobules (Kerley B septal lines) and patchy areas of ground-glass infiltrates. The high resolution CT is the radiological technique of choice for diagnosing PL, although only the lung biopsy identifies specific features of this disease. The treatment of choice is the conservative one for the support of the patient: pleural drain, total parenteral nutrition, and administration of octreotide.1 5 Chest tubes are required in patients with rapidly expanding pleural effusion.5 Parenteral nutrition and formula rich in medium chain triglycerides is important in the reduction of lymphatic production.

ve one for the support of the patient: pleural drain, total parenteral nutrition, and administration of octreotide.1 5 Chest tubes are required in patients with rapidly expanding pleural effusion.5 Parenteral nutrition and formula rich in medium chain triglycerides is important in the reduction of lymphatic production. Nonunivocal data are available regarding the use of octreotide.9 We decided to use it intravenously but the increment of the doses was limited by the rise of syncopes. We planned to monitor vital parameters (pressure and frequency rate) and glucose levels every 4 hours to identify and avoid the drug's collateral effects. During the treatment, the patient was also given plasma and albumin that he had lost with the large amount of fluid drained. Surgery should be considered after 2 to 4 weeks of conservative management if the patients remain stable.10 Some authors suggest to limit the length of the conservative treatment stating that its efficacy is related to the effusion drained during the first few days.11 Actually no prognostic factors have been identified yet. As suggested by Beghetti et al,10 we used a significant cutoff below 10 mL/kg/die of fluid drained as indicator of success. Surgery is required when pleural effusions persist (intractable chylothorax). In literature, various surgical techniques are proposed: direct ligation of the thoracic duct (if the point of rupture is readily identified), pleurodesis, pleurectomy, position of pleuro–peritoneal shunts.12 13 On the basis of our experience, we decided to treat the patient with a thoracoscopic approach that was useful both for diagnosis and therapy and produces all the advantages of a mini-invasive surgery. Considering the possible complications of talc pleurodesis (most of all pneumonitis and chest pain) and the absence of a precise diagnosis, we decided to avoid a nonreversible maneuver preferring massive ligation. Actually, the daily drain was really abundant and there was no evidence of a precise leakage point, so that the talc instillation could also be considered as a first approach.

ll pneumonitis and chest pain) and the absence of a precise diagnosis, we decided to avoid a nonreversible maneuver preferring massive ligation. Actually, the daily drain was really abundant and there was no evidence of a precise leakage point, so that the talc instillation could also be considered as a first approach. Long-term outcome of patients affected by PL remain uncertain.4 In most cases, symptoms gradually improve over time, especially in forms with later onset (childhood). Conflict of Interest None

Introduction Neonatal intestinal perforation (NIP) is an extremely rare complication of intestinal hemangioma (IH). Neonates younger than 30 days and having perforation include more common etiologies such as necrotizing enterocolitis, meconium ileus, and spontaneous idiopathic as well as gastric perforation.1 NIP has been associated with mortality rates of 40% to 70%.2 This report presents a rare case of NIP resulting from IH. Case Report A 27-day-old male neonate was admitted with low-grade fever, abdominal distension, and bilious vomiting associated with bouts of diarrhea of 2 days duration. Baby was a product of full-term spontaneous vaginal delivery with normal prenatal follow-up. Before coming to our hospital, the infant had been treated as gastroenteritis in other hospitals. On admission, the patient weighed 4.1 kg and was febrile, irritable, and moderately dehydrated. Abdominal examination revealed marked distension with mild tenderness in the paraumbilical region. Laboratory investigations showed hemoglobin 12 g/dL and leukocyte 15,000 c/mm. Other laboratory investigations were unremarkable. Plain abdominal radiographs suggested bowel obstruction versus ileus, therefore a barium meal follow through was obtained, which showed persistently dilated jejunal and proximal ileal loops but normal flow of contrast. Computed tomographic scan with intravenous contrast was not done because of family history of allergic reaction to the dye. On the basis of the diagnosis of intestinal obstruction, a decision for exploratory laparotomy was made.

h showed persistently dilated jejunal and proximal ileal loops but normal flow of contrast. Computed tomographic scan with intravenous contrast was not done because of family history of allergic reaction to the dye. On the basis of the diagnosis of intestinal obstruction, a decision for exploratory laparotomy was made. Laparotomy revealed dense inflammatory adhesions surrounding a solitary hemangioma located at the antimesenteric border 30 cm from the ileocaecal valve and on close inspection a small perforation was seen on one side of the hemangioma, which was sealed by extensive adhesions (Fig. 1). There was minimal contamination of the peritoneal cavity. Other areas of the bowels inspected were normal and resection with primary anastomosis was done. Fig. 1 Solitary ileal hemangioma with perforation site. Histopathological examination of the surgical specimen revealed capillary hemangioma (Fig. 2). Postoperative period was uneventful and the patient was discharged on the 10th day. Fig. 2 Dilated capillary spaces lined by single layer of endothelium with proliferation of endothelial cells in between.

Fig. 1 Solitary ileal hemangioma with perforation site. Histopathological examination of the surgical specimen revealed capillary hemangioma (Fig. 2). Postoperative period was uneventful and the patient was discharged on the 10th day. Fig. 2 Dilated capillary spaces lined by single layer of endothelium with proliferation of endothelial cells in between. Discussion Hemangiomas of the gastrointestinal tract are rare and account for only 0.05% of all intestinal neoplasms.3 They are commonly found within the small bowel, jejunum being the commonest site and compose 7 to 10% of all benign tumors.3 4 They have a tendency toward multiplicity, with solitary tumors being extremely rare.3 5 Microscopically, IH may be classified as cavernous, capillary, or mixed types, commonest being the cavernous.6 The usual presentation is intestinal bleeding usually insidious presenting as anemia7 or sometimes acute and potentially life threatening.4 Other forms of rare presentation include intussusception, obstruction, and perforation.8 9 10

be classified as cavernous, capillary, or mixed types, commonest being the cavernous.6 The usual presentation is intestinal bleeding usually insidious presenting as anemia7 or sometimes acute and potentially life threatening.4 Other forms of rare presentation include intussusception, obstruction, and perforation.8 9 10 Ileal perforation is a rare complication of IH in neonates, and so far, only one case has been reported in the literature, by McGaughey et al,11 who discovered evidence of ileal perforation due to IH while operating on a neonate with intestinal obstruction. Ours is the second reported case of NIP due to IH, and what makes it unique is the fact that the hemangioma belonged to the rare capillary type whereas the previously reported case was of the commoner cavernous type. Diagnosis in both reported cases, including ours, were made only at surgery for presumed acute intestinal obstruction. Although various causes of NIP including the rare IH have been reported, diagnosis can be difficult and exploratory laparotomy has often proved to be the final diagnostic tool. Conclusions Although a rare cause of intestinal perforation in neonates, IH should be considered in the differential diagnosis. Acknowledgements We wish to express special thanks to Dr. Hanan Al Khateeb and Dr. Soha Al-Tamimi from the Histopathlogy department, also to Dr. Fatma Al-Aqeil, Dr. Osama Abu Ghazala, and Dr. Yasser Sabry for their cooperation in this case study. Conflict of Interest None

Introduction Congenital hepatic fibrosis (CHF) is a rare disorder of the portobiliary system characterized histologically by defective remodeling of the ductal plate, abnormal branching of the intrahepatic portal veins, and progressive fibrosis of the portal tracts.1 Typically, CHF is associated with autosomal recessive polycystic kidney disease.2 Although liver transplantation is curative, it should be limited to the minority of patients with chronic cholangitis or progressive hepatic dysfunction. In most instances, CHF is a disorder characterized by well-preserved hepatic function and a good prognosis if complications such as variceal bleeding and cholangitis are controlled.3 Pancreaticobiliary maljunction (PBM) is a congenital anomaly defined as a union of the pancreatic and biliary ducts outside the duodenal wall. PBM is associated with a high risk for biliary tract carcinoma because of the continuous reflux of pancreatic juice into the biliary tract.4 To prevent biliary tract carcinogenesis in PBM patients, the cystic-dilated choledochus should be excised in childhood before the development to a precancerous stage.5 A case of CHF and PBM who suffered recurrent fever of unknown origin not responsive to several antibiotics after Roux-en-Y reconstruction is reported.

4 To prevent biliary tract carcinogenesis in PBM patients, the cystic-dilated choledochus should be excised in childhood before the development to a precancerous stage.5 A case of CHF and PBM who suffered recurrent fever of unknown origin not responsive to several antibiotics after Roux-en-Y reconstruction is reported. Case Report A 1-year-old girl was referred to our hospital due to prolonged diarrhea. She had mild hepatosplenomegaly. Her kidneys were not palpable. Her height and weight were within the reference ranges, and her developmental history was age-appropriate. Her abdominal enhanced computed tomography (CT) showed hepatomegaly, dilation of intrahepatic bile ducts, and a polycystic kidney. Magnetic resonance cholangiopancreatography and drip infusion cholangiographic CT revealed PBM and a choledochal cyst. She had no variceal bleeding or jaundice. Viral markers of hepatitis A virus, hepatitis B virus, hepatitis C virus, cytomegalovirus, and Epstein–Barr virus were all negative. Her renal function was normal and renal biopsy was not performed. The parents were healthy, non-consanguineous Japanese. The patient had a healthy elder sister and younger brother, and her family had no liver or renal diseases.

titis B virus, hepatitis C virus, cytomegalovirus, and Epstein–Barr virus were all negative. Her renal function was normal and renal biopsy was not performed. The parents were healthy, non-consanguineous Japanese. The patient had a healthy elder sister and younger brother, and her family had no liver or renal diseases. At the age of 4 years, she was treated by resection of the choledochal cyst and Roux-en-Y reconstruction because of the cyst's risk of cancer. Operative cholangiography showed PBM and a choledochal cyst (Fig. 1). On liver biopsy specimen examination, portal areas greatly expanded by fibrous tissue and increased bile ducts were seen. The bands sometimes connected with adjacent portal tracts. Distorted irregular bile ducts in the portal tracts were noted (Fig. 2a). She was diagnosed as having CHF based on the histological findings. After Roux-en-Y reconstruction, she suffered recurrent fever of unknown origin not responsive to several antibiotics (sulbactam/cefoperazone, panipenem/betamipron, piperacillin, amikacin, etc.). When she had a high fever, although C-reactive protein was high (∼10 mg/dL), her aspartate aminotransferase, alanine aminotransferase, lactate dehydrogenase, alkaline phosphatase, gamma-glutamyl transpeptidase, prothrombin time, total bilirubin, direct bilirubin, and immunoglobulin levels were all normal. She had no jaundice, cough, nasal discharge, diarrhea, pyuria, or otitis media. Repeated blood and urine cultures were all negative. The symptoms were intractable, and her systemic condition gradually deteriorated and her quality of life was reduced. Therefore, she underwent living donor liver transplantation (LDLT) from her father at the age of 6 years. The recipient liver was dark brown and green (Fig. 2b). A lymph node in the hepatic portal region was enlarged. On histological examination, neutrophilic cholangitis with diffuse infiltration of neutrophils in the bile duct and hepatic vein and reactive lymphadenitis with infiltration of neutrophils and macrophages in sinusoids were seen. Three species of multi-drug-resistant Pseudomonas aeruginosa were cultured in the recipient's liver. After LDLT, the patient had no episodes of recurrent fever, and her outcome was good.

in the bile duct and hepatic vein and reactive lymphadenitis with infiltration of neutrophils and macrophages in sinusoids were seen. Three species of multi-drug-resistant Pseudomonas aeruginosa were cultured in the recipient's liver. After LDLT, the patient had no episodes of recurrent fever, and her outcome was good. Fig. 1 Operative cholangiography shows pancreaticobiliary maljunction and a choledochal cyst. Fig. 2 (a) On histological examination of the liver biopsy specimen, portal areas greatly expanded by fibrous tissue and increased bile ducts are seen. The bands sometimes connect with adjacent portal tracts. Distorted irregular bile ducts are noted in the portal tracts (hematoxylin and eosin stain). (b) The recipient liver shows dark brown and green.

ation of the liver biopsy specimen, portal areas greatly expanded by fibrous tissue and increased bile ducts are seen. The bands sometimes connect with adjacent portal tracts. Distorted irregular bile ducts are noted in the portal tracts (hematoxylin and eosin stain). (b) The recipient liver shows dark brown and green. Discussion In the present case, complete choledochal cyst resection and Roux-en-Y reconstruction were performed in a 4-year-old girl with PBM and a choledochal cyst. At the time of the operation, hepatic histological findings were hyperplasia of the interlobular bile duct and diffuse fibril formation around the portal vein, compatible with a diagnosis of CHF. Postoperatively, the patient repeatedly suffered from fever of unknown origin. Although an investigation was performed from various perspectives to identify the cause of the fever, the focus could not be determined. While blood test findings did not include elevated transaminase levels or elevated bile duct–related enzymes such as gamma-glutamyl transpeptidase, it was considered that the cause of the fever was ascending cholangitis with foci at the liver and bile ducts. Therefore, LDLT from the patient's father was performed when the patient was 6 years old. In Japan, the number of deceased donors is still extremely low, especially for pediatric recipients. LDLT was efficacious for a patient with intractable cholangitis following Roux-en-Y reconstruction for CHF and PBM.

d bile ducts. Therefore, LDLT from the patient's father was performed when the patient was 6 years old. In Japan, the number of deceased donors is still extremely low, especially for pediatric recipients. LDLT was efficacious for a patient with intractable cholangitis following Roux-en-Y reconstruction for CHF and PBM. Since there are risks of biliary tract carcinoma in patients with PBM and a choledochal cyst, surgery at an early stage is recommended for such cases.5 Choledochal cyst resection in Japan is usually performed when the patient is between 1 and 4 years of age.6 Therefore, choledochal cyst resection and Roux-en-Y reconstruction were performed when the patient was 4 years old, because PBM was observed in the present case.

at an early stage is recommended for such cases.5 Choledochal cyst resection in Japan is usually performed when the patient is between 1 and 4 years of age.6 Therefore, choledochal cyst resection and Roux-en-Y reconstruction were performed when the patient was 4 years old, because PBM was observed in the present case. CHF can be associated with polycystic kidney, which is a rare disease. It is considered that CHF and Caroli disease are ductal plate malformations from an embryological point of view.1 CHF is often accompanied by portal hypertension. Even if portal hypertension is clearly present, hepatic function usually remains normal, and the patient's prognosis is relatively good.3 Liver transplantation is indicated in those children who develop liver failure in association with portal hypertension.7 Also, recurrent cholangitis in CHF is an indication for liver transplantation.8 In the present case, the patient suffered repeatedly from intractable cholangitis after Roux-en-Y reconstruction; therefore, this may have caused the liver transplantation to be performed earlier than otherwise. In Alagille syndrome accompanying hypoplasia of the intrahepatic bile duct, performing the Kasai procedure may result in a poor outcome.9 One possible mechanism could be that exposure to intestinal contents and/or ascending cholangitis via the portoenterostomy could result in a second hit on already abnormal bile ducts. Therefore, in such cases, the Kasai procedure is not recommended. In ductal plate malformation, as for CHF, the intrahepatic bile duct has a complicated form, and, therefore, once an infection is established, it becomes intractable. Similar to Alagille syndrome, Roux-en-Y reconstruction should be avoided for ductal plate malformations such as CHF. If polycystic kidneys are identified, liver biopsy should be performed before any operative intervention to rule out/diagnose CHF.

nd, therefore, once an infection is established, it becomes intractable. Similar to Alagille syndrome, Roux-en-Y reconstruction should be avoided for ductal plate malformations such as CHF. If polycystic kidneys are identified, liver biopsy should be performed before any operative intervention to rule out/diagnose CHF. Conflict of Interest None

Introduction Pharyngeal and esophageal perforation secondary to nasogastric (NG) or orogastric (OG) tube placement is rare and occurs most frequently in preterm infants who have weaker pharyngeal muscular structures and are at increased risk of iatrogenic esophageal perforation.1 2 Esophageal perforation in a full-term infant may occur with repeated attempts to place NG, OG, or endotracheal tubes. Eklöf et al first described iatrogenic esophageal perforation in 1968.3 Review of the literature reveals misdiagnosed esophageal atresia (EA) in the setting of esophageal perforation. Some infants have mistakenly undergone unnecessary thoracotomy for the same.1 4 However, esophageal perforation with EA is exceedingly rare. Perforation of the upper pouch in tracheoesophageal fistula (TEF) with EA was reported by Wright and Noblett, almost 35 years ago (1978).5 Here, we report a case of pure EA that had this rare complication of perforation of blind upper pouch of esophagus.

.1 4 However, esophageal perforation with EA is exceedingly rare. Perforation of the upper pouch in tracheoesophageal fistula (TEF) with EA was reported by Wright and Noblett, almost 35 years ago (1978).5 Here, we report a case of pure EA that had this rare complication of perforation of blind upper pouch of esophagus. Case Report A newborn baby weighing 1,400 g was transferred to our neonatal intensive care unit on day 3 after birth with symptoms of frothing after birth, with suspected EA. The baby was born at 32 weeks' gestation by normal vaginal delivery to a 22-year-old gravida 1, para 1 mother who went in premature labor. She had a normal prenatal ultrasound scan. She was hemodynamically unstable and required vasopressors. Baseline hematological investigations were normal except C reactive protein, which was 31 (N = 0–6 mg/L). To our surprise, the stiff rubber catheter (10 fr), passed to document the atresia and for taking the X-ray, went smoothly and effortlessly for 25 cm. But, the X-ray showed the tube ending in the right pleural cavity with right pneumothorax and gasless abdomen suggestive of pure EA with possible iatrogenic perforation of the upper pouch (Fig. 1). The catheter was removed immediately. Owing to increasing respiratory distress, the baby was intubated and ventilator support was provided. Right-sided intercostal drainage tube was inserted. The patient gradually improved and stabilized with ventilatory support. Fig. 1 X-ray showing catheter in right pleural cavity and gasless abdomen.

But, the X-ray showed the tube ending in the right pleural cavity with right pneumothorax and gasless abdomen suggestive of pure EA with possible iatrogenic perforation of the upper pouch (Fig. 1). The catheter was removed immediately. Owing to increasing respiratory distress, the baby was intubated and ventilator support was provided. Right-sided intercostal drainage tube was inserted. The patient gradually improved and stabilized with ventilatory support. Fig. 1 X-ray showing catheter in right pleural cavity and gasless abdomen. Cervical esophagostomy and feeding gastrostomy without thoracotomy were done. There was 0.5 × 0.3 cm perforation at the tip of the pouch with irregular margins (Fig. 2). Left cervical esophagostomy was done after mobilizing upper pouch completely. Fig. 2 Intraoperative photograph showing perforation at the tip of upper pouch.

Cervical esophagostomy and feeding gastrostomy without thoracotomy were done. There was 0.5 × 0.3 cm perforation at the tip of the pouch with irregular margins (Fig. 2). Left cervical esophagostomy was done after mobilizing upper pouch completely. Fig. 2 Intraoperative photograph showing perforation at the tip of upper pouch. Discussion Pharyngeal and esophageal perforations are rare but well-known complications of NG and OG catheter placement. Infants weighing less than 1,500 g have weaker pharyngeal muscular structures and are at increased risk of iatrogenic esophageal perforation.1 4 Neonatal esophageal perforation can present as pharyngeal or esophageal pseudodiverticulum secondary to a contained cervical perforation (less common), pneumothorax, and perhaps most commonly as esophageal obstruction. This results from compression of the lumen by the mass effect created by the false passage. It is this presentation that can be misdiagnosed as EA.6 Unlike atresia, treatment for esophageal perforation typically is medical, with antibiotics, parenteral or gastrostomy feedings, and tube thoracostomy for pneumothorax or pleural effusion.2 6 The differentiation of these two entities therefore is very important.

It is this presentation that can be misdiagnosed as EA.6 Unlike atresia, treatment for esophageal perforation typically is medical, with antibiotics, parenteral or gastrostomy feedings, and tube thoracostomy for pneumothorax or pleural effusion.2 6 The differentiation of these two entities therefore is very important. Perforation of upper pouch in EA with TEF (type C) was reported by Wright and Noblett in 1978. They reported it as a complication of using plastic sump catheter for continuous suction of upper pouch.5 However, in our case, perforation may have occurred due to the use of stiff catheter for the diagnosis of EA. The use of a stiff catheter can result in one of the following possibilities: (i) uneventful passage into the stomach through a normal esophagus, ruling out EA; (ii) hold up in the upper pouch confirming EA; (iii) iatrogenic perforation of a normal esophagus producing a clinical picture that mimics EA, a false-positive situation; and (iv) iatrogenic perforation of the upper pouch in the case of EA, with the catheter passing into the mediastinum and pleural cavity (as in our case).7

hold up in the upper pouch confirming EA; (iii) iatrogenic perforation of a normal esophagus producing a clinical picture that mimics EA, a false-positive situation; and (iv) iatrogenic perforation of the upper pouch in the case of EA, with the catheter passing into the mediastinum and pleural cavity (as in our case).7 Alternatives to the use of stiff catheter for diagnosis of EA are available, such as fluoroscopic swallow study and endoscopy. The risk of aspiration pneumonitis and adequate delineation of upper blind pouch with stiff catheter radiograph makes routine fluoroscopic swallow study less popular.7 Bronchoscopy may help in verification of the diagnosis, determination of fistula level, and detection of an occult second fistula with rigid esophagoscopy, which can further clarify the diagnosis and appears safe in experienced hands.8 Bronchoscopy and esophagoscopy allow direct visualization of a TEF and blind-ending esophagus.1 However, none of these studies are without risk and possibly have greater risk than a catheter radiograph. Conclusion A radiograph after placing catheter (NG) in the upper pouch is an accepted way of diagnosing EA with TEF. The treating surgeon should be vigilant especially in a premature infant or infant weighing less than 1,500 g, as a small force may perforate the upper pouch due to inherent weakness of the dilated tissue and musculature. Conflict of Interest None.

Introduction Lymphangiomas are benign congenital malformations of vascular origin that arise from sequestrated lymphatic tissue, resulting in a cystic mass that fails to communicate with the normal lymphatic drainage system.1 Lymphangiomas constitute 5% of all benign tumors in infants and children2 with 95% occurring in the neck and axilla. Others areas can be affected including the abdominal viscera and retroperitoneum. Lymphangiomas do not regress and cause symptoms due to their mass effect on surrounding structures or secondary infection. Patients with an intra-abdominal cystic lymphangioma may present with an abdominal mass. We describe two pediatric cases that were initially misdiagnosed as having appendicitis. Their presentations highlight the broad range of pathologies that may present as an acute abdomen in childhood. Case 1 A 5-year-old boy suffering from lethargy, vomiting, central abdominal pain, and mild right iliac fossa tenderness in the previous 2 days presented to a local hospital. Inflammatory blood markers were moderately raised. Over the following day, the child became progressively more unwell with increased pain and several episodes of vomiting. The abdomen was reexamined showing tenderness and fullness of the right iliac fossa. A provisional diagnosis of acute appendicitis with associated appendix mass was made. He was commenced on intravenous antibiotics with admission pending on ultrasound investigation.