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Angiographic diagnosis for accurate assessment of CPSS and EHPVO in children. BACKGROUND: Congenital porto-systemic shunt (CPSS) is a rare disease and can cause fatal complications. Accurate angiographic assessment is mandatory for proper treatment. Although technically difficult, we developed assessment techniques and assessed their accuracy. One technique came from evaluating patients with extrahepatic portal vein obstruction (EHPVO). METHODS: We conducted a single center retrospective study to evaluate the efficacy of angiographic diagnostic procedure for the assessment of CPSS and EHPVO, and its impact on patients' subsequent interventions and clinical course. Eight patients with CPSS and two patients with EHPVO who underwent diagnostic angiography were included. Assessment of intrahepatic portal vein was performed in all patients. The route of the shunt, and portal vein pressure under shunt occlusion were also evaluated for patients with CPSS. Evaluation was first attempted with balloon angiographic catheter (standard method). Three additional techniques, 1) direct wedge-catheter injection without balloon inflation, 2) use of occlusion balloon in two patients, and 3) hybrid angiography with sheath placement directly into the superior mesenteric vein were performed as needed. RESULTS: The standard method was sufficient in four patients. Contrarily, all three techniques were required in two patients each. One lost contact during follow up, but all other patients underwent optimal intervention. There was no complication related to the angiographic procedure. CONCLUSIONS: Use of direct wedge-catheter injection without balloon inflation, occlusion balloon, and hybrid catheterization improved the diagnostic yield in patients with CPSS or EHPVO.