膵胆管合流異常症を合併した先天性肝外門脈狭窄による門脈圧充進症の1例

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  • A CASE OF PORTAL HYPERTENSION DUE TO CONGENITAL EXTRAHEPATIC PORTAL STENOSIS WITH PANCREATICO-BILIARY MALJUNCTION

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A 17-year-old girl had suffered jaundice at the age of six and her illness had been diagnosed as idiopathic portal hypertension with symptoms of liver dysfunction, splenomegaly, and esophageal varices. She was admitted to our hospital with left hypochondralgia on June 5, 1997. Endoscopic retrograde cholangiopancreaticography revealed pancreatico-biliary maljunction. Abdominal computed tomography showed splenomegaly and cavernous transformation in the hepatic hilus. Protography revealed extrahepatic portal stenosis. From these examinations, we diagnosed her illness as portal hypertension due to extrahepatic portal stenosis with pancreatico-biliary maljunction. Surgery was performed on September 24, 1997. After splenectomy, cholecystectomy, and resection of the extrahepatic bile duct, the portal vein was incised, and it was found that extrahepatic portal stenosis had been caused by a web of fibrous round membrane. The web was resected and patch angioplasty was performed using an autograft of the splenic vein, after which portal pressure decreased from 39cm H2O to 19cm H2O. The web was pathologically found to be of collagenous tissue with a one-way valvar lamina, which was recognized as a congenital anomaly.

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