A Case Report of Congenital Multiple Intrahepatic Arterio-Portal Shunts with Esophago-Gastric Varices

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A 27 year-old male was admitted to our hospital due to hemorrhagic shock with sudden hematemesis. Endoscopic findings revealed ruptured esophago-gastric varices and oozing bleeding in the proximal gastric mucosa. The patient showed temporary improvement after endoscopic treatment. Abdominal arterial angiography was carried out because congenital portal hypertension was suspected. Multiple intrahepatic arterio-portal shunts in the liver were observed and reflux of portal flow from the hepatic hilum to the periphery was discovered. Repeated hematemesis (once or more per a week) occurred even if endoscopic treatment was performed. The patient was transferred to another hospital for a split liver transplantation. Liver transplantation, however, was aborted because of dysfunction of the donor liver. Particularly, the oozing bleeding from the proximal gastric mucosa had continued during the period in which endoscopic treatment was performed. The patient was returned into our hospital. A trial of complete occlusion of the common hepatic artery with a balloon catheter (over 24 h) failed to stop the bleeding. Repeated hematemesis caused us to choose palliative surgery and Hassab's procedure was performed. The patient was discharged in 41st postoperative day. We, herein, report a very rare case of congenital multiple intrahepatic A-P shunts with esophago-gastric varices
The Japan Society for Portal Hypertensionの論文