[Portosystemic A-V fistula and portal hypertension associated with islet-cell tumor of the pancreas].

A 50-year-old woman was admitted on Feb. 25, 1983, complaining of hematemesis and melena. Endoscopic examination revealed the rupture of the esophageal varices. At first she was treated with pressure hemostasis using a S-B tube, but it was failed. Then she underwent esophageal transection under emergency thoracotomy. After the operation, she developed ascites and watery diarrhea, though there was no episode of hematemesis and melena. A bruit was audible at the epigastric region. Abdominal angiograms demonstrated a fist-sized portosystemic A-V fistula (PAVF) locating at the pancreas head, and suggested concomitant existence of portal hypertension. On May 16, 1983, laparotomy was carried out in order to reduce portal pressure, but it was not successful. She died 3 weeks after the second operation. Autopsy and pathological findings revealed that PAVF associated with a hen-egg-sized islet-cell tumor of the pancreas head. Etiology and clinical features of this rare entity may be characterized by the nature of the islet-cell tumor having rich and rapidly growing vascular supply.