Portal hypertensive enteropathy in biliary atresia

Abstract

This paper describes a rare case of biliary atresia (BA) in which massive postoperative bleeding developed due to portal hypertensive enteropathy. A 15-year-old boy had been doing well after Kasai's operation for BA. At around the age of 6 years he developed recurrent episodes of esophageal variceal bleeding and underwent esophageal transection, splenectomy, sclerotherapy, and embolization of the left gastric vein. At the age of 15 years he suddenly developed abdominal pain, hematemesis, and massive tarry stools. His hemoglobin level was 6.3 g/dl. Endoscopy showed several small, polypoid lesions in the jejunum beyond Treitz' ligament. The source of the bleeding was one of these lesions located in the proximal jejunojejunostomy of the Roux-en-Y loop. Because he had repeated episodes of melena, he underwent partial resection of the jejunum under endoscopic guidance. He has since been free of gastrointestinal (GI) bleeding. From this experience, we conclude that the polypoid lesions of an enteropathy may be a cause of massive GI bleeding in the postoperative portal hypertension of BA.