CRUVEILHIER-BAUMGARTEN SYNDROME IN WHICH VENOUS HUM DISAPPEARED FOLLOWING ENDOSCOPIC VARICEAL SCLEROTHERAPY

We report a case of Cruveihier-Baumgarten syndrome associated with por tal vein thrombosis that developed, slowly during a 2-year period afte r endoscopic variceal sclerotherapy. The thrombosis led to the disappe arance of the venous hum and the dilated abdominal wall veins characte ristic of this syndrome. A 73-year-old woman was hospitalized for trea tment of esophageal varices in April 1988. Her spleen was markedly enl arged, and the histologic findings of her liver were not consistent wi th hepatic cirrhosis, but with idiopathic portal hypertension. A venou s hum was audible in the upper abdomen. Superior mesenteric angiograph y revealed a porto-systemic shunt vessel under the abdominal wall, ori ginating from the umbilical vein. She was injected four times with a s clerosant, and this brought about disappearance of the esophageal vari ces. Two years after the first admission, the venous hum was no longer audible, but there was a recurrence of the esophageal varices. More t han 2 years later (4 years after the first admission), ultrasonographi c study, computed tomography, and angiography showed a large thrombus, which completely obstructed the portal vein at the origin of the umbi lical vein, and the development of collateral vessels, seen as a ''cav ernous transformation.''