Imaging Techniques

Two cases of Cruveilhier-Baumgarten syndrome not clinically evident and without esophageal varices in patients with liver cirrhosis and portal hypertension are presented. The diagnosis was made by real-time ultrasonography, which showed echographic caput medusae with large afferent umbilical veins and efferent inferior superficial epigastric veins. Doppler flowmetry documented high blood flow rates in these collateral portal-systemic circulations, and this explained the absence of large varices at endoscopy. The role of massive spontaneous portal-systemic shunts in preventing the formation of other shunts and particularly esophageal variceal bleeding is discussed.