Cruveilhier Baumgarten syndrome: Report of nine cases

Abstract

Nine cases of Cruveilhier Baumgarten syndrome (C.B.S.) are reported. Two out of these had clinical evidence of excessive umbilical circulation i.e. caput medusae, engorged abdominal veins, venous hum and thrill, while in seven, the diagnosis of C.B.S. was entertained on demonstrability of left umbilical vein on portovenography. In one case ductus venosus was also outlined. All the nine patients had cirrhosis, micro-nodular or macronodular type. Esophageal varices were present throughout the length of esophagus in most of the cases. Two patients presented with hepatic encephalopathy. It is postulated that C.B.S. is an advanced stage in the life history of portal hypertension. Two forms of C.B.S., one that is complete, having caput medusae, engorged abdominal veins, venous hum and thrill and one incomplete, showing only left umbilical vein on portovenography are described. Probable factors leading to these two forms of C.B.S. are suggested. A brief review of the subject is given.