THE CRUVEILHIER-BAUMGARTEN SYNDROME; REVIEW OF THE LITERATURE AND REPORT OF TWO ADDITIONAL CASES

Abstract

Restudy of the original reports of Cruveilhier (1829) and Baumgarten (1908) indicates that their patients exhibited a distinct disease process. The clinical picture is dominated by evidence of excessive umbilical circulation, including murmur and thrill, plus the implied evolution of portal hypertension leading to death. Final diagnosis would depend on necropsy, which should reveal patency of the umbilical vein itself (with communication with the epigastric veins) and an atrophic liver, probably related to hypoplasia of the portal system. A certain amt. of fibrosis is inferred from Baumgarten''s descriptions, secondary to the atrophic state of the liver, but cirrhosis is not considered part of the picture. The persistent patency of the umbilical vein itself is a central feature and is of interest because of the importance of the umbilical vein in the embryonal development of the portal system. Clinical analysis of the reported cases indicates that in only 5 instances (including authors'' 2d case) do all the criteria of Cruveilhier and Baumgarten appear to be satisfied. In the other 50 instances, the diagnosis is based on either clinical evidence alone or, as the necropsies, when available, themselves indicate, some other pre-existing disease of the liver, hepatic veins or portal system. This, plus excessive utilization of the umbilical area through varied venous collaterals lead to consideration of these cases as belonging to this group. The authors suggest that at best such cases can only be considered as examples of Cruveilhier-Baumgarten Syndrome. The term Cruveilhier-Baumgarten disease should be reserved for the much rarer cases fulfilling both the clinical and autopsy criteria outlined by the original authors.