Cruveilhier-Baumgarten syndrome: Review of the literature and report of a case

Abstract

A case of Cruveilhier-Baumgarten syndrome is presented, with a discussion of the important clinical features of this entity, which include a prominent para-umbilical vein, a venous hum and a thrill at the site of the para-umbilical circulation, splenomegaly with or without hepatomegaly, anemia, eosinophilia and persistent leukopenia. The pathogenesis of the disease is also briefly discussed. The diagnosis depends on the physician's awareness of this entity and can usually be made by careful observation and auscultation of the abdomen when any disease resulting in portal hypertension, notably cirrhosis, is present. We believe this is the first case of Cruveilhier Baumgarten syndrome in which definitive confirmation by a glucose tolerance test of the portal anastomotic nature of a dilated abdominal vein was rendered. The case here presented is probably another example—therefore the seventh in the literature —of Cruveilhier-Baumgarten disease (rather than syndrome) in view of the normal findings at liver biopsy and other criteria. However, since only necropsy affords definitive confirmation, we designate our case as Cruveilhier-Baumgarten syndrome, the eighty-sixth such case described in the literature.