Cruveilhier-Baumgarten syndrome

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aditya Govindavarjhulla, M.B.B.S. [2]

Overview

A rare medical condition in which the umbilical or paraumbilical veins are distended.

Historical perspective

It was first described by Pégot in 1833, and then by Jean Cruveilhier (1835) and Paul Clemens von Baumgarten (1907). Armstrong et al. (1942) and Steinburg and Galambos (1967) described two different types of the condition: Cruveilhier-Baumgarten syndrome and Cruveilhier-Baumgarten disease.

Pathophysiology

• Cruveilhier-Baumgarten syndrome: liver cirrhosis or portal hypertension is the cause of the distension of the paraumbilical veins (i.e. an acquired condition in which the veins reopen due to high portal pressure).
• Cruveilhier-Baumgarten disease: the distension of the paraumbilical veins is due to failure of umbilical vein closure, with little or no evidence of liver disease found on liver biopsy (i.e. a congenital patency of the umbilical vein leading to portal hypertension).

=Physical examination

• Abdominal wall bruit (the Cruveilhier-Baumgarten bruit)
• Palpable thrill
• Portal hypertension oesophageal varices
  • Hypersplenism
  • oesophageal varices
• Normal liver