Cruveilhier–Baumgarten disease

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Cruveilhier–Baumgarten disease or Pégot-Cruveilhier–Baumgarten disease is a rare medical condition in which the umbilical or paraumbilical veins are distended, with an abdominal wall bruit (the Cruveilhier-Baumgarten bruit) and palpable thrill, portal hypertension with splenomegaly, hypersplenism and oesophageal varices, with a normal or small liver. The presence of the Cruveilhier-Baumgarten venous hum is highly suggestive of portal hypertension, and is never a normal physical examination finding.[1]

It was first described by Pégot in 1833, and then by Jean Cruveilhier (1835) and Paul Clemens von Baumgarten (1907).[2]

Armstrong et al. (1942) and Steinburg and Galambos (1967) described two different types of the condition:

  • 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).[2]


  1. 2.0 2.1

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