Cruveilhier–Baumgarten disease

Cruveilhier–Baumgarten disease
Other names: Pégot-Cruveilhier–Baumgarten disease
a) Caput medusae b,c) paraumbilical vein being fed by left branch of portal vein
SpecialtyInternal medicine
Named after
  • Jean Cruveilhier
  • Paul Clemens von Baumgarten

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]

References

  1. ↑ Hardison, JE (November 1977). "Venous hum of the Cruveilhier-Baumgarten syndrome: response to the Valsalva maneuver". Archives of Internal Medicine. 137 (11): 1623–4. doi:10.1001/archinte.1977.03630230095027. PMID 921454.
  2. 1 2 Bisseru B, Patel JS (January 1989). "Cruveilhier-Baumgarten (C-B) disease". Gut. 30 (1): 136–7. doi:10.1136/gut.30.1.136. PMC 1378244. PMID 2920918.
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