Cruveilhier–Baumgarten disease explained

Synonym:	Pégot-Cruveilhier–Baumgarten disease
Specialty:	Hepatology

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:^[3]

• 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).

Signs and symptoms

Symptoms of Cruveilhier–Baumgarten disease include the manifestation of a venous hum at the para-umbilical circulation site, often accompanied by a thrill, splenomegaly, atrophy of the liver, portal hypertension, and prominent para-umbilical veins.^[4]

See also

• Portal hypertension
• Splenomegaly

Notes and References

1. Hardison . JE . Venous hum of the Cruveilhier-Baumgarten syndrome: response to the Valsalva maneuver. . Archives of Internal Medicine . November 1977 . 137 . 11 . 1623–4 . 921454. 10.1001/archinte.1977.03630230095027 .
2. Bisseru B, Patel JS . Cruveilhier-Baumgarten (C-B) disease . Gut . 30 . 1 . 136–7 . January 1989 . 2920918 . 1378244 . 10.1136/gut.30.1.136.
3. The Cruveilhier-Baumgarten Syndrome; Review of the Literature and Report of Two Additional Cases . Annals of Internal Medicine . American College of Physicians . 16 . 1 . January 1, 1942 . 0003-4819 . 10.7326/0003-4819-16-1-113 . 113 .
4. Cheng . Tsung O. . Sutton . George C. . Sutton . Don C. . Cruveilhier-Baumgarten syndrome . The American Journal of Medicine . Elsevier BV . 17 . 1 . 1954 . 0002-9343 . 10.1016/0002-9343(54)90215-6 . 143–150. 13171401 .