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]
| Cruveilhier–Baumgarten disease | |
|---|---|
| Other names | Pégot-Cruveilhier–Baumgarten disease |
| Specialty | Hepatology |
| Named after | |
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).[2]
Signs and symptoms
editSymptoms 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
editReferences
edit- ^ 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.
- ^ a b 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.
- ^ "The Cruveilhier-Baumgarten Syndrome; Review of the Literature and Report of Two Additional Cases". Annals of Internal Medicine. 16 (1). American College of Physicians: 113. January 1, 1942. doi:10.7326/0003-4819-16-1-113. ISSN 0003-4819.
- ^ Cheng, Tsung O.; Sutton, George C.; Sutton, Don C. (1954). "Cruveilhier-Baumgarten syndrome". The American Journal of Medicine. 17 (1). Elsevier BV: 143–150. doi:10.1016/0002-9343(54)90215-6. ISSN 0002-9343. PMID 13171401.