Cruveilhier–Baumgarten disease
| Cruveilhier–Baumgarten disease | |
|---|---|
| Other names | Pégot-Cruveilhier–Baumgarten disease |
| Specialty | Hepatology |
| Named after | |
Cruveilhier–Baumgarten disease or Pégot-Cruveilhier–Baumgarten disease is a rare medical condition in which the
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).[2]
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
References
External links
- Cruveilhier-Baumgarten disease at Who Named It?