Cruveilhier–Baumgarten disease
Rare condition with distended umbilical veins and portal hypertension.
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Cruveilhier–Baumgarten disease, also known as Pégot-Cruveilhier–Baumgarten disease, is a rare medical condition characterized by distended umbilical or paraumbilical veins, an abdominal wall bruit (the Cruveilhier-Baumgarten bruit), and palpable thrill. It involves portal hypertension with splenomegaly, hypersplenism, and oesophageal varices, with a normal or small liver. The condition was first described by Pégot in 1833, then by Jean Cruveilhier in 1835, and later by Paul Clemens von Baumgarten in 1907.
Background
The disease was first documented by Pégot in 1833, followed by independent descriptions by Jean Cruveilhier in 1835 and Paul Clemens von Baumgarten in 1907. These early accounts established the core features of the condition, including the characteristic venous hum and thrill at the paraumbilical site. Later, Armstrong et al. in 1942 and Steinburg and Galambos in 1967 distinguished two forms: Cruveilhier–Baumgarten syndrome, where liver cirrhosis or portal hypertension causes the vein distension, and Cruveilhier–Baumgarten disease, where failure of umbilical vein closure leads to congenital patency and portal hypertension with little or no liver disease.
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Sources
Compiled from Wikipedia and the sources listed below. Text from Wikipedia is available under CC BY-SA 4.0; this entry is adapted from it.
- Wikipedia: Cruveilhier–Baumgarten disease (CC BY-SA 4.0).
- Word definitions: the , each quoted from its Wikipedia article.
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