Cruveilhier–Baumgarten disease or Pégot-Cruveilhier–Baumgarten disease is a rare medical condition in which the umbilical or

paraumbilical vein In the course of the round ligament of the liver, small paraumbilical veins are found which establish an anastomosis between the veins of the anterior abdominal wall and the portal vein, hypogastric, and iliac veins. These veins include Burrow's ...

s are distended, with an abdominal wall

bruit Bruit, also called vascular murmur, is the abnormal sound generated by turbulent flow of blood in an artery due to either an area of partial obstruction or a localized high rate of blood flow through an unobstructed artery. The bruit may be hea ...

(the Cruveilhier-Baumgarten bruit) and palpable thrill,

portal hypertension Portal hypertension is defined as increased portal venous pressure, with a hepatic venous pressure gradient greater than 5 mmHg. Normal portal pressure is 1–4 mmHg; clinically insignificant portal hypertension is present at portal pressures 5� ...

with

splenomegaly Splenomegaly is an enlargement of the spleen. The spleen usually lies in the left upper quadrant (LUQ) of the human abdomen. Splenomegaly is one of the four cardinal signs of ''hypersplenism'' which include: some reduction in number of circulat ...

hypersplenism Splenomegaly is an enlargement of the spleen. The spleen usually lies in the left upper quadrant (LUQ) of the human abdomen. Splenomegaly is one of the four cardinal signs of ''hypersplenism'' which include: some reduction in number of circulatin ...

and

oesophageal varices Esophageal varices are extremely dilated sub-mucosal veins in the lower third of the esophagus. They are most often a consequence of portal hypertension, commonly due to cirrhosis. People with esophageal varices have a strong tendency to develop ...

, with a normal or small

liver The liver is a major metabolic organ (anatomy), organ exclusively found in vertebrates, which performs many essential biological Function (biology), functions such as detoxification of the organism, and the Protein biosynthesis, synthesis of var ...

. The presence of the Cruveilhier-Baumgarten venous hum is highly suggestive of portal hypertension, and is never a normal physical examination finding. It was first described by Pégot in 1833, and then by

Jean Cruveilhier Jean Cruveilhier (; 9 February 1791 – 7 March 1874) was a French anatomist and pathologist. Academic career Cruveilhier was born in Limoges, France. As a student in Limoges, he planned to enter the priesthood. He later developed an intere ...

(1835) and Paul Clemens von Baumgarten (1907). 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).

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,

, atrophy of the liver,

, and prominent para-umbilical veins.

References

External links

* Diseases of liver Rare diseases Diseases named after discoverers {{Digestive system diseases

Signs and symptoms

See also

References

External links