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

Umbilical vein

The umbilical vein is a vein present during fetal development that carries oxygenated blood from the placenta to the growing fetus.The blood pressure inside the umbilical vein is approximately 20 mmHg.-Development:...

 or paraumbilical veins are distended, with an abdominal wall bruit

Bruit

Bruit is the term for the unusual sound that blood makes when it rushes past an obstruction in an artery when the sound is auscultated with the bell portion of a stethoscope.The term "bruit" simply refers to the sound...

 (the Cruveilhier-Baumgarten bruit

Cruveilhier-Baumgarten bruit

The Cruveilhier-Baumgarten bruit is a clinical sign which can be found in Cruveilhier-Baumgarten disease. In this condition there are prominent umbilical and paraumbilical veins; auscultation over these vessels may reveal a humming sound ....

) and palpable thrill, portal hypertension

Portal hypertension

In medicine, portal hypertension is hypertension in the portal vein and its tributaries.It is often defined as a portal pressure gradient of 10 mmHg or greater.-Causes:Causes can be divided into prehepatic, intrahepatic, and posthepatic...

 with splenomegaly

Splenomegaly

Splenomegaly is an enlargement of the spleen. The spleen usually lies in the left upper quadrant of the human abdomen. It is one of the four cardinal signs of hypersplenism, some reduction in the number of circulating blood cells affecting granulocytes, erythrocytes or platelets in any...

, hypersplenism and oesophageal varices

Esophageal varices

In medicine , esophageal varices are extremely dilated sub-mucosal veins in the lower esophagus...

, with a normal or small liver

Liver

The liver is a vital organ present in vertebrates and some other animals. It has a wide range of functions, including detoxification, protein synthesis, and production of biochemicals necessary for digestion...

.

It was first described by Pégot in 1833, and then by Jean Cruveilhier

Jean Cruveilhier

Jean Cruveilhier was a French anatomist and pathologist.In 1816 he earned his doctorate in Paris, where in 1825 he succeeded Pierre Augustin Béclard as professor of anatomy...

 (1835) and Paul Clemens von Baumgarten

Paul Clemens von Baumgarten

Paul Clemens von Baumgarten was a German pathologist.He was the son of a physician, and was a pupil of Christian Wilhelm Braune and Ernst Leberecht Wagner in Leipzig, and Ernst Neumann . He received his doctorate in Leipzig on August 22, 1873...

(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).