Cruveilhier–Baumgarten disease

Cruveilhier–Baumgarten disease is a rare medical condition characterized by the presence of portal hypertension without the occurrence of liver cirrhosis. The disease was first described by French pathologist Jean Cruveilhier and German pathologist Paul Clemens von Baumgarten, hence the name.

Etymology

The disease is named after Jean Cruveilhier (1791–1874), a French pathologist who was a professor of pathological anatomy at the University of Paris, and Paul Clemens von Baumgarten (1848–1928), a German pathologist who was a professor at the University of Tübingen.

Symptoms

The main symptom of Cruveilhier–Baumgarten disease is portal hypertension, which is high blood pressure in the portal vein. This can lead to various complications, including esophageal varices, splenomegaly, and ascites. Other symptoms may include caput medusae, which is the appearance of distended and engorged superficial epigastric veins, and a positive Cruveilhier-Baumgarten bruit, which is a venous hum heard in the epigastric region or over the liver.

Diagnosis

Diagnosis of Cruveilhier–Baumgarten disease is typically made through a combination of clinical examination and imaging studies. Ultrasound, CT scan, and MRI may be used to visualize the liver and portal vein. In some cases, a liver biopsy may be performed to rule out cirrhosis.

Treatment

Treatment for Cruveilhier–Baumgarten disease primarily involves managing the symptoms and complications of portal hypertension. This may include medications to lower blood pressure, endoscopic procedures to treat esophageal varices, and in severe cases, liver transplantation.

Prognosis

The prognosis for individuals with Cruveilhier–Baumgarten disease varies depending on the severity of portal hypertension and the presence of other underlying conditions. With appropriate management, many individuals can live a normal lifespan.

See also

• Portal hypertension
• Liver cirrhosis
• Esophageal varices
• Splenomegaly
• Ascites

Cruveilhier–Baumgarten disease Resources
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