Cruveilhier-Baumgarten disease


Cruveilhier-Baumgarten disease

Description, Causes and Risk Factors:

Cruveilhier-Baumgarten disease is a rare condition, characterized by the presence of prominent umbilical or paraumbilical veins, abdominal venus hum with thrill, splenomegaly, normal or small liver without evidence of cirrhosis, portal hypertension with esophageal varices and hypersplenism.

Researchers identified two different clinical entities: (1) the Cruveilhier-Baumgarten disease where primary liver disease, mainly cirrhosis or portal hypertension is responsible for the extensive dilatation of the paraumbilical veins and recanalization of the umbilical vein and (2) Cruveilhier-Baumgarten disease where the clinical picture is the result of a congenitally patent umbilical vein with little demonstrable liver disease or portal tract abnormality.

Cruveilhier-Baumgarten disease

In classic Cruveilhier-Baumgarten disease, the umbilical portion of left portal vein feeds a paraumbilical vein, which leaves the liver and then heads towards the umbilicus. The paraumbilical vein is seen as a tubular structure arising in the falciform ligament between the left lobe of liver, leading from the left portal vein to veins of the anterior abdominal wall. The development of a large re-canalized paraumbilical vein has been found to prevent formation of bleeding esophageal varices and predisposes to hepatic encephalopathy. These subcutaneous collaterals may undergo spontaneous hemorrhage or inadvertent significant bleeding during abdominal paracentesis.

Symptoms:

    Portal hypertension.

  • Hepatic atrophy.

  • Patent umbilical vein.

Diagnosis:

On CT, Cruveilhier-Baumgarten disease is characterized by the presence of a dilated tortuous paraumbilical vein in patients with cirrhosis, which arises from the left portal vein branch, traverses along the falciform ligament towards the umbilicus, forming a network of dilated periumbilical veins giving a "caput medusae" appearance. The blood eventually drains into the systemic circulation via the superficial and deep epigastric veins reaching the external iliac or femoral vein. Multidetector computed tomography (MDCT) is the imaging modality of choice which delineates this portosystemic collateral pathway with exceptional precision. The clinical relevance of this syndrome lies in the fact that unintended iatrogenic injury of these vessels can cause potentially life-threatening hemorrhage during abdominal surgeries or paracentesis.

Treatment:

Although portosystemic shunting and splenectomy have been implemented in the past to treat this condition, liver transplantation becomes the treatment of choice by re-establishing physiologic portal circulation and appropriate organ function.

NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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