What is Budd-Chiari Syndrome?

Budd-Chiari syndrome is a blood vessel disorder that affects the hepatic veins of the liver. It occurs when one or more veins become blocked or significantly narrowed, restricting blood flow back to the heart. As a result, blood and fluid back up into the liver and spleen and cause swelling, pain, and eventual organ failure. Early diagnosis and treatment in the form of medications are important to prevent serious complications. Some patients require surgery to repair or replace livers that have suffered major damage.

Many different factors can contribute to vein occlusion and the onset of Budd-Chiari syndrome. Individuals who have acquired conditions or genetic disorders that cause blood clots are at the highest risk. Protein C or S deficiency, high red blood cell counts, sickle cell disease, and lupus are common underlying conditions. Injury to the liver from surgery, medication use, or physical trauma can also precede Budd-Chiari syndrome. Rarely, a major infection or cancerous tumor can damage hepatic veins and lead to obstructions.

In most cases, the symptoms of Budd-Chiari syndrome develop slowly over the course of several months. A person may experience fatigue and slight abdominal pains that worsen over time. Ascites, or fluid buildup in the abdominal cavity, is a common complication that causes aches and distension. Many people also have swelling in their lower extremities, nausea, and vomiting.

The liver expands and becomes irritated as blood continues to back up into the organ. Over time, irritation and inflammation can cause permanent tissue scarring and compromise normal liver functioning. It is possible for a person to develop jaundice, severe abdominal pain, and extreme fatigue as liver problems worsen.

A doctor might suspect Budd-Chiari syndrome based on a patient's medical history and symptoms. Blood tests, ultrasounds, and specialized x-rays are usually taken to confirm the presence of a vein occlusion and gauge the severity of organ damage. If the liver is functioning poorly, a biopsy might be scheduled to check for scarring.

Treatment depends on the type and severity of symptoms. If Budd-Chiari syndrome is detected early, a patient might simply need to take anti-clotting medications and schedule periodic health checkups. Later-stage complications may require fluid drainage from the abdomen and surgery to expand or bypass a damaged vein. Liver transplantation is only necessary when the organ has been permanently and severely compromised. Most patients who receive prompt treatment and follow their doctors' instructions about lifestyle choices and medication use are able to make full recoveries.