Budd-Chiari syndrome

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at March 21, 2016
StartDiseasesBudd-Chiari syndrome

Budd-Chiari syndrome is a rare liver condition. It is categorized by the blockage of blood veins in the liver.

Contents

Definition & Facts

Budd-Chiari syndrome is a rare condition characterized by the obstruction of the veins out of the liver. Hepatic veins are responsible for carrying blood away from the liver. Blockages of hepatic veins cause blood to become congested in the liver leading to its enlargement. Blockages to hepatic veins are caused by tumors putting pressure on veins, blood clots or other obstructions.

Hepatic vein blockage is the most common cause of Budd-Chiari syndrome although the exact cause may ultimately be unknown. If Budd-Chiari syndrome is left untreated it could lead to liver failure. The syndrome is named after English physician, George Budd and Austrian pathologist, Hans Chiari.

Symptoms & Complaints

Depending on the speed of onset of Budd-Chiari syndrome symptoms may develop gradually or come on suddenly. Most cases are gradual with serious symptoms presenting later. Budd-Chiari syndrome may not even present symptoms in some cases. The symptoms most associated with it include:

The kidneys may play a part in causing fluid to accumulate in the abdomen by causing water and salt to be retained. This is not limited to the abdominal area. Edema (the collection of fluid in tissue or cavities of the body) can sometimes occur in the legs.

Causes

In 70% of Budd-Chiari syndrome diagnoses, the exact cause of the condition is unknown. Polycythemia vera (a slow-progressing cancer which causes bone marrow to overproduce red blood cells) is present in approximately 10% of patients with Budd-Chiari syndrome.

There are several conditions which may lead to hepatic obstruction such as a blood clotting disorder, hereditary blood clotting issues, some types of cancer, chronic inflammatory and autoimmune diseases, chemotherapy or anti-cancer drugs, the use of birth control pills, pregnancy, blood poisoning or exposure to radiation. Sickle-cell disease or parasitic infections of the kidneys and liver are also suspect. Pregnant women may experience issues with blood clotting as such conditions often become apparent during pregnancy.

There is also evidence to suggest that pyrrolizidine alkaloids may cause Budd-Chiari syndrome. Pyrrolizidine alkaloids are a naturally occurring alkaloid found in several plants and are ingested by humans in substances such as herbal teas. Comfrey, coltsfoot and borage leaf are notable examples of plants that can cause pyrrolizidine alkaloidosis or poisoning from the alkaloids. Cases of Budd-Chiari syndrome that results from consumption of pyrrolizidine alkalioids are more common in regions of the world where such teas are regularly consumed.

Diagnosis & Tests

A diagnosis of Budd-Chiari syndrome is made after a physical examination and a review of the patient's medical history. Tell-tale signs of Budd-Chiari syndrome include the swelling of the abdomen and ascites. If Budd-Chiari syndrome is suspected the following tests may be ordered to ensure a correct diagnosis.

  • CT scan or MRI scan of the abdomen to see if there is any obstruction of blood vessels and veins.
  • An ultrasound of the abdomen (this is the preferred method of detecting Budd-Chiari syndrome as it shows obstructions in much more detail.)
  • A venography may be ordered if treatment includes surgery or an operation to widen the veins or reroute blood flow. This involves an X-ray being taken after a radiopaque dye is injected into a vein in the leg. This allows the veins to show up on an X-ray.
  • Liver function tests.
  • A liver biopsy may be ordered to correctly rule out other liver diseases or conditions causing the symptoms.

Treatment & Therapy

Serious complications from Budd-Chiari syndrome can be avoided with early treatment of the condition. The course of treatment for Budd-Chiari syndrome mostly depends on the speed with which the condition sets in. Chronic onset symptoms may be managed with medications such as blood thinners (Coumadin® or heparin). These drugs prevent clots from forming or enlarging.

Thrombolytic drugs may be administered to dissolve blood clots as well. In some cases management of symptoms such as ascites can be managed with sodium restrictions. Many cases of Budd-Chiari syndrome require surgical intervention. An angioplasty may be performed to widen a blocked vein. This procedure involves threading a catheter with a small balloon at the end into the blocked vein and widening it. A mesh stent will be inserted to keep the vein open.

Another treatment option includes diverting blood flow around the liver to prevent portal hypertension. This is known as a transjugular intrahepatic portal-systemic shunt (TIPS). In this procedure a catheter with a needle is threaded to the hepatic vein to form a shunt between it and the portal vein. A stent is then placed to keep the shunt open and allow blood to flow around the liver. A liver transplant is reserved as a last resort treatment method for advanced cases of Budd-Chiari syndrome and is usually a successful treatment option.

Prevention & Prophylaxis

There are no steps that one can take to ensure prevention of Budd-Chiari syndrome. Individuals with blood clotting conditions, a genetic clotting disorder or sickle-cell disease have increased chances of developing Budd-Chiari syndrome. The most effective way for them to prevent its development is to monitor and control these conditions.

A family history of Budd-Chiari syndrome or another blood clotting condition does mean that an individual can take measures to prevent blood clots and seek a physician's help in preventing future complications. Blood thinners may be prescribed to lower chances of developing the disease.