Budd-Chiari Syndrome Definition, Reason, Symptoms, Treatment & Prevention

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Budd-Chiari Syndrome Definition, Reason, Symptoms, Treatment & Prevention

Definition

Budd-Chiari syndrome is a disorder in the abdominal cavity which is characterized by a blockage in the blood vessels in the liver, causing symptoms in the form of an increasingly enlarged stomach due to accumulation of fluid in the abdominal cavity, abdominal pain, and an enlarged liver. Budd-Chiari syndrome is a rare disease.

Budd-Chiari Syndrome Symptoms

Symptoms of Budd-Chiari syndrome vary widely. In people who experience acute symptoms, complaints may arise in the form of sudden severe abdominal pain, the stomach is getting bigger because fluid accumulates in the abdominal cavity (medically it is called ascites), yellow eyes and skin, and decreased urination due to kidney failure.

Budd-Chiari syndrome can also cause chronic symptoms. In this chronic phase, the symptoms are milder but occur in the long term, and day by day the symptoms are getting bigger. The symptoms are similar to those in the acute stage, except that the abdominal pain is less pronounced, and the eyes and skin do not usually turn yellow.

In people who are still young and in people who have severe blood coagulation, Budd-Chiari syndrome can be life-threatening because it causes symptoms of impaired consciousness, enlarged stomach, swollen legs, and severely impaired liver and kidney function.

Budd-Chiari Syndrome Reason

The cause of Budd-Chiari syndrome is not fully understood. However, it is known that the disease is more prone to occur in people who experience blood coagulation (or medically called hypercoagulable), such as in the following conditions:

  • Certain tumors or cancers such as hepatoma, kidney cancer, Willms tumor
  • Blood disorders such as polycythemia, myeloproliferative disease, antiphospholipid syndrome, etc.
  • Pregnancy and the postpartum period
  • Oral contraceptive (birth control pill) users
  • Chronic infections such as syphilis, tuberculosis, and amoebiasis
  • Chronic inflammatory diseases such as lupus, Sjogren’s syndrome, and inflammatory bowel disease
  • Stenosis (narrowing) of the veins in the liver
  • Malnutrition
  • Receive nutrition through an IV 

Diagnosis

Determining the presence of Budd-Chiari syndrome is not easy. It takes a lot of examinations and the expertise of a gastroenteric-hepatologist to determine it.

There are several checks that are generally required, namely:

  • Doppler ultrasound examination (USG) to see the condition of the blood vessels in the liver.
  • Sometimes a CT- scan or MRI examination of the abdomen is also necessary, especially if the Doppler ultrasound results do not show anything clear.
  • Analysis of ascitic fluid (fluid that accumulates in the abdominal cavity) is done by first taking a sample of the fluid by inserting a large syringe into the abdominal area.
  • A liver biopsy sometimes needs to be done to see the condition of the damaged cells in the liver.

Budd-Chiari Syndrome Treatment

Budd-Chiari syndrome must be treated quickly, appropriately, and aggressively. Delay in treatment leads to a high risk of death.

The target of treatment for Budd-Chiari syndrome is to treat the blockage in the blood vessels of the liver which is the cause of all the symptoms that occur. To overcome this blockage, there are several things to do, namely:

  • Administration of anti-coagulant drugs

Anticoagulants are a type of blood thinner that work to prevent and reduce blockages in the veins, including in the liver area. This medication is given by infusion or injected under the skin.

  • Thrombolytic therapy

Thrombolytics are also a type of blood thinner which works differently from anti-coagulants. The medicine can be streptokinase, recombinant tissue-type plasminogen activator (rt-PA), and so on.

  • Angioplasty

Angioplasty is performed by inserting a camera into the blood vessel of the liver, then ‘inflating’ the blockage in the blood vessel of the heart using a balloon-like device. 

This aims to open previously blocked blood vessels. Currently, angioplasty is the main treatment used to treat Budd-Chiari Syndrome.

If not treated properly and appropriately, Budd-Chiari disease has a high risk of causing death.

Prevention

To reduce a person’s risk of experiencing Budd-Chiari syndrome, there are several things that should be done, namely:

  • In people who experience blood clots, blood thinners (anticoagulants) must be taken according to the dosage and doctor’s instructions. In addition, routine control to the doctor must always be done.
  • Women who experience blood clots should avoid using birth control pills.
  • In people who have experienced blockage of blood vessels in certain organs, anti-coagulants must be consumed in the long term, even for life.

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