Liver Foundation of WA | Budd-Chiari Syndrome
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Budd-Chiari Syndrome

Budd-Chiari Syndrome (BCS)

BCS is a disorder affecting the liver and blood vessels, where blood flowing into the liver has difficulty in being able to flow out due to a blockage in the hepatic vein. This results in blood building up in the liver as there is more flowing in than flowing out. Due to the build-up of blood the liver becomes swollen, tender to the touch and a source of discomfort.

Blockage in the hepatic vein is usually caused by an imbalance in the blood. The blood needs to keep a fine balance between being too thin and too thick. Blood must be thick enough to allow for clotting, though people that suffer from BCS tend to have blood that clots too easily. This clotting causes the blockage.

There are many reasons why blood may be clotting too easily. Sometimes the reason is genetic or it can be because there is not enough of the substance that regulates blood clotting. It is important for doctors to identify the cause of the clotting in order to treat correctly. Although, in some cases there is no obvious explanation for the problem.

Symptoms

  • Abdominal pain in the upper right hand side of the abdomen (referred to as right upper quadrant)
  • Ascites
  • Enlarged liver (hepatomegaly)
  • Swelling of legs & ankles
  • Cramp in legs & feet
  • Itching

Diagnosis

Liver Function Tests (LFTs), ultrasound or CT scans are used to identify blockages in the hepatic veins.

A liver biopsy to determine the source of the problem may also be necessary. During a liver biopsy a tiny piece of the liver is taken for study. A fine, hollow needle is passed through the skin into the liver and a small sample of tissue is withdrawn.

Treatment

The aim of treatment is to ensure that liver function is maintained by assisted blood to flow out of the liver in equilibrium with the blood flowing into the liver. This can be done in many ways and treatment may depend on the reason as to why blood is clotting.

Doctors generally are looking to:

  • Re-channel blocked veins, if possible
  • Prevent the recurrence or progression of thrombosis
  • Ease or ‘decompress’ the congestion of blood in the liver
  • Manage ascites (fluid build-up)
  • Prevent further damage to the liver & allow liver cells to regenerate

These goals can be met in many different ways:

  • Anticoagulant therapy – this involves taking blood thinning medicine and ongoing monitoring of blood flow
  • Thrombolytic therapy – this treatment involves breaking up existing blood clots
  • Venoplasty or Angioplasty – this is a process where a clot in removed and a stent is put in place to hold open a vein
  • Transjugular Intrahepatic Portosystemic Shunt (TIPS) – if there are too many clots for venoplasty or angioplasty to be useful a tube called a TIPS may be put in place of the blocked vein for blood to flow in and out of.
  • Diet – a low-sodium diet is usually recommended to control ascites
  • Liver transplantation – this is only used as a treatment is liver cirrhosis has occurred due to BCS

Whichever treatment path is taken, sufferers of BCS need to look after their health and have regular check-ups with their doctors. Living with BCS is not an easy path but luckily medicine is progressively improving and there are more and more ways to manage symptoms and side effects of the syndrome.