Reviewed By: Pramod Kerkar, MD, FFARCSI

What Is Hepatic Veno-Occlusive Disease?

Hepatic veno-occlusive disease is a pathological condition in which there is blockage of minute veins in the liver. This results in various symptoms of hepatic veno-occlusive disease. The blockage caused by hepatic veno-occlusive disease is limited only to the small veins of the liver and not the large hepatic veins.

Hepatic veno-occlusive disease can occur at any age. Since the blood flow due to hepatic veno-occlusive disease is blocked, the blood backs up in the liver which in turn reduces the amount of blood entering the liver causing damage to the cells of the liver and due to supply of insufficient oxygen ischemia occurs. This results in the liver to get enlarged. Hepatic veno-occlusive disease also causes increased pressure in the portal veins which can result in portal hypertension. Due to portal hypertension and the accumulation of blood in the liver, it causes fluid accumulation in the abdomen causing another medical condition called ascites. There is also enlargement of the spleen. As the disease condition progresses there is less flow of blood in the liver ultimately resulting in liver cirrhosis or even liver failure due to hepatic veno-occlusive disease.

What Are The Causes Of Hepatic Veno-Occlusive Disease?

Some of the commonest causes of hepatic veno-occlusive disease are:

  • Ingestion of chemical called pyrrolizidine alkaloids which are generally found in plants and are used to make herbal tea, especially in countries like Jamaica can cause hepatic veno-occlusive disease.
  • One of the possible causes of hepatic veno-occlusive disease include certain classes of medications that can cause toxic effects on the liver. These medications include cyclophosphamide and azathioprine, basically immune suppressants.
  • Radiation therapy. People undergoing radiation therapy for treatment of some form of cancer are also at risk for developing hepatic veno-occlusive disease.
  • Hepatic veno-occlusive disease is also a reaction to procedures like bone marrow transplantation or stem cell transplantation.

What Are The Symptoms Of Hepatic Veno-Occlusive Disease?

The symptoms of hepatic veno-occlusive disease may be sudden in onset as the liver gets enlarged and becomes tender. There is fluid accumulation with swelling of the abdomen. Other symptoms of hepatic veno-occlusive disease may include jaundice with the skin and the whites of the eyes becoming yellow. Some people may also have hematemesis due to rupture of varicose veins in the esophagus. Black tarry stool is also a symptom of hepatic veno-occlusive disease due to blood passing through the digestive tract. This condition is also called as melena. Some people may experience symptoms like decreased function of the brain which is a condition called as hepatic encephalopathy. This may result in complicated symptoms like severe confusion and even at times coma. Cirrhosis is a condition that develops with time as hepatic veno-occlusive disease progresses and advances.

How is Diagnosis of Hepatic Veno-Occlusive Disease Made?

Based on the symptoms described by the patient, the physician may suspect hepatic veno-occlusive disease. Diagnosis of hepatic veno-occlusive disease can be made with the help of medical evaluation and certain investigations. The physician will order a liver function test to check the function of the liver and abnormal results can point to hepatic veno-occlusive disease. This is further substantiated if the patient has a history of having undergone bone marrow or stem cell transplant in the recent past. The physician will then order a Doppler ultrasound which can help in confirming the diagnosis of hepatic veno-occlusive disease. Sometimes, a liver biopsy may also be required to confirmed diagnosis of hepatic veno-occlusive disease.

What Is The Treatment Of Hepatic Veno-Occlusive Disease?

As of now there is no specific treatment for hepatic veno-occlusive disease. If an underlying cause is found for the condition then that cause needs to be treated. For example, if herbal tea is the factor in development of hepatic veno-occlusive disease then the patient is advised to stop taking it immediately. Post bone marrow or stem cell transplantation, Ursodeoxycholic acid is given to prevent development of hepatic veno-occlusive disease. The patient may be advised low sodium diet to control fluid accumulation in abdomen.

For portal hypertension, treatment of hepatic veno-occlusive disease, a procedure called as transjugular intrahepatic portal-systemic shunting can be done in which an alternate way for blood flow is created but the effectiveness of this procedure is not established yet.

In extreme cases, treatment of hepatic veno-occlusive disease where the patient has cirrhosis of the liver and the disease is advancing then liver transplant is the only option left for treatment.

What Is The Prognosis For Hepatic Veno-Occlusive Disease?

The prognosis for hepatic veno-occlusive disease depends on the extent of damage that has been caused and whether the condition causing hepatic veno-occlusive disease is treated or not and even if treated what are the chances of it recurring. All in all, about 25% of people die of liver failure caused due to hepatic veno-occlusive disease. In cases of hepatic veno-occlusive disease caused by bone marrow or stem cell transplantation, hepatic veno-occlusive disease resolves on its own within a few weeks of the onset of hepatic veno-occlusive disease.

Also Read:

Pramod Kerkar

Written, Edited or Reviewed By:

, MD,FFARCSI

Pain Assist Inc.

Last Modified On: July 26, 2018

This article does not provide medical advice. See disclaimer

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