What is Budd-Chiari syndrome?
This is a rare disorder which is caused by the obstruction of blood flowing from the liver – most of the time because of a blood clot. This syndrome occurs most often in individuals having some underlying disorders that leads to blood clotting, such as antiphosphoolipid syndrome and myeloproflierative disorders. Chronic inflammatory disorders for instance Behcet disease, sarcoidosis, inflammatory bowel disease Sjogren syndrome or lupus can also cause this syndrome.
This syndrome normally affects individuals of all ethnic groups and seems to affect men and women equally. Since it is known as a disorder that is rare exactly how often it occurs is not really known.
It is also referred to as hepatic vein obstruction and can also be caused due to a tumor or growth pressing on the vessel.
Budd-Chiari syndrome Symptoms
The majority of individuals who develop this syndrome have these symptoms:
- Ascites where liquid collects in the cavity of the abdominal region, frequently causing the abdomen to swell or stretch
- Abdominal pain – especially in the right upper abdomen
- Vomiting blood
A liver that is enlarged known as hepatomegaly is due to blood flowing into the liver but not out.
This accumulation of blood may cause damage to the cells of the liver. When the liver is not working correctly, the individual can develop jaundice which is the yellowing of the skin and eyes, as well as a kidney problem.
Budd-Chiari syndrome Causes
There are multiple reasons for the cause of this syndrome all mainly due to disorders that increase the body to form blood clots. Usually there is an underlying problem that predisposes individuals to blood clotting. But, in many cases, the exact cause of this syndrome is not known.
Some of these conditions that can lead to this syndrome consist of:
- Polycythemia or Sickle cell disease
- Use of oral contraceptives
- Liver cancer
- Phlebitis or inflammation of a vein
- Liver trauma
- Autoimmune-mediated processes
- Growth of abnormal cells in the bone marrow – myeloproliferative disorders
- Chronic inflammatory disease
- Inherited or acquired problems with blood clotting
Budd-Chiari syndrome Diagnosis
The usual symptoms and signs of Budd-Chiari are not always clues to its diagnosis, since these symptoms could be the cause of a number of disorders. If an individual has any disorder that can cause this syndrome – this information can aid in diagnosing.
Whenever Budd-Chiari is suspected, lab studies of the measurements of liver enzyme, and other organ markers including urea, creatinine, LDH, electrolytes are made.
Ultrasound studies are also done of the abdomen as well as retrograde angiography. Ultrasound can show obliteration of hepatic veins, stenosis or thrombosis, spiderweb vessels, hyperechoic cord replacing a normal, or large collateral vessels. CT or computer tomography or magnetic resonance imaging referred to as MRI is sometimes also used though these procedures are usually not as sensitive. A biopsy of the liver is nonspecific but is often needed to distinguish between this syndrome as well as other reasons of ascites and hepatomegaly, for instance Reye’s syndrome or galactosemia.
A definitive diagnosis is made after using the following tests:
- CT scan or MRI of the abdomen
- Liver biopsy
- Doppler ultrasound of the liver veins
- Liver function tests
- Liver ultrasound
Budd-Chiari syndrome Treatment
If even left not treated, this syndrome can lead to liver damage that is fatal. Medications can be prescribed that can dissolve any blood clots existing in the liver as well as reduce any further clots. A diet that is low in salt can help to control ascites.
Treatments vary and are dependent on the cause of the blockage.
- Anticoagulation or blood-thinning medications
- Thrombolytic or clot-busting drugs or thrombolytic treatment
Surgical treatments will probably be needed by the majority of patients. Some of these procedures include:
- Stent placement and angioplasty
- Transjugular intrahepatic portosystemic shunt known as TIPS
Liver transplantation is an effective treatment that is usually reserved for those individuals with failure of all other options or the progression of cirrhosis that has reduced the life expectancy to one (1) year.
The prognosis for individuals detected with this syndrome will depend on location of the blockage and the total number of veins involved. The underlying causes of this disease, diagnosis time, duration of symptoms and what form of treatment are all also critically important facts. Long-term survival is probable when treatment is started before any permanent liver damage is done. Liver cells can heal themselves if there is no damage that is permanent.