Budd Chiari syndrome is a condition caused due to the occlusion or obstruction of the hepatic vein due to various causes. This leads to the triad of symptoms like hepatomegaly (enlarged liver), ascites (fluid in the abdomen), pain in abdomen, nausea and vomiting, blood vomits, swelling in lower limbs etc. The disorder was first described by Budd and Chiari hence the name.
Budd Chiari syndrome is not a common condition. 1 out of 100000 populations may suffer from this syndrome. Females are more prone to this syndrome. Most patients with Budd-chiari syndrome have tendency for blood clotting.
- In other patients main cause is thrombosis of veins of the liver. This means blood gets clotted in the hepatic veins.
- Compression of the liver vein due to tumor, or abscess, or hydatid cyst etc is the other cause.
- Infections like tuberculosis, sarcoidosis, hydatid cyst and amoebic liver abscess.
- Pregnancy, contraceptives pills and use of immunosuppressive drugs.
- Hematological disorder like polycythemia vera and sickle cell disease.
- Cancer of hepatic cells.
- Carcinoma of renal cell.
- Trauma and injury to liver.
- Obstruction of inferior venacava.
- Postsurgical obstruction.
Budd Chiari can be acute, chronic and fulminant. Acute form presents with rapid development of symptoms.
Symptoms Of Budd Chiari Syndrome
- Icterus( yellow discoloration of the sclera of the eye)
- Enlarged liver
- Ascites
- Enlarged spleen
- Abdominal pain
- Edema of foot
- Fatigue
- Varicose veins in the esophagus which may rupture and the patient may bleed. This is life threatening condition and needs urgent treatment.
- Gradually cirrhosis of liver may develop and person may suffer from liver failure. His brain functions start deteriorating. He becomes confused.
Diagnosis: When Budd Chiari syndrome is suspected diagnosis is confirmed by ultrasound of liver, MRI, liver enzymes study, serum creatinine, blood urea and serum electrolytes. MRI is very sensitive and shows enlargement of liver, reduction in the caliber of hepatic veins.
Treatment And Prognosis For Budd Chiari Syndrome
Medical treatment will be restriction of salt intake, diuretics to control ascites, anticoagulants to control formation of blood clots. In long term anticoagulants are useful.
Surgical intervention such as: Venous shunt, angioplasty of the inferior vena cava if there is obstruction. Angioplasty is useful to widen the narrowed vein. Liver transplant may be suggested in some advanced cases. It is particularly life saving in severe liver failure cases.
Prognosis: Generally 2/3 percent of the patients are alive for 10 years. Prognosis of the patients will also depend on the underlying cause. When there is complete blockage of vein, and patient remains untreated, the patient may die of liver failure within 3 years.
In short it is potentially fatal if it is not treated on time.