What is Budd Chiari syndrome?

Budd chiari syndrome (BCS) is a disease characterized by obstruction of the hepatic venous outflow tract which is the main channel taking blood from the liver to the heart. Liver receives the blood supply from the portal vein and hepatic artery. After circulating through the liver, the blood then flows out via the hepatic veins to the inferior vena cava (large blood vessel emptying into the heart). There are total three hepatic veins. The obstruction to the blood flow can be in the hepatic veins or in the inferior vena cava. If the obstruction is in the smaller veins of liver (hepatic venules), then it is called as sinusoidal obstruction syndrome.

How does BCS affect liver functions?

Due to obstruction of the blood flow, there is back pressure in the blood vessels and spaces in the liver. This leads to damage to the adjacent liver cells as there is poor oxygenation. Gradually there will be liver cell death leading to scarring and permanent liver damage (Cirrhosis). Since the outflow of blood flow from the liver is affected, there is buildup of pressure in the portal vein called as portal hypertension.

What are the causes of Budd chiari syndrome?

Hepatic vein obstruction is commonly due to formation of blood clots (thrombosis) in the hepatic veins. This can be due to congenital or acquired disorder in which there is tendency of blood to clot. The congenital causes are Factor V Leiden mutation, protein C or protein S and antithrombin III deficiency, antiphospholipid syndrome and use of oral contraceptives. These are substances in the blood which prevent it from easily clotting. The acquired causes are conditions in which there is increased production of blood cells which make it more viscous and easy to get clot. These are a group of myeloproliferative diseases and malignancies which can make blood easy to clot.

Occasionally there may be anatomical malformation in the inferior vena cava causing obstruction like webs or stenosis (narrowing of blood vessel lumen). This variety was thought to be more common in the past but not currently.

How does Budd chiari syndrome manifest?

BCS may be asymptomaticin approximately 15–20% of cases. In 75–80% of patients with BCS will have clinical manifestations; the most common include fever, abdominal pain, abdominal distention, accumulation of fluid in abdominal cavity (ascites), liver failure, lower leg swelling, gastrointestinal bleeding, and disturbed sensorium. Clinical presentation of BCS may be fulminant(5%), acute (20%), and sub-acute or chronic (60%).

Fulminant BCS: Fulminant BCS develops in few days to weeks. Patients present with severe liver failure with elevation of liver enzymes (SGOT, SGPT), jaundice, altered sensorium and coagulopathy (failure of blood to clot due to liver dysfunction). The liver is massively enlarged and painful.

Acute BCS: Acute BCS develops usually within 1 month and is characterized by fluid in abdomen which does not repond to drugs, abdominal pain, liver enlargement, elevation of hepatic enzymes and failure of the blood to clot.

Sub-acute BCS: Sub-acute BCS is the most common clinical type. This form of BCS has a slow onset and it may take as long as 3 months to becomesymptomatic. Biopsy shows minimal hepatic damage. There is no ascites. The development of the anatomic problem is slow and allows time for decompressive collaterals (alternative pathways draining blood away from the liver) to develop.

Chronic BCS: There is portal hypertension. Liver biopsy shows congestive cirrhosis. There is progressive abdominal distention due to ascites. The liver function tests may be minimally affected or normal. Esophageal bleeding is seen in 5–15% of these patients. Enlarged spleen is commonly seen.

What are the investigation required in the diagnosis and management of BCS?

Your doctor will ask for following investigations:

Blood reports: complete blood counts, liver function tests (SGPT, SGOT, alkaline phosphatase, total serum bilirubin, albumin and prothrombin time.

Prothrombotic workup (To find out the cause of excessive clotting of blood)

Ascitic fluid examination: microscopy to look for cells, albumin to calculate SAAG (serum ascetic albumin gradient) which is high in BCS.

Ultrasound abdomen along with Doppler: USG can give details of liver enlargement and accumulation of fluid. Spider web appearance of the hepatic veins is characteristic findings. Doppler can detect the obstruction of the hepatic veins in 85 % of the cases.

Contrast enhanced CT scan: CT scan can better delineate the anatomy of the hepatic veins and the extent of liver involvement.

MRI venography: It has the advantage of being free of ionizing radiation but it is costly and lesser available.It can give better details of intra hepatic collaterals. Children may need sedation for this procedure.

Hepatic venogram: This is the gold standard for the diagnosis of BCS. This is done by interventional radiologist through the jugular vein in the neck. The hepatic venous flow obstruction can be better documented.  Liver biopsy may be recommended during the procedure to analyze the liver damage and also confirm the diagnosis. The procedure has the advantage of therapeutic intervention which can be done if required.

What is the treatment of Budd chiari syndrome?

The treatment of BCS is best done by a team involving a pediatric gastroenterologist, interventional radiologist, pediatrician and a liver surgeon. The treatment can be divided into supportive and definitive management:

Supportive care: The nutrition of the patient is improved. Large ascites can cause difficulty in breathing. This condition requires removal of fluid from the abdomen by inserting a needle (paracentesis) only if there is difficulty in breathing or for the sake of testing the fluid. In order to decrease the hypercoagulable state,  patients are usually started on oral anticoagulants. The common drug used is Warfarin.

Definitive management:

Radiological intervention: During hepatic venogram (study of venous blood flow of liver), the anatomy  and pressure in the hepatic veins and their caliber is noted. Depending upon the diameter of the hepatic veins, either angioplasty (balloon dilatation of the vein), hepatic vein stenting (a stent is passed into the hepatic vein) or TIPSS is done (a stent is placed between the portal vein and the inferior vena cava bypassing the liver). These procedures are less invasive and successful in more than 90% patients but they will require lifelong anticoagulants. Regular Doppler examination is necessary as  shunt blockage may require recurrent intervention. The long term outcome of these procedures in children is not known.

Surgical treatment: These are surgically created shunts between portal vein and inferior vena cava or mesenteric vein. These procedures have a high morbidity and therefore have been generally overtaken by radiological interventions.

Liver transplant: Liver transplant is required in patients with Cirrhosis and decompensation of liver functions. The survival rate is around 65% at 5 years post surgery.

What is the outcome of children with BCS?

Budd Chiari in our experience is the commonest cause of ascites in children especially in infants in the developing world. In the last decade due to availability of various minimal invasive radiological interventions,  the short and medium term outcome is good in more than 90% cases. The quality of life post procedure is good and children grow well with a spurt in their growth and milestones. They can go to school and play like other children except that they should avoid sports with body contact and cricket on anticoagulation. Anticoagulation in children is a challenge as INR monitoring is not easy and the tendency to fall in toddlers increases the bleeding risk. Long term results of these interventions need to be seen

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