What is Budd-chiari syndrome?

Budd-Chiari syndrome

It is a syndrome characterized by obstruction to the venous outflow of the liver due to the occlusion of the hepatic vein and sometimes IVC. This syndrome comprises hepatomegaly, abdominal pain, ascites. Site of obstruction is usually in the hepatic vein at any site from the efferent vein of the acinus to the entry of inferior vena cava to the right atrium. 

Causes of Budd-Chiari syndrome:

1. The haematological disorder that causes thrombosis. It may be due to hypercoagulability states like:

• Polycythemia Rubra vera
• Myelofibrosis
• Paroxysmal nocturnal hemoglobinuria
• Deficiency of antithrombin III, protein C and protein S
• Pregnancy and oral contraceptive pill
• Antiphospholipid syndrome
• Leukaemia

2. Compression of the hepatic vein due to:

• Carcinoma of liver, kidney or adrenal gland
• Posterior abdominal wall sarcoma
• Hepatic infections (like hydatid cyst)
• Congenital venous webs
• Inferior vena caval obstruction/stenosis

3. Others: Radiotherapy, trauma to the liver, Behcet’s syndrome.

4. Unknown in 1/3rd to half of the cases.

Clinical features of Budd-Chiari syndrome:

In the acute form, there is a rapid development of upper abdominal pain, nausea and vomiting, tender hepatomegaly with marked ascites. Peripheral oedema may present if there is IVC obstruction. Occasionally, may present with acute liver failure.

In chronic form or if there is gradual occlusion, there may be gross ascites with upper abdominal discomfort, mild jaundice, splenomegaly with portal hypertension, later turn into cirrhosis of the liver.

Investigations for Budd-Chiari syndrome:

• LFT: In case of an acute event, features of acute hepatitis may be seen
• Ascitic fluid analysis: In the early stage, it shows exudative fluids (a protein concentration is above 25 g/L or 2.5 g/dL). But later the concentration fall
• Doppler ultrasound: It shows obliteration of the hepatic veins and reversed flow. It may also reveal associated portal vein thrombosis
• Caudate lobe imaging: CT scan may show enlargement of the caudate lobe. Most often it has a separate venous drainage system and is not affected by the syndrome
• CT and MRI: Show occlusion of the hepatic veins and IVC
• Liver biopsy: Centrilobular congestion with fibrosis (depending upon the duration of the illness)
• Venography: Needed if CT and MRI fail to demonstrate the hepatic venous anatomy clearly
• Screening for thrombophilia is mandatory

Treatment of Budd-Chiari syndrome:

• Treatment of predisposing conditions or underlying disorders 
• If recent thrombosis is suspected, then treatment with streptokinase followed by heparin and oral anticoagulation should be considered
• The followings may be considered to relieve obstruction:
• Short hepatic venous strictures can be treated with angioplasty.
• More extensive hepatic vein occlusion may be treated with insertion of a covered TIPSS followed by lifelong anticoagulation.
• Surgical portocaval shunts are reserved for those who fail TIPSS treatment, providing that there is no caval obstruction. 
• Congenital webs should be resected surgically.
• IVC stenosis may be dilated.
• Ascites is initially treated medically
• Liver transplantation is the treatment of choice for chronic Budd-Chiari syndrome and for the fulminant form, followed by life-long anticoagulation. Progressive liver failure is an indication for liver transplantation and life-long anticoagulation

Prognosis of Budd-Chiari syndrome:

• Prognosis depends on the cause. It is poor without liver transplantation or shunting, particularly following an acute presentation with liver failure, but some patients can survive for several years. Among the survivors of initial events, 3 years of survival rate is 50%.
• Following liver transplantation, 1 and 10 years of survival is 85% and 69% respectively
• Following surgical shunting, 5- and 10-year of survival is 87% and 37% respectively

Source: 

• Davidsons Principles and Practice of Medicine 23rd Edition; page: 898
• 2- Step Up to MRCP Review Notes for P1 & P2 by Dr Khaled El Magraby 1st Edition; page: 283
• Kumar & Clark’s Clinical Medicine 9th Edition; page: 1225 (482)
• Harrison’s Principles of Internal Medicine (2 volumes) 19th Edition; page: 287