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Diseases and Conditions › Pediatric GI and Hepatology › Budd Chiari Syndrome

Budd Chiari Syndrome
Himali Meshram
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Introduction
Budd Chiari syndrome (BCS) is a clinical condition due to hepatic venous outflow obstruction. The obstruction can be in the small hepatic veins, inferior vena cava (IVC) and even in the right atrium. Primary Budd Chiari syndrome is due to an endoluminal lesion such as thrombosis or IVC web. Secondary Budd Chiari syndrome is due extrinsic compression by tumor, cysts, and abscesses or due to invasion of the hepatic veins or IVC by tumor.

Post hepatic venous obstruction leads to increased sinusoidal pressure and congestion leading to hepatomegaly, hepatic pain and ascites. Due to congestion, there is perisinusoidal necrosis of hepatocytes in zone 3 which eventually leads to liver failure. Patients also develop portal hypertension.

• 1 in 100,000 of the population worldwide and is more common in adults.
• It is infrequently seen in infants and children less than age 10 years account for 1–7% of all cases of BCS.
• Prevalence varies as per area – higher rate in Nepal, rare in Japan and France
• In infants, the etiology would be primary BCS due to acquired and inherited thrombophilias.

• Hypercoagulable states
• Infections,
• Malignancies,
• IVC web
• Polycystic liver disease
• Trauma to hepatic veins
• Inflammatory bowel disease (IBD)

Budd Chiari Syndrome Budd Chiari Syndrome 09/05/2018
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