Biliary Atresia

Accurate diagnosis involves a combination of clinical signs, lab tests, and imaging:

• Blood tests: Elevated direct (conjugated) bilirubin, Elevated liver enzymes (ALT, AST, GGT)
• Abdominal ultrasound: To assess gallbladder and bile ducts
• Hepatobiliary scintigraphy: To check bile flow from liver to intestine
• Liver biopsy: Shows bile duct proliferation and fibrosis
• Intraoperative cholangiography: Confirms biliary obstruction during surgery

Biliary atresia progression can be described in phases:

• Early stage: Obstruction begins; jaundice and pale stool appear
• Fibrotic stage: Bile accumulates, liver inflammation worsens
• Cirrhotic stage: Irreversible scarring, portal hypertension, liver failure

Early surgery can delay or prevent progression to cirrhosis.

The main treatment is surgical intervention followed by long-term management:

Kasai portoenterostomy

A surgery that creates a path for bile to drain directly from the liver to the intestine. Best outcomes occur if performed before 60 days of age.

Liver transplant

Required if Kasai fails or liver damage becomes severe.

Supportive care

• Fat-soluble vitamin supplementation (A, D, E, K)
• Nutritional support to promote growth
• Monitoring for complications (e.g., cholangitis, portal hypertension)

With timely surgery and careful monitoring, many children survive into adulthood—some even without needing a transplant.