What is the management of extrahepatic biliary atresia?

Management of Extrahepatic Biliary Atresia

The Kasai portoenterostomy is the initial treatment of choice for extrahepatic biliary atresia and must be performed within the first 2 months of life by an experienced surgeon, as success rates dramatically decline after 3 months of age. 1

Initial Surgical Management

Kasai Portoenterostomy (Hepatoportoenterostomy)

• Early intervention is critical: The procedure should be performed before 60 days of life to maximize success, with outcomes significantly worse when delayed beyond 90 days. 1, 2

• Success rates with optimal timing: When performed early by experienced surgeons, the Kasai procedure achieves prolonged survival in up to 70% of infants and bile drainage in 50-60% of cases. 1, 3

• Surgical technique requirements: The procedure demands precision with minimally invasive tissue anastomoses using fine suture needles (5-0 or 6-0) for bile duct reconstruction. 1

• Prognostic indicator: If total serum bilirubin falls below 2 mg/dL (or ≤1.5 mg/dL by some protocols) within 3 months following the Kasai procedure, up to 70% of patients may achieve prolonged transplant-free survival. 1, 4

Post-Kasai Medical Management

Pharmacologic Adjuvant Therapy

Prophylactic antibiotics:

• Reduce recurrent cholangitis rates and improve survival according to the American Association for the Study of Liver Diseases. 1
• Despite guideline recommendations, recent evidence shows conflicting results, with one study demonstrating no significant difference in cholangitis rates (33% without antibiotics vs 28% with antibiotics, P=0.18). 4

Ursodeoxycholic acid (UDCA):

• Commonly used post-operatively to promote bile flow, though regimens are not standardized. 1
• Should be given 2-4 hours apart from cholestyramine if both are used to avoid binding interactions. 5

Corticosteroids:

• Used post-Kasai to reduce inflammatory bile duct changes and improve bile flow, NOT to treat pruritus. 5
• Evidence for their effectiveness remains unclear and lacking. 3

Monitoring and Complication Management

Essential surveillance includes:

• Cholangitis (occurs in approximately 30% of patients in first 6 months post-operatively). 4
• Portal hypertension and variceal bleeding. 1
• Fat-soluble vitamin deficiencies requiring supplementation. 1
• Growth failure necessitating aggressive nutritional support. 1

Pruritus management algorithm (if it develops):

1. First-line: Cholestyramine 4 g/day, titrating to maximum 16 g/day, given 2-4 hours apart from UDCA. 5
2. Second-line: Rifampicin 150 mg once to twice daily, titrating to maximum 600 mg/day with liver function monitoring every 2-4 weeks. 5
3. Third-line: Naltrexone 12.5 mg/day titrating to 50 mg/day, or sertraline 100 mg/day. 5

Liver Transplantation

Indications for transplantation:

• Failure of the Kasai procedure (persistent jaundice, progressive liver dysfunction). 1
• Development of intractable portal hypertension or variceal bleeding. 1
• Progressive liver failure or end-stage cirrhosis. 1, 2
• Delayed diagnosis presenting with advanced disease. 1
• Intractable pruritus refractory to all medical therapies (often resolves within 24 hours post-transplant). 5

Transplantation outcomes:

• 1-year survival: 93%. 1
• 5-year survival: exceeds 85%. 1
• Options include reduced-size deceased donor organ, living-related liver transplantation, and split liver transplantation. 1

Timing considerations:

• Delaying transplantation until at least 6 years of age is associated with better graft and patient survival, highlighting the critical importance of a successful initial Kasai procedure. 1
• Real survival rates at 10 years approach 90% when the entire treatment pathway is optimized. 3

Prognosis Without Treatment

Without intervention, biliary atresia is universally fatal, with death typically occurring within the first 1-2 years of life. 1

Critical Pitfalls to Avoid

• Late diagnosis: The persistence of jaundice for 6 weeks or more after birth should prompt immediate evaluation, as delays beyond 60-90 days dramatically reduce Kasai success rates. 2, 1

• Inadequate surgical expertise: The procedure should only be performed by experienced surgeons at high-volume centers, as technical precision directly impacts outcomes. 1, 3

• Missed complications: Ascending cholangitis was historically a major recurrent complication with sequelae of permanent jaundice and irreversible liver damage; vigilant monitoring and prompt treatment are essential. 6

• Underlying liver involvement: Even with apparently normal bile drainage, the hepatobiliary infrastructure may remain grossly abnormal, suggesting intrinsic liver disease that may progress despite successful surgery. 6