What is the treatment for biliary atresia?

Treatment of Biliary Atresia

The definitive treatment for biliary atresia is Kasai portoenterostomy performed within the first 2 months of life by an experienced surgeon, followed by liver transplantation if the Kasai procedure fails or complications develop. 1

Initial Management: Kasai Portoenterostomy

• Kasai portoenterostomy is the initial treatment of choice for biliary atresia and should be performed within the first 2 months of life by an experienced surgeon 1
• When performed early, the Kasai procedure can result in prolonged survival in up to 70% of infants 2, 1
• Success rates significantly decrease when the procedure is performed after 3 months of age 2, 1
• Children who are not offered surgery due to delayed diagnosis, or those with unsuccessful Kasai procedures, typically die before their second birthday without transplantation 2

Post-Kasai Management

• Ursodeoxycholic acid is commonly administered post-operatively to promote bile flow 1
• Prophylactic antibiotics are essential to reduce recurrent cholangitis rates and improve survival 1
• Close monitoring is required for complications including:
  • Cholangitis
  • Portal hypertension
  • Variceal bleeding
  • Fat-soluble vitamin deficiencies
  • Growth failure 1

Prognostic Indicators

• If total serum bilirubin falls below 2 mg/dL within 3 months following Kasai procedure, up to 70% of patients may have prolonged transplant-free survival 1
• Elevated gamma-glutamyl transferase (>100 U/L) and bilirubin levels (>5 mg/dL) at 5 weeks post-Kasai may indicate poor outcome and need for earlier liver transplantation evaluation 3

Liver Transplantation

• Liver transplantation is indicated in the following scenarios:
  • Failure of the Kasai procedure
  • Development of intractable portal hypertension
  • Progressive liver failure despite successful portoenterostomy
  • Delayed diagnosis beyond optimal timing for Kasai procedure 2, 1
• Transplantation options include:
  • Reduced-size deceased donor organ
  • Living-related liver transplantation
  • Split liver transplantation 2, 1
• One-year survival rates following liver transplantation for biliary atresia exceed 90% 1
• Delaying transplantation until at least 6 years of age, when possible, is associated with better graft and patient survival 1

Important Considerations and Pitfalls

• Early diagnosis is critical: The success of Kasai procedure is significantly higher when performed before 60 days of life 4, 5
• Surgical expertise matters: The procedure requires precision with minimally invasive tissue anastomoses using fine suture needles for bile duct reconstruction 1
• Long-term outcomes: Even with successful Kasai procedures, only about 45% of patients achieve 10-year native liver survival 6
• Sequential approach: Treatment should be viewed as a complementary sequence of interventions (Kasai followed by transplantation if needed) rather than competing options 6
• Avoid delayed referral: Infants with cholestatic jaundice, pale stools, and hepatomegaly should be promptly evaluated for biliary atresia to avoid missing the optimal surgical window 5

Prognosis

• Without treatment, biliary atresia is universally fatal, with death usually occurring within the first 1-2 years of life 1, 7
• With optimal management (timely Kasai procedure followed by transplantation when needed), overall survival rates can exceed 90% 1
• Even with successful bile drainage after Kasai procedure, many patients will eventually develop cirrhosis and progressive portal hypertension over years, potentially requiring liver transplantation for long-term survival 2