What is the treatment for biliary atresia?

Treatment of Biliary Atresia

The treatment of biliary atresia requires a sequential approach with Kasai portoenterostomy performed within the first 2 months of life by an experienced surgeon as the initial treatment of choice, followed by liver transplantation if the Kasai procedure fails or complications develop. 1

Initial Management: Kasai Portoenterostomy

• Kasai portoenterostomy should be performed as early as possible, ideally within the first 2 months of life, as success rates significantly decrease when performed after 3 months of age 1
• The procedure involves removal of the fibrotic extrahepatic bile ducts and creation of a roux-en-Y anastomosis between the porta hepatis and small intestine to restore bile flow 2
• When performed early by experienced surgeons, the Kasai procedure can result in prolonged survival in up to 70% of infants 1
• The procedure is performed as an open surgery in all European centers with expertise in biliary atresia 3

Post-Kasai Management

• Ursodeoxycholic acid is commonly used post-operatively to promote bile flow, though regimens are not standardized 1, 3
• Prophylactic antibiotics (such as trimethoprim/sulfamethoxazole or neomycin) reduce recurrent cholangitis rates and improve survival 1
• Steroid therapy is used in many centers, but with significant variation in dosing and duration 3
• Close monitoring for complications including cholangitis, portal hypertension, variceal bleeding, fat-soluble vitamin deficiencies, and growth failure is essential 1

Liver Transplantation

• Liver transplantation is indicated in the following scenarios: 1

  • Failure of the Kasai procedure to achieve adequate bile drainage
  • Development of intractable portal hypertension
  • Progressive liver failure despite successful initial bile drainage
  • Diagnosis delayed beyond feasibility of successful Kasai procedure

• Transplantation options include: 1

  • Reduced-size deceased donor organ
  • Living-related liver transplantation (LRLT)
  • Split liver transplantation

• Children with biliary atresia have excellent post-transplant outcomes with 1-year survival of 93% and 5-year survival exceeding 85% 1

Timing Considerations and Outcomes

• If the 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
• Even with successful Kasai procedures, many children eventually develop cirrhosis and progressive portal hypertension over years, requiring liver transplantation for long-term survival 1, 4
• Delaying transplantation until at least 6 years of age is associated with better graft and patient survival, highlighting the importance of a successful initial Kasai procedure 1

Special Considerations

• Children who undergo Kasai procedure followed by liver transplantation may experience more surgical complications during transplantation compared to those who undergo primary transplantation, including increased blood loss and longer operative time 5
• However, the Kasai procedure allows children to grow and develop before potentially needing transplantation, which can improve overall outcomes 4
• Biliary atresia splenic malformation (BASM) syndrome may be associated with less favorable transplant-free survival rates 1

Prognosis

• Without treatment, biliary atresia is universally fatal, with death usually occurring within the first 1-2 years of life 1
• With the sequential approach of Kasai procedure followed by liver transplantation when needed, overall survival rates of 55-75% can be achieved 4
• The earlier the diagnosis and Kasai procedure, the better the outcomes and the longer the potential delay before liver transplantation becomes necessary 2, 6