What is the management of biliary atresia?

Management of Biliary Atresia

The management of biliary atresia requires Kasai portoenterostomy as the initial treatment of choice, which should be performed within the first 2 months of life by an experienced surgeon, followed by liver transplantation if necessary. 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
• When performed early by experienced surgeons, the Kasai procedure can result in prolonged survival in up to 70% of infants 1, 2
• The procedure involves removal of extrahepatic bile duct remnants and reconstruction to restore biliary flow to the intestine 3
• Surgical technique requires precision with minimally invasive tissue anastomoses using fine suture needles (5-0 or 6-0) for bile duct reconstruction 4

Post-Kasai Management

• Ursodeoxycholic acid is commonly administered post-operatively to promote bile flow, though dosing regimens are not standardized 1, 5
• Prophylactic antibiotics are recommended to reduce recurrent cholangitis rates and improve survival 1
• Close monitoring is essential for complications including:
  • Cholangitis (occurs in approximately 30% of patients in the first 6 months) 5
  • Portal hypertension 1
  • Variceal bleeding 1
  • Fat-soluble vitamin deficiencies 1
  • Growth failure 1
• Total serum bilirubin level below 2 mg/dL within 3 months following Kasai procedure is a positive prognostic indicator, with up to 70% of these patients having prolonged transplant-free survival 1

Liver Transplantation

• Liver transplantation becomes necessary in several scenarios:
  • Failure of the Kasai procedure 1
  • Development of intractable portal hypertension 1
  • Progressive liver failure 1
  • Delayed diagnosis 1, 6
• Transplantation options include:
  • Reduced-size deceased donor organ 1
  • Living-related liver transplantation 1
  • Split liver transplantation 1
• Current outcomes show 1-year survival of 93% and 5-year survival exceeding 85% 1
• 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

Timing Considerations

• Early diagnosis is critical - systematic screening for biliary atresia is warranted given the time-sensitive nature of treatment 2
• Untreated biliary atresia is universally fatal, with death usually occurring within the first 1-2 years of life 1, 6
• The earlier the Kasai procedure is performed, the later a liver transplantation is usually needed 2
• Approximately 25-35% of patients who undergo Kasai portoenterostomy survive more than 10 years without liver transplantation 6
• One-third of patients achieve bile drainage but develop complications of cirrhosis requiring liver transplantation before age 10 6
• The remaining one-third develop inadequate bile flow following portoenterostomy, leading to progressive fibrosis and cirrhosis 6

Special Considerations

• In cases with anatomical variations or previous surgeries, modifications to the standard Kasai procedure may be necessary, such as using colon segments as biliary conduits in patients with short bowel syndrome 7
• Biliary atresia represents the most common indication for pediatric liver transplantation, accounting for more than 50% of cases 6
• The American Academy of Pediatrics recommends development of improved screening methods for earlier detection of biliary atresia 1