What is the recommended treatment for an infant diagnosed with biliary atresia?

Treatment of Biliary Atresia

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

Initial Surgical Management: Kasai Portoenterostomy

Timing is critical for the Kasai procedure—success rates plummet when performed after 3 months of age, making early diagnosis and referral essential. 1 When performed early by experienced surgeons, up to 70% of infants can achieve prolonged survival without immediate transplantation. 1

Technical Considerations

• The procedure involves transection of atretic ducts high in the porta hepatis with creation of a Roux-en-Y hepatoportoenterostomy. 2
• Surgical technique requires precision with fine suture needles (5-0 or 6-0) for bile duct reconstruction. 1
• Open Kasai portoenterostomy is strongly preferred over laparoscopic approaches—laparoscopic techniques show a 66.6% early failure rate compared to 38.5% for open procedures, with higher complication rates including intestinal volvulus and internal herniation. 3

Prognostic Indicators Post-Kasai

• If total serum bilirubin falls below 2 mg/dL within 3 months following the Kasai procedure, up to 70% of patients may achieve prolonged transplant-free survival. 1
• Conversely, if total bilirubin remains above 6 mg/dL at 3 months post-Kasai, only 16% will survive long-term without transplantation. 4
• Ductal histology on resected tissue correlates significantly with postoperative outcome—presence of patent bile ductules predicts better bile drainage. 2

Post-Kasai Medical Management

Pharmacologic Therapy

• Ursodeoxycholic acid should be administered post-operatively to promote bile flow, though standardized dosing regimens remain undefined. 1
• Prophylactic antibiotics are essential and reduce recurrent cholangitis rates while improving survival. 1
• Corticosteroids may be used to reduce inflammatory bile duct changes and improve bile flow, but are NOT indicated for pruritus management. 5

Nutritional Support

• Infants require aggressive nutritional support with medium-chain triglyceride (MCT)-containing formulas, as they need 20-80% more calories than healthy children due to hypermetabolic state and malabsorption. 6
• Do NOT restrict protein intake despite liver disease—normal protein intake is required. 6
• All patients require fat-soluble vitamin (A, D, E, K) supplementation with regular monitoring. 6
• Escalate to nasogastric tube feeding when oral intake proves insufficient, and consider parenteral nutrition if enteral feeding fails. 6
• Use triceps skinfold thickness and mid-arm circumference for nutritional assessment rather than weight alone. 6

Surveillance for Complications

Close monitoring is mandatory for: 1

• Recurrent cholangitis (most common complication)
• Portal hypertension and variceal bleeding
• Fat-soluble vitamin deficiencies
• Growth failure and malnutrition
• Progressive liver dysfunction

Liver Transplantation Criteria

Indications for Urgent Referral

Immediate contact with a pediatric liver transplant center should be initiated for: 4

• Total bilirubin greater than 6 mg/dL beyond 3 months post-Kasai (prompt referral required) 4
• Total bilirubin between 2-6 mg/dL at 3 months (transplant evaluation should be considered) 4
• Acute decompensation or liver failure 4

Indications for Transplantation

Liver transplantation becomes necessary when: 1, 7

• Kasai procedure fails to establish adequate bile drainage
• Development of intractable portal hypertension
• Progressive liver failure with deteriorating synthetic function
• Recurrent cholangitis refractory to medical management
• Variceal hemorrhage
• Intractable ascites
• Poor weight gain and growth failure despite aggressive nutritional support 4
• Intractable pruritus severely impacting quality of life 4

Transplant Outcomes

• Biliary atresia represents over 50% of pediatric liver transplantation cases. 7
• Current transplant outcomes show 93% one-year survival and over 85% five-year survival. 1
• Transplant options include reduced-size deceased donor organs, living-related liver transplantation, and split liver transplantation. 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

Sequential Treatment Strategy

The optimal approach uses Kasai portoenterostomy in infancy followed by selective liver transplantation for progressive hepatic deterioration, yielding an overall survival rate of 86%. 8 This sequential strategy outperforms primary transplantation due to limited donor availability and increased complications in infants less than 1 year of age. 8

Approximately 25-35% of patients who undergo Kasai portoenterostomy will survive more than 10 years without transplantation, one-third will drain bile but develop cirrhosis complications requiring transplantation before age 10, and one-third will have inadequate bile flow requiring earlier transplantation. 7

Critical Pitfall to Avoid

Without treatment, biliary atresia is universally fatal, with death occurring within the first 1-2 years of life (median survival 8 months). 1, 7 Any infant older than 14 days with jaundice requires prompt evaluation for conjugated hyperbilirubinemia—delays in diagnosis beyond 2-3 months of age dramatically worsen outcomes. 7, 9