What is the prognosis for an infant under 6 months old diagnosed with biliary atresia?

Prognosis of Biliary Atresia in Infants Under 6 Months

Without treatment, biliary atresia is universally fatal, with death typically occurring within the first 1-2 years of life (median survival 8 months), but with appropriate surgical intervention and liver transplantation when needed, the majority of patients can achieve long-term survival. 1, 2

Untreated Disease Outcomes

• Biliary atresia without intervention leads to progressive cholestasis, cirrhosis, and hepatic failure, with death occurring within 2 years in virtually all cases 1, 2
• The median survival without treatment is approximately 8 months 2

Outcomes with Kasai Portoenterostomy (Initial Surgery)

Early Success Rates

• When performed within the first 2 months of life by an experienced surgeon, the Kasai procedure can result in prolonged survival in up to 70% of infants 1
• Success rates significantly decrease when performed after 3 months of age 1
• Surgery before 30 days of age provides the best outcomes, with a 22% improvement in native liver survival compared to surgery after 31 days 3

Long-term Native Liver Survival

• Approximately 25-35% of patients who undergo Kasai portoenterostomy will survive more than 10 years without liver transplantation 2
• 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
• Conversely, if total bilirubin remains above 6 mg/dL at 3 months post-Kasai, only 16% will survive long-term without transplantation 1

Intermediate Outcomes

• One-third of patients drain bile successfully but develop complications of cirrhosis and require liver transplantation before age 10 2
• One-third have inadequate bile flow following portoenterostomy and develop progressive fibrosis requiring earlier transplantation 2

Liver Transplantation Outcomes

Timing and Necessity

• At least 80% of patients with biliary atresia are transplanted by 20 years of age, with the majority transplanted under 4 years of age 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

Survival Rates Post-Transplant

• In the United States, the overall 10-year actuarial graft survival for liver transplant in biliary atresia is 73% 4
• The overall 10-year patient survival is 86% 4
• One-year survival after transplantation is 93%, and 5-year survival exceeds 85% 1

Critical Prognostic Factors

Age at Surgery

• Surgery performed before 60 days of life results in significantly longer median survival compared to surgery after 60 days 5
• The benefit is most pronounced when surgery occurs before 30 days of life 3

Post-Kasai Bilirubin Levels

• Total bilirubin below 2 mg/dL at 3 months post-Kasai predicts excellent long-term outcomes 1
• Total bilirubin 2-6 mg/dL at 3 months warrants transplant evaluation 1
• Total bilirubin above 6 mg/dL at 3 months indicates only 16% long-term survival without transplantation and requires immediate transplant center contact 1

PELD Score

• PELD score less than 15 at the time of Kasai procedure predicts favorable 24-month survival 6
• PELD score is a better predictor than age at surgery or histological fibrosis score 6

Common Complications Affecting Prognosis

• Recurrent cholangitis occurs post-Kasai and impacts survival; prophylactic antibiotics reduce rates and improve outcomes 1
• Portal hypertension and variceal bleeding may develop and can be sentinel events prompting transplant evaluation 4
• Growth failure and nutritional deficiencies occur commonly; aggressive nutritional support prior to transplantation improves both patient and graft survival 4, 1
• Hepatocellular carcinoma risk exists but is low, with anecdotal cases reported even in patients less than 1 year of age 4

Key Pitfalls to Avoid

• Delayed diagnosis is the most critical pitfall—any infant older than 14 days with jaundice requires prompt evaluation for conjugated hyperbilirubinemia 2
• Waiting beyond 60 days for surgery significantly worsens prognosis 3, 5
• Failing to provide aggressive nutritional support pre-transplant compromises outcomes 4, 1
• Not promptly referring for transplant evaluation when bilirubin remains elevated at 3 months post-Kasai delays life-saving intervention 1