What is the treatment for biliary atresia?

Treatment of Biliary Atresia

The Kasai portoenterostomy is the initial treatment of choice for biliary atresia and must be performed within the first 2 months of life by an experienced surgeon, as success rates drop dramatically after 3 months of age. 1, 2

Initial Surgical Management: Kasai Portoenterostomy

The Kasai procedure (hepatoportoenterostomy) represents the first-line surgical intervention and should be performed as early as possible:

• Timing is critical: Surgery performed before 7 weeks of life significantly increases success rates, while procedures after 3 months have substantially reduced outcomes 1, 2, 3
• When performed early by experienced surgeons, the Kasai procedure achieves prolonged survival in up to 70% of infants 1, 2
• The surgical technique requires precision with fine suture needles (5-0 or 6-0) for bile duct reconstruction 2
• Children diagnosed before 2 months of age have better outcomes than those diagnosed later (5.6 weeks vs 8.2 weeks in one analysis) 3

Critical pitfall: If diagnosis is delayed beyond 3 months or if established cirrhosis is already present at diagnosis, primary liver transplantation should be considered instead of attempting Kasai 1, 4

Post-Kasai Medical Management

After successful portoenterostomy, aggressive medical management is essential:

• Prophylactic antibiotics reduce recurrent cholangitis rates and improve survival 2
• Ursodeoxycholic acid is commonly used to promote bile flow, though standardized dosing regimens are not yet established 2
• Aggressive nutritional support with fat-soluble vitamin supplementation is critical for growth and development 2

Monitoring and Prognostic Indicators

Close surveillance post-Kasai is mandatory to identify treatment failure early:

• Bilirubin levels at 3 months post-Kasai: If total serum bilirubin falls below 2 mg/dL within 3 months, up to 70% of patients may achieve prolonged transplant-free survival 2
• Five-week markers: Patients with gamma-GT >100 U/L and bilirubin >5 mg/dL at 5 weeks post-Kasai should be evaluated early for liver transplantation, as these predict poor outcomes 3
• Monitor for complications including cholangitis, portal hypertension, variceal bleeding, fat-soluble vitamin deficiencies, and growth failure 2

Liver Transplantation Indications

Liver transplantation becomes necessary in specific scenarios:

• Failure of Kasai procedure to establish adequate bile flow 1, 2
• Development of intractable portal hypertension or variceal bleeding despite successful initial Kasai 1, 2
• Progressive liver failure with decompensated cirrhosis 1, 2
• Delayed diagnosis beyond 3 months when Kasai success is unlikely 1, 2

Transplantation options include reduced-size deceased donor organs, living-related liver transplantation, and split liver transplantation, with 1-year survival of 93% and 5-year survival exceeding 85% 2. Delaying transplantation until at least 6 years of age when possible is associated with better graft and patient survival 2.

Sequential Treatment Approach

The evidence strongly supports a sequential strategy rather than primary transplantation:

• First intervention: Kasai portoenterostomy in early infancy (ideally before 2 months) 1, 2, 4
• Second intervention: Selective liver transplantation only for those with progressive hepatic deterioration 4
• This sequential approach yields overall survival rates of 82-86% at 5 years 4
• Approximately one quarter of patients achieve long-term survival (>10 years) with their native liver after Kasai alone 5

Important caveat: Limited donor availability and increased complications after liver transplantation in infants less than 1 year of age argue against primary transplantation without prior portoenterostomy 4. However, when biliary atresia is not recognized in infancy and established cirrhosis has already developed, primary transplantation should be offered as the initial surgical treatment 4.

Long-Term Outcomes

Even successful Kasai patients require lifelong monitoring:

• Approximately two-thirds of long-term survivors experience some manifestation of portal hypertension (variceal bleeding, hypersplenism, ascites) 5
• About 79% of 10-year survivors remain anicteric with normal liver synthetic function and achieve age-appropriate development 5
• Children with successful Kasai procedures can develop cirrhosis and progressive portal hypertension over years, potentially requiring late transplantation 1

Without treatment, biliary atresia is universally fatal, with death occurring within the first 1-2 years of life 2, making early diagnosis and intervention absolutely critical.