What is the treatment for biliary atresia?

Treatment for Biliary Atresia

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

Initial Management: Kasai Portoenterostomy

Timing and Indications

• Kasai portoenterostomy should be performed before 8-10 weeks of age for optimal outcomes 2
• Success rates decline significantly when diagnosis is delayed beyond 3 months 1
• Children who do not undergo surgery due to delayed diagnosis invariably die before their second birthday 1

Surgical Technique

• The procedure involves:
  • Thorough examination of the liver and extrahepatic biliary system
  • Intraoperative cholangiogram to confirm diagnosis if not previously established
  • Dissection along the posterior edge of liver segment IVb along the hilar transverse groove
  • Removal of extrahepatic bile duct remnants
  • Creation of a biliary conduit to restore bile flow 2

Postoperative Management

• Prophylactic antibiotics (trimethoprim/sulfamethoxazole or neomycin) to prevent cholangitis 2
• Ursodeoxycholic acid to promote bile flow 2
• Close monitoring for complications, especially ascending cholangitis 2

Outcomes of Kasai Procedure

• Can restore bile flow in approximately 80% of children operated on before 60 days of life 3
• Long-term outcomes:
  • Approximately 25-35% of patients survive more than 10 years without liver transplantation 4
  • One-third develop complications of cirrhosis and require liver transplantation before age 10 4
  • One-third have inadequate bile flow and develop progressive fibrosis and cirrhosis 4

Liver Transplantation

Indications for Transplantation

• Failed Kasai procedure (inadequate bile drainage) 1
• Development of intractable portal hypertension or liver failure despite successful portoenterostomy 1
• Recurrent cholangitis, progressive jaundice, portal hypertension complications, ascites, decreased synthetic function, and growth/nutritional failure 4

Transplantation Considerations

• Small children can be successfully transplanted using:
  • Reduced-size deceased donor organ
  • Portion of the liver from a living related donor 1
• Biliary atresia is the most common indication for pediatric liver transplantation, representing more than 50% of cases 4
• Outcomes after transplantation are excellent with 1-year survival of 93% and 5-year survival of more than 85% 1

Prognostic Factors and Caveats

Poor Prognostic Indicators for Kasai Procedure

• Vitamin K nonresponsive coagulopathy
• Hypoalbuminemia
• Advanced cirrhosis
• Ascites
• Portal hypertension
• Poor nutritional status 2

Important Considerations

• The earlier the Kasai is performed, the later a liver transplantation is usually needed 5
• Prompt evaluation is indicated for any infant older than 14 days with jaundice to determine if conjugated hyperbilirubinemia is present 4
• There is no effective medical therapy for biliary atresia; surgical intervention is essential 1

Sequential Treatment Approach

1. Early diagnosis (before 8-10 weeks of age)
2. Kasai portoenterostomy as first-line treatment
3. Post-Kasai management to prevent and treat complications
4. Liver transplantation when indicated by progressive disease or complications

This sequential approach has significantly improved survival rates for children with this otherwise fatal condition 5, 6.