What is the recommended treatment for an infant diagnosed with biliary atresia, specifically regarding the Kasai procedure?

Kasai Procedure for Biliary Atresia

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

Timing is Critical

• Perform the Kasai procedure before 60 days of age to maximize the chance of bile drainage and prolonged survival with the native liver 1
• Success rates significantly decrease when performed after 3 months of age, making early diagnosis and referral to an experienced center essential 1
• When performed early by experienced surgeons, up to 70% of infants achieve prolonged survival with their native liver 1
• Without treatment, biliary atresia is universally fatal within the first 1-2 years of life 1

Surgical Technique Requirements

• The procedure requires precision with minimally invasive tissue anastomoses using fine suture needles (5-0 or 6-0) for bile duct reconstruction 1
• Transection must occur high in the porta hepatis to identify and expose microscopic bile ductules in the fibrous tissue 2, 3
• The standard approach is a Roux-en-Y hepatoportoenterostomy connecting the porta hepatis to a jejunal limb 2, 4

Post-Operative Management Protocol

Medications

• Start ursodeoxycholic acid immediately post-operatively to promote bile flow, though standardized dosing regimens remain under investigation 1
• Initiate prophylactic antibiotics to reduce recurrent cholangitis rates and improve survival 1

Nutritional Support (Critical for Outcomes)

• Provide 20-80% more calories than healthy infants using MCT-containing formulas due to hypermetabolic state and malabsorption 5
• Do NOT restrict protein despite liver disease 5
• Supplement all fat-soluble vitamins (A, D, E, K) with regular monitoring 5
• Escalate to nasogastric tube feeding if oral intake is insufficient, and consider parenteral nutrition if enteral feeding fails 5
• Aggressive nutritional support directly improves patient survival, graft survival, and neurodevelopmental outcomes 5

Monitoring for Complications

• Watch for cholangitis (fever, worsening jaundice, acholic stools), portal hypertension, variceal bleeding, fat-soluble vitamin deficiencies, and growth failure 1
• Monitor total serum bilirubin closely: if it falls below 2 mg/dL within 3 months post-Kasai, up to 70% of patients achieve prolonged transplant-free survival 1

When Liver Transplantation Becomes Necessary

Transplantation is indicated when: 1

• The Kasai procedure fails (persistent jaundice, progressive liver dysfunction)
• Intractable portal hypertension develops
• Progressive liver failure occurs
• Diagnosis was delayed beyond the optimal surgical window

Delaying transplantation until at least 6 years of age is associated with better graft and patient survival, highlighting why a successful initial Kasai procedure is so valuable 1

Transplantation outcomes are excellent: 1-year survival of 93% and 5-year survival exceeding 85% 1

Long-Term Outcomes for Kasai Survivors

• Approximately 25% of patients achieve long-term survival (>10 years) without transplantation 6
• Two-thirds of long-term survivors experience some manifestation of portal hypertension (variceal bleeding, hypersplenism, ascites) 6
• Among those surviving more than 10 years, 79% remain anicteric with normal liver synthetic function and achieve age-appropriate development 6

Critical Pitfalls to Avoid

• Do not delay referral to an experienced pediatric surgical center—every week counts before 60 days of age 1
• Do not restrict protein intake in these patients despite liver disease 5
• Do not rely on weight alone for nutritional assessment—use triceps skinfold thickness and mid-arm circumference 5
• Do not delay escalation of nutritional support—early aggressive intervention optimizes outcomes 5
• Do not perform the Kasai procedure without adequate surgical experience, as outcomes are operator-dependent 1, 2