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Biliary Atresia Kasai Procedure Failure

Persistent jaundice or progressive cholestatic liver disease after Kasai portoenterostomy in biliary atresia - the leading indication for pediatric liver transplantation.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Çocuk Sağlığı ve Hastalıkları department. Book Appointment →

What is Biliary Atresia Kasai Procedure Failure?

Biliary atresia (BA) is an obliterative cholangiopathy of unknown etiology that involves intra/extrahepatic bile ducts in newborns; incidence 1/15,000-19,000 live births. The pathogenesis is thought to be multifactorial - viral infection (CMV, reovirus), genetic predisposition, immune dysregulation. Untreated, mortality is 100% by 2 years (cirrhosis, hepatic failure).

Kasai hepatic portoenterostomy (HPE) is the standard treatment - performed within the first 60 days. Roux-en-Y intestinal loop is anastomosed to porta hepatis dissection field for bile drainage. Success criteria: serum bilirubin <2 mg/dL at 6 months. Success rates: <30 days 80%, 30-60 days 60%, 60-90 days 40%, >90 days 25%. Long-term native liver survival: 5 years 30-40%, 20 years 10-20%.

Kasai failure: 1) Early failure - persistent jaundice (bilirubin >2 mg/dL at 6 months) - liver transplantation immediately; 2) Late failure - progressive cholestasis, recurrent cholangitis (40-60%), portal hypertension complications (varices bleeding, ascites), hepatopulmonary syndrome, hepatocellular carcinoma (rare). Liver transplantation post-Kasai 5-year survival 90-95%. Predictors: surgery age, surgeon experience, postoperative cholangitis frequency, ductal plate paucity.

Symptoms

Persistent jaundice (>6 months)
Recurrent cholangitis attacks
Hepatomegaly and splenomegaly
Failure to thrive
Acholic stools
Bleeding from esophageal varices

Risk Factors

Late Kasai surgery (>60 days)
Severe ductal plate paucity
Recurrent postoperative cholangitis
Family history of biliary atresia
BASM (BA splenic malformation syndrome)
Cytomegalovirus association

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Persistent jaundice 6 months post-Kasai
  • Recurrent cholangitis attacks
  • Failure to thrive
  • Variceal bleeding
  • Ascites and hepatic failure signs
  • Pre-liver transplant evaluation

Treatment Methods

01
Early Kasai (within first 60 days)
02
Postoperative ursodeoxycholic acid
03
Cholangitis prophylaxis (antibiotic)
04
Fat-soluble vitamin (ADEK)
05
Liver transplantation (Kasai failure)
06
Living-donor LT preferred

Which Department to Visit?

You can visit our Çocuk Sağlığı ve Hastalıkları department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Çocuk Sağlığı ve Hastalıkları Department

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.