A Novel Approach to the Management of Late-Onset Liver Failure in Biliary Atresia

Saleem Islam, Narasimham Dasika, Ronald B. Hirschl, Arnold G. Coran

Research output: Contribution to journalArticlepeer-review

5 Citations (Scopus)


Purpose: Late-onset hyperbilirubinemia in patients who have undergone a successful portoenterostomy (PE) for biliary atresia (BA) is usually considered evidence of ongoing severe liver failure. The authors recently have treated 2 patients who had acute hyperbilirubinemia years and months after a successful PE and had dilated intrahepatic cysts. A combined operative and percutaneous approach reestablished drainage and a reduction in their bilirubin levels. Methods: Data from 2 cases of BA and late-onset hyperbilrubinemia from obstruction were reviewed and analyzed. Results: Two patients (15-year-old boy and a 2.5-year-old girl) presented with increasing serum bilirubin after a PE for BA in infancy. Both had extensive preoperative workup, which showed intrahepatic biliary dilatation in one and a large bile lake in the other. They underwent attempted percutaneous transhepatic cholangiography and stenting, followed by an intraoperative transhepatic approach in which the dilated ducts were connected to the PE. A rapid and sustained reduction in the serum bilirubin level was noted in these patients. Conclusions: When patients with BA after a successful PE present with sudden onset of hyperbilirubinemia, imaging for biliary obstruction should be carried out. If biliary dilatation is found, then a combined radiologic and operative approach may help improve the bile flow and delay the need for liver transplant.

Original languageEnglish
Pages (from-to)371-374
Number of pages4
JournalJournal of Pediatric Surgery
Issue number3
Publication statusPublished - Mar 2004
Externally publishedYes


  • Biliary atresia
  • Liver failure
  • Percutaneous cholangiography


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