A novel approach to the management of late-onset liver failure in biliary atresia.
OBJECTIVE
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 hyperbilirubinemia 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.
Islam
Saleem
S
Section of Pediatric Surgery, CS Mott Children's Hospital, University of Michigan Health System, Ann Arbor, MI 48109, USA.
Dasika
Narasimham
N
Hirschl
Ronald B
RB
Coran
Arnold G
AG
D000293:Adolescent; D001653:Bile Ducts, Intrahepatic / Q000000981:diagnostic imaging / Q000601:surgery*; D001656:Biliary Atresia / Q000150:complications* / Q000601:surgery; D002758:Cholangiography / Q000379:methods; D002780:Cholestasis, Intrahepatic / Q000150:complications / Q000000981:diagnostic imaging / Q000601:surgery*; D005260:Female; D006801:Humans; D006932:Hyperbilirubinemia / Q000209:etiology / Q000601:surgery; D007223:Infant; D017114:Liver Failure, Acute / Q000209:etiology / Q000601:surgery*; D008297:Male; D011171:Portoenterostomy, Hepatic; D011183:Postoperative Complications