A Novel Approach to the Management of Late-Onset Liver Failure in Biliary Atresia By Saleem Islam, Narasimham Dasika, Ronald B. Hirschl, and Arnold G. Coran Ann Arbor, Michigan Purpose: Late-onset hyperbilirubinemia in patients who have undergone a successful portoenterostomy (PE) for biliary atresia (BA) is usually considered evidence of ongoing se- vere 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 drain- age and a reduction in their bilirubin levels. Methods: Data from 2 cases of BA and late-onset hyperbil- irubinemia 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 per- cutaneous transhepatic cholangiography and stenting, fol- lowed 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 dilata- tion is found, then a combined radiologic and operative approach may help improve the bile flow and delay the need for liver transplant. J Pediatr Surg 39:371-374. © 2004 Elsevier Inc. All rights reserved. INDEX WORDS: Biliary atresia, liver failure, percutaneous cholangiography. T HERE ARE a number of reports documenting the appearance of intrahepatic cysts or biliary lakes in patients after the Kasai procedure for biliary atresia (BA). 1-4 These can be seen in conjunction with repeated attacks of cholangitis and have been treated with aspira- tion and or percutaneous transhepatic cholangiograms and drainage. 4 The clinical implications of these cysts are not completely understood but may portend a poor prognosis, with a high incidence of subsequent transplantation. 2 We report the use of a combined approach with sur- gical and interventional radiology techniques to help reestablish bile flow in the portoenterostomy (PE) and perhaps delay the need for liver transplantation. To our knowledge, this combined technique of opening cysts or bile lakes into the portoenterostomy has not been previ- ously reported. CASE REPORTS Case 1 A 15-year-old boy presented with a month-long history of increasing fatigue, fevers, and progressive jaundice. This was associated with pruritis, light-colored stools, and dark urine. He had undergone a successful Kasai procedure (portoenterostomy or PE) in infancy, and was otherwise doing well. His total bilirubin level had increased to 5.7 mg/dL from less than 1 mg/dL a year before presentation. Abdominal ultrasound scan showed dilated intrahepatic ducts, and a HIDA scan showed no excretion into the bowel. He underwent a percutaneous liver biopsy, which showed bile ductule proliferation and bile lakes consis- tent with an obstructive process. A liver transplant workup was initi- ated, and a percutaneous transhepatic cholangiogram (PTC) was per- formed. An attempt was made on PTC to place a transhepatic drainage catheter into the PE, but was unsuccessful because of obstruction at the portal plate (Fig 1A). The patient then underwent a laparotomy, and the Roux-en-Y limb of jejunum was isolated. A small enterotomy was performed, and a 6F sheath was introduced, followed by a catheter and guide wire. The PTC site was also accessed and a wire introduced to the portal plate. Under fluoroscopic guidance, the portal plate stricture was negotiated with blunt and sharp wire manipulation from both sides. A snare was placed through the liver side and the wire pulled through, creating a tract. The tract then was dilated with a balloon catheter, and an 8.5F transhepatic drainage catheter was placed (Fig 1B). The enterotomy and abdomen were closed. The patient made an uneventful recovery with a dramatic reduction in bilirubin levels to less than 1 mg/dL. All the preoperative symptoms resolved and over the course of 6 months, the catheters were exchanged under fluoroscopy to a 12F size, and then removed, leaving a patent tract (Fig 1C). He is now 17 months postprocedure with no recurrence of symptoms. Case 2 A 20-month-old girl presented with rapidly worsening jaundice, pale-colored stools, and fevers. She had a history of biliary atresia as From the Section of Pediatric Surgery and Interventional Radiology, CS Mott Children’s Hospital, University of Michigan Health System, Ann Arbor, MI. Presented at the 34th Annual Meeting of the American Pediatric Surgical Association, Fort Lauderdale, Florida, May 25-28, 2003. Address reprint requests to Arnold G. Coran, MD, F3970 Mott Children’s Hospital, 1500 East Medical Ctr Dr, Ann Arbor, MI 48109. © 2004 Elsevier Inc. All rights reserved. 0022-3468/04/3903-0022$30.00/0 doi:10.1016/j.jpedsurg.2003.11.041 371 Journal of Pediatric Surgery, Vol 39, No 3 (March), 2004: pp 371-374