Background

For adult liver transplants, choledochocholedochostomy or “duct-to-duct” biliary reconstruction is the standard of care. In contrast to a Roux-en-Y reconstruction, it maintains physiologic bile flow, limits operative intestinal manipulation, and facilitates postoperative endoscopic access. However, its efficacy in pediatric transplants has seldom been studied, let alone in patients with cystic fibrosis. These patients have a poorly characterized pattern of bile duct changes that includes lymphocyte infiltration and eosinophilic inspissated secretions. Given these specialized changes, our purpose was to conduct a review of pediatric patients at our institution who have undergone liver transplantation for cystic fibrosis, and evaluate the effect of duct-to-duct reconstruction on biliary complications.

Methods

A retrospective analysis was conducted by identifying all patients who received a liver transplant for cystic fibrosis related end stage liver disease at our pediatric institution. A pathologist reviewed explant pathology for all subjects. Post-operative outcomes included biliary complications as well as graft and patient survival. Biliary complications were defined as biliary leaks or strictures seen on endoscopic or radiographic imaging.

Results

A total of 17 patients with cystic fibrosis at our institution have undergone orthotopic deceased-donor liver transplantation with a duct-to-duct biliary reconstruction. Over half of the patients were female, and ages ranged from 9 to 19 years with a mean of 14. On pathology, nearly all explanted livers demonstrated extensive portal fibrosis with duct proliferation and bile duct dilatation with bile plugs or eosinophilic granular material. The time to follow up ranged from 1 to 116 months, with a median follow up of 36 months. At 1 year follow-up, there have been no graft failures and patient survival was 100%. Furthermore, we have no reported cases of biliary strictures or leaks during this follow up period.

Conclusions

Despite profound pathological changes in the bile ducts of cystic fibrosis patients, we found no incidence of biliary complications after duct-to-duct biliary reconstruction including strictures or biliary leaks. These findings validate the use of choledochocholedochostomy as a safe and effective method of biliary reconstruction in this patient population.

CITATION INFORMATION: Rizk P, Moffett J, Vu M, Ackah R, Sigireddi R, Finegold M, O'Mahony C, Rana A, Goss J. Biliary Complications in Pediatric Liver Transplantation for Cystic Fibrosis: A Single-Center Experience with Choledochocholedochostomy. Am J Transplant. 2017;17 (suppl 3).

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