C. Ibarra1, D. C. Mulligan1, P. Yoo1, K. Giles1, K. Cartiera1, G. Babas1, C. DelaSancha1, A. Liapakis1, M. Schilsky1, C. Caldwell1, S. Emre1, M. I. Rodriguez-Davalos1 1Yale University School Of Medicine,Surgery – Transplant,New Haven, CT, USA
Introduction: Primary Sclerosing Cholangitis (PSC) is a chronic cholestatic liver disease secondary to a fibrotic inflammation of the intra and/or extra hepatic biliary tree that progresses to cirrhosis, portal hypertension and liver failure, and in some cases cholangiocarcinoma. Liver transplantation remains the only effective treatment for this disorder. Living liver donor transplant (LDLT) offers an option for timely transplantation of these patients. The aim of this study is to review patient and graft survival in a cohort of patients with PSC who underwent liver transplant at our center
Methods: Retrospective review of data from patients transplanted with LDLT or deceased donor organs for PSC between August 2006 and August 2016. Demographics, type of donor, relationship between recipient and donor, type of transplant anastomosis and number of ducts, post-operative surgical complications, recurrence, patient and graft survival were analyzed
Results:Of the last 390 transplants at our center over 10 years, 19 transplants (4.8%) in 18 patients were performed for PSC. Eleven (57.8%) were LDLT; [7 Right lobes in 7 adults, 4 Left lobes in 3 adults and one pediatric patient] and eight (42.2%) received deceased donor organs (DD). Two underwent liver transplantation at other institutions and received graft for retransplantation (1-LD, 1-DD). 14 patients were males (77.7%) with a mean age of 39.9 years (9 – 66yr) and 80% were over the age of 18 yr. IBD was associated in 15 patients; 13 Ulcerative Colitis (72.2%), 2 Crohn’s (11.1%) and 3 patients had no bowel disease (16.6%). Of 19 transplants performed at our institution, 18 survived [median f/u was 48 months, range 10-77 months]. One patient died (due to chronic rejection from non-compliance with immunosuppression) and 1 lost her left lobe donated graft within 90 days from antibody mediated rejection leading to graft loss and small for size syndrome, and was successfully re-transplanted with a DD; 5 year patient and graft survival was excellent at 89 and 90% respectively. No cases of Cholangiocarcinoma (CCA) were found on explant pathology in this series. Biliary reconstruction was carried out by Roux en-Y hepaticojejunostomy to a single duct in 14 patients (77.7%), and to two ducts in 1 patient (5.5%), with duct-to-duct in 3 patients (16.6%).
Conclusion:Living Donor Liver Transplantation is a excellent option for patients who have PSC to permit timely liver transplant and prevent severe complications from infections, liver failure or development of CCA. Right lobe LDLT in adults may offer a greater margin of safety in preventing small for size syndrome with post op complications, however both right and left lobe LDLT was effective for PSC.