Liver Transplantation and Auxiliary Partial Orthotopic Transplantation Outcomes in Wilson’s Disease

Abstract

Introduction Liver transplantation (LT) is an effective option for Wilson's disease (WD) patients with neurologic symptoms, acute liver failure and with advance decompansated liver disease. In this retrospective study we aimed to review our LT results for WD patients. Materials and Methods Between December 1988 and October 2017 we performed 580 LT procedures at our center (age range, 6 months-64 years). We performed 53 LT in WD (45 living donor LT, 8 deceased donor LT) and after discharge we followed them for 12-350 months. We evaluated the regression of neurologic symptoms and reported survival, graft function of WD patients after LT. Results We performed LT for acute liver failure in 7 WD patients and for end stage liver failure in 41 WD patients. Five patients had LT for neurological WD. One of these neurological WD patients had auxiliary partial orthotopic LT (APOLT) due to neurological WD without hepatic failure (child A). After APOLT he is doing well with normal liver functions and ceruloplasmin levels. All neurologic symptoms were regressed in all of these 5 neurological WD patients. Four of these 53 WD patients had retransplantation (due to 1 primary non-function and 3 chronic rejections) and 3 retransplant patients are doing well with normal liver functions. Thirteen of 53 WD patients died; 6 patients died in early postoperative period, 7 patients died during their follow up due to complications unconnected to WD. The remaining 40 patients have normal liver functions in their follow up (range 12-350 months). Our long term survival is 85%. Conclusion Liver transplantation for WD with end stage liver failure and neurological WD has good outcomes. We may also consider auxiliary partial orthotopic LT for progressive neurological WD patients with no hepatic insufficiency.