Piggyback orthotopic liver transplantation in the treatment of Wilson′s disease:a report of 29 cases

Objective To summarize the clinical experience of standard piggyback liver transplantation(SPBLT) and modified piggy-back liver transplantation(MPBLT) in the treatment of Wilson′s disease(WD).Methods The clinical records of 29 cases of WD who underwent piggyback liver transplantation over the recent 12 years were analyzed retrospestively.Among them,there were 22 male and 7 female patients,with 6cases aged 8-14y and 23 cases aged 24-37y,who underwent liver transplantation because of chronic advanced liver disease(18cases),fulminant hepatic failure(2 cases),or with normal liver function(9 case),and among them,24 cases showed neurological dysfunction.The modes of operation included SPBLT(13 cases) and MPBLT(16 cases).Results The patients were followed up for 1 month-12 years with the median follow-up time of 47 months.Four patients died in the perioperative period,of which 2 died of hepatic failure due to obstruction of hepatic venous return after SPBLT,and 2 died of hepatic artery thrombosis and bile leakage combined with severe infection.The recipient survival rate at 1 year and 3 years was 86% and 79% respectively.One patient has survived for 12 years.All the levels of serum copper and copper-protein recovered to normal in four weeks post-operatively.Neurological symptoms improved in varying degrees after operation.Conclusions WD patients who undergo PBLT can expect a satisfactory clinical outcome and a good quality of life,and MPBLT can effectively reduce the complications of operation and improve the operation survival rate.