Split liver transplantation for acute Wilson's disease: new option for urgent recipient?
Wilson's disease is a rare metabolic disorder that may lead to fulminant hepatitis and subsequent liver failure. Herein, we present a case of split liver transplantation performed on a patient with acute Wilson's disease. A 27-year-old female with acute presentation of Wilson's disease and advanced neurological impairment, received a Right Split liver Graft (Segments: IV, V, VI, VII and VIII) transplant. The graft was obtained by an in situ splitting technique. The graft implantation was performed in a standard fashion. No acute rejection episodes of the organ occurred. The postoperative course was uneventful. The graft function, ceruloplasmine level and copper levels progressively normalized. The patient totally recovered from neurological symptoms and the Kayser-Fleischer rings disappeared within one month. At 13 months of follow-up, the patient presented with no symptoms and in good condition. The current literature reports high preoperative mortality rate in patients that underwent partial liver graft for acute hepatic failure. However, our experience indicates that in situ split technique of liver may be a feasible and effective alternative to whole graft transplantation in urgent cases. Moreover, to our knowledge, this is the first successfully case of in situ split liver transplantation for acute Wilson's disease described in literature.
Andorno
E
E
Department of General and Transplant Surgery, St. Martino Hospital, University of Genoa, Genoa, Italy.
Miggino
M
M
Panaro
F
F
Morelli
N
N
Bottino
G
G
Casaccia
M
M
Jarzembowski
T M
TM
Valente
U
U
D000208:Acute Disease; D000328:Adult; D004638:Emergency Treatment; D005260:Female; D006527:Hepatolenticular Degeneration / Q000601:surgery*; D006801:Humans; D016031:Liver Transplantation / Q000379:methods*