[Orthotopic liver transplantation in polycystic liver disease]

There is currently no effective medical therapy for polycystic liver (PCL). Cyst puncture and sclerotherapy, cyst fenestration, or partial hepatic resections have been used as palliative treatments. Orthotopic liver transplantation (OLT) has become the treatment of choice for terminal PCL, being indicated in patients with limiting symptoms not susceptible to any other medical treatment. It is also difficult to determine the priority on the waiting list using the Model for End-Stage Liver Disease (MELD).

A retrospective analysis of OLT for PCL was conducted in our centre. Inclusion criteria were patients with limiting symptoms, bilateral cysts liver, and insufficient remaining liver. In all cases a deceased donor liver transplantation with piggy-back technique without veno-venous bypass was performed.

Six patients underwent liver transplantation for PCL between April 1992 and April 2010, one of them a combined liver-kidney transplantation. The mean intraoperative packed red blood cell transfusion was 3.25 L and fresh frozen plasma was 1.200 cc. Mean operation time was 299 min, and 498 min in the liver-kidney transplantation. There was no peri-operative mortality. The mean hospital stay was 6.5 days. All patients are healthy after a mean follow-up of 71 months.

OLT offers an excellent overall survival. Results are better when OLT is performed early; thus these patients should receive additional points to be able to use the MELD score as a valid prioritisation system for waiting lists.