Choice of Transplantation Techniques and Indications for Liver
Transplantation in Polycystic Liver Disease in Patients With No Signs
of End-Stage Liver Disease
O. Kornasiewicz, K. Dudek, M. Bugajski, B. Najnigier, and M. Krawczyk
ABSTRACT
Objective. Since the initiation of the Liver Transplant Program, 500 liver procedures
have been performed. Polycystic liver disease (PLD) and polycystic kidney-liver disease
(PKLD) have been rare indications for orthotopic liver transplantation (OLT). Only 7
patients (1.4%) underwent transplantation due to PLD and PKLD.
Materials and Methods. The group consisted of 4 patients who underwent OLT
(0.8%) and 3 patients who received simultaneous liver kidney transplantation (LKT;
0.6%). Our objective was to analyze the indications for either OLT or combined LKT
as well as indications for surgical techniques during OLT among patients with PLD or
PKLD.
Results. The main indication for OLT was massive hepatomegaly causing severe physical
handicaps, fatigue, and clinically advanced malnutrition. All 3 patients with indications for
combined LKT were dialysis-dependent. None of the patients had symptoms of end-stage
liver disease and/or hepatic failure. In 4 cases, a portal bypass was applied, and the
piggy-back method used in the other 3 cases. The hepatectomy caused no uncommon
difficulty. In cases of simultaneous transplantations, the kidney was implanted separately
after OLT. All patients are alive following the transplantation; major surgical complica-
tions have occurred.
Conclusions. Patients with PLD can undergo OLT safely with good results. They benefit
from the relief of abdominal distension and anorexia. Patients with PKLD who are
dialysis-dependent should undergo simultaneous LKT. The surgical technique was solely
dependent on the intraoperative conditions determined during the dissection phase.
P
OLYCYSTIC liver disease (PLD) is a rare, benign,
inherited disorder characterized by multiple cystic
lesions throughout the liver and kidney.
1,2
Most patients
with PLD have concomitantly associated renal disease.
Liver failure rarely occurs. Many patients with PLD present
with abdominal pain and other symptoms that are due to
cyst enlargement and compression.
3
The development of
PLD appears to correlate with age, female gender, and
gravidity. During the last 17 years, we have performed 500
orthotopic liver transplantations (OLT) with PLD and
polycystic kidney-liver disease (PKLD) as rare indications
for OLT. The purpose of this study was to analyze the
indications for either OLT or combined liver kidney trans-
plantation (LKT) as well as surgical techniques applied in
these patients.
MATERIALS AND METHODS
Between 2003 and 2006, we operated on 5 women and 2 men with
highly symptomatic PLD. In this retrospective study, we reviewed
the medical records. The mean interval from the diagnosis of PLD
to surgical treatment was 16.5 years, ranging from 12 to 20 years.
Patient ages ranged from 37–57 years, averaging 50 years.
Seven patients (1.4%) underwent transplantation for polycystic
disease including 4 patients (0.8%) who underwent OLT, and 3
(0.6%) who underwent simultaneous LKT. The group had concom-
From the Department of General, Transplant and Liver Sur-
gery, Medical University of Warszaw, Warsaw, Poland.
Address reprint requests to Oskar Kornasiewicz, Department
of General, Liver and Transplantation Surgery, Medical Univer-
sity of Warsaw, ul. Banacha 1a, 02-097 Warszawa, Poland.
E-mail: oskar.kornasiewicz@gmail.com
0041-1345/08/$–see front matter © 2008 by Elsevier Inc. All rights reserved.
doi:10.1016/j.transproceed.2008.02.080 360 Park Avenue South, New York, NY 10010-1710
1536 Transplantation Proceedings, 40, 1536 –1538 (2008)