Introduction: Polycystic liver disease (PCLD) can lead to massive, highly symptomatic hepatomegaly and a reduced quality of life. While hepatic resection and cyst fenestration can be used selectively for palliation of patients with well-preserved hepatic and renal function, liver transplantation (LT) has been proposed as primary therapy for patients with bilobar disease, malnutrition, and concomitant renal dysfunction.

Objective: To report our institutional experience of LT for PCLD and identify predictors of outcome.

Setting: Large university medical center, October 1992 through August 2012

Patients: 27 patients with PCLD underwent LT; 25 (93%) had polycystic kidneys (PCLKD), and 11(41%) underwent concomitant kidney transplantation. Mean age was 50 (range 13-64) years, and 81% were female. 17 patients had prior disease-directed surgical therapy including liver cyst fenestration (10), liver resection (3), nephrectomy (9), and kidney transplantation (6). Indications for LT included chronic abdominal pain (96%), early satiety (52%), renal failure (41%), dyspnea (37%), and ascites (30%). Mean follow-up was 64 months.

Results: Overall survival after LT was 81%. Comparing patients with and without prior surgery, the former group had significantly greater length of stay, lower overall survival, and a trend to more post-transplant reoperations, as shown:

In the sub-group of patients with prior surgery, patients who died (n=5) had more than twice the number of pre-transplant operations (3.0 vs 1.3; P=0.03) and were the only patients to require reoperation (80% vs 0%; P=0.02), compared to the long-term survivors (n=12).

Conclusions: This is one of the largest series of LT for PCLD/PCKLD. Although long-term survival is excellent, prior palliative surgical therapy significantly increases the risk for post-transplant morbidity and mortality and should be avoided in patients best treated with LT.

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