Polycystic liver disease: A critical appraisal of hepatic resection, cyst fenestration, and liver transplantation

OBJECTIVE: To identify operative morbidity, mortality, and long-term outcome after operative treatment for symptomatic polycystic liver disease (PLD) and develop a treatment algorithm for patients with PLD. BACKGROUND: PLD represents a challenging clinical problem that can result in massive hepatomegaly and various complications, leading to significant decline in health status and quality of life. The optimal surgical treatment for this disease is still evolving. METHODS: All patients who underwent hepatic resection, cyst fenestration, or liver transplantation for PLD from 1985 to 2006 were identified retrospectively. Long-term outcomes were evaluated by patient survey. Mean follow-up was 8 ± 0.5 years. RESULTS: Of 141 patients (122 women; age: 51 ± 1 years) with PLD, 117 had concomitant polycystic kidney disease. All patients suffered from symptomatic hepatomegaly with 85% being functionally impaired (Eastern Cooperative Oncology Group Performance Status: 1-3). Despite significant inferior vena cava or hepatic venous compression in 65%, hepatic function was commonly preserved. A total of 124 patients underwent partial hepatectomy with cyst fenestration, 10 underwent cyst fenestration alone, and 7 underwent liver transplantation for primary treatment of PLD. Overall operative morbidity and mortality was 58% and 4%, respectively, with major complications (Clavien grade: III-V) in 30%. Five- and 10-year survival was 90% and 78%, respectively. Eastern Cooperative Oncology Group Performance Status performance status normalized or improved in 75% of patients and 73% returned to work full-time. At follow-up, health survey scores were similar to the general population despite subsequent recurrence of symptoms in 73% of patients. CONCLUSION: Selective patients with massive hepatomegaly from PLD benefit from operative intervention. The type of operation performed is mainly dependent on the distribution of the cysts, coincident sectoral vascular patency and parenchymal preservation, and hepatic reserve. Hepatic resection can be performed with acceptable morbidity and mortality, prompt and durable relief of symptoms, and maintenance of liver function. Cyst fenestration and liver transplantation, though effective in selected patients, are less broadly applicable.