The status of polycystic liver disease in Japan: a questionnaire survey of patients

Abstract

Polycystic liver disease (PLD) is genetic disorder characterized by the progressive development of multiple liver cysts. Because of rarity of PLD, therapeutic consensus is controversial. The aim of this study was to investigate a situation of PLD treatment by a questionnaire survey. In this study, 422 patients with PLD participated, and 223 patients underwent treatment. The most common symptom was abdominal distension (73%). The treatments were performed as follows; percutaneous aspiration in 27% of patients, liver resection in 12%, cyst fenestration in 9%, and liver transplantation in 3%. The efficacies of these treatments were recognized in 77%, 96%, 92%, and 100% of each patient, respectively. Because of no effect of primary therapy, additional treatment was performed in only 4.3% of patients. In general, the choice of primary treatment for PLD was considered reasonable and proper, in Japan.