Current treatment status of polycystic liver disease in Japan

Aim: Polycystic liver disease (PLD) is a genetic disorder characterized by the progressive development of multiple liver cysts. No standardized criteria for the selection of treatment exist because PLD is a rare condition and most patients are asymptomatic. We here aimed to clarify the status of treatment and to present a therapeutic strategy for PLD in Japan. Methods: From 1 June 2011 to 20 December 2011, we administered a questionnaire to 202 PLD patients from 86 medical institutions nationwide. Results: The patients included 45 men and 155 women, and the median age was 63 years. Two hundred and eighty-one treatments were performed for these patients, as follows: cyst aspiration sclerotherapy (AS) in 152 cases, cyst fenestration (FN) in 53, liver resection (LR) in 44, liver transplantation (LT) in 13 and other treatments in 19. For cases of type I PLD (mild form) according to Gigot's classification, the therapeutic effects of AS, FN and LR were similar. For type II (moderate form), LT demonstrated the best therapeutic effects, followed by LR and FN. For type III (severe form), the effects of LT were the best. The incidences of complications were 23.0% in AS, 28.4% in FN, 31.8% in LR and 61.5% in LT. Conclusion: Considering the therapeutic effects and complications, AS, LR and LT showed good results for type I, type II and type III PLD, respectively. However, LT for PLD was performed in a small number of patients. In Japan, the transplantation therapy is expected to be common in the future.