Adult Polycystic Liver Disease

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Abstract
The aim of this study was to evaluate the immediate and long-term results in a retrospective series of patients with highly symptomatic adult polycystic liver disease (APLD) treated by extensive fenestration techniques. A classification of APLD was developed as a stratification scheme to help surgeons conceptualize which operation to offer to patients with APLD. Treatment options for APLD remain controversial, with partisans of fenestration techniques or combined liver resection-fenestration. Clinical symptoms, performance status, liver volume measurement by computed tomography (CT), and morbidity were recorded before surgery and after surgery. Adult polycystic liver disease was classified according to the number, size, and location of liver cysts and the amount of remaining liver parenchyma. Follow-up was obtained by clinical and CT examinations in all patients. Ten patients with highly symptomatic APLD were operated on using an extensive fenestration technique (by laparotomy in 8 patients and by laparoscopy in 2 patients, 1 of whom conversion to laparotomy was required). The mean preoperative liver volume was 7761 cm3. There was no mortality. Postoperative morbidity occurred in 50%, mainly from biliary complications, requiring reintervention in two cases. Massive intraoperative hemorrhage occurred in one patient. During a mean follow-up time of 71 months (range, 17 to 239 months), all patients were improved clinically according to their estimated performance status. The mean postoperative liver volume was 4596 cm3, which represents a mean liver volume reduction rate of 43%. However, in type III APLD, despite absence of clinical symptoms, a significant increase in liver volume was observed in 40% of the patients. Extensive fenestration is effective in relieving symptoms in patients with APLD. Hemorrhage and biliary complications are possible consequences of such an aggressive attempt to reduce liver volume. The procedure can be performed laparoscopically in type I APLD. A longer follow-up period is mandatory in type II APLD, to confirm the usefulness of the fenestration procedure. In type III APLD, significant disease progression was observed in 40% of the patients during long-term follow-up. Fenestration may not be the most appropriate operation for long-term management of all types of APLD.