ADULT POLYCYSTIC LIVER-DISEASE - IS FENESTRATION THE MOST ADEQUATE OPERATION FOR LONG-TERM MANAGEMENT

Objective The aim of this study was to evaluate the immediate and long -term results in a retrospective series of patients with highly sympto matic adult polycystic liver disease (APLD) treated by extensive fenes tration techniques. A classification of APLD was developed as a strati fication scheme to help surgeons conceptualize which operation to offe r to patients with APLD. Summary Background Data Treatment options for APLD remain controversial, with partisans of fenestration techniques or combined liver resection-fenestration.Methods Clinical symptoms, pe rformance 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 parench yma. Follow-up was obtained by clinical and CT examinations in all pat ients. Results Ten patients with highly symptomatic APLD were operated on using an extensive fenestration technique (by laparotomy in 8 pati ents and by laparoscopy in 2 patients, 1 of whom conversion to laparot omy was required). The mean preoperative liver volume was 7761 cm(3). There was no mortality. Postoperative morbidity occurred in 50%, mainl y from biliary complications, requiring reintervention in two cases. M assive intraoperative hemorrhage occurred in one patient. During a mea n follow-up time of 71 months (range, 17 to 239 months), ail patients were improved clinically according to their estimated performance stat us. The mean postoperative liver volume was 4596 cm(3), which represen ts a mean liver volume reduction rate of 43%. However, in type III APL D, despite absence of clinical symptoms, a significant increase in liv er volume was observed in 40% of the patients. Conclusions Extensive f enestration 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 pe rformed laparoscopically in type I APLD. A longer follow-up period is mandatory in type II APLD, to confirm the usefulness of the fenestrati on procedure. In type III APLD, significant disease progression was ob served in 40% of the patients during long-term follow-up. Fenestration may not he the most appropriate operation for long-term management of all types of APLD.