Surgical therapy options in polycystic liver disease

Polycystic liver disease (PLD) is a rare affliction frequently observed in association with polycystic kidney disease. Only symptomatic patients require treatment, which can be conservative or surgical, i.e. laparoscopic or conventional. We report the results of our experience in the surgical management of polycystic liver disease.

Between 1994 and 2003, 19 patients (16 female, 3 male) were referred to our center for the management of PLD. Their median age was 50 years (range 33-72). All were symptomatic and their cysts had a median diameter of 11 cm (range 5-22).

Laparoscopic management was undertaken in eight patients, with one conversion to open technique because of bleeding from a superficial hepatic vein. An open procedure was performed in 11 patients: one left hemihepatectomy, deroofing in two patients, segment resection 2/3 plus deroofing in six patients, and segment resection 5/6 plus deroofing in two patients. Four patients had complications: one case of biliary leakage was managed conservatively; two patients had pneumothorax caused by the cava catheter inserted for anesthesia, and one patient's abdominal drain tore off and had to be removed by relaparotomy on the fourth postoperative day. Median follow-up of all patients was 49 months (range 7-98). In one patient there was symptomatic recurrence with hepatomegaly and compression of the inferior vena cava 84 months after the first operation.

Careful selection of patients and meticulous surgical technique are recommended in the management of PLD. The treatment of choice for symptomatic Gigot or Morino type 1 PLD is laparoscopic surgery, and for advanced stage PLD combined hepatic resection and cyst fenestration.