Highly symptomatic adult polycystic liver disease: options and results of surgical management.

BACKGROUND

The majority of patients afflicted with adult polycystic liver disease (APLD) are asymptomatic. For those who are symptomatic, there are a variety of treatment procedures that have been proposed but these lack verification through long-term studies with respect to safety and long-term effectiveness. Choice of surgical procedure is related to the severity of APLD and morphology of the cysts within the liver. The aim of the present study was to analyse the immediate and long-term results of fenestration and combined resection-fenestration at Singapore General Hospital.

METHODS

A retrospective analysis of clinical, operative, imaging and follow-up data was carried out for 12 patients (10 women and two men) with symptomatic APLD who underwent surgery from January 1992 to December 2000. The primary outcome measures assessed were postoperative alleviation of symptoms, performance status, complications, mortality and long-term recurrence of symptoms.

RESULTS

Nine patients underwent 12 fenestration procedures and three patients had combined resection-fenestration. Fenestration was carried out for eight of nine patients with a dominant cyst morphology and combination resection-fenestration was carried out for those three patients with diffuse cyst morphology. There was no operative mortality and all patients were discharged from hospital free of their preoperative symptoms. Overall morbidity rate was 58%. The mean follow up for the present cohort was 29.3 months. Only two patients had recurrence of symptoms. One patient with dominant cyst morphology who underwent laparoscopic fenestration had recurrence at 26 and 43 months but this was successfully treated finally with open fenestration. The other patient had diffuse cyst morphology and was treated with fenestration for recurrent cyst infection that recurred 1 month postoperatively. This required subsequent intravenous antibiotics and percutaneous drainage for resolution of symptoms.

CONCLUSION

Treatment for symptomatic APLD should be based on the morphology of the liver cysts. Fenestration is a safe and acceptable procedure for patients with a dominant cyst pattern where liver size can be reduced after the cysts collapse. A combination of resection-fenestration is suitable for those with a diffuse cyst pattern where grossly affected segments are resected in combination with fenestration to allow for reduction in liver size.