Therapeutic dilemmas in patients with symptomatic polycystic liver disease.

Most reports of the operative treatment of symptomatic polycystic liver disease (PCLD) are anecdotal or consist of only a small subset of patients in an institution's overall experience treating hepatic cysts. We have reviewed our experience with nine consecutive patients with symptomatic PCLD undergoing operative treatment from 1981 to 1987. Indications for operation include chronic abdominal pain (4 patients), cyst infection (2 patients), biliary obstruction (2 patients), inferior vena cava obstruction (2 patients), and symptomatic abdominal distention (2 patients). The average duration of symptoms leading to operation was 7.8 months. Three types of cystic disease were identified based on gross morphology: dominant cystic disease (3 patients), diffuse cystic disease (4 patients), and mixed cystic disease (2 patients). Operations to treat symptomatic PCLD included unroofing and external drainage of infected cysts (2 operations), simple unroofing (1 operation), cyst fenestration alone (4 operations) and fenestration combined with resection (3 operations). Treatment directed at principally dominant cysts (five patients) was associated with resolution of symptoms and low morbidity and mortality. Treatment directed at diffusely cystic disease (four patients) resulted in significant morbidity and mortality including three deaths. Successful surgical treatment of symptomatic patients with PCLD depends on accurate preoperative identification of patients with symptoms related to one or more dominant cysts. In this setting fenestration or simple unroofing of the dominant cyst is safe and effective treatment. By comparison, extensive fenestration with or without hepatic resection in patients with symptoms attributed to a diffusely cystic liver may be associated with unacceptable morbidity and mortality.(ABSTRACT TRUNCATED AT 250 WORDS)