Clinical experience with adult polycystic liver disease

Adult polycystic liver disease is characterized by multiple diffuse cystic lesions of the liver, usually in association with polycystic kidney disease. Rarely, significant liver disease will produce enough symptoms to call for surgical attention. Symptoms range from simple compression to fatal liver failure. Treatment of seven patients (six women) with symptomatic polycystic liver disease over 18 months is reported. Rapidly progressing liver failure and sepsis from infected ascites following puncture developed in one patient and precluded surgery. Another presented with an acute abdomen and anaemia from liver cyst bleeding and was treated conservatively. Five patients with massive hepatomegaly and compression symptoms underwent fenestration and resection to debulk the cystic liver mass. No intra-abdominal drains were used after operation and there were no hospital deaths. One patient developed a bile leak which was managed by percutaneous drainage. Clinical approach, complications and treatment options in adult polycystic liver disease are reviewed.