Symptomatic polycystic liver disease: a rare indication of hepatectomy.

ABSTRACT

We present the case of forty-four-year-old woman with autosomal dominant Polycystic liver disease (PLD) without kidney disease diagnosed for 18 years ago. She experienced progressive abdominal distension and malnutrition over the previous twelve months. The patient had no other comorbidities. Physical examination revealed an 8 cm hepatomegaly in the right hypochondrium region. Liver function analysis was normal. CT scan showed multiple liver cysts with smooth and regular walls, predominantly in the right lobe which was considered as type III according to Gigot´s classification. Due to the significant hepatomegaly, conventional surgery was performed. Exploration showed multiple cysts of varying sizes on the liver surface, with diffuse involvement of the right hemiliver. Standard right lobe mobilization was technically not possible. Fenestration was performed in dominant superficial cysts, to allow access to the deeper cysts and liver parenchyma. A right hepatectomy was carried up using ultrasound devices and ultrasonic aspirator. And the biggest cysts located in the surface of left hepatic lobe were surgically unroofed. No surgical complications occurred, and the patient was discharged 5 days after the intervention.