“Given the relative rarity of PCLD, current data on the outcomes of surgical debulking for advanced PCLD are scarce, particularly within the realm of laparoscopic hepatic
resection techniques. We therefore sought to evaluate the characteristics of patients undergoing operative debulking for advanced PCLD and analyze the perioperative and long-
term postoperative outcomes of hepatic resection for PCLD.”
“Adult polycystic liver disease (PCLD) is an autosomal dominant condition commonly associated with autosomal dominant polycystic kidney disease (ADPKD). However
in the last decade, it has been recognized that there is a distinct form of autosomal dominant PCLD that arises without concomitant ADPKD. Early knowledge of the pathogenesis was gained from the study of hepatic cysts in patients with ADPKD.”
“Polycystic liver disease (PLD) is the result of embryonic ductal plate malformation of the intrahepatic biliary tree.The phenotype consists of numerous cysts spread throughout the liver parenchyma. Cystic bile duct malformations originating from the peripheral biliary tree are called Von Meyenburg complexes (VMC). In these patients embryonic remnants develop into small hepatic cysts and usually remain silent during life. Symptomatic PLD occurs mainly in
the context of isolated polycystic liver disease (PCLD) and autosomal dominant polycystic kidney disease (ADPKD).”
“Management of adult PLD is based on liver phenotype, severity of clinical features and quality of life. Conservative treatment is recommended for the majority of PLD patients. The primary aim is to halt cyst growth to allow abdominal decompression and ameliorate symptoms. Invasive procedures are required in a selective patient group with advanced PCLD, ADPKD or
liver failure. Pharmacological therapy by somatostatin analogues lead to beneficial outcome of PLD in terms of symptom relief and liver volume reduction.”
Surgical Focus 