Polycystic liver disease: Classification, diagnosis, treatment process, and clinical management

Abstract

Polycystic liver disease (PLD) is a rare hereditary disease that independently exists in isolated PLD, or as an accompanying symptom of autosomal dominant polycystic kidney disease and autosomal recessive polycystic kidney disease with complicated mechanisms. PLD currently lacks a unified diagnostic standard. The diagnosis of PLD is usually made when the number of hepatic cysts is more than 20. Gigot classification and Schnelldorfer classification are now commonly used to define severity in PLD. Most PLD patients have no clinical symptoms, and minority with severe complications need treatments. Somatostatin analogues, mammalian target of rapamycin inhibitor, ursodeoxycholic acid and vasopressin-2 receptor antagonist are the potentially effective medical therapies, while cyst aspiration and sclerosis, transcatheter arterial embolization, fenestration, hepatic resection and liver transplantation are the options of invasion therapies. However, the effectiveness of these therapies except liver transplantation are still uncertain. Furthermore, there is no unified strategy to treat PLD between medical centers at present. In order to better understand recent study progresses on PLD for clinical practice and obtain potential directions for future researches, this review mainly focuses on the recent progress in PLD classification, clinical manifestation, diagnosis and treatment. For information, we also provided medical treatment processes of PLD in our medical center.

Keywords: Polycystic liver disease; Autosomal dominant polycystic kidney disease; Autosomal recessive polycystic kidney disease; Isolated polycystic liver disease; Diagnosis; Treatment

Core tip: Polycystic liver disease (PLD) is a rare hereditary disease. However, there is no unified strategy in the treatment of PLD so far. In order to better understand recent progresses on clinical practice of PLD and contribute to potential directions for future researches, we conducted this review mainly focusing on recent progresses of PLD classification, clinical manifestation, diagnosis and treatments. For information, we also provided medical treatment process of PLD that is being used in our medical center.