Ongaro Marie, Bronstein Francois-Nathan, Goossens Nicolas, Spahr Laurent, Bresson Hadni Solange
Service de gastroentérologie et hépatologie, Hôpitaux universitaires de Genève, 1211 Genève 14.
Rev Med Suisse. 2022 Aug 31;18(793):1599-1605. doi: 10.53738/REVMED.2022.18.793.1599.
Polycystic liver disease (PLD) includes three entities in adults : biliary hamartomas which develop as a result of ductal plate malformation, autosomal dominant polycystic liver disease (ADPLD) and autosomal dominant polycystic kidney disease (ADPKD) which occur in the setting of genetic disorders. Hamartomas are asymptomatic and benign. PLD are marked by a steady growth of cysts over time, clinically silent in the majority of cases. Symptomatic forms mainly affect women due to the influence of estrogens on the growth of cysts therefore estrogen treatments are contraindicated in this setting. Diagnosis is based on imaging. Complications are rare but must be identified early in order to offer appropriate care in an expert center.
多囊肝病（PLD）在成人中包括三种类型：因导管板畸形而形成的胆管错构瘤、常染色体显性遗传多囊肝病（ADPLD）以及发生于遗传性疾病背景下的常染色体显性遗传多囊肾病（ADPKD）。错构瘤无症状且为良性。PLD的特征是囊肿随时间持续生长，大多数情况下临床无症状。有症状的类型主要影响女性，因为雌激素会影响囊肿生长，所以在此情况下禁忌使用雌激素治疗。诊断基于影像学检查。并发症罕见，但必须尽早识别，以便在专业中心提供适当治疗。
