Abernethy畸形肝脏结节的发病机制和诊疗进展

Advances in the mechanisms,diagnoses and treatments of liver nodules in Abernethy malformation

Abernethy畸形是一种罕见的先天性血管畸形,门静脉血流于肝外经异常通道完全或部分绕过肝脏进入腔静脉系统,进而导致肺动脉高压、肝肺综合征、肝性脑病及肝脏结节等广泛临床表现。本文首先阐述Abernethy畸形肝脏结节的临床特点,然后对肝脏结节形成的发生机制及诊疗手段研究进展进行综述。

Abernethy malformation is a congenital vascular anomaly in which portal blood completely or partially bypasses liver through a congenital portosystemic shunt,resulting in diverse clinical manifestations,such as pulmonary hypertension,hepatopulmonary syndrome,hepatic encephalopathy and liver nodules.Starting with clinical characteristics of liver nodules in Abernethy malformation,this review focused upon the formative mechanism of liver nodules and the latest advances of diagnosing and treating liver nodules in Abernethy malformation.