儿童先天性门-体分流的诊断和治疗

Diagnosis and management of congenital portosystemic shunts in children

先天性门-体分流是一种非常罕见的门静脉畸形,可导致严重并发症,包括婴幼儿胆汁淤积、肝脏肿瘤、门静脉相关性脑病、肝肺综合征和门脉性肺动脉高压等。早期识别与治疗可预防并逆转严重的临床并发症,改善远期预后。

As a rare portal vein vascular malformation,congenital portosystemic shunt leads to severe complications,including infantile cholestasis,hepatic tumor,portosystemic encephalopathy,hepatopulmonary syndrome and portopulmonary hypertension.Early recognition and prompt therapy prevent and reverse clinically significant complications and improve the long-term outcomes in children.