PDF(Pubmed)
Abstract：
The Abernethy malformation is an extremely rare congenital, extrahepatic, portosystemic shunt. There are many problems associated with this abnormal portovenous shunting and subsequent reduced hepatic portal venous flow. With the advances in non-invasive imaging technologies, these cases are diagnosed in more numbers; however, the presentation of patients is varied and the natural history is not completely known. The presenting symptom of the portosystemic shunt is mainly hyperammonemia, leading to encephalopathy. Management varies depending on the type of shunt and its clinical course; hence, the classification of the congenital portosystemic shunt is important in these patients.
摘要：
Abernethy畸形是一种极为罕见的先天性畸形,肝外,门体分流术.这种异常的门静脉分流和随后的肝门静脉流量减少存在许多问题。随着非侵入性成像技术的进步，这些病例的诊断数量更多；然而，患者的表现多种多样，自然史尚不完全清楚。门体分流的表现症状主要是高氨血症，导致脑病.管理因分流类型及其临床过程而异；因此，先天性门体分流术的分类在这些患者中很重要。
