先天性肝外门体静脉分流Abernethy畸形1例并文献复习

A case report of congenital extrahepatic portosystemic venous shunt—Abernethy malformation and literatures review

Abernethy畸形是一种异常的肝外门体静脉分流的血管畸形。该疾病相对罕见,临床表现多样,可表现为门静脉高压症、肝脏多发结节及消化道出血等症状。腹部超声检查有助于Abernethy畸形的早期筛查,CT血管造影(CT angiography,CTA)、磁共振血管成像(magnetic resonance angiography,MRA)及数字减影血管造影(digital subtraction angiography,DSA)均可作为Abernethy畸形的最佳确诊及分型方法。现报道本院1例首诊肝硬化最终确诊为Abernethy畸形患者的临床资料并回顾相关文献进行分析总结。

Abernethy malformation is an abnormal extrahepatic portosystemic venous shunt vascularmalformation which is relatively rare and has a variety of clinical manifestations. It can manifest as portalhypertension, multiple nodules of the liver and gastrointestinial bleeding. Abdominal ultrasonographyis helpful for early screening of Abernethy malformation. CT angiography （CTA）, magnetic resonanceangiography （MRA） and digital subtraction angiography （DSA） can be used as the best diagnosis and typingof Abernethy malformation. In this paper, a case of Abernethy malformation firstly diagnosed as liver cirrhosisin our hospital was reported and the involved literatures were reviewed and analyzed.