A basic understanding of congenital extrahepatic portosystemic shunt: incidence, mechanism, complications, diagnosis, and treatment

作者：Haowen Tang, Peipei Song, Zhiqiang Wang, Bing Han, Xiangfei Meng, Yingwei Pan, Xuan Meng, Weidong Duan · 发表于：Intractable & Rare Diseases Research · 年份：2020 · DOI：10.5582/irdr.2020.03005 · 被引用次数：27 · 研究领域：Liver Disease and Transplantation、Organ Transplantation Techniques and Outcomes、Liver Disease Diagnosis and Treatment

Extrahepatic portosystemic shunt belongs to a family of rare vascular abnormalities. The clinical importance and manifestations of this vascular abnormality range from asymptomatic cases to liver or metabolic dysfunctions of various degrees. Congenital extrahepatic portosystemic shunt, also termed as Abernethy malformation, is a very rare congenital vascular malformation in which splenomesenteric blood drains into a systemic vein, bypassing the liver through a complete or partial extrahepatic shunt. So far, limited cases of congenital extrahepatic portosystemic shunt have been reported. In this review, incidence, mechanisms, complications, diagnoses and treatments of congenital extrahepatic portosystemic shunt are described.