Intractable Rare Dis Res. 2020;9(2):64-70. (DOI: 10.5582/irdr.2020.03005)

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

Tang HW, Song PP, Wang ZQ, Han B, Meng XF, Pan YW, Meng X, Duan WD


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.

KEYWORDS: Congenital extrahepatic portosystemic shunt, incidence, mechanism, complications, diagnosis, treatment, review

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