Congenital portosystemic vascular malformations.
Congenital portosystemic shunts are developmental abnormalities of the portal venous system resulting in the diversion of portal blood away from the liver to the systemic venous system. Such malformations are believed to come from an insult occurring between the fourth and eighth week of gestation during the development of hepatic and systemic venous systems, and could explain their frequent association with cardiac and other vascular anomalies. They are currently categorized into end-to-side shunts (type I) or side-to-side shunts (type II). This article aims to review the common symptoms and complications encountered in congenital portosystemic shunts, the surgical and endovascular treatment, and the role of liver transplantation in this disease. We will also focus on the current controversies and the areas where there is potential for future studies.
Department of Pediatric Surgery, Bicêtre Hospital, Paris, France. florent.guerin-chir@bct.aphp.fr
Florent
F
Guérin
Thomas
T
Blanc
Frédéric
F
Gauthier
Stephanie Franchi
SF
Abella
Sophie
S
Branchereau
D057510:Endovascular Procedures; D006503:Hepatic Veins / Q000196:embryology; D006801:Humans; D016031:Liver Transplantation; D011169:Portal Vein / Q000002:abnormalities* / Q000196:embryology / Q000601:surgery; D014471:Umbilical Veins / Q000196:embryology; D054079:Vascular Malformations / Q000150:complications / Q000175:diagnosis / Q000196:embryology / Q000601:surgery; D014682:Vena Cava, Inferior / Q000196:embryology