Diagnóstico imagenológico en el Síndrome de Abernethy

Congenital portosystemic shunt (CEPS) or Abernethy Syndrome is a rare condition that was first reported by John Abernethy in 1793. Two types of CEPS are described: type I (side to end anastomosis) or congenital absence of the portal vein, and type II (side to side anastomosis) with portal vein suppl...

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Publicado en:Revista Médica Universitaria
Autores principales: Bufaliza, Jorge, Correa, Roberto, Gutierrez, Mario, Manzino, Ricardo, Pérez Monteleone, Leonardo E.
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Acceso en línea:https://bdigital.uncu.edu.ar/fichas.php?idobjeto=9705
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Sumario:Congenital portosystemic shunt (CEPS) or Abernethy Syndrome is a rare condition that was first reported by John Abernethy in 1793. Two types of CEPS are described: type I (side to end anastomosis) or congenital absence of the portal vein, and type II (side to side anastomosis) with portal vein supply partially conserved. Type I CEPS is usually seen in girls and associates multiple malformations as polysplenia, malrotation, and cardiac anomalies. Type II is even rarer with no sex preference and no malformations associated. Hepatic encephalopathy is a common complication of both types in adulthood. Liver transplantation is the only effective treatment for symptomatic type I CEPS. A therapeutic approach for type II could be surgical closure of the shunt. Objective: To present the imagenological diagnosis an adult patient with Abernethy Syndrome type 1B.