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Case Reports
. 2023 Apr 1;121(2):e202202570.
doi: 10.5546/aap.2022-02570.eng. Epub 2022 Nov 17.

Portosystemic shunt, a rare cause of neonatal cholestatic hepatitis. A case report

[Article in English, Spanish]
Sofía C Geffner  1 , Macarena L Atun  1 , Sebastián García López  1 , María P Carrascal Gutiérrez  1
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Free article
Case Reports

Portosystemic shunt, a rare cause of neonatal cholestatic hepatitis. A case report

[Article in English, Spanish]
Sofía C Geffner et al. Arch Argent Pediatr. .
Free article

Abstract
in English, Spanish

Congenital portosystemic shunt is a venous vascular abnormality that connects portal and systemic circulation, resulting in diversion of the blood flow, bypassing the hepatic passage. It is a rare malformation; its incidence varies from 1:30 000 to 1:50 000 newborns. It may be asymptomatic or present with complications in the pediatric age or, less frequently, in the neonatal age. Upon diagnosis, the need for a surgical or an intravascular intervention for closure should be defined. This decision depends on the malformation anatomical characteristics, clinical manifestations, and complications. We present the case of a 1-month-old patient referred to our center for the study of neonatal cholestatic hepatitis, with a diagnosis of extrahepatic portosystemic shunt. Intravascular closure of the defect was performed with significant subsequent improvement.

El shunt portosistémico congénito es una anomalía vascular venosa que comunica circulación portal y sistémica, por la que se deriva el flujo sanguíneo, salteando el paso hepático. Es una entidad poco frecuente, cuya incidencia varía entre 1/30 000 y 1/50 000 recién nacidos. Puede cursar de forma asintomática o presentarse con complicaciones en la edad pediátrica o, menos frecuente, en la edad neonatal. Ante el diagnóstico, se deberá definir la necesidad de intervención quirúrgica o intravascular para el cierre. Esta decisión depende de las características anatómicas de la malformación, de las manifestaciones clínicas y complicaciones presentes. Se presenta el caso de un paciente de un mes de vida derivado a nuestro centro para estudio de hepatitis colestásica neonatal, con diagnóstico de shunt portosistémico extrahepático. Se realizó cierre intravascular de la lesión con mejoría significativa posterior.

Keywords: cholestasis; liver; neonatal hyperbilirubinemia; portosystemic shunt; vascular malformations.

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