Ausencia Congénita de la Vena Porta Hepática Asociada a Malrotación Intestinal y a Vena Cava Inferior Izquierda. Reporte de Caso

Inzunza, Oscar

Keywords: inferior caval vein, intestinal malrotation


SUMMARY: Congenital absence of the hepatic portal vein is an extremely rare malformation that was first described by John Abernethy in 1793. Only 101 cases had been described in the literature until 2015 and most affected females. In this anomaly, also known as congenital extrahepatic porto-systemic shunt (CEPS), intestinal venous drainage is derived towards the territory of the systemic veins and could be associated with other birth defects, including heart and biliary tract anomalies, metabolic diseases, mental retardation. We present the case of a 2-year-old boy who died because of multifocal pneumonia and necropsy showed anatomical findings consistent with this malformation as an incidental finding. The portal vein followed an anomalous course and joined the right renal vein, forming the "mesenteric-splenic-renal" venous collector, which then received the right gonadal vein and ended at the confluence of the common iliac veins. In our case this anomaly was associated to intestinal malrotation, aberrant hepatic artery and persistent left inferior vena cava, situation described once in the literature. The finding of these anatomical abnormalities in cadavers has a great teaching value for our undergraduate and graduate students who are learning anatomy and they can also assess the associated clinical.

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Título de la Revista: International Journal of Morphology
Volumen: 34
Número: 2
Editorial: In Press.
Fecha de publicación: 2016
Página de inicio: 665
Página final: 670
Idioma: Español