Polysplenia and anomaly of the hepatic portal system associated with the absence of inferior vena cava in a child: a case report

Abstract

Agenesis of IVC is a rare cardiovascular malformation that occurs between the sixth and tenth week of embryogenesis. It may be associated with cardiac and abdominal malformations, in addition to complications such as DVT. The present work reports the case of a patient randomly diagnosed with total IVC agenesis associated with malformation of the portohepatic system and polysplenia. Female patient, 9 years old, being monitored for hemorrhagic dengue, with CT and abdominal US revealing anomaly of the development of the hepatic portal system associated with the absence of inferior vena cava with continuation of the retrocrural azygos vein, in addition to multiple accessory spleens. When the anastomoses of the VCI primitive veins are not formed properly, partial or total agenesis of this vessel may occur. Consequently, the blood is diverted to the retrocrural azygos. Identification of these malformations is important for the patients receive proper guidance about DVT prevention, in addition to monitoring them for diagnosis of other possible malformations.

Keywords: inferior vena cava, cardiovascular abnormalities, vascular malformations, deep vein thrombosis, polysplenia.