POLIESPLENIA E ANOMALIA DO SISTEMA PORTAL HEPÁTICO ASSOCIADAS À AUSÊNCIA DE VEIA CAVA INFERIOR EM CRIANÇA: RELATO DE CASO

Agenesis of inferior vena cava (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, besidescomplications such as deep vein thrombosis (DVT). The present study 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, withcomputed tomographyand abdominal ultrasound revealing anomaly of the development of the hepatic portal system associated with the absence of inferior vena cava with continuation inretrocrural azygos vein, in addition to multiple accessory spleens. When the anastomoses of the IVCprimitive 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 additionto monitoring them for diagnosis of other possible malformations.