Agenesis of Retrohepatic Inferior Vena Cava with Congenital Cavo-Portal Shunt

Background Congenital anomalies of the inferior vena cava are extremely rare with agenesis of retrohepatic segment of the inferior vena cava (RHIVC) with azygos continuation being a relatively wellknown congenital anomaly. We present the rare case of absence of the RHIVC, with venous return entirely through the liver parenchyma by way of a cavo-portal shunt. This to our knowledge is only the second such case reported in English medical literature. Case report A eleven year old girl with normal mile stones, presented with abdominal pain of four days duration. She gave no previous history of medical problems. She was evaluated and her abdominal examination and blood investigations were unremarkable. An Ultrasonogram revealed a cavo-portal shunt, with absent retrohepatic segment of IVC. CT angiogram was obtained which confirmed the findings. As she was asymptomatic, she was managed conservatively and continues to be asymptomatic on routine followup.Conclusion We highlight an extremely rare case of an absent segment of IVC with congenital cavo-portal shunt, wherein the patient was well compensated and was managed by masterly inactivity