Congenital Hepatic Arteriovenous Malformation With Infrahepatic Inferior Vena Cava as a Draining Vein: An Unusual Association

Shruti Thakur, Praveen Verma, Mukesh Surya, Anupam Jhobta

Abstract

Arteriovenous malformations (AVMs) are vascular anomalies consisting of dysplastic vessels lined by resting endothelium with direct arterial connection to the venous drainage system, bypassing the intervening perfusing capillary bed. Congenital hepatic AVM is a rare entity and is associated with a high mortality rate. Here, we report a case of congenital hepatic AVM showing a vascular nidus predominantly in segment V of the liver, with branches from the hepatic artery and right innominate artery as arterial feeders and having an anomalous draining vein communicating with the infrahepatic inferior vena cava (IVC). These malformations show typical ultrasonographic and computed tomography angiographic findings. The management is not standard and has to be individualized and depends upon the size, extent, and location of hepatic AVMs and the associated complications.

Congenital hepatic AVMs, although rare, can be diagnosed prenatally with a high index of suspicion. Prenatal diagnosis and determination of prognostic parameters are crucial for better parenteral counseling and treatment decisions. To the best of our knowledge, no case of congenital hepatic AVM between the hepatic artery, innominate artery, and infrahepatic IVC has been described in literature till date as was seen in this novel unusual case.