Bilateral Thrombophlebitis Revealing an Agenesis of the Inferior Vena Cava as Part of Polysplenum Syndrome

Abstract

Polysplenum syndrome is a rare malformation characterized by the association of multiple spleen with other abnormalities dominated by cardiovascular and visceral malformations. We report the observation of a young patient admitted to the emergency room for a clinical presentation of acute appendicitis. The abdominal ultrasound scan had invalidated the diagnosis and pushed to perform an abdominopelvian computed tomography detecting a bilateral iliac acute thrombophlebitis with agenesis of the inferior vena cava supplemented by a large azygos vein, short pancreas and multiple splenic lobules.