Heterotaxy syndrome and interrupted inferior vena cava (IVC) with azygos continuation

Heterotaxy syndrome or situs ambiguous is a rare congenital disease in which the pattern of anatomical organization of the thoraco-abdominal visceral and vascular structures is not arranged in normal position. Patients with heterotaxy syndrome represent a wide range of anatomical variations including thoraco-abdominal structures. Here we report a rare case of asymptomatic heterotaxy syndrome in an elderly female with multiple accessory spleens, stomach on right side of the abdomen, midline liver, azygos continuation of Inferior Vena Cava (IVC) and intestinal malrotation.