Ivemark syndrome with cor triatriatum, primum ASD, cleft mitrale and pulmonary stenosis

Ivemark syndrome is a rare anomaly with a reported incidence of 1 in 10000 – 20000 live births. It is characterized by agenesis of spleen, a dextropozed, hypoplastic or lobulated spleen or multiple spleens in association with complex cardiac anomalies. These malformations are usually associated with trilobulated or bilobulated lungs and abnormalities of other abdominal organs. Here we report a case of Ivemark syndrome, accompanied by polysplenia, accessory gall bladder, dilatation of the intrahepatic ducts, cor triatriatum, primum ASD, pulmonary stenosis, dysmorphic face and mild mental retardation.