Ivemark syndrome in association with congenital septum transversum defect and pancreatic divisum.

ABSTRACT

A four-month-old female baby presented with cyanosis and respiratory distress. A provisional diagnosis of congenital posterolateral diaphragmatic hernia was made but on exploration there was a defect in the septum transversum along with features of Ivemark syndrome - asplenia with visceroatrial heterotaxia, malrotation and pancreatic divisum - an association not yet reported in literature. The child did well after operative correction of the hernia. Echocardiography showed situs inversus with dextrocardia with double outlet right ventricle, atrial septal defect, ventricular septal defect, patent ductus arteriosus and pulmonary stenosis.