Heterotaxy Asplenia Syndrome.

Introduction: Heterotaxy asplenia syndrome (right isomerism or bilateral right-sidedness), which is associated with a centrally located liver, absent spleen, and two morphologic right lungs usually associated with dextrocardia and cyanotic congenital heart disease. Case Presentation: We report the case of dextrocardia with heterotaxy syndrome who presented with respiratory distress soon after birth. A chest X-ray showed dextrocardia, cardiomegaly with pulmonary congestion. The findings of an electrocardiogram and echocardiography confirmed dextrocardia and an abdominal Ultrasound showed liver central more on right side with mild hepatomagly, no spleen visualized, stomach on left side, pancreas both kidneys urinary bladder normal with free minimal fluid. Conclusions: Heterotaxy asplenia syndrome a rare group of congenital malformations that must be fully evaluated. There is need for a complete and elaborate diagnostic work up of suspected cases by using various imaging modalities so that they are not missed. Doctors should encourage routine medical examination for their patients which could help identify this anomaly, thereby preventing wrong diagnosis and possibly death due to delay in management.