Multiple Detector Computed Tomography Evaluation of Congenital Pulmonary Venolobar Syndrome: Scimitar Syndrome with Pulmonary Arterio-Venous Fistula.

ABSTRACT

The term dysmorphic lung has been introduced to describe any complex congenital malformation that involves both abnormal pulmonary vascular morphology and disordered growth of the entire lung. The major group within this definition of the dysmorphic lung is the scimitar syndrome. Scimitar syndrome is a rare anomaly consisting of partial anomalous pulmonary venous return into the supra or infradiaphragmatic portion of the inferior vena cava, right lung hypoplasia, dextroposition of the heart, and anomalous systemic arterial supply from the aorta or one of its branches to the right lung. It can be associated with congenital cardiovascular defects namely dextrocardia, coarctation of the aorta, Tetralogy of Fallot, patent ductus arteriosus, atrial or ventricular septal defect, and right pulmonary artery hypoplasia. Associated pulmonary anomalies are lung hypoplasia, sequestration, and tracheobronchial anomalies. Thus, it can manifest with heart failure and recurrent pneumonia. Other reported associated anomalies include bronchogenic cysts, horseshoe lung, accessory diaphragm, and hernias. We report an 8-year-old boy with complaint of chest pain, who was diagnosed as having scimitar syndrome with rare association of pulmonary arteriovenous fistula within the involved lung and role of newer faster multiple detector computed tomography scanner in its evaluation.