Scimitar syndrome is a rare but well defined disorder, involving anomalies of lung and pulmonary vein1. Children may present with tachypnea early in life or with repeated chest infection at any age. It is important to diagnose or at least to rule the syndrome as management depends upon the severity of different components. Treatment may be medical, surgical or combined in majority of cases. A c...

Fig. 2. (A) Three dimensional reconstructed computed tomography image showing the venous drainage of the right lower lobe directly in the inferior vena cava (white arrow). (B) Intra-operative view with aberrant artery of the intrapulmonary sequestration (Ar) and venous drainage of the right lower lobe (RLV) directly in the inferior vena cava (IVC) just above the diaphragm. The patient underwent...

Four infants with the scimitar syndrome presented with failure to thrive, cardiac failure, and pulmonary hypertension. In all cases systemic arteries arose from the upper abdominal aorta to perfuse the lower part of the right lung, and a large unobstructed anomalous pulmonary vein drained venous blood from almost the entire right lung to the inferior vena cava. An additional small pulmonary vei...