Introduction
Congenital Lobar Emphysema (CLE) is a rare and life-threatening anomaly
of the lung characterized by hyperinflation of usually one or sometimes
more of the lung lobes. It sincidence rate is around 1 in the 20,000 to
30,000 live births with 3:1 male preponderance1,2. The
hyperinflated lobe compresses the surrounding normal lung tissue causing
atelectasis leading to ventilation-perfusion mismatch and hypoxia. It is
usually manifested in early infancy with respiratory
distress3,4. The most common site of lobe involvement
is the left upper lobe (43%), followed by the right middle lobe(32%)
and right upper lobe (21%).Congenital Heart Defects (CHD) can present
in around 14 – 20% of cases of CLE, and Ventricular Septal Defect
(VSD) is the most commonly associated anomaly1,4. The
clinical presentation of CLE and most CHDs are similar and overlapping
in the infantile age, and CHD being far more common gets detected
early.Often the diagnosis of CLE may get overlooked in presence of the
CHD5,6. We encountered a case where the presence of
CLE was established after the corrective repair of the large
perimembranous VSD with moderate Patent Ductus Arteriosus (PDA).