Radiology Quiz: Congenital Lobar Emphysema

Abstract

Congenital lobar emphysema (CLE) is due to alveolar over-distension of a lung lobe frequently associated with intrinsic or extrinsic obstruction of the corresponding lobar bronchus. Causes of extrinsic obstruction include compression by vascular abnormalities or lymph node enlargement. Intrinsic causes can be due to granulation tissue, meconium aspiration or deficient cartilage of the bronchus.1 Ninety percent of cases involved upper lobes and left side slightly more common than right side.1 Some cases may be diagnosed in-utero and others may present as respiratory distress after birth. Most symptomatic patients will present in first six months of life. Cases being misdiagnosed as pneumothorax or pneumonia are not uncommon.2 Severity of symptoms depends on the extent of air-trapping and compression on the other parts of the lung which determine the management options.2 Lobectomy is the definitive management for patients with moderate severe symptoms and flexible bronchoscopy has been successfully used for acute management of CLE.2,3 Some patients may be managed conservatively with good prognosis as in our patient who have mild interval symptoms and gradual improvement in serial CXRs