Congenital lobar emphysema: Pitfalls in diagnosis

Abstract

Congenital lobar emphysema (CLE) is a rare but life-threatening congenital anomaly leading to respiratory distress in early childhood. Diagnosis requires a strong clinical suspicion. We report a case of a 31/2-month-old infant who was initially diagnosed with pneumonia requiring multiple hospital admissions. After computed tomography of the thorax, a diagnosis on CLE was made. The child was planned for surgery in the next available routine operation theatre. However, suddenly in the evening, she developed respiratory distress and needed emergency surgical intervention. The child improved dramatically after surgery, and the postoperative period was uneventful. Early diagnosis and treatment in such cases can lead to dramatic results.

An Unusual Presentation of Congenital Lobar Emphysema

“…As noted above, the pathophysiological development of congenital lobar emphysema has not yet ben elucidated, but abnormalities including bronchial cartilage, intrinsic and extrinsic bronchial obstructions, vascular anomalies, alveolar disease, and cytomegalovirus infection have been considered possible factors that can lead to a ball-valve obstruction where a bigger air volume enters the lobe during inhalation than leaves during expiration with following air trapping [ 1 , 8 , 9 ].…”

<p>Congenital lobar emphysema: diagnosis and treatment options</p>

“…Diseases stemming from organs adjacent to the lungs and bronchus may cause CLE. Vascular abnormalities such as pulmonary arterial sling anomalies and abnormal pulmonary venous return anomalies may cause CLE 5,35–37. Bronchogenic cysts and mediastinal tumors are rare extrinsic causes of CLE 12,19,38,39…”

An unusual case of unilateral hyperlucent lung

“…The aetiology of this abnormality is still unknown, although a variety of causes have been proposed which cause obstruction of the developing airway, leading to ball valve obstruction and air trapping causing emphysema, such as cartilaginous dysplasia of bronchi, vascular anomalies, kinking of the bronchioles, compression by large lymph nodes, mucus plugs, alveolar disease and bronchial stenosis [ 11 , 12 ]. The most accepted among the theories are bronchial cartilage defects like hypoplasia, flaccid tissue, or absence of cartilage.…”