The clinical and imaging spectrum of findings in patients with congenital lobar emphysema.

Congenital lobar emphysema (CLE) is an important cause of infantile respiratory distress that may require surgical intervention. We retrospectively reviewed the clinical and imaging findings in eight infants with CLE. In our series, CLE was more common in females, predominantly involved the right lung, often presented with an opaque lobe from retained fetal lung fluid, and frequently involved the lower lobes, multiple segments or lobes, and both lungs. Most patients with CLE were diagnosed and managed on the basis of clinical and plain radiographic findings alone. Computed tomography, and occasionally ventilation/perfusion scintigraphy, were helpful in confirming the diagnosis and in guiding management decisions in several cases; bronchoscopy showed that stenosis of the right mainstem bronchus was the cause of CLE in one case. Three patients experienced progressive worsening of respiratory distress and required surgical resection of the affected lobe for cure; the remaining five patients were managed medically with eventual remission of symptoms.