Bilateral congenital lobar emphysema: staged management

Background Only a few isolated cases in the literature exist to guide management of bilateral congenital lobar emphysema (CLE). Here, we review our experience in infants with bilateral CLE. Methods A case series of all infants presenting with bilateral CLE from 2014 to 2015 in a single institution. Results Four patients underwent intervention, with all having right middle lobe (RML) and left upper lobe (LUL) affected. Preoperative planning with computed tomography angiography (CTA) chest allowed a tailored approach based on specific radiologic features. All patients also underwent bronchoscopy to evaluate the anatomy and to assess for alternative causes of airway compression. Three patients underwent unilateral lobectomies, two RML and one LUL. All are growing normally and on room air more than one year later. The last patient underwent a staged procedure beginning with left upper lobectomy followed by right middle lobectomy two weeks later after exhibiting rebound hyperexpansion of the remaining diseased lobe. Thoracoscopy was precluded by mass effect in all patients. No patients underwent emergent lobectomies. One patient had pulmonary interstitial glycogenosis (PIG) in the setting of CLE, first reported case of bilateral CLE with PIG. Conclusions This study supports a staged, image-guided, physiology-based operative approach to bilateral CLE. Excision of both diseased lobes does not appear to be mandatory, at least in the short-term follow up, and comports with a "the least intervention that is the most effective" philosophy. CTA is critical for planning, but the role of V/Q scan is not defined. Thoracoscopy appears to have no role. Level of evidence Treatment Study, Level IV.