Abstract
Introduction: Congenital lobar emphysema or congenital large hyperlucent lobe (CLHL) is a rare lung malformation. Afflicted children typically develop progressive tachydyspnoea in the first 3 months of life and radiologically have hyperlucency of one or more lung segments. The current treatment recommendation in symptomatic infants is surgical resection of the affected lung lobe.
Objective: To present preliminary results of a novel interventional technique of endobronchial lobar or segmental occlusion as a less invasive treatment option in symptomatic infants with CLHL.
Material and Methods: Retrospective analysis of clinical parameters in 3 infants with symptomatic CLHL after bronchoscopically and fluoroscopically controlled placement of self-expanding occluders.
Results: Symptoms started on average at 4 weeks (3-5) of life. Two patients had left upper lobe (UL) bronchus malacia and one patient malacia of segment 1 in the right UL bronchus. Self-expanding occluders were deployed in the affected segment bronchus. One patient developed a pneumothorax during the procedure. All profited within 24 hours with a decrease in tachypnoea and radiological appearance. In 2 patients hyperlucency was incompletely resolved: inserting a second device full occlusion was achieved in 1 patient at 9 months. Because of the third infant's size, a second occluder could not yet be inserted. No significant complications were noted to date.
Conclusion: Endobronchial occlusion of a malacious lobar or segment bronchus in symptomatic infants with CLHL represents a minimally invasive alternative to surgical lobe resection; however it requires ongoing technical refinement with longer term follow-up to fully assess the clinical benefit.
- Copyright ©the authors 2016
