Abstract Introduction Severe pediatric bronchial stenosis is rare and may result from congenital anomalies, foreign bodies, trauma, infection, or systemic causes. Chronic complete mainstem obstruction often requires surgical resection with end-to-end anastomosis or pneumonectomy, which carries significant long-term morbidity. We present a 10-year-old girl with diffuse right-sided bronchial fibrosis and complete lung collapse, successfully managed with staged endoscopic airway recanalization. Case 10-year-old female with type 1 diabetes and positive celiac serologies developed right upper lobe pneumonia during admission for diabetic ketoacidosis at an outside hospital. Her exertional dyspnea, dry cough, and fatigue persisted, and a chest radiograph at 6 months showed complete right lung opacification. CT demonstrated an abrupt cutoff of the right mainstem bronchus with complete collapse and mediastinal shift. She was then transferred to our center, where four staged bronchoscopies were performed. The first revealed complete right mainstem occlusion and tracheomalacia. Endobronchial cryobiopsy showed squamous metaplasia with chronic inflammation, granulation tissue, and scarring, negative for tumor and amyloid. Infectious studies, including HPV, EBV, HIV, Tuberculosis, Coccidioides, BAL cultures, PCR panels, and Karius, were negative. Despite known autoimmune conditions, rheumatology found no evidence of GPA, sarcoidosis, IgG4-related disease, relapsing polychondritis, or lupus on extensive evaluation. In the absence of a systemic disease to target, cryodebulking, cryodevitalization, forceps extraction, and intralesional Kenalog were performed to restore airway patency. However, multilayered fibrotic membranes were present beyond the initial obstruction, extending distally into the bronchus intermedius and right lower bronchus, where balloon dilation was required. The third and fourth bronchoscopies mapped distal lobar branches, and re-expansion of the right lung was achieved. No foreign body was identified. Upon discharge, she was asymptomatic on room air with obstruction and normal lung volumes on spirometry and a normal 6-minute walk. Vest, inhaled fluticasone, and nocturnal CPAP were prescribed to enhance airway clearance, decrease inflammation, and provide noninvasive stenting, respectively, during the acute post-procedural phase. Working diagnosis is post-infectious, immune-mediated, or idiopathic, with expectation for continued surveillance and repeat bronchoscopies. Discussion This case highlights the diagnostic and therapeutic challenges of pediatric bronchial fibrosis. Staged bronchoscopic cryorecanalization allowed organ preservation, restoration of lobar anatomy, and functional recovery. Ongoing surveillance will guide the need for adjuvant strategies. High success rates are reported in adults, though pediatric experience remains limited. Conclusion Staged endoscopic airway recanalization offers a safe, effective, organ-sparing alternative to pneumonectomy in children with diffuse bronchial fibrosis. Early recognition, thorough evaluation, monitoring, and multidisciplinary care are essential. This abstract is funded by: none
Rico et al. (Fri,) studied this question.