(A936) Multidisciplinary Management of Congenital Subglottic Stenosis in the Setting of Cystic Fibrosis

Introduction: Subglottic stenosis (SGS) is a common laryngeal anomaly in infants and can be congenital, acquired, or acquired upon congenital. To date there have been no case reports of congenital SGS in the setting of cystic fibrosis (CF). This case report highlights the unified airway theory of CF and the need for multidisciplinary management of a congenital airway anomaly in this context.

Methods: A 4-month-old male with homozygous delF508 CF required multiple hospitalizations for CF exacerbations. He was later found to have congenital SGS complicating his clinical picture. His initial hospital course included multiple readmissions to the PICU, respiratory support including intubation due to stridor, and the inability to maintain adequate oxygenation. After minimal improvement, it was decided to evaluate his airway due to concern for acquired SGS.

Results: On microlaryngoscopy and bronchoscopy (MLB) he was found to have a congenital elliptical cricoid with an acquired membranous grade 3 SGS, thought to be due to inflammation from his CF illnesses and intubation. Initially the response to balloon dilation was good, however, the stenosis progressed, and he continued to have refractory symptoms resulting in frequent admissions. We elected to perform a single stage laryngotracheoplasty with thyroid ala graft. His postop course was complicated by increased peak inspiratory pressures on mechanical ventilation requiring frequent treatments, and tracheitis with H. influenzae and N. meningitidis treated with IV cefepime and ampicillin-sulbactam. His first postop MLB was performed on day 3 to upsize the tube to a 3.0 ETT from a 2.5 to allow for better ventilation. He was successfully extubated on postop day 4 but remained hospitalized with O2 requirement until postop day 10. He was discharged 12 days after surgery on room air.

Conclusions: This case emphasizes the unified airway theory and need for a broad differential of airway diagnoses in patients with CF who require repeated hospital admissions, unexplained O2 requirement, and intubation. A multidisciplinary approach is critical in the management of these complex airway patients.