Anesthetic Management of Progressive Deformity of Tracheal Cartilaginous Sleeve in a Pediatric Patient With Beare-Stevenson Syndrome: A Case Report.

Beare-Stevenson syndrome is a rare fibroblast growth factor receptor 2-related disorder characterized by craniosynostosis, midface hypoplasia, cutis gyrata, and developmental delay, with upper airway obstruction being a critical concern in early infancy. Tracheal cartilaginous sleeve is a congenital anomaly associated with fibroblast growth factor receptor 2-related syndromes, which often necessitates tracheostomy because of the potential for significant airway complications. However, serial airway imaging in patients with Beare-Stevenson syndrome with tracheal cartilaginous sleeve has not yet been documented. Our pediatric patient with Beare-Stevenson syndrome underwent airway evaluations using a rigid bronchoscope under general anesthesia at 33 days, 62 days, and then at 3 years of age. The initial rigid bronchoscopy demonstrated tracheal cartilaginous sleeve, and tracheostomy was performed. At 3 years of age, rigid bronchoscopy revealed progressive tracheal cartilage deformity with inward protrusion. This pouch-like structural change greatly affected anesthesia management because it posed a risk for airway obstruction due to potential tube misplacement. The image resolution of a rigid bronchoscope is superior to that of a flexible bronchoscope, allowing for more precise assessment. Despite the absence of abnormalities on routine flexible bronchoscopy, rigid bronchoscopy provided critical insights into airway changes. Precise imaging allowed multidisciplinary planning, and it highlighted tube malposition as a cause of intraoperative respiratory failure. Tracheal cartilaginous sleeve is a life-threatening condition that may occur in children with Beare-Stevenson syndrome. This case demonstrated progressive tracheal deformity over time, increasing the risk of sudden airway obstruction and presenting significant anesthetic challenges. Our findings highlight the clinical importance of repeated rigid bronchoscopic evaluations, which provide essential information for safe airway management.