Sleep surgery in syndromic and neurologically impaired children

Purpose: To examine surgery performed for obstructive sleep apnea (OSA) in children with syndromic or neurologic comorbidities. Material and methods: Medical records of 375 children with OSA were retrospectively reviewed, including 142 patients with trisomy 21, 105 with cerebral palsy, 53 with muscular dystrophy, 32 with spinal muscular atrophy, 18 with mucopolysaccharidoses, 14 with achondroplasia, and 11 with Prader-Willi. Outcome measures: Apnea-hypopnea index (AHI), complications, length of postoperative stay, and endoscopic findings. Results: 228 patients received 297 surgical interventions, with the remainder undergoing observation or positive pressure ventilation. Adenoidectomy was the most common procedure performed (92.1% of patients), followed by tonsillectomy (91.6%). Average AHI decreased following tonsillectomy, from 12.4 to 5.7 (p = 0.002). The most common DISE finding was the tongue base causing epiglottic retroflexion. Lingual tonsillectomy also resulted in an insignificant decrease in the AHI. Conclusions: Adenotonsillectomy, when there is hypertrophy, remains the mainstay of management of syndromic and neurologically-impaired children with OSA. However, additional interventions are often required, due to incomplete resolution of the OSA. DISE is valuable in identifying remaining sites of obstruction and guiding future management.