Management of obstructive sleep apnea in children with achondroplasia: Outcomes of surgical interventions.

ABSTRACT

INTRODUCTION: Children with achondroplasia are predisposed to obstructive sleep apnea (OSA), however little is known regarding surgical interventions and outcomes for this condition. The aim of this study was to evaluate the severity of OSA in children with achondroplasia and report outcomes of surgical interventions using polysomnography (PSG) parameters. METHODS: Retrospective chart review of children with achondroplasia with documented OSA from 2002 to 2018 that had pre- and post-operative PSG results. Additional data extracted included age, gender, and type of surgical interventions. The primary outcome was change in postoperative obstructive apnea hypopnea index (OAHI). RESULTS: Twenty-two children with achondroplasia were identified that underwent formal PSG before and after confirmed OSA. The median age was 12 months (range 4 days-15.3 years, IQR 2 years) at time of initial PSG evaluation. The majority (72.7%) of patients had severe OSA with a median preoperative OAHI of 14.25 (IQR 9.4). The most common surgical intervention was adenotonsillectomy (n = 15). Multilevel surgical intervention was required in 9 (41.0%) patients. Post-operatively, 16 (72.7%) children experienced a reduction in OAHI, of which four (18.2%) had complete OSA resolution. OAHI scores increased in six (27.3%) children. Patients with the most severe OSA at baseline had greater improvements in post-operative OAHI (P < 0.01). Neither type nor number of surgical interventions was associated with improved outcomes (P = 0.51, P = 0.89 respectively). CONCLUSIONS: Treatment of OSA in children with achondroplasia remains challenging. Although reduction of OAHI is possible, caregivers should be counseled about the likelihood of persistent OSA and the potential for multilevel airway surgery.