0614 Comparison and Outcomes of Adenotonsillectomy for Obstructive Sleep Apnea in Children with Achondroplasia

Abstract Introduction Achondroplasia, a genetic disorder arising from a FGFR3 mutation, is typically detected perinatally, and affects 1 in 40,000 children. The diagnosis of achondroplasia has many associated medical complications, one of which is obstructive sleep apnea (OSA). The objective was to compare demographics between children with achondroplasia and OSA with the general pediatric population with OSA, as well as present treatment outcomes for children with achondroplasia. Methods Retrospective chart review of 22 children with achondroplasia and OSA. A sample of 141 children with OSA without achondroplasia was used as a control. Parameters from polysomnography (PSG) were recorded and analyzed. Values before and after adenotonsillectomy (T 0.001.) Obstructive Apnea-Hypopnea index, a marker of OSA severity, trended toward being higher in the achondroplasia group (p-value = 0.06). The lowest SpO2 saturation was not significantly different in the achondroplasia group. When comparing baseline data in the achondroplasia population with respective T 0.05) and SpO2 (p-value 0.05) were significantly improved. Conclusion Patients with achondroplasia and OSA have an earlier age of onset compared to children without achondroplasia. For these patients, treatment with adenotonsillectomy shows a significant improvement in OSA severity. Future research needs would be to increase sample sizes, establish a standard treatment for OSA in children with achondroplasia, and also establish improvement and treatment frequency by OSA severity. There is also a need to compare the improvement of OSA in children undergoing other interventions and compare these to the general pediatric population. Support (if any)