Adenotonsillectomy in children with sickle cell disease and obstructive sleep apnea

Introduction: Obstructive sleep apnea (OSA) is prevalent and may be more severe in children with Sickle Cell Disease (SCD) compared to the general pediatric population. Objectives: The objective of this study was to describe the therapeutic effects and complications of tonsillectomy and adenoidectomy (T&A) for treatment of OSA in children with SCD. Methods: A comprehensive database of pediatric SCD patients was reviewed to identify all patients who underwent T&A between 2010 and 2016. An IRB-approved, retrospective review of laboratory values, perioperative course, pre- and post-T&A hospital utilization, and polysomnography was conducted. Results: There were 132 SCD children (108 HbSS) who underwent T&A. Mean age was 7.6 +/- 4.6 years. The mean baseline hemoglobin of these patients was 9.3 +/- 1.4 g/dL; 72.7% of patients had pre-operative transfusion, such that the mean Hb at time of T&A was 11.4 +/- 1.0 g/dL. The average admission length surrounding T&A was 3.5 +/- 1.2 days. Complications were documented in 11.4% of operative cases. Polysomnography was available in 104 pre-T&A and 45 post-T&A. The Apnea-Hypopnea Index decreased on post-T&A polysomnogram (7.6 +/- 8.7 vs. 1.3 +/- 1.9, p = 0.0001). The O-2 nadir improved on post-T&A polysomnogram (81.2 +/- 10.8 vs. 89.3 +/- 7, p = 0.0003). Emergency room visits (mean events per year) decreased post-operatively (2.6 +/- 2.8 vs. 1.8 +/- 2.2, p = 0.0002). Conclusions: T&A can be a safe and effective option to treat OSA in pediatric patients with SCD and was significantly associated with reduced AHI and fewer ER visits post-operatively.