Obstructive sleep apnea syndrome in sickle cell disease.

We present a series of 21 children with obstructive sleep apnea syndrome and sickle cell disease (SCD). Thirteen patients underwent adenotonsillectomy with resolution of symptoms and improvement in alveolar hypoventilation. Adenotonsillectomy involves significantly increased risks in patients with SCD. We present our protocol for management of these difficult surgical patients, including vigorous hydration and transfusion therapy. Polysomnography has proven valuable both in diagnosis and in identifying severely affected patients. Increased end tidal alveolar carbon dioxide during sleep has proven to be a significant predictor of disease, while oxygen saturation monitoring was shown to be unreliable. The incidence of adenotonsillar hypertrophy (ATH) in SCD appears increased and not related to infectious diseases. We suggest that ATH represents a part of the natural course of compensatory lymphoid tissue enlargement in children with SCD.