1023 Resolution of Idiopathic Central Sleep Apnea after Prolonged Treatment with Acetazolamide: A Case Report

Abstract

Introduction: Idiopathic central sleep apnea (ICSA) is caused by instability of the control of breathing particularly in the transition from wakefulness to sleep. Literature regarding management of ICSA in children is limited. Current reported management options include watchful waiting, supplemental oxygen, NIPPV or tracheostomy depending on severity of illness and there is limited information regarding the efficacy of acetazolamide treatment for ICSA in children. Report of Case: A 3-year-old girl with history Autism, and global developmental delay presented for evaluation of insomnia and restless sleep. She underwent diagnostic polysomnography for evaluation of sleep disordered breathing. Her initial polysomnography study (PSG) revealed a central apnea index (CAI) of 8.5/hour, obstructive AHI(AHIo) of 0.3/hour, without hypoventilation. Workup revealed a normal MRI, long term EEG monitoring was done, and blood work showed no evidence of daytime hypoventilation nor hypercarbia. Acetazolamide therapy (3 mg/kg/day PO initially) was started for treatment of her central sleep apnea. The patient had initial weekly monitoring of bicarbonate levels to reach target medication levels. She then had subsequent laboratory monitoring with dose titrations during the treatment phase (6 m/k/d, 8m/k/d, 10 m/k/d, 12 m/k/d). A second PSG at 6 months after starting acetazolamide therapy, revealed CAI of 5.2/hour), and AHIo 2.0/hour. Medical management for mild OSA was initiated. During follow-up visits, she reported significant improvement in insomnia, restless sleep and daytime mood symptoms. She was continued on the acetazolamide for 16 months from time of initial diagnosis of ICSA. She was seen for follow up and weaned off the acetazolamide. 2 months after discontinuing acetazolamide, a third PSG revealed sustained resolution of central sleep apnea (CAI=2.4/ hour). Conclusion: Low dose acetazolamide may be effective in sustained resolution of ICSA in children.