Abstract Introduction Breath-holding spells are benign paroxysmal events occurring in early childhood, most often triggered by emotional upset or painful stimuli. Continuous positive airway pressure (CPAP) therapy is commonly used in pediatric obstructive sleep apnea (OSA), though initiation may be stressful. To our knowledge, there are no prior reports of breath-holding spells associated with CPAP. Here, we describe what appears to be the first reported case of a cyanotic breath-holding spell occurring immediately after CPAP initiation in a 2-year-old child. Report of case(s) A 2-year-old boy with Cornelia de Lange syndrome and severe OSA (Obstructive apnea- hypopnea index of 65 events/hour) had sustained apnea and cyanosis immediately after CPAP initiation in the sleep lab. A rapid response was called and cardiopulmonary resuscitation was started, but patient quickly spontaneously recovered. The patient was subsequently admitted to the hospital for monitoring. CPAP was reattempted twice in the hospital and patient again had apnea with cyanosis and bradycardia. On the first attempt, recorded oxygen saturation decreased from 100% to 18% on CPAP and heart rate decreased from 143 to 70 beats per minute. On a repeat CPAP attempt after one hour, the patient again had apnea and oxygen saturation decreased to 66% and heart rate decreased from 180 to 51. The CPAP mask was removed and the patient spontaneously recovered both times. Given this CPAP intolerance, the patient was discharged home with oxygen at night for treatment of OSA. Overnight oximetry on 0.2 liter per minute of oxygen via nasal cannula showed an oxygen desaturation index of 11 events /hour and 6.1% of the night with an oxygen saturation below 90%. Conclusion Children with Cornelia de Lange syndrome and severe OSA are at a risk for breath – holding spells. CPAP initiation may trigger breath holding spells and sleep medicine clinicians should be aware of this to avoid unnecessary interventions. There is some evidence that behavioral interventions can lesson breath – holding spells in children with Cornelia de Lange syndrome, but treatments other than CPAP may be necessary in some cases. Support (if any)
Alabi et al. (Fri,) studied this question.