1149 Unravelling the Mysteries of Pediatric Central Sleep Apnea (CSA): A Unique Case Report

Abstract Introduction CSA in pediatric patients is characterized by pauses in breathing during sleep that result from a lack of drive to breathe. It is estimated to be about 10% of all pediatric sleep-disordered breathing cases. The most common type is primary, also known as idiopathic CSA, unrelated to another medical cause. Other causes include, CSA of prematurity, CSA in certain medical conditions such as congenital heart defects or neurological disorders, and CSA related to medications. We report a case of CSA in the setting of an acute neurological impairment secondary to atypical hemolytic uremic syndrome (HUS) in a pediatric patient. Report of case(s) A 3-year-old female with chromosome 3p deletion syndrome presents after a recent hospitalization with acute hypoxic respiratory failure status post tracheostomy, for a baseline PSG with capped tracheostomy to evaluate for decannulation. During this hospitalization, the patient developed acute neurological impairment with brain swelling and abnormal signaling in bilateral basal ganglia and thalami, findings consistent with HUS. A baseline PSG (table 1) with capped tracheostomy showed an AHI of 16 events per hour of sleep. She was uncapped per protocol but continued to have worsening AHI of 128 events per hour, predominantly central apneas. An interval brain imaging showed evolving parenchymal injury with encephalomalacia and some resolution of prior swelling, indicating the cause of her CSA to be neurological. She was placed on BIPAP ST with set rate 12, IPAP 10 cm H2O and EPAP 5 cm H2O during sleep. Tracheostomy remained capped during the day but decannulation was deferred due to intolerance of BIPAP via nasal mask on further attempts. Conclusion The prognosis for pediatric patients with CSA depends on the underlying cause and how well the patient responds to treatment. Mild CSA may require just observation or medications such as acetazolamide or specific respiratory stimulants to stimulate breathing during sleep. In severe cases, continuous positive airway pressure (CPAP) or bilevel positive airway pressure (BIPAP) therapy may be used to provide mechanical support for breathing during sleep. A multidisciplinary approach involving Pulmonary and Sleep specialists would thus be ideal to manage the complex nature of pediatric CSA. Support (if any) None