0804 Sleep-disordered Breathing in Myelomeningocele and Chiari II Malformation: Our Experience at a Tertiary Care Center

Abstract

Abstract Introduction Children with myelomeningocele (MMC) and Chiari Malformation type II (CMII) have some degree of sleep-disordered breathing (SDB), which may include central apnea, obstructive apnea, and hypoventilation. Untreated SDB may lead to growth failure, neurocognitive and behavioral abnormalities, cor pulmonale, and death. There is no standardized diagnostic and management protocol to be followed if SDB is suspected in a patient with CMII and MMC. Methods Variability in physician practice patterns was assessed for patients with CMII and MMC suspected of having SDB at a tertiary care center from January 2008 to December 2018. Results 180 patients with CM2 and MMC were identified. Pulmonology/Sleep providers were consulted on 63 of them. A sleep study was ordered for 73% of these patients, with snoring being the most common indication. Most patients had obstructive sleep apnea, with mean obstructive AHI of 6 events/h. Recommendations after sleep study included supplemental oxygen (20%), non-invasive positive airway pressure initiation (9%), subspecialty referral in 35% (otolaryngology and neurosurgery), and tracheostomy with mechanical ventilation in 10% of the patients. Conclusion In a large tertiary care center, there was significant variability in clinical practice patterns for children with CM2 and MMC who have SDB. Clinicians caring for these patients should consider PSG to enhance early diagnosis and management of SDB. A standardized diagnostic and management protocol could complement physician judgment to care for this complex patient population. Support (if any)