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Sleep-disordered breathing in children with myelomeningocele
K A Waters1, P Forbes, A Morielli
1Department of Pediatrics, Montreal Children's Hospital, Quebec, Canada.
Insights
Sleep-disordered breathing (SDB) is common in myelomeningocele patients, with central apneas more prevalent than obstructive ones. High-risk individuals require polysomnography and pulse oximetry for accurate diagnosis and classification of SDB.
Area of Science:
- Neurology
- Pulmonology
- Pediatrics
Background:
- Patients with myelomeningocele and Chiari II malformation often experience sleep apnea and respiratory control issues.
- The exact prevalence, types, severity, and associations of sleep-disordered breathing (SDB) in this population remain incompletely understood.
Purpose of the Study:
- To define the prevalence, types, severity, and associated factors of sleep-disordered breathing (SDB) in children with myelomeningocele.
- To correlate polysomnographic findings with clinical, imaging, and pulmonary function data.
Main Methods:
- Cross-sectional study of myelomeningocele clinic patients.
- Correlation of polysomnography results with historical data, Chiari malformation MRI, pulmonary function tests, and nocturnal pulse oximetry.
Main Results:
- Of 83 patients undergoing polysomnography, 37% had normal breathing, 42% mild SDB, and 20% moderate/severe SDB.
- Moderate/severe SDB was associated with thoracic/thoracolumbar myelomeningocele, prior posterior fossa decompression, severe brainstem malformations, and pulmonary function abnormalities.
- Nocturnal pulse oximetry demonstrated 100% sensitivity and 67% specificity for detecting moderate/severe SDB.
Conclusions:
- Sleep-disordered breathing in myelomeningocele is multifactorial, involving spinal lesion level, brainstem abnormalities, pulmonary function, and upper airway control.
- Polysomnography and nocturnal pulse oximetry are recommended for high-risk myelomeningocele patients to detect and classify SDB.
Background:
Although patients with myelomeningocele and the Chiari II malformation are known to have sleep apnea and respiratory control deficits, the prevalence, types, severities, and associations of sleep-disordered breathing (SDB) have not been adequately defined.
Methods:
A cross-sectional study of our myelomeningocele clinic population was undertaken to correlate polysomnographic results with historical data and findings from magnetic resonance imaging of the Chiari malformation, pulmonary function results, and nocturnal pulse oximetry.
Results:
A questionnaire survey of symptoms was available for 107 of 109 children (98% of the clinic population), and 83 patients agreed to undergo overnight polysomnography. Breathing during sleep was classified as normal in 31 cases (37%), mildly abnormal in 35 cases (42%), and moderately/severely abnormal in 17 cases (20%). Among the 17 patients with moderately/severely abnormal SDB, 12 patients had predominantly central apneas and 5 had predominantly obstructive apnea. Patients with a thoracic or thoracolumbar myelomeningocele, those who had previously had a posterior fossa decompression operation, those with more severe brain-stem malformations, and those with pulmonary function abnormalities were more likely to have moderately/severely abnormal SDB, relative risks (95% confidence intervals) 9.2 (2.9 to 29.3), 3.5 (1.3 to 8.9), 3.0 (0.9 to 10.5), and 11.6 (1.6 to 81.3), respectively. Failure of obstructive SDB to resolve after adenotonsillectomy in four patients suggested abnormal control of pharyngeal airway patency during sleep. Nocturnal pulse oximetry accurately predicted moderately/severely abnormal SDB with a sensitivity of 100% and a specificity of 67%.
Conclusions:
The pathogenesis of SDB in patients with myelomeningocele involves the functional level of the spinal lesions, congenital and acquired brainstem abnormalities, pulmonary function abnormalities, disorders of upper airway maintenance, and sleep state. Polysomnography and nocturnal pulse oximetry should be performed in high-risk patients to detect and classify SDB.