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Published on: December 6, 2016
Sleep disordered breathing in children and adolescents with Chiari malformation type I
Anna Losurdo1, Serena Dittoni, Elisa Testani
1Institute of Neurology, Catholic University, Rome, Italy. annalosurdo@hotmail.it
Insights
Sleep disordered breathing (SDB) affects 24% of children with Chiari malformation Type I (CM-I). SDB is linked to hydrocephalus and syringomyelia in these patients.
Area of Science:
- Neurology
- Pediatrics
- Sleep Medicine
Background:
- Chiari malformation Type I (CM-I) is a structural defect in the cerebellum.
- CM-I has been linked to sleep disordered breathing (SDB).
Purpose of the Study:
- To determine the prevalence of SDB in pediatric CM-I patients.
- To identify clinical factors associated with SDB in this population.
Main Methods:
- Fifty-three pediatric patients with CM-I underwent neurological examination, MRI, and polysomnography (PSG).
- Otorhinolaryngologic evaluation was performed for patients with evidence of upper airway obstruction.
Main Results:
- SDB was identified in 13 (24%) of the CM-I patients.
- SDB prevalence was higher in patients with hydrocephalus (p=0.002) and syringomyelia (p=0.001).
- Obstructive SDB correlated with syringomyelia, while central SDB correlated with hydrocephalus.
Conclusions:
- The prevalence of SDB in this CM-I cohort was 24%.
- Cerebrospinal fluid dynamic abnormalities, specifically syringomyelia and hydrocephalus, are associated with SDB in CM-I.
Study Objectives:
Chiari malformation Type I (CM-I) has been associated with sleep disordered breathing (SDB). The aim of this study was to evaluate the prevalence of SDB in CM-I and its clinical correlates in a population of children and adolescents.
Methods:
Fifty-three consecutive children and adolescents affected by CM-I were enrolled (27 girls and 26 boys, mean age 10.3 ± 4.3, range: 3-18 years). All patients underwent neurological examination, MRI, and polysomnography (PSG). Otorhinolaryngologic clinical evaluation was performed in patients with polysomnographic evidence of sleep-related upper airway obstruction.
Results:
Mean size of the herniation was 9.5 ± 5.4 mm. Fourteen patients had syringomyelia, 5 had hydrocephalus, 31 presented neurological signs, 14 had epileptic seizures, and 7 reported poor sleep. PSG revealed SDB in 13 subjects. Patients with SDB, compared to those without SDB, had a higher prevalence hydrocephalus (p = 0.002), syringomyelia (p = 0.001), and neurological symptoms (p = 0.028). No significant difference was observed in age, gender, prevalence of epilepsy, and size of the herniation. Obstructive SDB was associated with syringomyelia (p = 0.004), whereas central SDB was associated with hydrocephalus (p = 0.034).
Conclusions:
In our population of CM-I patients the prevalence of SDB was 24%, lower than that reported in literature. Moreover, our findings suggest that abnormalities in cerebrospinal fluid dynamics in CM-I, particularly syringomyelia and hydro-cephalus, are associated with SDB.
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