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Published on: December 6, 2016
Type I Chiari malformation presenting central sleep apnea
Takuro Kitamura1, Soichiro Miyazaki2, Hiroshi Kadotani3
1Department of Sleep Medicine, Shiga University of Medical Science, Seta Tsukinowa-cho, Otsu, Shiga 520-2192, Japan; Department of Otorhinolaryngology, University of Occupational and Environmental Health, 1-1, Iseigaoka, Yahatanishi-ku, Kitakyushu 807-8555, Japan.
Type I Chiari malformation can cause central sleep apnea in children, even without other neurological symptoms. Surgical decompression significantly improved this young patient's breathing during sleep.
Area of Science:
- Neurology
- Pediatrics
- Sleep Medicine
Background:
- Sleep apnea is a known, though rare, clinical feature of Type I Chiari malformation.
- It can manifest as obstructive or central sleep apnea.
- Typically, sleep apnea in this condition is accompanied by neurological signs.
Observation:
- A 10-year-old girl presented with central sleep apnea (CSA) as her sole symptom.
- She had no other accompanying neurological deficits.
- Initial treatment with acetazolamide provided only mild symptomatic relief.
Findings:
- The patient was diagnosed with Type I Chiari malformation.
- Posterior fossa decompression surgery led to dramatic clinical and polysomnographic improvement in her respiratory status during sleep.
- This case highlights CSA as a potential isolated presentation of Type I Chiari malformation in children.
Implications:
- Type I Chiari malformation should be considered in the differential diagnosis of pediatric central apneas, even in the absence of other neurological signs.
- Sagittal craniocervical magnetic resonance imaging (MRI) is crucial for definitive diagnosis.
- Early diagnosis and surgical intervention can significantly improve outcomes for affected children.
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