Central sleep apnea and Chiari 1 malformation in a pediatric patient with Klippel-Feil syndrome.
Klippel-Feil sequence
decompression
sleep apnea
sleep-disordered breathing
Journal
Journal of clinical sleep medicine : JCSM : official publication of the American Academy of Sleep Medicine
ISSN: 1550-9397
Titre abrégé: J Clin Sleep Med
Pays: United States
ID NLM: 101231977
Informations de publication
Date de publication:
15 10 2020
15 10 2020
Historique:
pubmed:
6
7
2020
medline:
24
6
2021
entrez:
5
7
2020
Statut:
ppublish
Résumé
Klippel-Feil sequence (KFS) is a rare congenital condition that presents with congenital cervical spine fusion, reduced cervical spine flexion, and low posterior hairline. Chiari malformation type 1 and sleep-disordered breathing (SDB) are frequent comorbidities of KFS. The pathologic basis of the connection between Chiari malformation type 1 and SDB in the setting of KFS is not clearly understood. Here we report a pediatric patient with KFS, SDB, and drooling who also had Chiari malformation type 1. Posterior fossa decompression of this patient significantly improved most symptoms including sleep disturbances. Repeat polysomnogram 8 weeks after posterior fossa decompression revealed worsening central sleep apnea despite the patient being clinically asymptomatic. Taken together, this case highlights the point that, although it is critical to recognize the association of SDB in the setting of KFS, decompression alone may not be sufficient to completely alleviate SDB and certain neurologic symptoms.
Identifiants
pubmed: 32620190
doi: 10.5664/jcsm.8650
pmc: PMC7954022
doi:
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
1805-1807Informations de copyright
© 2020 American Academy of Sleep Medicine.
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