Chiari malformation and sleep related breathing disorders.

Dauvilliers Y, Stal V, Abril B, Coubes P, Bobin S, Touchon J, Escourrou P, Parker F, Bourgin P. Chiari malformation and sleep related breathing disorders. Journal of Neurology, Neurosurgery, and Psychiatry. 2007 Mar 30;

OBJECTIVE: To estimate the frequency, mechanisms, and predictive factors of sleep apnea syndrome (SAS) in a large group of children and adults affected with type I and II chiari malformation (CM).

BACKGROUND: The anatomical and functional integrity of both respiratory circuits and lower cranial nerves controlling the upper airway is necessary for breathing control during sleep. These latter structures may be altered in CM, and a few investigations have reported CM- related sleep disordered breathing.

METHODS: Forty-six, consecutive, unrelated CM patients (40 CMI, 6 CMII), of which 20 were children (8 male) and 26 were adults (12 male), underwent physical, neurological, oto-rhino-laryngoscopic examination, MRI, and polysomnography.

RESULTS: SAS was present in 31 (67.4%) of the CM patients (70% of CMI, 50% of CMII, including mainly children). Sixty percent of children with CM exhibited SAS, including 35% with obstructive (OSAS) and 25% with central (CSAS) sleep apnea syndrome. SAS was observed in 73% of CM adults (57.7 % OSAS, 15.4% CSAS). Severe SAS was found in 23% of CM adults. Multiple regression analysis revealed that age, type II Chiari and vocal cord paralysis predicted the central apnea index.

CONCLUSION: SAS is highly prevalent in all age groups of patients suffering from CM. CSAS, a rare condition in the general population, was common among the CM patients in our study. SDB associated with CM may explain the high frequency of respiratory failures observed during curative surgery of CM. Our results suggest that SAS should be systematically screened in CM patients, especially before surgery.

PMID: 17400590