Obstructive Sleep Apnea Syndrome Associated with
Klippel-Feil Syndrome：Report of a Case

Masaki KOHNO, Yoshiyuki TAKATA, Masahiro YONEZAWA,
Tsutomu NOMURA，Tamio NAKAJIMA
Fisrt Department of Oral and Maxillofacial Surgery,
Niigata University School of Dentistry

Abstract:
The first case of sleep apnea syndrome occurring in a 55-year-old man with K1ippel-Feil syndrome is described. Polysomnography and lateral cephalograms revealed the presence of obstructive type of sleep apnea possibly resulting from obstruction of a narrowed pharyngeal airway space due to severe lordosis of fused cervical vertebrae by the elongated soft palate and uvula. The results were compatible with the findings of CT, MRI and SPECT which revealed the absence of Arnold-Chiari anomaly, syringobulbia and/or extension of syringomyelia to the bulb, compression of spina1 cord and/or bulb by odontoid process and low perfusion of blood in the brainstem which could cause central type of sleep apnea．The uvulopalatoplasty (UPP) with complete resection of the excessive soft palate was performed without enlargement of the airway space at the hypopharynx．Although preoperative sleep hypopnea was still remaining, the episodes of sleep apnea decreased from 159 to 14 postoperatively. Clinically, excessive daytime sleepiness disappeared and the patient returned to his job.

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