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

Jazyk: japonština
Rok vydání: 1997
Předmět:
Zdroj: 新潟歯学会雑誌. 27(2):49-53
ISSN: 0385-0153
Popis: The first case of sleep apnea syndrome occurring in a 55-year-old man with Klippel-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 spinal 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 59 to 14 postoperatively. Clinically, excessive daytime sleepiness disappeared and the patient returned to his job.
55歳男性のKlippel-Feil症候群に生じた睡眠時無呼吸症候群の初めての報告である。終夜睡眠ポリグラフと側方セフアログラムによって、癒合して著しく前弯した頚椎によって狭窄した咽頭部の気道が睡眠中に過剰な軟口蓋と口蓋垂によって閉塞されることによって閉塞型の睡眠時無呼吸が生じることが判った。この結果は,中枢型の睡眠時無呼吸症候群の原因となりうるArnold-Chiari奇形、延髄空洞症もしくは脊髄空洞症の延髄への波及、歯突起による脊髄または延髄の圧迫、脳幹部の血流低下などが、CT、MRI、SPECTによって認められなかったことに矛盾しなかった。下咽頭に於ける気道を拡大せずに軟口蓋の過剰量を完全に切除する口蓋垂軟口蓋形成術(UPP)を行ったところ、術後には睡眠中の低換気は残ったが、無呼吸は59回から14回に減少した。臨床的にも日中傾眠は消失し、患者は仕事に復帰した。
Databáze: OpenAIRE