Yosunkaya Sebnem, Pekcan Sevgi
Department of Chest Diseases, Necmettin Erbakan University Meram Faculty of Medicine, Konya, Turkey.
Turk J Pediatr. 2013 Jan-Feb;55(1):107-11.
There are few reports of a patient presenting with symptoms of obstructive sleep apnea syndrome (OSAS) as the sole manifestation of Chiari malformation type 1 (CM1). In the literature, complex sleep apnea syndrome (CompSAS) was also reported as a rare condition related to CM1 patients. We report the case of a 13-year-old patient with the complaint of snoring and difficulty in breathing during sleep, but otherwise healthy. After an initial polysomnography, the patient was diagnosed with OSAS and nocturnal continuous hypoxemia. The child underwent titration to pressure of continuous positive airway pressure (CPAP); obstructive apnea improved but central apnea (i.e., CompSAS) and nocturnal continuous hypoxemia persisted. Magnetic resonance imaging led to diagnosis of CM1. Her central apnea and nocturnal hypoxemia resolved following bi-level positive airway pressure-spontaneous-timed (S/T) (BiPAPS/ T) treatment. We emphasize that the CM1 cases can admit with only breathing problems during sleep without concomitant neurological findings, and this malformation can cause central apnea resistant to CPAP.
仅有少数报告称,患者以阻塞性睡眠呼吸暂停综合征(OSAS)症状作为 Chiari 畸形 1 型(CM1)的唯一表现。在文献中,复杂睡眠呼吸暂停综合征(CompSAS)也被报道为一种与 CM1 患者相关的罕见病症。我们报告了一例 13 岁患者,其主诉为打鼾及睡眠时呼吸困难,但其他方面健康。初次多导睡眠监测后,该患者被诊断为 OSAS 及夜间持续性低氧血症。患儿接受了持续气道正压通气(CPAP)压力滴定;阻塞性呼吸暂停有所改善,但中枢性呼吸暂停(即 CompSAS)及夜间持续性低氧血症仍持续存在。磁共振成像检查确诊为 CM1。在接受双水平气道正压通气-自主定时(S/T)(BiPAPS/T)治疗后,她的中枢性呼吸暂停及夜间低氧血症得到缓解。我们强调,CM1 病例可能仅表现为睡眠期间的呼吸问题,而无伴随的神经系统表现,且这种畸形可导致对 CPAP 抵抗的中枢性呼吸暂停。