Reimão R, Papaiz E G, Papaiz L F
Department of Neurology, Hospital das Clínicas, University of São Paulo Medical School and Sleep Disorders Center, Brasil.
Arq Neuropsiquiatr. 1994 Dec;52(4):554-9. doi: 10.1590/s0004-282x1994000400017.
The case of a 12-year-old female patient with Pierre Robin sequence is reported, in which reduction of the pharyngeal airway leads to obstructive sleep apnea syndrome (OSAS) and excessive daytime sleepiness. Radiological evaluation, computerized tomography and magnetic resonance image showed bilateral temporomandibular ankylosis. Cephalometric data evidenced marked reduction of the posterior airway space. Three all-night polysomnographic evaluations detected severe OSAS with decrease in oxygen saturation. The Multiple Sleep Latency Test (MSLT) performed on two separate days objectively quantified the excessive daytime sleepiness with short sleep latencies; stage REM was not present. Polysomnography, MSLT and thorough radiologic studies, in this case, made it possible to determine the severity of OSAS, the site of obstruction, and the associated malformations.
报告了一例12岁患有皮埃尔·罗宾序列征的女性患者，其咽气道狭窄导致阻塞性睡眠呼吸暂停综合征（OSAS）和日间过度嗜睡。放射学评估、计算机断层扫描和磁共振成像显示双侧颞下颌关节强直。头影测量数据表明后气道间隙明显减小。三次整夜多导睡眠图评估检测到严重的OSAS伴血氧饱和度下降。在两个不同日期进行的多次睡眠潜伏期试验（MSLT）客观地量化了日间过度嗜睡且睡眠潜伏期短；快速眼动期不存在。在该病例中，多导睡眠图、MSLT和全面的放射学研究使得确定OSAS的严重程度、阻塞部位及相关畸形成为可能。
