Kiely J L, Deegan P C, McNicholas W T
Dept of Respiratory Medicine, St Vincent's Hospital, Dublin, Ireland.
Eur Respir J. 1998 Aug;12(2):499-501. doi: 10.1183/09031936.98.12020499.
A 12 year old female with the Robin sequence presented with a one year history of snoring, witnessed apnoeas and daytime sleepiness. Surgery in early childhood had consisted of cleft palate repair, tonsillectomy and adenoidectomy and, later, revision palatoplasty. Overnight polysomnography (PSG) demonstrated severe obstructive sleep apnoea syndrome with an apnoea/hypopnoea index (AHI) of 49 events x h(-1), and repetitive oxygen desaturations below 50%. Nasal continuous positive airway pressure (nCPAP) effectively controlled her sleep abnormalities. After 3 yrs of nCPAP therapy, she requested discontinuation and was fully reassessed. PSG without nCPAP revealed an AHI <5 events x h(-1) with no desaturations below 90% and normal sleep quality. A repeat lateral cephalometrogram showed increased mandibular length and posterior airway space and reduced soft palate length. The patient remains asymptomatic 9 months following nCPAP discontinuation. This case indicates that nasal continuous positive airway pressure is an effective nonsurgical therapy in children with obstructive sleep apnoea syndrome and the Robin sequence. It is likely that mandibular growth, increase in mandibular length and enlargement of the posterior airway space was responsible for the resolution of obstructive sleep apnoea syndrome in this case.
一名患有罗宾序列征的12岁女性，有一年的打鼾、目击性呼吸暂停和日间嗜睡病史。幼儿期手术包括腭裂修复术、扁桃体切除术和腺样体切除术，后来又进行了腭裂修复术翻修。夜间多导睡眠图（PSG）显示严重阻塞性睡眠呼吸暂停综合征，呼吸暂停/低通气指数（AHI）为49次事件×小时⁻¹，且反复出现氧饱和度低于50%的情况。鼻持续气道正压通气（nCPAP）有效控制了她的睡眠异常。经过3年的nCPAP治疗后，她要求停用并进行了全面重新评估。未使用nCPAP的PSG显示AHI<5次事件×小时⁻¹，无低于90%的饱和度降低情况且睡眠质量正常。重复的头颅侧位片显示下颌长度增加、后气道间隙增大以及软腭长度缩短。在停用nCPAP 9个月后，该患者仍无症状。此病例表明，鼻持续气道正压通气是治疗患有阻塞性睡眠呼吸暂停综合征和罗宾序列征儿童的一种有效的非手术疗法。在该病例中，阻塞性睡眠呼吸暂停综合征的缓解可能是由于下颌生长、下颌长度增加以及后气道间隙增大所致。
