Järund M, Lauritzen C
Department of Otorhinolaryngology, Head and Neck Surgery, Sahlgrenska University Hospital, Göteborg University, Sweden.
Scand J Plast Reconstr Surg Hand Surg. 1996 Dec;30(4):275-9. doi: 10.3109/02844319609056405.
Craniofacial surgery in craniofacial dysostosis on airway obstruction was studied retrospectively in a consecutive series of patients. The records of 76 patients were reviewed, 27 with Apert syndrome, 47 with Crouzon's syndrome, and two with Pfieffer's syndrome. Of 172 operations, 148 were done for cosmetic reasons, hydrocephalus, or papillary oedema, and 24 were done for airway distress. Forty patients (23%) were children less than 13 years of age, and 22 underwent midface advancement procedures. Only 13 of these operations had been done for airway distress. Two were cured by operation and seven improved. After operation had failed to relieve the airway obstruction, a nasal continuous positive airway pressure device (nCPAP) was fitted to seven patients. The nCPAP relieved or improved airway obstruction recorded by a sleep study. As midface advancement in childhood rarely results in lasting improvement in breathing or aesthetics, it may well be advisable to postpone operation until the early teens. We conclude that with nCPAP operation can in most cases be deferred until a time when a permanent result can be achieved.
我们对一系列连续性病例进行回顾性研究，以探讨颅面骨发育不全患者行颅面外科手术治疗气道梗阻的情况。我们回顾了76例患者的病历，其中27例为Apert综合征，47例为Crouzon综合征，2例为Pfeiffer综合征。在172例手术中，148例是出于美容、脑积水或乳头水肿的原因进行的，24例是出于气道窘迫进行的。40例患者（23%）年龄小于13岁，其中22例接受了面中部前移手术。这些手术中只有13例是为了解决气道窘迫问题。2例通过手术治愈，7例有所改善。在手术未能缓解气道梗阻后，为7例患者安装了鼻持续气道正压通气装置（nCPAP）。nCPAP缓解或改善了睡眠研究记录的气道梗阻情况。由于儿童期面中部前移很少能在呼吸或美学方面带来持久改善，因此很可能建议将手术推迟到青少年早期。我们得出结论，使用nCPAP，在大多数情况下可以将手术推迟到能够取得永久性效果的时候。
