CRANIOFACIAL DYSOSTOSIS - AIRWAY-OBSTRUCTION AND CRANIOFACIAL SURGERY

Craniofacial surgery in craniofacial dysostosis on airway obstruction was studied retrospectively in a consecutive series of patients. The r ecords of 76 patients were reviewed, 27 with Apert syndrome, 47 with C rouzon's syndrome, and two with Pfieffer's syndrome. Of 172 operations , 148 were done for cosmetic reasons, hydrocephalus, or papillary oede ma, and 24 were done for airway distress. Forty patients (23%) were ch ildren less than 13 years of age, and 22 underwent midface advancement procedures. Only 13 of these operations had been done for airway dist ress. Two were cured by operation and seven improved. After operation had failed to relieve the airway obstruction, a nasal continuous posit ive airway pressure device (nCPAP) was fitted to seven patients. The n CPAP relieved or improved airway obstruction recorded by a sleep study . As midface advancement in childhood rarely results in lasting improv ement in breathing or aesthetics, it may well be advisable to postpone operation until the early teens. We conclude that with nCPAP operatio n can in most cases be deferred until a time when a permanent result c an be achieved.