0890 INDIVIDUALIZED THERAPY FOR TREATING OBSTRUCTIVE SLEEP APNEA IN PEDIATRIC CROUZON SYNDROME PATIENTS

Abstract

Pediatric patients with Crouzon syndrome have great possibilities in suffering from obstructive sleep apnea (OSA), which is mainly due to midfacial hypoplasia and facial deformities. For most patients, a multi-disciplinary and sequential treatment plan is necessary to make for Crouzon syndrome often has different phenotypes of different severity in OSA and facial deformities. Typical patients were selected in this study to illustrate the necessity of individualized therapy for treating OSA. In the study, four Crouzon syndrome children of different severity in suffering from OSA and maxillofacial deformities were introduced. Detailed information in individualized treatment options was given including clinical manifestations, radiological findings, polysomnography detectings and treatment combinations. Based on the above findings, different but effective treatment options for these children’s OSA problems were adopted, either by surgeries including distraction osteogenesis and craniomaxillofacial surgeries with or without tonsillectomy, or by noninvasive continuous positive airway pressure (CPAP) therapy. Patient 1 was performed with LeFort IIIosteotomy and external distraction osteogenesis. Patient 2 was given LeFort IIIosteotomy and external distractor fixation and concurrent bilateral mandibular body osteotomy and internal distractor fixation for bimaxillary distraction. Patient 3 received LeFort I osteotomy for maxillary advancement with concurrent bilateral tonsillectomy. Differently, patient 4 with mild OSA disturbance adopted initial continuous positive airway pressure (CPAP) with good adherence and body development. Six years later, he began orthodontic treatment for preparing for orthognathic surgery. Recently bimaxillary surgery was performed on him to enlarge upper airway anatomically as well as improve facial profile. Follow-up studies showed problems of OSA and nocturnal hypoxia of those four patients were all alleviated greatly, as well as maxillofacial deformities. Combined with preoperative and postoperative orthodontics, patient 1 and 4 also got optimal results in better facial profile and dental occlusion. Thus, given Crouzon patients had great possiblilities in suffering from OSA and hypoxia problems, individualized therapy should be made and performed carefully to obtain optimized treatment results based on adequate clinical evaluations and patients’ conditions including age, disease severity and esthetic considerations. This work was supported by the National Natural Science Foundation of China (Grant number: 81470786).