Craniofacial team management in Apert syndrome.

INTRODUCTION: Apert syndrome is one of the rarest of the craniosynostosis syndromes. Affected persons have extensive structural and functional impairments, some of which can be life threatening. Management requires team care from infancy to adulthood. The purposes of this article are to assess the outcomes in individuals with Apert syndrome after completion of treatment and to review current protocols for craniofacial team care and dental, orthodontic, and orthognathic surgical management.

METHODS: This was a retrospective cohort study of 8 subjects with Apert syndrome. Cephalograms at 2 time points were compared: adolescence (before midface advancement) and at least 1 year after advancement. The cephalometric values were compared with paired t tests. Team protocols are delineated.

RESULTS: Measurements indicating forward positioning of the maxilla increased significantly: SNA by 10.7° (P = 0.002) and midface length by 9.6 mm (P = 0.002). Sagittal jaw relationship improved significantly as well: ANB by 14° (P = 0.004) and the Wits appraisal by 8 mm (P = 0.003). Vertical dimensions also increased.

CONCLUSIONS: All individuals had significantly improved and stable positions of the midface and normalized facial profiles after treatment.