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VOLUME 22 , ISSUE 10 ( October, 2021 ) > List of Articles


Long-term Management of a Patient with Apert Syndrome

Shinya Horiuchi, Hiroko Sato, Akihiko Iwasa, Aki Ichihara, Hirofumi Tenshin, Keiichiro Watanabe, Masahiro Hiasa, Ichiro Hashimoto, Eiji Tanaka

Keywords : Apert syndrome, Craniosynostosis, Distraction osteogenesis, Le Fort III osteotomy, Maxillary growth deficiency

Citation Information : Horiuchi S, Sato H, Iwasa A, Ichihara A, Tenshin H, Watanabe K, Hiasa M, Hashimoto I, Tanaka E. Long-term Management of a Patient with Apert Syndrome. J Contemp Dent Pract 2021; 22 (10):1184-1190.

DOI: 10.5005/jp-journals-10024-3201

License: CC BY-NC 4.0

Published Online: 07-02-2022

Copyright Statement:  Copyright © 2021; The Author(s).


Aim and objective: To present an Apert syndrome patient with midfacial growth deficiency treated with Le Fort III distraction osteogenesis and subsequent two-jaw surgery. Background: Apert syndrome is expressed as a severe and irregular craniosynostosis, midfacial hypoplasia, and symmetric syndactyly in the fingers and toes. For craniosynostosis syndromes, treatment planning is complex due to the disharmony between facial profile and occlusion. Case description: A 4-year-and-5-month-old boy, diagnosed with Apert syndrome, showed a concave profile accompanied with midfacial hypoplasia, moderate exorbitism, a reversed occlusion of −10.0 mm, an anterior open bite of −5.0 mm, and skeletal class III jaw-base relationship. The patient, aged 15 years and 4 months, underwent a Le Fort III osteotomy, and subsequent osteodistraction was performed via a rigid external distraction (RED) device. His midfacial bone was advanced by approximately 7.0 mm. One year after the distraction, preoperative treatment with 0.018-in preadjusted edgewise appliances was initiated. Two-jaw surgery with a Le Fort I osteotomy and bilateral sagittal split ramus osteotomy was performed after 42 months of preoperative orthodontic treatment. At the age of 20 years and 9 months, his facial profile dramatically changed to a straight profile, and an acceptable occlusion with an adequate interincisal relationship was obtained. A functional occlusion with an excellent facial profile was maintained throughout the 2-year retention period, although the upper dental arch width was slightly decreased, resulting in the recurrence of the left posterior crossbite. Conclusion: Our report indicates the necessity of long-term follow-up in patients with craniosynostosis because of syndrome-specific growth and methodologically induced relapse. Clinical significance: The two-stage operation combining early distraction osteogenesis and postgrowth orthognathic surgery proves to be an effective therapy for correcting midfacial hypoplasia and skeletal mandibular protrusion caused by Apert syndrome.

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