The Journal of Contemporary Dental Practice

Register      Login

SEARCH WITHIN CONTENT

FIND ARTICLE

Volume / Issue

Online First

Archive
Volume  25 (2024)
Volume  24 (2023)
Volume  23 (2022)
Volume  22 (2021)
Volume  21 (2020)
Volume  20 (2019)
Volume  19 (2018)
Volume  18 (2017)
Volume  17 (2016)
Volume  16 (2015)
Volume  15 (2014)
Volume  14 (2013)
Volume  13 (2012)
Volume  12 (2011)
Volume  11 (2010)
Volume  10 (2009)
Volume  9 (2008)
Volume  8 (2007)
Volume  7 (2006)
Volume  6 (2005)
Volume  5 (2004)
Volume  4 (2003)
Volume  3 (2002)
Volume  2 (2001)
Volume  1 (1999)
Related articles

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

CASE REPORT

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).


Abstract

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.

HTML PDF Share
  1. Cohen MM, Kreiborg S. Skeletal abnormalities in the Apert syndrome. Am J Med Genet 1993;47(5):624–632. DOI: 10.1002/ajmg.1320470509.
  2. Park WJ, Theda C, Maestri NE, et al. Analysis of phenotypic features and FGFR2 mutations in Apert syndrome. Am J Hum Genet 1995;57(2):321–328. PMID: 7668257.
  3. Cohen MM, Kreiborg S. A clinical study of the craniofacial features in Apert syndrome. Int J Oral Maxillofac Surg 1996;25(1):45–53. DOI: 10.1016/s0901-5027(96)80011-7.
  4. Ciurea AV, Toader C. Genetics of craniosynostosis: review of the literature. J Med Life 2009;2(1):5–17. PMID: 20108486.
  5. Ferraro NF. Dental, orthodontic, and oral/maxillofacial evaluation and treatment in Apert syndrome. Clin Plast Surg 1991;18(2):291–307. PMID: 2065490.
  6. Cohen SR, Rutrick RE, Burstein FD. Distraction osteogenesis of the human craniofacial skeleton: initial experience with new distraction system. J Craniofac Surg 1995;6(5):368–374. DOI: 10.1097/00001665-199509000-00007.
  7. Polley JW, Figueroa AA. Management of severe maxillary deficiency in childhood and adolescence through distraction osteogenesis with an external, adjustable, rigid distraction device. J Craniofac Surg 1997;8(3):181–185; discussion 186. DOI: 10.1097/00001665-199705000-00008.
  8. Krimmel M, Cornelius CP, Roser M, et al. External distraction of the maxilla in patients with craniofacial dysplasia. J Craniofac Surg 2001;12(5):458–463. DOI: 10.1097/00001665-200109000-00011.
  9. Kuroda S, Araki Y, Oya S, et al. Maxillary distraction osteogenesis to treat maxillary hypoplasia: comparison of an internal and an external system. Am J Orthod Dentofacial Orthop 2005;127(4):493–498. DOI: 10.1016/j.ajodo.2004.08.014.
  10. Figueroa AA, Polley JW. Management of severe cleft maxillary deficiency with distraction osteogenesis: procedure and results. Am J Orthod Dentofacial Orthop 1999;115(1):1–12. DOI: 10.1016/S0889-5406(99)70310-0.
  11. Kuroda S, Watanabe K, Ishimoto K, et al. Long-term stability of Le Fort III distraction osteogenesis with a rigid external distraction device in a patient with Crouzon syndrome. Am J Orthod Dentofacial Orthop 2011;140(4):550–561. DOI: 10.1016/j.ajodo.2009.12.038.
  12. Matsumoto K, Urano Y, Kubo Y, et al. Mutation of the fibroblast growth factor receptor 2 gene in Japanese patients with Apert syndrome. Plast Reconstr Surg 1998;101(2):307–311. DOI: 10.1097/00006534-199802000-00007.
  13. Tanimoto Y, Yokozeki M, Hiura K, et al. A soluble form of fibroblast growth factor receptor 2 (FGFR2) with S252W mutation acts as an efficient inhibitor for the enhanced osteoblastic differentiation caused by FGFR2 activation in Apert syndrome. J Biol Chem 2004;279(44):45926–45934. DOI: 10.1074/jbc.M404824200.
  14. Matsumoto K, Nakanishi H, Koizumi Y, et al. Correction of a deformed thumb by distraction of the phalanx. Scand J Plast Reconstr Surg Hand Surg 2002;36(6):368–372. DOI: 10.1080/028443102321096375.
  15. Wada K, Matsushita K, Shimazaki S, et al. An evaluation of a new case analysis of a lateral cephalometric roentgenogram. J Kanazawa Med Univ 1981;6:60–70.
  16. Posnik JC, Ruiz RL, Tiwana PS. Craniofacial dysostosis syndromes: stages of reconstruction. Oral Maxillofacial Surg Clin N Am 2004;16(4): 475–491. DOI: 10.1016/j.coms.2004.08.004. PMID: 18088749.
  17. Posnick JC, Armstrong D, Bite U. Crouzon and Apert syndromes: Intracranial volume measurements before and after cranio-orbital reshaping in childhood. Plast Reconstr Surg 1995;96:539–548. PMID: 7638278.
  18. Oberoi S, Hoffman WY, Vargervik K. Craniofacial team management in Apert syndrome. Am J Orthod Dentofacial Orthop 2012;141(4 Suppl):S82–S87. DOI: 10.1016/j.ajodo.2012.01.003.
  19. Figueroa AA, Polley JW, Friede H, et al. Long-term skeletal stability after maxillary advancement with distraction osteogenesis using a rigid external distraction device in cleft maxillary deformities. Plast Reconstr Surg 2004;114(6):1382–1392; discussion 1393–1394. DOI: 10.1097/01.prs.0000138593.89303.1b.
  20. Satoh K, Mitsukawa N, Hosaka Y. Dual midfacial distraction osteogenesis: Le Fort III minus I and Le Fort I for syndromic craniosynostosis. Plast Reconstr Surg 2003;111(3):1019–1028. DOI: 10.1097/01.PRS.0000047440.06788.72.
  21. Mitsukawa N, Kaneko T, Saiga A, et al. Early midfacial distraction for syndromic craniosynostotic patients with obstructive sleep apnoea. J Plast Reconstr Aesthet Surg 2013;66(9):1206–1211. DOI: 10.1016/j.bjps.2013.04.061.
  22. Breik O, Mahindu A, Moore MH, et al. Apert syndrome: Surgical outcomes and perspectives. J Craniomaxillofac Surg 2016;44(9): 1238–1245. doi: 10.1016/j.jcms.2016.06.001.
  23. Susami T, Fukawa T, Miyazaki H, et al. A survey of orthodontic treatment in team care for patients with syndromic craniosynostosis in Japan. Cleft Palate Craniofac J 2018;55(4):479–486. DOI: 10.1177/1055665617747703.
  24. Saltaji H, Altalibi M, Major MP, et al. Le Fort III distraction osteogenesis versus conventional Le Fort III osteotomy in correction of syndromic midfacial hypoplasia: a systematic review. J Oral Maxillofac Surg 2014;72(5):959–972. DOI: 10.1016/j.joms.2013.09.039.
  25. Shetye PR, Boutros S, Grayson BH, et al. Midterm follow-up of midface distraction for syndromic craniosynostosis: a clinical and cephalometric study. Plast Reconstr Surg 2007;120(6):1621–1632. DOI: 10.1097/01.prs.0000267422.37907.6f.
  26. Lee DW, Ham KW, Kwon SM, et al. Dual midfacial distraction osteogenesis for Crouzon syndrome: long-term follow-up study for relapse and growth. J Oral Maxillofac Surg 2012;70(3): e242– e251. DOI: 10.1016/j.joms.2011.11.010.
  27. Shetye PR, Davidson EH, Sorkin M, et al. Evaluation of three surgical techniques for advancement of the midface in growing children with syndromic craniosynostosis. Plast Reconstr Surg 2010;126(3): 2061–2070. DOI: 10.1097/PRS.0b013e3181e6051e.
  28. Iannetti G, Ramieri V, Pagnoni M, et al. Le Fort III external midface distraction: surgical outcomes and skeletal stability. J Craniofac Surg 2012;23(3):896–900. DOI: 10.1097/SCS.0b013e31824e2549.
  29. Meazzini MC, Allevia F, Mazzoleni F, et al. Long-term follow-up of syndromic craniosynostosis after Le Fort III halo distraction: a cephalometric and CT evaluation. J Plast Reconstr Aesthet Surg 2012;65(4):464–472. DOI: 10.1016/j.bjps.2011.09.048.
PDF Share
PDF Share

© Jaypee Brothers Medical Publishers (P) LTD.