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VOLUME 21 , ISSUE 10 ( October, 2020 ) > List of Articles


Hemifacial Microsomia Caused by First and Second Brachial Arch Syndrome Treated with Orthodontic Approach: A Case Report

Ayami Ito, Kazuna Tachiki, Ryoko Shioyasono, Mohannad Ashtar

Citation Information : Ito A, Tachiki K, Shioyasono R, Ashtar M. Hemifacial Microsomia Caused by First and Second Brachial Arch Syndrome Treated with Orthodontic Approach: A Case Report. J Contemp Dent Pract 2020; 21 (10):1189-1195.

DOI: 10.5005/jp-journals-10024-2957

License: CC BY-NC 4.0

Published Online: 01-12-2020

Copyright Statement:  Copyright © 2020; The Author(s).


Aim and objective: To present a growing patient with unilateral mandibular hypoplasia and microtia involved in the first and second branchial arch syndrome (FSBAS) treated with functional appliance. Background: The FSBAS comprises several developmental facial hypoplasia in ear and maxillofacial bones, resulting in hemifacial microsomia. Treatment for hemifacial microsomia varies greatly depending on the grade of mandibular deformities. Functional appliance treatment during growth period is available for mild to moderate mandibular deformities. However, there are few reports of hemifacial microsomia treated with functional appliance. Case description: The patient, an 8-year-and-5-month-old girl, had a chief complaint of mandibular deviation. She had been diagnosed with the FSBAS at birth. Her facial profile was straight and panoramic radiograph indicated that the mandibular ramal height of the affected side was about 60.4% compared to the unaffected side. The occlusal cant was 6°, and the right maxilla and mandible showed severe growth deficiency. At the age of 10 years, functional appliance with expander was used; for 2 years 6 months, the maxillomandibular growth was controlled and from panoramic radiograph, the ramus height of the affected side was increased to 65.0% compared to the unaffected left mandibular ramus. At the age of 12 years and 8 months, multibracket treatment was initiated. After 32 months of active treatment, proper occlusion with functional Class I canine and molar relationships was obtained, although facial asymmetry associated with the difference of ramus heights still existed. The resulting occlusion was stable during 1.5-year retention period. Conclusion: Our results indicated the importance of orthopedic treatment during growth period in the patient with hemifacial microsomia involving the FSBAS. Clinical significance: This report proposes an efficacy of conventional orthodontic treatment for growing patients with hemifacial microsomia involved in the FSBAS.

  1. Stoll C, Roth MP, Dott B, et al. Discordance for skeletal and cardiac defect in monozygotic twins. Acta Genet Med Gemellol (Roma) 1984;33(3):501–504. DOI: 10.1017/s0001566000005961.
  2. Cohen Jr MM, Rollnick BR, Kaye CI. Oculoauriculovertebral spectrum: an updated critique. Cleft Palate J 1989;26(4):276–286.
  3. Hartsfield JK. Review of the etiologic heterogeneity of the oculo-auriculo-vertebral spectrum (hemifacial microsomia). Orthod Craniofac Res 2007;10(3):121–128. DOI: 10.1111/j.1601-6343.2007.00391.x.
  4. Rollnick BR, Kaye CI, Nagatoshi K, et al. Oculoauriculovertebral dysplasia and variants: phenotypic characteristics of 294 patients. Am J Med Genet 1987;26(2):361–375. DOI: 10.1002/ajmg.1320260215.
  5. Bielicka B, Necka A, Andrych M. Interdisplinary treatment of patient with Goldenhar syndrome-clinical reports. Dent Med Probl 2006;43:458–462.
  6. Pruzansky S. Not all dwarfed mandibles are alike. Birth Defects Orig Artic Ser 1969;5:120–129.
  7. Kaban LB, Moses MH, Mulliken JB. Surgical correction of hemifacial microsomia in the growing child. Plast Reconstr Surg 1988;82(1):9–19. DOI: 10.1097/00006534-198882010-00003.
  8. Meazzini MC, Mazzoleni F, Gabriele C, et al. Mandibular distraction osteogenesis in hemifacial microsomia: long-term follow-up. J Craniomaxillofac Surg 2005;33(6):370–376. DOI: 10.1016/j.jcms.2005.07.004.
  9. 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.
  10. James D, Ma L. Mandibular reconstruction in children with obstructive sleep apnea due to micrognathia. Plast Reconstr Surg 1997;100(5):1131–1137. DOI: 10.1097/00006534-199710000- 00007.
  11. Verlinden CR, van de Vijfeijken SE, Jansma EP, et al. Complications of mandibular distraction osteogenesis for congenital deformities: a systematic review of the literature and proposal of a new classification for complications. Int J Oral Maxillofac Surg 2015 Jan;44(1):37–43. DOI: 10.1016/j.ijom.2014.07.009.
  12. Zhang RS, Lin LO, Hoppe IC, et al. Early mandibular distraction in craniofacial microsomia and need for orthognathic correction at skeletal maturity: a comparative long-term follow-up study. Plast Reconstr Surg 2018;142(5):1285–1293. DOI: 10.1097/PRS.0000000000004842.
  13. Wang P, Zhang Z, Wang Y, et al. The accuracy of virtual-surgical-planning-assisted treatment of hemifacial macrosomia in adult patients: distraction osteogenesis vs. orthognathic surgery. Int J Oral Maxillofac Surg 2019;48(3):341–346. DOI: 10.1016/j.ijom.2018. 07.026.
  14. Wang X, Feng S, Tang X, et al. Incidents of mandibular distraction osteogenesis for hemifacial macrosomia. Plast Reconstr Surg 2018;142(4):1002–1008. DOI: 10.1097/PRS.0000000000004812.
  15. Wang J, Yuan L, Liu J, et al. Hemifacial microsomia treated with a hybrid technique combining distraction osteogenesis and a mandible-guided functional appliance: pilot study. Am J Orthod Dentofacial Orthop 2019;155(6):801–811. DOI: 10.1016/j.ajodo.2018. 06.020.
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