Journal of the Korean Association of Oral and Maxillofacial Surgeons

The Korean Association of Oral and Maxillofacial Surgeons (대한구강악안면외과학회)
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Congenital syngnathia: a case report

  • Kim, Chul-Hwan (Department of Oral and Maxillofacial Surgery, College of Dentistry, Dankook University) ;
  • Kim, Moon-Young (Department of Oral and Maxillofacial Surgery, College of Dentistry, Dankook University)
  • Received : 2011.08.19
  • Accepted : 2011.10.12
  • Published : 2012.06.30
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Abstract

Congenital syngnathia refers to the fusion of bony tissues, a rare disorder with only 41 cases reported in the international literature from 1936 to 2009. The occurrence of syngnathia without any other associated systemic disease or congenital anomaly is extremely rare. This report presents a case of congenital syngnathia with unilateral maxillomandibular bony adhesion without any other oral or maxillofacial anomaly. No recommended protocol for surgery exists due to the rarity of the disorder. There is a very low survival rate for the few patients who have forgone surgical management. This case describes a 74-year-old female patient who was suffering from limitation of mouth opening and was subsequently diagnosed with congenital syngnathia. The surgical staff performed separation surgery and reconstructed the malformed oral vestibule and cheek using the radial forearm free flap operation.

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References

  1. Naikmasur VG, Sattur AP, Joshi SK, Rai A. Congenital syngnathia: case report and review of literature. Cleft Palate Craniofac J 2010;47:654-60.
  2. Burket LW. Congenital bony temporomandibular ankylosis and facial hemiatrophy. JAMA 1936;106:1719-22.
  3. Dawson KH, Gruss JS, Myall RW. Congenital bony syngnathia: a proposed classification. Cleft Palate Craniofac J 1997;34:141-6.
  4. Laster Z, Temkin D, Zarfin Y, Kushnir A. Complete bony fusion of the mandible to the zygomatic complex and maxillary tuberosity: case report and review. Int J Oral Maxillofac Surg 2001;30:75-9.
  5. Poswillo D. The pathogenesis of the first and second branchial arch syndrome. Oral Surg Oral Med Oral Pathol 1973;35:302-28.
  6. Hegtvedt AK. Diagnosis and management of facial asymmetry. In: Peterson LJ, ed. Principles of oral and maxillofacial surgery. Philadelphia: Lippincott; 1992:1400-1.
  7. Rao S, Oak S, Wagh M, Kulkarni B. Congenital midline palato mandibular bony fusion with a mandibular cleft and a bifid tongue. Br J Plast Surg 1997;50:139-41.
  8. Kamata S, Satoh K, Uemura T, Onizuka T. Congenital bilateral zygomatico-mandibular fusion with mandibular hypoplasia. Br J Plast Surg 1996;49:251-3.
  9. Goodacre TE, Wallace AF. Congenital alveolar fusion. Br J Plast Surg 1990;43:203-9.
  10. Nanda R. Maxillomandibular ankylosis and cleft palate in rat embryos. J Dent Res 1970;49:1086-90.
  11. Humphrey T. The relation between human fetal mouth opening reflexes and closure of the palate. Am J Anat 1969;125:317-44.
  12. Subramanian B, Agrawal K, Panda K. Congenital fusion of the jaws: a management protocol. Int J Oral Maxillofac Surg 2010;39:925-9.
  13. Agrawal K, Chandra SS, Sreekumar NS. Congenital bilateral intermaxillary bony fusion. Ann Plast Surg 1993;30:163-6.
  14. Choi JY, Min CG, Myoung H, Hwang SJ, Kim MJ, Lee JH. Acquired syngnathia. Br J Oral Maxillofac Surg 2004;42:448-50.

Cited by

  1. Congenital Bilateral Zygomatico-Maxillo-Mandibular Fusion Associated With Gum Fusion vol.27, pp.1, 2012, https://doi.org/10.1097/scs.0000000000002293
  2. Congenital Maxillomandibular Syngnathia: Review of Literature and Proposed New Classification System vol.20, pp.1, 2012, https://doi.org/10.1007/s12663-019-01308-8