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Segmental odontomaxillary dysplasia

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Segmental odontomaxillary dysplasia is a painless, unilateral enlargement of the upper jaw. The cause is unknown, and the disease affects the jaws, teeth, and adjacent soft tissue. The premolars may be congenitally missing, and the primary teeth (baby teeth) may be smaller than usual. It is a relatively recent discovery.


It is a rare developmental disorder that affects maxilla, also known as the upper jaw in most vertebrates. The disorder is often diagnosed in early childhood. Since its original description as hemimaxillofacial dysplasia by doctors in 1987,[1] less than 40 cases have been reported in the English literature.[2]


All cases reported appear to represent sporadic occurrence.[3][4] There is no specific inheritance pattern.[5][6][7] The male-to-female ratio of affected is 1.8:1 and is often diagnosed before the age of 9.[8][9][10][11] The disorder affects the right and left sides of the maxilla almost equally.[12][13]


  1. ^ Miles DA, Lovas JL, Cohen MM., Jr Hemimaxillofacial dysplasia: A newly recognized disorder of facial asymmetry, hypertrichosis of the facial skin, unilateral enlargement of the maxilla, and hypoplastic teeth in two patients. Oral Surg Oral Med Oral Pathol. 1987;64:445–8.
  2. ^ Shah, Ajaz; Latoo, Suhail; Ahmed, Irshad; Malik, Altaf H.; Hassan, Shahid; Bhat, Abraar; Mir, Shazia; Jain, Nitul (2012-01-01). "Midline segmental odontomaxillary dysplasia". Annals of Maxillofacial Surgery. 2 (2): 185–189. doi:10.4103/2231-0746.101358. ISSN 2231-0746. PMC 3591049. PMID 23483745.
  3. ^ Welsch MJ, Stein SL. A syndrome of hemimaxillary enlargement, asymmetry of the face, tooth abnormalities, and skin findings (HATS) Pediatr Dermatol. 2004;21:448–51
  4. ^ Becktor KB, Reibel J, Vedel B, Kjaer I. Segmental odontomaxillary dysplasia: Clinical, radiological and histological aspects of four cases. Oral Dis. 2002;8:106–10.
  5. ^ Prusack N, Pringle G, Scotti V, Chen SY. Segmental odontomaxillary dysplasia: a case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2000;90:483–8.
  6. ^ Paticoff K, Marion RW, Shprintzen RJ, Shanske AL, Eisig SB. Hemimaxillofacial dysplasia: A report of two new cases and further delineation of the disorder. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1997;83:484–8.
  7. ^ DeSalvo MS, Copete MA, Riesenberger RE, Cleveland DB, Chen SY. Segmental odontomaxillary dysplasia (hemimaxillofacial dysplasia): Case report. Pediatr Dent.1996;18:154–6.
  8. ^ Jones AC, Ford MJ. Simultaneous occurrence of segmental odontomaxillary dysplasia and Becker's nevus. J Oral Maxillofac Surg. 1999;57:1251–4.
  9. ^ Drake DL. Segmental odontomaxillary dysplasia: an unusual orthodontic challenge. Am J Orthod Dentofacial Orthop. 2003;123:84–6.
  10. ^ Gavalda C. Segmental odontomaxillary dysplasia. Med Oral. 2004;9:181.
  11. ^ Packota GV, Pharoah MJ, Petrikowski CG. Radiographic features of segmental odontomaxillary dysplasia: A study of 12 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1996;82:577–84.
  12. ^ Danforth RA, Melrose RJ, Abrams AM, Handlers JP. Segmental odontomaxillary dysplasia. Report of eight cases and comparison with hemimaxillofacial dysplasia. Oral Surg Oral Med Oral Pathol. 1990;70:81–5.
  13. ^ Armstrong C, Napier SS, Boyd RC, Gregg TA. Histopathology of the teeth in segmental odontomaxillary dysplasia: new findings. J Oral Pathol Med. 2004;33:246–8.
  • Kahn, Michael A. Basic Oral and Maxillofacial Pathology. Volume 1. 2001.
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Segmental odontomaxillary dysplasia
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