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Regional Odontodysplasia Affecting the Maxilla


Regional odontodysplasia (RO) is a rare dental anomaly of unknown etiology that can affect both deciduous and permanent dentition. RO is characterized by severe hypoplasia of enamel and dentin, and teeth affected are friable and more susceptible to caries and fractures. Most of the lesions occur in the anterior maxilla and correlation with clinical and radiographic features is essential to provide a correct diagnosis. The major criteria for diagnosis are predominantly based on radiography, which shows presence of large pulp chambers and a marked reduction in the radiopacity of enamel and dentin, making the distinction between these mineralized structures difficult. Early diagnosis is important to minimize future sequels and allow preventive or conservative treatment. The therapeutic approach of the RO should be based on the degree of severity of the anomaly and in the individual functional and aesthetic needs of each case. A classic case of RO affecting the maxilla is exemplified in this Sine Qua Non Radiology–Pathology article.

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  1. 1.

    de Sa Cavalcante D, Fonteles CSR, Ribeiro TR, Kurita LM, Pimenta AVM, Carvalho FSR, et al. Mandibular regional odontodysplasia in an 8-year-old boy showing teeth disorders, gubernaculum tracts, and altered bone fractal pattern. Int J Clin Pediatr Dent. 2018;11(2):128–34. https://doi.org/10.5005/jp-journals-10005-1498.

  2. 2.

    Bowden E, Carroll C, Gill M, Llewelyn R. Cervicofacial infection in a 3-year-old child with regional odontodysplasia: a case report and literature review. J Oral Maxillofac Pathol. 2018;22(Suppl 1):117–20. https://doi.org/10.4103/jomfp.JOMFP_146_17.

  3. 3.

    Jahanimoghadam F, Pishbin L, Rad M. Clinical, radiographic, and histologic evaluation of regional odontodysplasia: a case report with 5-year follow-up. J Dent. 2016;17(2):159–63.

  4. 4.

    Hitchin AD. Unerupted deciduos teeth in a youth aged 15 1/2. Br Dent J. 1934;56:631–3.

  5. 5.

    Mc Call JO, Wald SS. In clinical dental roentgenography. Philadelphia: W.B. Saunders Co; 1947. pp. 169–70.

  6. 6.

    Zegarelli EV, Kutscher AH, Applebaum E, Archard HO. Odontodysplasia. Oral Surg Oral Med Oral Pathol. 1963;16:187–93.

  7. 7.

    Al-Mullahi AM, Toumba KJ. Regional odontodysplasia with generalised enamel defect. Case Rep Dent. 2016;2016:4574673. https://doi.org/10.1155/2016/4574673.

  8. 8.

    de Fays L, Van Malderen K, De Smet K, Sawchik J, Verlinden V, Hamdani J, et al. Use of paracetamol during pregnancy and child neurological development. Dev Med Child Neurol. 2015;57(8):718–24.

  9. 9.

    Murthy PS, Deshmukh S. Regional odontodysplasia and its treatment maneuver. Int J Health Allied Sci. 2013;2:153–8.

  10. 10.

    Mathew A, Dauravu LM, Reddy SN, Kumar KR, Venkataramana V. Ghost teeth: regional odontodysplasia of maxillary first molar associated with eruption disorders in a 10-year-old girl. J Pharm Bioallied Sci. 2015;7(Suppl 2):S800–3. https://doi.org/10.4103/0975-7406.163570.

  11. 11.

    Babu NS, Smriti RJ, Pratima DB. Regional odontodysplasia: report of an unusual case involving mandibular arch. Contemp Clin Dent. 2015;6(2):237–9. https://doi.org/10.4103/0976-237X.156054.

  12. 12.

    Toupenay S, Fournier BP, Manière MC, Ifi-Naulin C, Berdal A, de La Dure-Molla M. Amelogenesis imperfecta: therapeutic strategy from primary to permanent dentition across case reports. BMC Oral Health. 2018;18(1):108. https://doi.org/10.1186/s12903-018-0554-y.

  13. 13.

    Shields ED, Bixler D, el-kafrawy AM. A proposed classification or heritable human dentin defects with a description of a new entity. Arch Oral Biol. 1973;18:543–53.

  14. 14.

    Chen D, Li X, Lu F, Wang Y, Xiong F, Li Q. Dentin dysplasia type I-A dental disease with genetic heterogeneity. Oral Dis. 2018. https://doi.org/10.1111/odi.12861.

  15. 15.

    Khandelwal S, Gupta D, Likhyani L. A case of dentin dysplasia with full mouth rehabilitation: a 3-year longitudinal study. Int J Clin Pediatr Dent. 2014;7(2):119–24. https://doi.org/10.5005/jp-journals-10005-1248.

  16. 16.

    Perlea P, Dragomir C, Bodeanu A, Temelcea AN, Iliescu AA. A rare case diagnosed as dentin dysplasia type II. Rom J Morphol Embryol. 2018;59(1):359–65.

  17. 17.

    Barron MJ, McDonnell ST, Mackie I, Dixon MJ. Hereditary dentine disorders: dentinogenesis imperfecta and dentine dysplasia. Orphanet J Rare Dis. 2008;3:31. https://doi.org/10.1186/1750-1172-3-31.

  18. 18.

    Feeney C, Stanford N, Lee S, Barry S. Hypophosphatasia and the importance of the general dental practitioner: a case series and discussion of upcoming treatments. Br Dent J. 2018;224(12):937–43. https://doi.org/10.1038/sj.bdj.2018.441.

  19. 19.

    Mornet E, Hypophosphatasia. Orphanet J Rare Dis. 2007;2:40. https://doi.org/10.1186/1750-1172-2-40.

  20. 20.

    Millán JL, Whyte MP. Alkaline phosphatase and hypophosphatasia. Calcif Tissue Int. 2015;98(4):398–416. https://doi.org/10.1007/s00223-015-0079-1.

  21. 21.

    Smith CEL, Poulter JA, Antanaviciute A, et al. Amelogenesis imperfecta; genes, proteins, and pathways. Front Physiol. 2017;8:435. https://doi.org/10.3389/fphys.2017.00435.

  22. 22.

    Carlos R, Contreras-Vidaurre E, Almeida OP, Silva KR, Abrahão PG, Miranda AM, Pires FR. Regional odontodysplasia: morphological, ultrastructural, and immunohistochemical features of the affected teeth, connective tissue, and odontogenic remnants. J Dent Child. 2008;75(2):144–50.

  23. 23.

    Naik LK, Shetty P, Girish Babu KL. Regional odontodysplasia crossing the midline: a unique case associated with bilateral impacted supernumerary teeth. J Pediatr Dent. 2014;2:96–100. https://doi.org/10.4103/2321-6646.145585.

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Correspondence to John Lennon Silva Cunha.

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Silva Cunha, J.L., Barboza Santana, A.V., Alves da Mota Santana, L. et al. Regional Odontodysplasia Affecting the Maxilla. Head and Neck Pathol 14, 224–229 (2020). https://doi.org/10.1007/s12105-019-01031-3

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  • Regional odontodysplasia
  • Ghost teeth
  • Primary dentition
  • Permanent dentition