Mucopolysaccharidosis

Radiographic findings in a series of 16 cases

Dmitry José De Santana Sarmento, Sérgio Henrique Gonçalves De Carvalho, Saulo Sousa Melo, Fátima Roneiva Alves Fonseca, Denise Nóbrega Diniz, Patrícia Meira Bento, Germana De Queiroz Tavares Borges Mesquita, Daniela Pita De Melo

Research output: Contribution to journalArticle

8 Citations (Scopus)

Abstract

Objectives To investigate the radiographic findings of mucopolysaccharidoses (MPS) on panoramic radiographs in a series of 16 patients. Study Design This retrospective cross-sectional study included panoramic radiographs of 16 patients with cytogenetically diagnosed MPS. Two blinded, previously calibrated observers evaluate the presence or absence of the following features: type of dentition; stage of dental age in comparison with chronologic age; delayed exfoliation of deciduous teeth; supernumerary teeth; teeth impaction; enlarged, cyst-like dental crypt; hypercementosis; taurodontism; generalized enamel hypoplasia; enlargement of the bone marrow spaces; thinning of cortical bone; unusual morphology of condyles; and flattening of the condylar head. Results The final sample was composed of 8 MPS IV, 7 MPS VI, and 1 MPS I cases. All patients presented unusual morphology of condyles. Cyst-like dental crypt (75%), taurodontism, and teeth impaction (68.75% each) were also overall common findings. Generalized enamel hypoplasia was exclusively identified in patients with MPS IV (75% of those patients). Conclusions Radiographic features of oral and maxillofacial manifestations in patients with MPS frequently encountered by dentists may help recognize the disorder. However, because of sample size limitations, it was not possible to infer any statistical relationship between the radiographic features and the types of MPS.

Original languageEnglish (US)
Pages (from-to)e240-e246
JournalOral surgery, oral medicine, oral pathology and oral radiology
Volume120
Issue number6
DOIs
StatePublished - Jan 1 2015
Externally publishedYes

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Mucopolysaccharidoses
Tooth
Mucopolysaccharidosis IV
Dental Enamel Hypoplasia
Mucopolysaccharidosis I
Hypercementosis
Cysts
Mucopolysaccharidosis VII
Mucopolysaccharidosis VI
Supernumerary Tooth
Oral Manifestations
Bone and Bones
Dentition
Deciduous Tooth
Dentists
Sample Size
Cross-Sectional Studies
Bone Marrow

ASJC Scopus subject areas

  • Surgery
  • Oral Surgery
  • Pathology and Forensic Medicine
  • Dentistry (miscellaneous)
  • Radiology Nuclear Medicine and imaging

Cite this

De Santana Sarmento, D. J., De Carvalho, S. H. G., Sousa Melo, S., Fonseca, F. R. A., Diniz, D. N., Bento, P. M., ... De Melo, D. P. (2015). Mucopolysaccharidosis: Radiographic findings in a series of 16 cases. Oral surgery, oral medicine, oral pathology and oral radiology, 120(6), e240-e246. https://doi.org/10.1016/j.oooo.2015.08.009

Mucopolysaccharidosis : Radiographic findings in a series of 16 cases. / De Santana Sarmento, Dmitry José; De Carvalho, Sérgio Henrique Gonçalves; Sousa Melo, Saulo; Fonseca, Fátima Roneiva Alves; Diniz, Denise Nóbrega; Bento, Patrícia Meira; De Queiroz Tavares Borges Mesquita, Germana; De Melo, Daniela Pita.

In: Oral surgery, oral medicine, oral pathology and oral radiology, Vol. 120, No. 6, 01.01.2015, p. e240-e246.

Research output: Contribution to journalArticle

De Santana Sarmento, DJ, De Carvalho, SHG, Sousa Melo, S, Fonseca, FRA, Diniz, DN, Bento, PM, De Queiroz Tavares Borges Mesquita, G & De Melo, DP 2015, 'Mucopolysaccharidosis: Radiographic findings in a series of 16 cases', Oral surgery, oral medicine, oral pathology and oral radiology, vol. 120, no. 6, pp. e240-e246. https://doi.org/10.1016/j.oooo.2015.08.009
De Santana Sarmento, Dmitry José ; De Carvalho, Sérgio Henrique Gonçalves ; Sousa Melo, Saulo ; Fonseca, Fátima Roneiva Alves ; Diniz, Denise Nóbrega ; Bento, Patrícia Meira ; De Queiroz Tavares Borges Mesquita, Germana ; De Melo, Daniela Pita. / Mucopolysaccharidosis : Radiographic findings in a series of 16 cases. In: Oral surgery, oral medicine, oral pathology and oral radiology. 2015 ; Vol. 120, No. 6. pp. e240-e246.
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abstract = "Objectives To investigate the radiographic findings of mucopolysaccharidoses (MPS) on panoramic radiographs in a series of 16 patients. Study Design This retrospective cross-sectional study included panoramic radiographs of 16 patients with cytogenetically diagnosed MPS. Two blinded, previously calibrated observers evaluate the presence or absence of the following features: type of dentition; stage of dental age in comparison with chronologic age; delayed exfoliation of deciduous teeth; supernumerary teeth; teeth impaction; enlarged, cyst-like dental crypt; hypercementosis; taurodontism; generalized enamel hypoplasia; enlargement of the bone marrow spaces; thinning of cortical bone; unusual morphology of condyles; and flattening of the condylar head. Results The final sample was composed of 8 MPS IV, 7 MPS VI, and 1 MPS I cases. All patients presented unusual morphology of condyles. Cyst-like dental crypt (75{\%}), taurodontism, and teeth impaction (68.75{\%} each) were also overall common findings. Generalized enamel hypoplasia was exclusively identified in patients with MPS IV (75{\%} of those patients). Conclusions Radiographic features of oral and maxillofacial manifestations in patients with MPS frequently encountered by dentists may help recognize the disorder. However, because of sample size limitations, it was not possible to infer any statistical relationship between the radiographic features and the types of MPS.",
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AU - Diniz, Denise Nóbrega

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