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Mucopolysaccharidosis: radiographic findings in a series of 16 cases
Dmitry José de Santana Sarmento1, Sérgio Henrique Gonçalves de Carvalho1, Saulo Leonardo Sousa Melo2
1Department of Dentistry, Paraíba State University, Araruna, Paraíba, Brazil.
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.
Insights
Panoramic radiographs revealed common oral and maxillofacial findings in mucopolysaccharidoses (MPS) patients, including condyle abnormalities and cyst-like dental crypts. These dental radiographic features may aid in recognizing MPS disorders.
Area of Science:
- Dentistry
- Medical Imaging
- Genetics
Background:
- Mucopolysaccharidoses (MPS) are rare genetic disorders.
- Oral and maxillofacial manifestations are common in MPS patients.
- Radiographic evaluation is crucial for diagnosis and management.
Purpose of the Study:
- To investigate radiographic findings on panoramic radiographs in patients with mucopolysaccharidoses (MPS).
- To identify common oral and maxillofacial features associated with MPS.
Main Methods:
- Retrospective cross-sectional study of 16 patients with cytogenetically diagnosed MPS.
- Evaluation of panoramic radiographs for specific dental and skeletal features by two blinded observers.
- Assessment included dentition, dental age, tooth impaction, taurodontism, and condyle morphology.
Main Results:
- All 16 patients exhibited unusual condyle morphology.
- Common findings included cyst-like dental crypts (75%), taurodontism, and tooth impaction (68.75% each).
- Generalized enamel hypoplasia was specific to MPS IV (75% of MPS IV patients).
Conclusions:
- Radiographic features in the oral and maxillofacial region are frequent in MPS patients.
- Dentists may recognize MPS through these common radiographic findings.
- Statistical relationships between specific radiographic features and MPS subtypes could not be established due to sample size.
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