Dental and maxillomandibular incidental findings in panoramic radiography among individuals with mucopolysaccharidosis: a cross-sectional study

Abstract Mucopolysaccharidosis (MPS) is a group of rare and inherited metabolic disorders caused by the accumulation of macromolecule glycosaminoglycans inside lysosomes. Affected individuals may have dental and craniofacial tissue alterations, facilitating the development of several oral diseases. Objectives To assess, with panoramic radiographic images, the frequency of dental and maxillomandibular incidental findings among MPS individuals and compare them with non-MPS individuals. Methodology A cross-sectional study evaluating a sample of 14 MPS individuals and 28 non-MPS individuals aged from 5 to 26 years was carried out. They were matched for sex and age on a 2:1 proportion. Panoramic radiographs were assessed for the presence/absence of the following dental and maxillomandibular alterations: dental anomalies of number (hypodontia/dental agenesis, supernumerary teeth); anomalies of form (microdontia, macrodontia, conoid teeth, taurodontism, and root dilaceration); anomalies of position (impacted tooth, inverted tooth, tooth migration, partially bony teeth, complete bony teeth); periapical alterations (furcation lesion, circumscribed bone rarefaction); other alterations (radiolucent bone lesions, radiopaque bone lesions, radiopacity in the maxillary sinus, condylar hypoplasia). Differences between groups were tested by the Fisher’s exact test and chi-square test (p<0.05). Results For intrarater agreement, Kappa values were 0.76 to 0.85. The presence of supernumerary teeth (p=0.003); conoid teeth (p=0.009); taurodontism (p<0.001); impacted teeth (p<0.001); partial bony teeth (p=0.040); complete bony teeth (p=0.013); and root dilaceration (p=0.047) were statistically more frequent in MPS individuals compared to non-MPS individuals. Bone rarefaction/furcation lesions (p=0.032), condylar hypoplasia (p<0.001), radiolucent bone lesions (p=0.001), and dentigerous cysts (p=0.002) were also more frequent in MPS individuals. Conclusion The presence of specific oral manifestations is more common in MPS individuals than non-MPS individuals.

lesions, and condylar defects in individuals with MPS as typical. [5][6][7][8] MPS individuals are also a group with a higher vulnerability to dental caries, therefore, requiring better guidance and access to preventive oral health programs. 9,10 It has been stated in the literature that the presence of dental and/or maxillomandibular anomalies can be considered a hampering factor for some dental treatments, such as root canal therapy, or it may also cause pain, sensitivity or aesthetic problems to patients. 11  A single-blinded radiologist with over 10 years of experience performed the interpretation of the panoramic radiographs. The rater was previously calibrated for data collection. The panoramic radiographs of 15 non-MPS individuals were examined, for the calibration process. They were re-examined two weeks later for the intra-rater agreement estimation.
Those radiographs were not included in the main study.
Before the main study, a pilot study was also

Results
For the intra-rater agreement, Kappa values obtained ranging from 0.76 to 0.85 were very good. 14 Among the 29 MPS individuals who were assessed regarding eligibility, 14 were included and evaluated. Regarding the type of MPS, the individuals were classified as follows: MPS I (n=4), MPS II (n=2), MPS IV (n=1) and MPS VI (n=7). No individuals with MPS type III and IX were identified. Figure 1 and Figure 2 shows the panoramic radiographs of individuals with    Radical examinations, such as panoramic radiographs, are still considered a significant dental screening tool. Radiographic equipment is easy to use, provides cost-effective low-dose radiation, and it is used in epidemiological studies to evaluate the presence of dental and maxillomandibular anomalies. 16 Among dental abnormalities, the most relevant radiographic manifestations found in MPS individuals compared to non-MPS individuals were supernumerary tooth, conoid teeth, taurodontism, and impacted   In the present study, the authors suggest diagnosing dentigerous cysts in the studied sample due to the presence of a well-circumscribed, unilocular radiolucency around the crown of an impacted or developing tooth, with their largest dimension ranging from 2.0 cm to 4.0 cm of diameter. However, to confirm a cystic lesion presence, complementary examinations, such as three-dimensional (3D) computed tomography (CT), biopsy, and cytopathologic analysis are recommended. [22][23][24] Generally, dentigerous cysts are asymptomatic and they are usually diagnosed during routine dental care. However, they can become large in some cases, leading to tooth malposition or even causing tooth and bone resorption. The most frequently suggested treatment is the complete surgical enucleation and extraction of the associated impacted tooth or, in some cases, decompression or marsupialization in a developing tooth. 25 Due to some physical or mental impairment inherent to MPS disease, clinicians may face some difficulties in conducting regular dental treatment in these individuals. In some cases, general anesthesia -to perform dental surgical procedures -may be necessary. However, dental practitioners should be aware that airway problems are common in MPS individuals, and the risks imposed during perioperative intubation should be considered. Therefore, in individuals with MPS, preventive and conservative dental treatments should be a priority to avoid some possible systemic injuries. 26.27 In this study, increased condylar hypoplasia  practice with a better understanding of the clinical consequences that might be inherent in this rare disease. Some surgical interventions or orthodontic treatment might be necessary to correct or to minimize dental problems, and the clinicians must be aware of how to perform therapeutic interventions, considering the systemic and physical limitations that these individuals might present.

Maxillomandibular alterations MPS individuals Non-MPS individuals
Some limitations of this study should be highlighted.
The first is inherent to the design of a cross-sectional study. The results presented cannot be used to assess causality. They suggested an increased prevalence of dental alterations among MPS individuals. Secondly, a qualitative assessment of the presence and absence of dentofacial anomalies was performed. The authors suggest that future studies quantifying the extent of those alterations should be performed. Twodimensional panoramic radiographs were used to measure dental and maxillomandibular alterations, and it is known that these exams present geometric distortion and structural superimposition. However, this study can be considered a starting point for future epidemiological studies regarding dental and maxillomandibular features in MPS individuals using a 3D tool. Finally, it is noteworthy that sample size should not be considered a major limitation as MPS has a relatively low overall prevalence, making large sample recruitment quite challenging.

Conclusion
MPS individuals showed an increased frequency of dental and maxillomandibular incidental findings when compared to non-MPS individuals. The increased presence of supernumerary teeth, conoid teeth, taurodontism, impacted teeth, and root dilaceration was observed. The presence of bone rarefaction/ furcation lesions, condylar hypoplasia, and radiolucent bone lesions were also more frequent among MPS individuals.