Differences in maxillomandibular morphology among patients with mucopolysaccharidoses I, II, III, IV and VI: a retrospective MRI study

Koehne, Till; Köhn, Anja; Friedrich, Reinhard E; Kordes, Uwe; Schinke, Thorsten; Muschol, Nicole; Kahl‐Nieke, Bärbel

doi:10.1007/s00784-017-2240-x

Cited by 7 publications

(23 citation statements)

References 42 publications

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“…In fact, many of the MPS patients develop a severe open bite causing impaired bite function, uneven tooth wear and limited mouth opening. It is therefore important to recognize that the mandibular morphology of MPS patients significantly differs not only as compared to those of individuals without MPS but also among MPS subtypes 14 . This finding is in line with our results, suggesting that condylar resorption is more frequent and more severe in MPS I patients than in those of MPS II and IV individuals.…”

Section: Discussionsupporting

confidence: 90%

“…It is interesting that we observed condylar changes in all MPS I patients irrespective of patients' age, which indicates that this finding has an early onset in MPS I. Indeed, we have previously observed that condylar resorption in MPS I patients can occur from up to 4 years of age 14 . Another relevant finding of our present study is that condylar resorption also exists in the mild MPS I‐Scheie form.…”

Section: Discussionsupporting

confidence: 79%

“…The age of MPS patients ranged from 5 to 37 years. MPS III patients were not included because they do not exhibit skeletal anomalies, 14 and the examination of these patients was extremely difficult because of their limited compliance. The age of the non‐MPS individuals ranged from 10 to 17 years.…”

Section: Resultsmentioning

confidence: 99%

“…The condyles of MPS patients are often described as hypoplastic, flattened or destructed 8–13 . Importantly, these findings should not be considered as a developmental defect, but as the consequence of a progressive condylar resorption due to the accumulation of glycosaminoglycans in the temporomandibular joint 14 . Condylar resorption in MPS patients can cause an extreme vertical growth pattern associated with a class II malocclusion, anterior open bite and limited mouth opening 10,15 …”

Section: Introductionmentioning

confidence: 99%

“…There are several case reports or case series showing condylar resorption in MPS I, 7–9,12,14,16–19 II, 7,10,14,17,20 IV 7,8,13,21 and VI 8,11,17,22–24 patients. To our knowledge, however, there is no quantitative analysis of the severity of condylar changes on panoramic radiographs in MPS I, nor any study comparing panoramic radiographs according to MPS subtypes.…”

Section: Introductionmentioning

confidence: 99%

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Mandibular condyle morphology among patients with mucopolysaccharidosis: An observational study of panoramic radiographs

Schmid-Herrmann

Muschol

Fuhrmann

et al. 2022

Int J Paed Dentistry

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Background: Mucopolysaccharidoses (MPS) are a group of rare metabolic diseases characterized by a wide spectrum of symptoms including progressive condylar resorption. Aim:The aim of this study was to quantify the severity of condylar involvement in MPS I individuals in comparison with a group of non-MPS individuals and to describe how condylar changes may vary among the different types of MPS.Design: Fifty panoramic radiographs of MPS patients (13.4 ± 6.2 years) with MPS I (n = 14), MPS II (n = 2), MPS IV (n = 8) and MPS VI (n = 2) were compared with forty panoramic radiographs of non-MPS individuals. The severity of condylar resorption was evaluated using a qualitative score (grades 0-3) and using the ratio of condylar height to ramus height (CH: RH).Results: All MPS I and VI individuals showed pronounced bilateral degenerative condylar resorption. In contrast, individuals with MPS II and IV exhibited heterogeneous findings. The quantification of condylar height to ramus height revealed that CH: RH was significantly decreased in MPS I as compared to that of non-MPS individuals (P < .001). In contrast, the CH: RH ratios of MPS II and IV showed great variability. Conclusion:Mucopolysaccharidoses subtypes differ with regard to the severity of condylar resorption.

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Section: Discussionsupporting

confidence: 90%

Section: Discussionsupporting

confidence: 79%

Section: Resultsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Mandibular condyle morphology among patients with mucopolysaccharidosis: An observational study of panoramic radiographs

Schmid-Herrmann

Muschol

Fuhrmann

et al. 2022

Int J Paed Dentistry

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Orofacial abnormalities in mucopolysaccharidosis and mucolipidosis typeIIandIII: A systematic review

et al. 2022

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Mucopolysaccharidoses (MPSs) and mucolipidosis II and III (ML II and III) often manifest with orofacial (progressive) abnormalities, which may have a major impact on quality of life. However, because these patients have multiple somatic health issues, orofacial problems are easily overlooked in clinical practice and available literature on this topic solely consists of case reports, small case series, and small cohort studies. The aim of this systematic review was to gain more insight in the nature and extent of orofacial abnormalities in MPS, ML II, and III. A systematic review of all previously published articles addressing orofacial abnormalities in MPS, ML II, and III was performed. Both clinical studies and case reports were included. Outcome was the described orofacial abnormalities, subdivided into abnormalities of the face, maxilla, mandible, soft tissues, teeth, and occlusion. The search resulted in 57 articles, describing orofacial features in 340 patients. Orofacial abnormalities were present in all subtypes of MPS, ML II, and III, and consisted of thickened lips, a hypoplastic midface, a high‐arched palate, hypoplastic condyles, coronoid hyperplasia, macroglossia, gingival hyperplasia, thick dental follicles, dentigerous cysts, misshapen teeth, enamel defects, and open bite. Orofacial abnormalities are present in all subtypes of MPS, ML II, and III. As orofacial abnormalities may cause complaints, evaluation of orofacial health should be part of routine clinical care.

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Examples of Congenitally Acquired Pathology in Pediatric Dental Practice

Aps

2019

Imaging in Pediatric Dental Practice

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Differences in maxillomandibular morphology among patients with mucopolysaccharidoses I, II, III, IV and VI: a retrospective MRI study

Abstract: Clinicians should be aware of specific differences in mandibular morphology and condylar involvement among the MPS subtypes.

Cited by 7 publications

References 42 publications

Mandibular condyle morphology among patients with mucopolysaccharidosis: An observational study of panoramic radiographs

Mandibular condyle morphology among patients with mucopolysaccharidosis: An observational study of panoramic radiographs

Orofacial abnormalities in mucopolysaccharidosis and mucolipidosis typeIIandIII: A systematic review

Examples of Congenitally Acquired Pathology in Pediatric Dental Practice

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