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Clinical Oral Investigations

, Volume 22, Issue 3, pp 1541–1549 | Cite as

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

  • Till Koehne
  • Anja Köhn
  • Reinhard E. Friedrich
  • Uwe Kordes
  • Thorsten Schinke
  • Nicole Muschol
  • Bärbel Kahl-Nieke
Original Article
  • 205 Downloads

Abstract

Objective

The aims of this study were to analyze the maxillomandibular morphology of patients with mucopolysaccharidosis (MPS) type I, II, III, IVa and VI and to evaluate the craniofacial effect of hematopoietic stem cell transplantation (HCST) in MPS I.

Materials and methods

One hundred head magnetic resonance images were retrospectively analyzed from 41 MPS and 27 control individuals. The width, height and length of the maxilla and mandible were plotted against age and the means of controls, MPS I, MPS II and MPS III were statistically compared. To determine the effect of HSCT in MPS I, jaw morphology was compared between MPS I patients with full donor chimerism versus patients with mixed/no donor chimerism.

Results

Maxillary dimensions were not statistically different between the MPS types. The height and length of the mandible were clearly smaller in MPS I as compared to those in controls, MPS II and MPS III. This was associated with progressive resorption of the mandibular condyles in MPS I, which was also observed in MPS II and VI, but not in MPS III or IVa. Whereas the success of HCST did not affect these changes, mandibular width was significantly smaller in MPS I individuals with full donor chimerism.

Conclusion

MPS I individuals have a smaller mandible as compared to control, MPS II and MPS III individuals due to progressive condylar degeneration. These abnormalities are also evident following successful HSCT.

Clinical relevance

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

Keywords

Mucopolysaccharidosis MRI Craniofacial Cephalometry OSAS Hematopoetic stem cell transplantation 

Notes

Funding

This research received no specific grant from any funding agency.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethics approval

All analyses were performed retrospectively on routinely acquired images and all data were fully anonymized before analysis. Therefore, in accordance with the federal hospital laws (Hamburgisches Krankenhausgesetz) no approval by the local ethics committee was necessary.

Informed consent

For this type of study formal consent is not required.

Supplementary material

784_2017_2240_MOESM1_ESM.pdf (11.7 mb)
ESM 1 (PDF 11934 kb).

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Copyright information

© Springer-Verlag GmbH Germany 2017

Authors and Affiliations

  • Till Koehne
    • 1
  • Anja Köhn
    • 2
  • Reinhard E. Friedrich
    • 3
  • Uwe Kordes
    • 4
  • Thorsten Schinke
    • 5
  • Nicole Muschol
    • 2
  • Bärbel Kahl-Nieke
    • 1
  1. 1.Department of OrthodonticsUniversity Medical Center Hamburg-EppendorfHamburgGermany
  2. 2.Department of PediatricsUniversity Medical Center Hamburg-EppendorfHamburgGermany
  3. 3.Department of Oral and Maxillofacial SurgeryUniversity Medical Center Hamburg-EppendorfHamburgGermany
  4. 4.Department of Pediatric Hematology and OncologyUniversity Medical Center Hamburg-EppendorfHamburgGermany
  5. 5.Department of Osteology and BiomechanicsUniversity Medical Center Hamburg-EppendorfHamburgGermany

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