Display options
Share it on

Int J Paediatr Dent. 2021 Dec 30; doi: 10.1111/ipd.12952. Epub 2021 Dec 30.

Condyle morphology among patients with mucopolysaccharidosis: an observational study of panoramic radiographs.

International journal of paediatric dentistry

Carmen Ulrike Schmid-Herrmann, Nicole Muschol, Vera Fuhrmann, Anja Friederike Koehn, Susanne Lezius, Bärbel Kahl-Nieke, Till Koehne

Affiliations

  1. Department of Orthodontics, Center for Dental and Oral Medicine, University Medical Center Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Germany.
  2. Department of Pediatrics, University Medical Center Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Germany.
  3. Department of Medical Biometry and Epidemiology, University Medical Center Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Germany.
  4. Department of Orthodontics, University Medical Center Leipzig, Liebigstrasse 12, 04103, Leipzig, Germany.

PMID: 34967064 DOI: 10.1111/ipd.12952

Abstract

BACKGROUND: Mucopolysaccharidoses (MPS) are a group of rare metabolic diseases characterized by a wide spectrum of symptoms including progressive condylar resorption.

AIM: The aim was to quantify the severity of condylar involvement in MPS I individuals in comparison to 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 (grade 0 to 3) and using the ratio of condylar height to ramus height (CH:RH ratio).

RESULTS: All MPS I and MPS VI individuals showed pronounced bilateral degenerative condylar resorption. In contrast, individuals with MPS II and MPS IV exhibited heterogeneous findings. 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<0.001). In contrast, the CH:RH ratios of MPS II and MPS IV showed great variability.

CONCLUSIONS: MPS subtypes differ with regard to the severity of condylar resorption.

This article is protected by copyright. All rights reserved.

Keywords: Bone Resorption; Dental care for the disabled; Lysosomal Storage Diseases; Mandibular Condyle; Mucopolysaccharidosis; Osteoarthritis; Rare disease

Publication Types