Carmen Ulrike Schmid-Herrmann1, Nicole Maria Muschol2, Vera Ulrike Fuhrmann1, Anja Friederike Koehn2, Susanne Lezius3, Bärbel Kahl-Nieke1, Till Koehne4. 1. Department of Orthodontics, Center for Dental and Oral Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. 2. Department of Pediatrics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. 3. Department of Medical Biometry and Epidemiology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. 4. Department of Orthodontics, University Medical Center Leipzig, Leipzig, Germany.
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 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.
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.
Keywords:
bone resorption; dental care for the disabled; lysosomal storage diseases; mandibular sondyle; mucopolysaccharidosis; osteoarthritis; rare disease