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.