Dental Follow-Ups of Children With Mucopolysaccharidoses (MPS): a Cohort Study

Objectives: Mucopolysaccharidoses (MPS) represent a rare group of inherited metabolic disorders categorized under lysosomal storage disorders (LSD), characterized by systemic manifestations that affect multiple organs. These conditions arise from the absence or dysfunction of specific lysosomal enzymes required for glycosaminoglycan (GAG) degradation. Seven distinct clinical types and various subtypes of MPS have been identified. This study evaluates the dental health and caries progression over three years in a cohort of children with MPS.
Methods: Ethical approval was obtained from the Clinical Research Ethics Committee of Marmara University School of Dentistry (No: 2020-451). Initially, 40 pediatric patients treated at the Metabolic Diseases Clinic of Marmara University Medical School were invited for dental evaluations. Data collection included demographic information, oral examinations, DMFT/dft indices, and panoramic radiographs when feasible. 25 patients had attended the initial evaluation, while after three years, only 17 (aged 4-16 years at baseline) returned for follow ups.
Results: The three-year change in caries indices was -2.06 ± 4.28 for dft and +1.47 ± 2.42 for DMFT. Tooth impaction was observed in 58.3% of cases and generalized enamel hypoplasia in 41.7%, and anterior open bite in 35.3%. All Type 1 and Type 6 patients in the study were males. Delayed tooth eruption was a consistent observation in most cases.
Conclusions: The results indicate that children with MPS exhibit elevated oral health care needs, compounded by the complexities of their systemic condition. Compliance with dental care is low, partly due to the burden of managing multiple comorbidities and the need for regular attendance at metabolism clinics for enzyme replacement therapy or bone marrow transplantation. These challenges emphasize the importance of integrating routine dental evaluations, preventive strategies, and timely interventions into their multidisciplinary healthcare regimen to address oral health challenges.