Oral health status in patients with mucopolysaccharidoses

Özet

The aim of this study is to assess oral clinical and radiological findingsof patients with mucopolysaccharidoses (MPS) which are a group of rareinherited disorders of glycosaminoglycan catabolism. In this retrospective anddescriptive study, records of all patients with MPS over 3 years of age managedat Division of Pediatric Metabolism, who were all referred to Department ofPediatric Dentistry for evaluation of their oral health status were assessed.Oral examinations were performed in 30 patients with determination ofDMFT/S, dmft/s, dental plaque and gingival indices and panoramic radiologicalassessment was performed in 12 patients. Twenty-seven (90.0%) patientshad dental caries and 19/21 (90.5%) had gingival inflammation. Radiologicalexamination in 12 patients with panoramic image showed short mandibularramus (100.0%), enlarged dental follicles (83.3%), taurodontism (75.0%),flat mandibular notch (66.7%). In association with the bone structure andmorphology of the jaws, 11 patients (91.7%) had trabecular variation similarto osteoporotic disease. Condylar defect was observed in all patients, whichmight lead to temporomandibular joint disorders. Pulp chamber with threehorns, which had never been reported in MPS before, was observed inpanoramic images of 2 of 7 patients with MPS-VI. Oral clinical and radiologicalchanges are very common in this population of MPS patients. These changesmay impair opening of the mouth, eating and chewing and thus affect theirquality of life. Regular dental counseling, preventive applications and dentaltreatments must be included in multidisciplinary team approach of MPSpatients to provide better oral health.