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Mucopolysaccharidosis: MPS VI and associated delayed tooth eruption
K S Smith1, K B Hallett, R K Hall
1Department of Plastic and Maxillofacial Surgery, Royal Children's Hospital, Melbourne, Australia.
International Journal of Oral and Maxillofacial Surgery
|April 1, 1995
Summary
Mucopolysaccharidosis Type VI (MPS VI) patients frequently exhibit dental anomalies like impacted teeth. Early dental care is crucial for managing these issues and preventing serious complications such as infective endocarditis.
Area of Science:
- Dentistry
- Genetics
- Pediatrics
Background:
- Mucopolysaccharidosis Type VI (Maroteaux-Lamy syndrome) is a rare genetic disorder.
- Patients with MPS VI often present with complex medical and dental challenges.
Observation:
- Developmental dental anomalies are common in MPS VI, including unerupted and impacted permanent teeth.
- Hyperplastic tooth follicles frequently accompany these dental abnormalities.
Findings:
- This review analyzes eight cases of MPS VI, with two detailed case presentations.
- Significant cardiovalvular disease was a consistent finding across all reviewed patients.
Implications:
- Early dental evaluation and basic care are essential for MPS VI patients.
- Proactive management of delayed dentition can minimize risks of infective endocarditis.
- Timely treatment of impacted teeth is critical for patient outcomes.