MUCOPOLYSACCHARIDOSIS - MPS-VI AND ASSOCIATED DELAYED TOOTH ERUPTION

Eight cases of mucopolysaccharidosis Type VI (Maroteaux-Lamy syndrome) are reviewed and two cases are presented in detail. Developmental den tal anomalies including unerupted and impacted permanent teeth and ass ociated hyperplastic tooth follicles are seen frequently in MPS patien ts. The surgical implications and management are discussed. All patien ts reviewed had significant cardiovalvular disease. It is essential fo r the primary medical provider to establish early basic dental care an d evaluation for delayed eruption of primary and permanent dentition. This will probably minimize the possibility of infective endocarditis and allow for early treatment of impacted teeth.