Bucomaxillofacial Manifestations of Maroteaux-Lamy Syndrome and Its Relationship with Dentigerous Cyst: An Integrative Literature Review

Abstract

Introduction: Maroteaux-Lamy Syndrome (MPS VI) is a rare genetic disorder characterized by the accumulation of glycosaminoglycans in tissues, leading to various systemic and orofacial manifestations such as macroglossia, gingival hypertrophy, and malocclusions. The occurrence of dentigerous cysts, although rare, is significant in some patients with MPS VI, requiring special attention in diagnosis and treatment. Objective: To evaluate the main bucomaxillofacial manifestations in patients with MPS VI and their relationship with the presence of dentigerous cysts, analyzing the frequency and impact of these alterations. Methods: An integrative literature review was conducted following PRISMA guidelines in the PubMed, SciELO, and BVS databases, using the keywords "Mucopolysaccharidosis VI" and "Dentigerous Cyst." Studies in English and Portuguese without time restrictions were included, focusing on case reports, cohort studies, and clinical trials. Five studies met the inclusion criteria. Results and Discussion: Most studies identified macroglossia, gingival hypertrophy, impacted teeth, and malocclusions in patients with MPS VI. In two cases, multiple dentigerous cysts were reported, indicating a possible correlation with the disease. Cone-beam computed tomography (CBCT) proved valuable in accurately identifying these lesions. Conclusion: Orofacial manifestations in MPS VI are extensive and significantly impact patients' quality of life. The presence of dentigerous cysts, though not common to all cases, presents an additional challenge in clinical management. The variability in the clinical expression of the syndrome suggests the need for further studies to better understand the prevalence of dentigerous cysts and optimize treatment for these patients.