Les maladies rares et leurs manifestations cliniques orales dans deux formations hospitalières de Yaoundé

INTRODUCTION: Rare diseases have, in certain cases, oral manifestations. These are often understudied; this helps to limit oral care services to this category of patients. This study aims to determine the epidemiology and clinical features of oral manifestations in patients with rare diseases in our environment. METHODS: We conducted a cross-sectional descriptive study in two referral hospitals in Yaoundé over a period of 7 months. Data were collected from patients' medical records, parents' or guardians' interview and oral examination. The threshold significance level was p< 0.05. RESULTS: Oral manifestations associated with rare diseases vary based on the group of conditions, teeth and often have functional consequences. Functional impairment was diagnosed in 97.2% of cases. Patients with deciduous dentition (59.4%) most commonly developed anomalies of tooth shape and position (conicity 7/22 cases, 32%) while patients with permanent teeth most commonly developed carious lesions (7/10 cases, 70%) and anomalies in tooth structure (4/10 cases with dental wear). A significant link between type of tooth, anomalies in tooth structure (p=0.001) and tooth numbers was found (p=0.018). Mastication problems (p=0.023) as well as suction problems (p=0.033) were linked to rare disease patient groups. CONCLUSION: Oral lesions in patients with rare diseases, although present in our environment, are often neglected. Oral care should be integrated within the basic package of oral health care trainings after the establishment of free or affordable health care for patients with disabilities or with specific needs.