Oro-Dental Management of a Recessive Case of Thévenard Disease in Childhood: A Case Report

Introduction: Thévenard disease, or hereditary sensory neuropathy type 1, is a rare genetic disorder characterized by sensory loss, recurrent ulcerations, and progressive osteoarticular destruction. While systemic manifestations are well described, oral involvement—especially in recessive forms-remains poorly understood. This article aims to report the oral features of a child with recessive Thévenard disease and to outline specific challenges in dental management. Case Presentation: A 7-year-old female with recessive Thévenard disease was referred for management of a dental infection. Medical history revealed early-onset febrile episodes with neuropathic pain, chronic cutaneous ulcers, and osteoarticular deformities. Oral examination identified mandibular deviation, incisor-molar hypomineralization (MIH), severe tooth wear, and necrosis of mandibular molars associated with cellulitis. Radiographs showed delayed root maturation and multiple pulp and periapical lesions. Dental management included extractions, preventive, and restorative treatments, pulpotomy, and selective occlusal adjustments. A removable partial denture was fabricated to restore occlusion, and an anterior crossbite was later corrected using a composite inclined plane. The patient was followed for one year, with stable outcomes and improved oral function. Discussion: This case highlights the potential oral complications of Thévenard disease and the need for individualized, multidisciplinary care. Dental management must integrate preventive strategies, behavior guidance, and careful monitoring due to altered pain perception, infection risk, and psychological factors. Early recognition of oral manifestations may improve quality of life and reduce morbidity in affected patients.