Dental management of a patient with LRP6 gene mutation and oligodontia: case report

ABSTRACT Introduction: Oligodontia is a rare dental anomaly defined as the congenital absence of six or more permanent teeth (excluding third molars). It has significant clinical consequences for mastication, phonation, aesthetics, and psychosocial well-being. Genetic factors are central to its etiology, with common mutations involving MSX1, PAX9, WNT10A, AXIN2, and EDAR. Mutations in the LRP6 gene (low-density lipoprotein receptor-related protein 6, OMIM *603507), however, are very rare. LRP6 encodes a coreceptor of the WNT/β-catenin pathway, essential for craniofacial and dental development. Case report: We report on a patient (12 years and 10 months) with the LRP6 gene 3–11 deletion associated with selective tooth agenesis type 7, comprising teeth 12, 15, 22, 25, 31, 32, and 41. In this case, the first phase of treatment included prosthetic rehabilitation of the edentulous area of the mandible using a removable resin prosthesis. After permanent eruption, fixed prosthetic rehabilitation and ameloplasty of the upper canines will be scheduled. Conclusion: This case emphasizes the importance of early diagnosis, regular follow-up, and multidisciplinary dental treatment in patient with oligodontia.