Type I Dentinal Dysplasia—Proposal of A New Classification System Combined with A Variant Case Presentation

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Dentine dysplasia is an extremely rare autosomal dominant hereditary condition primarily characterized by defective dentine formation affecting both the deciduous and permanent dentitions. The aetiology of dentinal dysplasia remains imprecise to date, in spite of the numerous hypotheses put forward by various authors. Also, of late, new case reports of type I dentine dysplasias are emerging with clinical and radiographic fi ndings which are unique and variant from the classical characteristic features of this anomaly, rendering it diffi cult to fi t into the previously established classifi cation systems. In this article, after a thorough review and understanding of the drawbacks of the previous classifi cation systems and the cumulative fi ndings from the published case reports in literature, we propose a new classifi cation system for dentine dysplasia type I. We also present a case report which exhibited an absence of familial hereditary pattern, the absence of periradicular radiolucent lesions and osseous pathologies as well as atypical morphological defects of the molar roots which were diverse from the classical fi ndings of the various sub types of dentine dysplasia type I reported to date. Early diagnosis and implementation of preventive and curative therapy is crucial for avoiding premature exfoliation of deciduous and permanent dentition and associated residual ridge resorption, thereby overcoming functional and aesthetic defi cits. The new classifi cation proposed in this article will help clinicians to diagnose and categorize the patients effectively, facilitating proper treatment and supportive care for all.