Purpose/Aim: The aim of this study was to explore whether dentinogenesis imperfecta (DGI)-related aberrations are detectable in odontogenic tissues. Materials and Methods: Morphological and histological analyses were carried out on 3 teeth (two maxillary 1st molars, one maxillary central incisor) extracted from a patient with DGI Type II. A maxillary 2nd molar teeth extracted from a healthy patient was used as control. A micro-computed tomographic (μCT) data-acquisition system was used to scan and reconstruct samples. Pentachrome and picrosirius red histologic stains were used to analyze odontogenic tissues and their collagenous matrices. Results: Our findings corroborate DGI effects on molar and incisor root elongation, and the hypo-mineralized state of DGI dentin. In addition to these findings, we discovered changes to the DGI pulp cavity: Reactionary dentin formation, which we theorize is exacerbated by the early loss of enamel, nearly obliterated an acellular but still-vascularized DGI pulp cavity. We also discovered an accumulation of lamellated cellular cementum at the root apices, which we hypothesize compensates for the severe and rapid attrition of the DGI tooth. Conclusions: Based on imaging and histological data, we propose a novel hypothesis to explain the complex dental phenotypes observed in patients with DGI Type II.
Keywords: DSPP gene; Dentinogenesis imperfecta; enamel loss; pulp obliteration; reactionary dentin.