Dentinogenesis Imperfecta Associated with Osteogenesis Imperfecta in Children: phenotypic features and Dental Management
Keywords:
N\AAbstract
Dentinogenesis Imperfecta (DI) is a hereditary dental condition commonly associated with Osteogenesis Imperfecta (OI), a genetic connective tissue disorder characterized by bone fragility and skeletal deformities. This article presents a case report of a child diagnosed with OI Type I and concurrent DI, highlighting the clinical, radiographic, and histological features that support the diagnosis. The patient exhibited classical signs of DI, including brown-grey opalescent teeth, generalized attrition, crossbite, and significant enamel chipping. Imaging revealed bulbous crowns, large pulp chambers, and a hypoplastic maxilla consistent with DI Type I. A comprehensive dental management plan involving preventive care, restorative treatment, and orthodontic planning was implemented to preserve oral function and aesthetics. This case reinforces the importance of recognizing dental anomalies as phenotypic indicators of systemic conditions and underscores the need for an interdisciplinary approach in managing children with OI and associated orodental manifestations.
Downloads
Metrics
References
Trejo P, Rauch F. Osteogenesis imperfecta in children and adolescents. Osteoporos Int. 2016;27:427–37.
Gajko-Galicka A. Mutations in type I collagen genes resulting in osteogenesis imperfecta. Acta Biochim Pol. 2002;49(2):433-41.
Forlino A, Marini JC. Osteogenesis imperfecta. Lancet. 2016;387:1657–71.
Malmgren B, Lindskog S. Assessment of dysplastic dentin in OI and DI. Acta Odontol Scand. 2003;61:72–80.
Majorana A, Bardellini E, Brunelli PC, Lacaita M, Cazzolla AP, Favia G. Dentinogenesis imperfecta in children with osteogenesis imperfecta: a clinical and morphological study. Int J Paediatr Dent. 2010;20(2):112–118.
Malmgren B, Norgren S. Dental aberrations in children and adolescents with osteogenesis imperfecta. Acta Odontol Scand. 2002;60(2):65–71.
Renders GA, Lafferty KA, O’Connell AC. Bisphosphonate treatment in children with osteogenesis imperfecta: dental findings. Pediatr Dent. 2007;29(5):409–413.
Tolar J, Teitelbaum SL, Orchard PJ. Osteogenesis imperfecta: translating mechanism to therapy. J Bone Miner Res. 2015;30(6):1027–1036.
Forlino A, et al. New perspectives on osteogenesis imperfecta. Nat Rev Endocrinol. 2011;7:540–57.
Rizkallah J, et al. Evaluation of malocclusion in children with osteogenesis imperfecta. Am J Orthod Dentofac Orthop. 2012;143:336–41.
Downloads
Published
How to Cite
Issue
Section
License

This work is licensed under a Creative Commons Attribution 4.0 International License.
You are free to:
- Share — copy and redistribute the material in any medium or format
- Adapt — remix, transform, and build upon the material for any purpose, even commercially.
Terms:
- Attribution — You must give appropriate credit, provide a link to the license, and indicate if changes were made. You may do so in any reasonable manner, but not in any way that suggests the licensor endorses you or your use.
- No additional restrictions — You may not apply legal terms or technological measures that legally restrict others from doing anything the license permits.