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Abstract

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.