Osteogenesis imperfecta (OI) is a rare genetic disorder characterized by a defect in type I collagen that leads to bone fragility and connective tissue disintegration. The orofacial manifestations of OI include dentinogenesis imperfecta (DI), tooth and jaw malocclusion, and dental anomalies.
This case report shows a four-year-old female patient with OI who presented with brown discolouration typical of DI on all deciduous teeth on oral examination. The teeth were already severely abraded and the bite height was reduced. The patient was symptom-free and practiced optimised oral hygiene.
Patients with these genetic structural anomalies require lifelong, close, interdisciplinary dental care to maintain the results of treatment.
Keywords: osteogenesis imperfecta, dentinogenesis imperfecta, rare genetic disorders
