Dentinogenesis imperfecta type I, case report

Abstract

Introduction: Dentinogenesis imperfecta (DI) is a rare autosomal dominant hereditary condition that affects both dentitions. It is characterized by translucent teeth, bluish-gray or brown in color, prone to fracturing, with normal enamel but defective dentin. Radiographically, bulbous crowns, marked cervical constriction,  short and thin roots are observed. DI type I is an oral anomaly associated with Osteogenesis imperfecta (OI) type IV. Objective: To describe the clinical and radiographic characteristics of DI type I to facilitate its diagnosis and treatment. Case report: A 12-year-old female patient, diagnosed via genetic study with OI type IV and DI type I, attends the Pediatric Dentistry postgraduate clinic for comprehensive treatment. Clinically, normal sclera, normal to low stature, overweight, late mixed dentition, translucent-opalescent teeth with bluish-gray-brown coloration,tooth wear, enamel fractures, glassy dentin, infraoccluded teeth, extruded antagonists and dentomaxillary anomaly were observed. Radiographically, bulbous crowns, marked cervical constriction, obliterated pulp chambers and root canals, short and thin roots of primary teeth and dental agenesis were evident. Conclusions: Due to its low prevalence, both the diagnosis and clinical management of DI pose a challenge. Early recognition of its characteristics will facilitate and enhance management in pediatric dental practice