Summary: Introduction: Dentinogenesis imperfecta (DI) is an inherited dental diaseasein which dental crowns are affected. DI originates by defects on the histo-differenciation stage ofodontogenesis, constituting a localized mesodermal dysplasia characterized by an expressedalteration of dentin proteins. There are three types of dentinogenesis imperfecta: Type I(associated with osteogenesis imperfecta OI), type II (no associated with OI), and type III(Brandywine’s). Objectives: To determine the type of dentinogenesis imprefecta, the family historyand clinical characteristics of a patient within extensive coronal destruction, to establish aprecise diagnosis and a treatment to recover masticatory function. Case Report: A 3-year-old malechild was referred to the clinic with dental hypersensitivity and pain during mastication. Hepresented generalized and extensive crown destructions and discoloration of erupted teeth,abscesses, attrition and loss of vertical dimension. Radiographs showed obliterated pulp chambers,marked cervical constriction and short roots. After clinical and radiographic analysis, familyhistory and consultation with a Geneticist, a relation with OI was not established. In contrast, adiagnosis of dentinogenesis imperfecta type II was confirmed. DE type II is an autosomyc dominanttrait, without gender predilection. Restorative treatment involved stainless steel crowns, requiredextractions, space maintainers and removable partial dentures placement. Conclusion: Early diagnosisand treatment of DI is recommended because the severe destruction of primary dentition that mayaffect child chewing and nutrition. Consultation wit a Geneticist in DI cases is reccomended todiscard any association with OI or other genetic syndromes. Key words: Dentinogenesis imperfecta.Odontogenesis imperfecta. Opalescent dentin. Hereditary opalescent dentin.
Bilateral cracked permanent first molars in dentinogenesis imperfecta type II: A case report