Imperfecta Dentinogenesis: a case report

Abstract

Summary: Introduction: Dentinogenesis imperfecta (DI) is an inherited dental diasease
in which dental crowns are affected. DI originates by defects on the histo-differenciation stage of
odontogenesis, constituting a localized mesodermal dysplasia characterized by an expressed
alteration 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 history
and clinical characteristics of a patient within extensive coronal destruction, to establish a
precise diagnosis and a treatment to recover masticatory function. Case Report: A 3-year-old male
child was referred to the clinic with dental hypersensitivity and pain during mastication. He
presented 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, family
history and consultation with a Geneticist, a relation with OI was not established. In contrast, a
diagnosis of dentinogenesis imperfecta type II was confirmed. DE type II is an autosomyc dominant
trait, without gender predilection. Restorative treatment involved stainless steel crowns, required
extractions, space maintainers and removable partial dentures placement. Conclusion: Early diagnosis
and treatment of DI is recommended because the severe destruction of primary dentition that may
affect child chewing and nutrition. Consultation wit a Geneticist in DI cases is reccomended to
discard any association with OI or other genetic syndromes. Key words: Dentinogenesis imperfecta.
Odontogenesis imperfecta. Opalescent dentin. Hereditary opalescent dentin.