Introduction

Dentinogenesis imperfect (DI) is an inherited condition originated in the histodifferentiation stage during odontogenesis, constituting a localized mesodermal dysplasia form, characterized by an expressed alteration of dentin proteins. There are three types of dentinogenesis imperfect: Type I (associated with osteogenesis imperfect Ol), type II (not associated with Ol), and type III (Brandywine's).

Objectives

To determine the type of dentinogenesis, family history trend and clinical characteristics of the patient, to establish early diagnosis and heredi tary pattern.

Case report

A 3-year-old boy patient was referred to the clinic with dental hypersensitivity and pain during mastication. He presented a structure anomaly 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 Genetics, there was no relation with Ol. A diagnosis of dentinogenesis imperfect type II was made, transmitted as an autosomal dominant trait, without gender predilection. Restorative treatment involved stainless steel crowns, extractions, space maintainer and removable partial denture placement.

Conclusion

Early diagnosis and treatment of DI is recommended because of the severe alterations of primary dentition. In this kind of patients it is very important to carry out consultations with Genetics to discard any association with other disorders.

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