Imaging Sci Dent.  2017 Jun;47(2):129-133. 10.5624/isd.2017.47.2.129.

Dentinogenesis imperfecta type II: A case report with 17 years of follow-up

• Gama FJ
• Corrêa IS
• Valerio CS
• Ferreira E
• Manzi FR

• KMID: 2389894
• DOI: http://doi.org/10.5624/isd.2017.47.2.129

Abstract

Dentinogenesis imperfecta is a dominant autosomal hereditary disorder of dentin formation that affects the deciduous and permanent teeth. Its etiology is characterized by inadequate cell differentiation during odontogenesis. The clinical characteristics of dentinogenesis imperfecta are discolored teeth with a translucency that varies from gray to brown or amber. Radiographically, the teeth exhibit pulp obliteration, thin and short roots, bell-shaped crowns, and periapical bone rarefaction. The aim of this report was to present a case of dentinogenesis imperfecta type II that was followed up over a 17-year period. This report also presents scanning electron microscopy images of the enamel and dentin, showing that both were altered in the affected teeth. The disease characteristics and the treatments that were administered are reported in this study to guide dentists with respect to the need for early diagnosis and adequate follow-up to avoid major sequelae.

Keyword

Dentinogenesis Imperfecta; Dental Enamel; Dentin; Microscopy, Electron, Scanning

MeSH Terms

Amber
Cell Differentiation
Crowns
Dental Enamel
Dentin
Dentinogenesis Imperfecta*
Dentinogenesis*
Dentists
Early Diagnosis
Follow-Up Studies*
Humans
Microscopy, Electron, Scanning
Odontogenesis
Tooth
Amber

Figure

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  Fig. 1 Pedigree of dentinogenesis imperfecta in the patient's family medical record, covering 3 generations.

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  Fig. 2 Radiographic images of the 7-year-old patient. Temporary stainless steel crowns are present in the permanent first molars, and direct composite resin restorations are present in the primary mandibular molars.

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  Fig. 3 Panoramic radiograph of the patient at 20 years of age, showing obliterated pulp chambers, shortened crowns restored with resin, and thin roots with constriction in the cervical region.

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  Fig. 4 Scanning electron microscopy images (×1000) show the enamel and dentin of both a normal tooth and a tooth affected by dentinogenesis imperfecta. A. Parallelism of the prismatic enamel crystals can be observed. B. Absence of parallelism in the prismatic enamel crystals is found. C. Normal dentin structure with a tubular direction throughout its entire structure is found. D. Affected dentin structure with the obliteration and reduction of the quantity of dentin tubules is seen.

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