Comparison of Long-term Prognosis in Siblings with Dentinogenesis Imperfecta depending on the Timing of the Treatment Intervention : Case Reports

Author: Gimin KIM ¹ ; Jaesik LEE
Author Information

1. Department of Pediatric Dentistry, School of Dentistry, Kyungpook National University
Publication Type:3
From: Journal of Korean Academy of Pediatric Dentistry 2021;48(2):237-244
CountryRepublic of Korea
Language:Korean
Abstract: Dentinogenesis imperfecta (DI) is a hereditary disorder of dentinal defect. It is generally inherited as a single autosomal dominant trait. DI usually affects both the primary and permanent dentition. Affected teeth have various types of discolorations, rapid destruction of the dentin, and severe attrition. In radiologic view, the affected teeth have bulbous crowns, short roots and narrow or closed pulp chambers. The treatment objective is to prevent additional attrition and recover the vertical dimension of occlusion.The aim of this report was to present the long-term prognosis in 15 years in a pair of siblings. Both the patients had DI with tooth attrition and discoloration. Different treatment procedures were used, depending on the difference in the timing of intervention. The first patient saved most of his teeth. The second patient had all of her teeth extracted. This report could be helpful for early diagnosis and overall treatment of DI.