Review Article

Dentinogenesis imperfecta: An update

Efthimia Tsoukala¹, Georgia Chochtoula¹, Iosifina Theodorou¹, George I. Lambrou^1,2,3

1. Postgraduate Program “Metabolic Bone Diseases”, National and Kapodistrian University of Athens, Medical School, Athens, Greece
2. Laboratory for the Research of the Musculoskeletal System “Th. Garofalidis”, National and Kapodistrian University of Athens, Medical School, Athens, Greece
3. Choremeio Research Laboratory, First Department of Pediatrics, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece, Athens, Greece

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Keywords: Amelogenesis, Dentin, Dentinogenesis, Metabolic disease, Osteogenesis

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Abstract

The term Dentinogenesis Imperfecta refers to diseases that affect the dental tissue, consisting of enamel or dentin. The disease leads to insufficient quantitative or qualitative enamel and dentin tissue formation, leading to Amelogenesis Imperfecta or Dentinogenesis Imperfecta, respectively. These diseases have a similar clinical appearance and their differential diagnosis requires good knowledge of their characteristics. They are hereditary, have a great diversity in clinical, radiological and histological appearance and require a long-term and combined treatment plan. Finally, they are often found as part of syndromes and in-depth understanding of their characteristics can help in the early diagnosis of these congenital disorders. A very common metabolic disease, in which incomplete dentinogenesis is found as a subsequent finding in more than 50% of patients, is Osteogenesis Imperfecta. In the present work, we have attempted, to review the latest data on Dentinogenesis Imperfecta, thus enabling a specialist clinician to more easily diagnose and treat these patients.

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