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References

Kim JW, Simmer JP. Hereditary dentin defects. J Dent Res. 2007; 86:392-399
Rushton MA. A case of dentinal dysplasia. Guy's Hosp Rep. 1939; 89:369-373
Witkop CJ Hereditary defects of dentin. Dent Clin North Am. 1975; 19:25-45
O'Carroll MK, Duncan WK. Dentin dysplasia type I. Radiologic and genetic perspectives in a six-generation family. Oral Surg Oral Med Oral Pathol. 1994; 78:375-381
Melnick M, Levin LS, Brady J. Dentin dysplasia type I: a scanning electron microscopic analysis of the primary dentition. Oral Surg Oral Med Oral Pathol. 1980; 50:335-340
Wesley RK, Wysocki GP, Mintz SM, Jackson J. Dentin dysplasia Type I. Clinical, morphologic, and genetic studies of a case. Oral Surg Oral Med Oral Pathol. 1976; 41:516-524
Wesley RK, Wysocki GP, Mintz Jackson J. Dentin dysplasia type I. Clinical, morphologic, and genetic studies of a case. Oral Surg Oral Med Oral Pathol. 1976; 41:516-524
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Type-1 dentine dysplasia – diagnostic and clinical challenges in restorative management

From Volume 44, Issue 3, March 2017 | Pages 174-180

Authors

Lakshmi Rasaratnam

BDS Lond(Hons), MJDF,

Specialist Registrar, King's College London and William Harvey Hospital, Ashford, Kent

Articles by Lakshmi Rasaratnam

Serpil Djemal

BDS, MSc, MRD, RCS, FDS (Rest dent), RCS Dip Ed

Consultant in Restorative Dentistry, King's College Hospital, London SE5 9RS, UK

Articles by Serpil Djemal

Abstract

Type-1 dentine dysplasia is a rare hereditary condition, associated with an abnormality in dentine formation. Deceptively, teeth have the clinical appearance of normality, however, radiographically, a different picture is seen; with multiple periapical radiolucencies associated with non-carious, unrestored teeth. This article reports the diagnostic and management challenges associated with dentine dysplasia in adults.

CPD/Clinical Relevance: Early diagnosis and preventive advice within primary care are imperative in the long-term outcomes.

Article

Dentine dysplasia (DD) is a rare, autosomal dominant condition that manifests itself clinically with discoloured and malformed teeth that can have a significant impact on a patient's confidence and self-esteem if untreated. Despite normal formation of enamel during tooth development, there is a hereditary disturbance in the formation in dentine with subsequent alterations in pulp development. The reason for this genetic disturbance is unknown and the condition can affect both the primary and secondary dentitions.1

The clinical manifestations were initially described by Ballschmiede,2 in 1930, and in 1939 Rushton3 described the clinical presentation and introduced the term ‘dentine dysplasia’ in a clinical case report. As more cases came to light in the dental literature, Witkop4 introduced the classification for dentine dysplasia in 1972 with 2 subtypes: Type-1 and Type-2.

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