Dentinogenesis imperfecta: A case report

Abstract

Dentinogenesis imperfecta is an autosomal dominant disorder of tooth development characterized by the presence of opalescent dentin, resulting in a dusky blue to brownish discoloration of the teeth. This condition is genetically and clinically heterogeneous; it may affect only the teeth or it may be associated with the osteogenesis imperfecta. Dentinogenesis imperfecta has been subdivided into three types: type I is associated with osteogenesis imperfecta; in type II there is no associated osteogenesis imperfecta; and when the condition is associated with the Brandywine triracial isolate and large pulp chambers it is classified as type III. This report describes a 16-year-old female patient who showed the characteristic dental features of dentinogenesis imperfecta type II. The etiology and prevalence of the disorder, and a comprehensive treatment plan, will be briefly reviewed.

Dentinogenezis imperfecta tip 2 ile ilgili sıradışı özellikler: ailesi üç kuşaktan fazla etkilenmiş iki olgu

“…The term 'hereditary opalescent dentin' was first used by Skillen, Finn and Hodge. They used this particular term to explain the brown translucent teeth with opalescent sheen which lacked pulp chambers 4 . DI is known as hereditary opalescent dentin or Capdepont dysplasia 2 .…”

“…Radiographically, the characteristic appearance is the bulbous crowns with constricted short roots 1,6 . Initially the pulp chambers are usually abnormally spacious and resemble "shell teeth," but then they will progressively obliterate giving the teeth solid appearance 4 . The teeth lack pulp chambers and root canals.…”

“…DI is a hereditary disorder of dentin formation 3 . It has a high degree of penetrance and a very low mutation rate 4 . It is associated with localized mesodermal dysplasia of dental papilla of both deciduous and permanent dentitions 4,5 .…”

Comparative Study of Dentinogenesis Imperfecta in Different Families of the Same Topographical Region

“…The term was coined by Robert and Schor in 1939 4. Witkop reported that it was most common autosomal dominant disease affecting westerners 67…”

“…Radiographically type I and II exhibit identical changes 4-6. Opacification of dental pulp occur due continued deposition of abnormal dentin.…”