Dentinogenesis Imperfecta: Clinical Features and Management

Dentinogenesis Imperfecta is a genetic disease which affects the tooth development, especially the dentin of the tooth. The teeth that are affected by this disease are translucent and are discolored. By definition, this disease is called an inherited disease of dentin formation. This disease is also called as ‘DI’ and ‘Hereditary Opalescent Dentin’.

This is an autosomal dominant condition which affects both the primary teeth and the permanent teeth alike. People who suffer from Dentinogenesis Imperfecta tend to have weaker teeth which wear and break easily. Because of this, there is a higher chance of early teeth loss in these individuals than the normal ones.

Occasionally people with this disease may also suffer from speech-related problems due to malpositioned teeth. This is a rare disease seen in 1 out of 6000 to 8000 people.

Dentinogenesis Imperfecta Classification

There are three popular classifications of Dentinogenesis Imperfecta that are available today.

  • Shields Classification
  • Revised Classification
  • Witkop Classification

Shields Classification

Type 1: This type of Dentinogenesis Imperfecta occurs in people who are already suffering from osteogenesis imperfecta.

Type 2: This type isn’t associated with Osteogenesis Imperfecta.

Type 3: This type is also called ‘Brandywine type’. This is a very rare condition seen in racial isolates of Maryland. This disease is characterized by multiple pulp exposures and periapical lesions in deciduous (baby teeth) teeth.

dentinogenesis imperfecta

Dentinogenesis Imperfecta

Revised Classification

Dentinogenesis Imperfecta 1: This is found in people without Osteogenesis Imperfecta and hence this classification corresponds with the Shields classification type 2. This type of DI disease is also called as Opalescent Dentin and Capdepont Teeth.

Dentinogenesis Imperfecta 2: This is similar to type 3 of Shields classification of DI.

Dentinogenesis Imperfecta Cause

This disease is believed to be caused due to the mutation in a gene called the ‘Dentin Sialo Phospho Protein Gene’ otherwise known as the DSPP gene. This is the gene which encodes the Dentin Phosphoprotein and Dentin Sialoprotein.

Clinical Features of DI

Clinical features may vary from person to person depending on the severity. One thing that is common to all the cases of DI is translucent or opalescent hue found in the affected teeth. Unlike seen in many cases of Amelogenesis Imperfecta, in Dentinogenesis Imperfecta teeth are not prone to caries more than the normal ones.

Also, it has been observed that people having DI may suffer from frequent multiple periapical abscesses. This is caused due to pulpal strangulation which results from pulp obliteration caused due to the wear of the coronal part of the tooth (tooth crown).

DI affects both the males and females equally. Early loss of tooth enamel is a significant feature of DI. The layer of tooth enamel may start splitting from dentin when a load or occlusal pressure is applied.

Severe attrition of teeth is also a common finding in the people suffering from this disease. In some cases, hypo-mineralized areas on enamel may be seen.

The water content is found out to be more in teeth affected by Dentinogenesis Imperfecta. Also, the mineral content of the tooth is significantly reduced.

Radiographic Features:

  • Crowns of teeth appear bulb-shaped or bell-shaped, with constricted cervical areas.
  • Obliteration of tooth pulp in both the crown part (coronal) and the root part (radicular), is a common finding.
  • The components of the periodontium appear normal under the radiograph.
  • Roots of the teeth may appear thin.
  • Large pulp chambers in teeth with thinner shells of dentin and enamel result in ‘shell teeth‘.

Treatment for Dentinogenesis Imperfecta

  • Preserving the teeth, especially the teeth enamel and the dentin, is the main aim of the treatment.
  • It is necessary to prevent the enamel and dentin from undergoing attrition.
  • In milder forms of the disease, where there isn’t significant enamel loss, routine restorative treatments like amalgam and composite fillings, can be carried out.
  • For improving the aesthetic appeal of the person, dental veneers and bonding agents may be used. But please remember that teeth whitening is contraindicated in these cases.
  • In severe cases, where there is a significant loss of teeth enamel, full crown restorations may be needed.
  • If the teeth attrition has already occurred, an overdenture may be required to prevent further loss of teeth structure due to attrition as well as to prevent the loss of bone height (alveolar bone height) in the jaws.
  • In DI associated with Osteogenesis Imperfecta, Bisphosphonates may be required. Please alert your dentist if you are already under Bisphosphonates medication. Bisphosphonates are considered to be a high-risk drug for dental treatments because of its tendency to cause ‘Bisphosphonate-associated osteonecrosis of the jaw’.
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