Dentinogenesis Imperfecta

Dentinogenesis Imperfecta

Question. Write a short note on Dentinogenesis imperfecta.
Answer. Dentinogenesis imperfecta is also known as hereditary opalescent dentin or Capadepont’s teeth.

Dentinogenesis Imperfecta Classification

• Shield Type 1: Dentinogenesis imperfecta always occurs with osteogenesis imperfecta. As per Witko, it is known as dentinogenesis imperfecta.
• Shield Type 2: Dentinogenesis imperfecta, which is not associated with osteogenesis imperfecta. As per Witkop, it is known as hereditary opalescent dentin.
• Shield Type 3: It has shell shell-teeth appearance and multiple pulp exposures. As per Witko, it is known as the Brandywine type.

Dentinogenesis Imperfecta Clinical Features 

Dentinogenesis Imperfecta Clinical Features  Shield Type 1

• Presence of multiple bone fractures, hyperextensible joints, blue sclera, and there is progressive deafness.
• Deciduous teeth are affected more commonly as compared to permanent teeth.
• The color of teeth ranges from blue to brownish violet to yellow brown.
• Enamel is lost and dentition undergoes rapid attrition.
• Scalloping of DEJ is absent. Teeth are shorter in size. In some of the cases, total crown loss is present.
• In anterior teeth, the crown is square-shaped, but both mesial and distal borders are curved.
• In posterior teeth, both bicuspids and molars become flat, and normal circumferential curves get accentuated because of this, teeth show a bulbous appearance. The neck of the teeth narrows down.

Dentinogenesis Imperfecta Clinical Features  Shield Type 2

Features are the same as Shield Type I, but they are severe in form and are not associated with osteogenesis imperfecta.

Dentinogenesis Imperfecta Clinical Features  Shield Type 3

• In this, both permanent and deciduous dentition are affected.
• Here, the thickness of enamel is normal, while dentin is very thin.
• The tooth has an opalescent color, crown is bell-shaped, and there is multiple pulp exposures.

Dentinogenesis Imperfecta Radiographic Features 

• Affected teeth show cervical constriction and impart bulbous appearance.
• The pulp chamber is either partially or completely obliterated. Root canals can be absent or thread-like.
• There is a presence of periapical radiolucency without any pulpal involvement and widening of the periodontal ligament space.
• In some cases, the radicular part of pulp cavities is narrow, but the pulp chamber consists of a bulbous expansion that terminates at a point deep to the occlusal aspect, resembling a flame shape.
• In Brandywine type, tooth enamel is normal, but dentin is thin, and pulp chambers are enormous. Teeth look like shell teeth. The root of the tooth is very short.

Dentinogenesis Imperfecta Management

• An overdenture can be given to the patient, and the affected teeth should be restored with glass ionomer cement.
• Anteriorly, a porcelain jacket crown and posteriorly cast metal crown can be given.