Osteogenesis imperfecta

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Summary chapter 4

  • The most frequent dental abnormality in OI is dentinogenesis imperfecta, characterised by abnormal dentin formation. Affected teeth exhibit a grey or yellowish-brown colour and are more fragile than normal teeth.
  • OI can also lead to alterations in tooth eruption, including dental agenesis, supernumerary teeth, ectopic eruption, and dental impaction.
  • Facial bone development may be affected in OI, resulting in malocclusion. Class III malocclusion is the most prevalent form among OI patients.
  • Starting 6 months after the eruption of their first primary teeth, individuals with OI should receive dental evaluations for preventive measures and potential treatments if necessary.
  • While wormian bones are present in a healthy population, the presence of numerous wormian bones (typically exceeding 10) suggests a bone pathology, such as OI.
  • Platybasia refers to a skull shape anomaly that leads to flattening of the cranial base.
  • In basilar impression and basilar invagination, the skull base descends toward the upper cervical vertebrae. The protrusion of the vertebrae towards the foramen magnum (the hole in the skull) can result in compression of the spinal cord and the exiting nerves.

 

Last modified
14 July 2023