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Craniofacial Implications of Osteogenesis Imperfecta

I had the pleasure to speak with Dr. Jean-Marc Retrouvey and view his presentation on the craniofacial implications of osteogenesis imperfecta. This is a disease that causes weak bones that break easily. It is also known as brittle bone disease where bones sometimes break for no known reason. OI can also cause many other problems, such as weak muscles, brittle teeth, and hearing loss.

Dr. Retrouvey delved into the implications of treating patients that suffer from OI and what the possible treatments are.

Chiraz Guessaier, CDA Oasis Manager

Highlights

Dr. Jean-Marc Retrouvey presents various craniofacial aspects of osteogenesis imperfecta. This is a clinically relevant topic for dentists as the treatment is not always straightforward.

Osteogenesis imperfecta (OI) is a disease resulting from a mutation in collagent type 1 genes and there are several main types. The disease affects bones and teeth more than other organs.

Signs & Symptoms

  • Brittle bones
  • Small stature
  • Muscular weakness, multiple fractures
  • Joint hyperlaxity
  • Long bone deformation (bowing)
  • Scoliosis
  • Blue sclera
  • Deafness
  • Dentinogenesis imperfecta (DI)
  • Severe malocclusions

There is no cure for this condition so symptomatic treatment is indicated. IV bisphosphonates help to increase the mineral bone density but, this is less effective in adults. At times, surgical intervention may be required. Physiotherapy and physical exercise are key in managing this condition.

Dentinogensis Imperfecta

  • Not visibly present in all OI patients, but is one of the most significant aspects of OI
  • Not all teeth are affected in the same way in the same dentition
  • Requires clinical and radiographic examination as the pulp, size and shape of the teeth are affected
  • Primary dentition usually more affected than that permanent dentition
    • Important to maintain the vertical dimension of occlusion
    • Decrease the risk of premature tooth loss and loss of space

Expression of DI

  • Blue or brown opalescence
  • Obliteration of pulpal tissue
  • Microscopic disturbances in the dentin
  • Constriction of the CEJ
  • Short and narrow roots
  • Excessive wear of the dentition

Treatment of the pediatric dentition often involves full coverage restorations in the posterior and if required, extractions with the necessary space maintenance. Teeth that are borderline non-restorable may not be candidates for restoration and in the long-term it may be wiser to extract them. With respect to the treatment of malocclusions, early intervention is favoured and the dentition and malocclusion worsens over time. The ultimate goal of treatment it to restore the patient to functional occlusion. Dental implant treatment in OI patients can be done in Type 1 patients if the periodontal condition is good and bone density at the recipient site is adequate; however, Type III and IV patients are much riskier candidates for implant treatment and any treatment should be done in conjunction with advice from an OI centre.

 

 

 

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