How are spinal deformities treated in osteogenesis imperfecta (OI)?

Updated: Feb 24, 2020
  • Author: Manoj Ramachandran, MBBS, MRCS, FRCS; Chief Editor: Harris Gellman, MD  more...
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Answer

Bracing is not effective in treating spinal deformities such as scoliosis and kyphosis, because the rib cage is too fragile to transfer the brace pressure to the vertebral column. Moreover, the external pressure may worsen the chest deformities. Surgery is indicated when the following two conditions are present:

  • Acceptable bone quality
  • Progressive scoliosis with curvature of more than 45° if OI is mild or more than 30-35° if OI is severe

Posterior spinal arthrodesis is the treatment of choice and is best performed with segmental instrumentation. Often, significant correction and stable fixation are not achieved. Pretreatment with pamidronate appears to improve the surgical outcome.


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