Orthopaedic surgical methods

In the serious case of osteogenesis imperfecta when the malformation of long bones causes malfunction, it is corrigible with orthopaedic surgical intervention. This intervention can be executable in two ways: expertly treatment of fractures of malformed long bone or correction of malformed bones in order to prevent the fracture.
Most of the interventions are the Kirschner-wire tacking of the broken bone and casing it in plaster.
The treatment can include intramedullar (in-bone-marrow) fixation too. Furthermore segment resectio (excision) can be necessary which depends on the measure of the malformation. In several cases correction with rotation of the resected segment was needful. Once, an extremely large convex shin-curve was fixed by fixateur externe.
In short, we can say that the treatment of fractures of patients with osteogenesis imperfecta differs from normal fractures’. Fixation of engraved malformed bones and fixation of fractures of long bones:

  1. Implenting a rod into the neural crest
  2. Kirschner-wire tacking
  3. Fixateur externe is available.

AThe fractures are healing full well, but ensuring the right setting of the axle is very important to prevent malformations. Large curves and malformations can be corrigible by bone engraving.
Screws and sheets are not good for fixing these bones, because under the sheets and around the screws the bone becomes spoungy whereby the bone will be more weaker. Mainly during the childhood, in the case of fracture of thigh bone, the overhead extension is a very efficient method.
After the treatment, it is practical to mobilize the patient as soon as possible.
Additional therapeutic possibilities:

  1. ensuring positive calcium balance
    - calcium and vitamin D or calcitriol 

    • human recombinans GH

    - augments the growth and bone matrix, improves the mood

    • osteoprogenitor stem-cell transplant

    - isolated multipotent stem-cell from bone marrow

  2. mosaicismus phenotype is more moderate
  3. cell concentration is low.