Complications after surgical treatment for fractures and deformities of extremities in children with osteogenesis imperfecta

Osteogenesis imperfecta (OI) or Lobstein Vorlick disease is a heterogeneous hereditary dysplasia of the connective tissue, characterized by frequent fractures and skeleton deformities, short stature, discoloration of the sclera, dentinogenesis imperfecta, joint hyperlaxity, and other extra-skeletal manifestations. Frequent bone fractures resulting from low-energy exposure are associated with poor bone quality or the presence of congenital or post-traumatic deformities, which leads to fractures on its apex. Conservative treatment can lead to the development of a vicious circle: a fracture - immobilization - post-immobilization osteoporosis - fracture, which is often the reason for the limitation of patients' motor activity. Intramedullary osteosynthesis eliminates the displacement of fragments, restores the axis of the limb, provides intramedullary splinting and reduces the time of immobilization. The use of bisphosphonates to increase bone mineral density has greatly expanded surgical capabilities. But despite multidisciplinary approach and improvement of surgical techniques, the frequency of complications remains extremely high. Nevertheless, surgical treatment of long bones is a method of choice in these patients and in most cases leads to an improvement in their quality of life, allowing them to expand their motor activity.