Purpose of the study: The aim of the study was to analyze a group of patients who had undergone multilevel osteotomy of long bones and medication therapy for osteogenesis imperfecta (OI).
Material and methods: The group included 14 OI patients (nine girls and five boys) operated on in the years 1996 to 2006, who ranged in age from 3 to 17 years (average, 8.2 years). Due to residual deformation following a fracture of or because of treatment for acute trauma to long bones of the lower extremities, the patients underwent multilevel osteotomy with the use of osteosynthesis (Prevot's rod, six patients; Kirschner's wire, three patients; Küntcher's nail, three patients; Rush's nail, one patient; condylar plate, one patient). A special working and rehabilitation program played an important role in the therapeutic protocol. Four patients treated after 2003 received Pamidronate.
Results: Sufficient correction of axil deformity of the legs and equal leg length resulting in gait improvement were achieved in 11 patients. In one patient, osteosynthesis with a condylar plate failed and it was necessary to apply intramedullary elastic fixation. In one patient, tibia vara developed following Küntcher's nail osteosynthesis. In one patient, disunion of bone from osteosynthetic material, with a subsequent supracondylar fracture under the Küntcher's nail, was recorded. Pamidronate administered in pre- and post-operative periods to the four patients treated after 2003 reduced the need for their immobilization from 6 to 3 weeks, which permitted early rehabilitation and, in one patient, first standing and walking at the age of 12 years.
Discussion: The treatment of long bone fractures in OI patients is based on the assumptions that bone healing is not affected and that long immobilization leads to deterioration of osteopenia and to a risk of further fractures. For these reasons, surgical procedures using intramedullary fixation have recently been preferred. Pamidronate administration alleviates pain, improves muscle tonus, reduces the period of immobilization and enhances bone density.
Conclusions: The multidisciplinary, rational approach, which involves early surgical intramedullary fixation of fractures with subsequent rehabilitation and Pamidronate administration, is considered to provide a more effective therapy with better results and therefore better quality of life in patients with osteogenesis imperfecta.