The use of intramedullary telescopic fixateur for osteosynthesis of long bones of the lower limbs in children with osteogenesis imperfecta

Abstract

Osteogenesis imperfecta (OI) is a heterogeneous pathology which is accompanied by the development of multiple fractures and deformi­ties of the long bones of the extremities. Objective: to present out­comes of surgical treatment in patients with OI using intramedullary telescopic fixateur (ITF). Methods: 9 patients with bone deformities of the extremities due to OI were operated (4 boys, 5 girls aged 1,5–11 years). Correction was performed with using of percuta­neous semi-open osteoclasia on top of deformity. Bone fragments were fixed by means of ITF developed by authors which provides bone elongation during growth. After surgery we used an orthosis for external fixation. Rehabilitation envisaged work out active and passive movements in joints of the operated extremity, exercises for increasing of muscle strength, and hydrokynesiotherapy. Control checkups (clinical and radiological examination, determination of active movements in joints and muscle strength of the lower limbs) were performed every 3–4 months. Outcomes were assessed by FAQ Ambulation Scale. In all patients there were found consolidation of fragments in the area of osteotomy, preservation of achieved axial correction of parameters in lower extremities, increasing of active movements in hip and knee joints. Bearing-kinematic function of the lower extremities grew on 5 classes, and in two cases — on 7 ones. Improvement of functional class according to FAQ Ambula­tion Scale was found in 4 patients. In 6 patients which were not able to walk before surgery an ability for vertical standing and walking appeared. Conclusion: The proposed ITF provides restoration of anatomical axis of a limb, function of walk and bearing capacity in lower extremities, early start of the rehabilitation period, reduction of the percentage of complications, and also reduces the need for repeated surgeries, prevents the progression of deformation and a nascence of secondary fractures of long bones.