MANAGEMENT DELLE FRATTURE E DEFORMITÀ A CARICO DELLE OSSA LUNGHE DEGLI ARTI INFERIORI IN UN GRUPPO DI PAZIENTI AFFETTI DA OSTEOGENESI IMPERFETTA TIPO I, III E IV Management of long bones fractures and deformities of lower extremities in patients with osteogenesis imperfecta type I, III AND IV

Summar y Background. Osteogenesis imperfecta (OI), or brittle bone syn- drome, is a rare connective tissue disorder. The long bones of patients often are deformed with axial rota- tion or bent, and often at the apex of deformity for an obvious lever mechanism, there is a fracture that reinforce a vicious circle whereby deformation causes new fractures that, consolidating, worsen the same deformity. The goal of the orthopaedic treatment must be always to treat deformities to prevent acute fracture event, That we have to deal with urgent needs. We present our experience about treatment and follow-up intra and post-op of long bones fractures and deformities of lower extremities in patients with osteogenesis imperfecta type I, III and IV treated with corrective osteotomies and syntehsis with Fassier Duval telescopic rod or 2 TEN nails with sliding nails technique. Methods. 195 patients are treated in Congenital Osteodystrofic Desease clinical centre, in Department of Pediatrics of Universi