Diagnosis of Osteofibrous Dysplasy in Children

The first description in Russian literature of osteofibrous dysplasy in children is presented. It is grounded on the analysis of diagnostic peculiarities and treatment of 24 patients in the period from 1966 to 1996 The main diagnostic criteria by clinical, radiologic and pathomorphologic features are given. Osteofibrous dysplasy is a rare and insufficiently studied disease affecting mainly tibia in children of the first ten years. It has dysplastic congenital nature and is similar to the biastomatosis process. Saber-like deformity of the affected segment prevails in clinical picture. This deformity may be of different degree of manifestation and accompanied by painless. Eccentric intracortical location of pathologic focus is typical of radiologic picture. Morphologic examination is the main diagnostic method. Histologic study shows architectonics «zone» of the pathologic focus which consists of fibrous tissue with bone trabeculae inclusion (bone trabeculae with laminar structure) mainly surrounded by active osteoblasts.