Abstract
BackgroundResearch into the optimal treatment of fibrous dysplasia has been limited by the lack of an established classification system for the disease. The purposes of this study were to develop a radiographic classification for fibrous dysplasia of the proximal femur and to test this classification’s intra- and interobserver reliability as well as the effectiveness of our treatments.MethodsWe retrospectively reviewed radiographs and computed tomography (CT) of 227 femurs from 206 patients with fibrous dysplasia. The radiographs were evaluated in the coronal plane for neck-shaft angle, varus deformity in the proximal femoral shaft, and distal juxtaarticular valgus deformity. CT was evaluated in the axial plane for destruction of cortex. Reduction of bone strength was defined as the thickness of the remaining cortex less than 50 % of the original on axial CT. Two senior orthopedists evaluated each radiograph and CT twice at 8-week intervals. Intra- and interobserver reliability testing was performed using the kappa statistic. Treatments were assessed through mid-term follow-up.ResultsThe 227 femurs were classified into five reproducible types: type 1 (33 %), normal bone strength without angular deformity; type 2 (30 %), decreased bone strength without angular deformity; type 3 (12 %), isolated coxa vara with neck-shaft angle <120°; type 4 (11 %), isolated varus deformity in the proximal femoral shaft; and type 5 (14 %), coxa vara with varus deformity in the proximal femoral shaft. Intra- and interobserver kappa values were excellent, ranging from 0.85 to 0.88. Good clinical outcomes were achieved.ConclusionsThis radiographic classification of fibrous dysplasia is reproducible and useful for describing and assessing this disease. The treatments based on this classification were effective.
Highlights
Research into the optimal treatment of fibrous dysplasia has been limited by the lack of an established classification system for the disease
The proximal femur is a frequent site of fibrous dysplasia, resulting in pain, limping, deformity, and pathological fracture due to the weight of the body and the strong gluteal muscles
Patient information We searched the medical records of our department (Department of Orthopedics, West China Hospital, China) from January 1995 to January 2013 to identify all patients with fibrous dysplasia in the proximal femur treated in our department during that period
Summary
Research into the optimal treatment of fibrous dysplasia has been limited by the lack of an established classification system for the disease. The purposes of this study were to develop a radiographic classification for fibrous dysplasia of the proximal femur and to test this classification’s intra- and interobserver reliability as well as the effectiveness of our treatments. Fibrous dysplasia is an uncommon skeletal disorder in which normal bone and bone marrow are replaced by fibro-osseous tissue [1]. It may involve one bone (monostotic fibrous dysplasia) or multiple bones (polyostotic fibrous dysplasia). The proximal femur is a frequent site of fibrous dysplasia, resulting in pain, limping, deformity, and pathological fracture due to the weight of the body and the strong gluteal muscles. We further proposed different treatments according to these varying types and assessed their effectiveness
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