Deformity of the proximal femur in fibrous dysplasia
leads to deviation of the mechanical axis of the hip, which may lead
to the development of secondary osteoarthritis (OA). This study
investigated the prevalence and predisposing factors for the development
of OA in patients with fibrous dysplasia of the proximal femur.
We reviewed the records of 209 patients from our institutional database
with fibrous dysplasia of the proximal femur, investigating possible predisposing
factors including patient demographics, the extent of the coxa vara
deformity, the presence of peri-articular disease, and the overall
burden of skeletal disease. Of the 209 patients, 24 (12%) had radiological
evidence of OA in the ipsilateral hip. The prevalence was significantly
higher in patients with polyostotic fibrous dysplasia compared with
those with monostotic disease (p <
0.001). In a subgroup analysis of
patients with polyostotic disease, the extent of deformity (quantified
using the neck–shaft angle), and the presence of peri-articular
disease (whether in the head of the femur or the acetabulum) were
significant predictors of osteoarthritis (neck–shaft angle likelihood
ratio (LR) = 0.847 per 1° increase, p = 0.004; presence of lesion
in the head of the femur LR = 9.947, p = 0.027; presence of lesion
in the acetabulum LR = 11.231, p = 0.014). . Our data suggest that patients with polyostotic fibrous dysplasia
have a high risk of developing secondary OA of the hips. This risk
is higher in patients with peri-articular disease, and those with
a more
Aims
Patients and Methods
We present a retrospective study of patients suffering from a variety of benign tumours in whom external fixators were used to treat deformity and limb-length discrepancy, and for the reconstruction of bone defects. A total of 43 limbs in 31 patients (12 male and 19 female) with a mean age of 14 years (2 to 54) were treated. The diagnosis was Ollier’s disease in 12 limbs, fibrous dysplasia in 11, osteochondroma in eight, giant cell tumour in five, osteofibrous dysplasia in five and non-ossifying fibroma in two. The lesions were treated in the tibia in 19 limbs, in the femur in 16, and in the forearm in eight. The Ilizarov frame was used in 25 limbs, the Taylor Spatial Frame in seven, the Orthofix fixator in six, the Monotube in four and the Heidelberg fixator in one. The mean follow-up was 72 months (22 to 221). The mean external fixation period was 168 days (71 to 352). The mean external fixation index was 42 days/cm (22.2 to 102.0) in the 22 patients who required limb lengthening. The mean correction angle for those with angular deformity was 23° (7° to 45°). At final follow-up all patients had returned to normal activities. Four patients required a second operation for recurrent deformity of further limb lengthening. Local recurrence occurred in one patient, requiring further surgery.