Two-stage surgical treatment of complex femoral deformities with severe coxa vara in polyostotic fibrous dysplasia.

BACKGROUND In patients with polyostotic fibrous dysplasia with extensive femoral involvement, severe coxa vara may cause complex femoral deformities that are difficult to treat with a single-stage surgical procedure. We evaluated the results of treatment of such patients with a two-stage procedure. METHODS Eleven patients with polyostotic fibrous dysplasia and severe coxa vara (including one who required bilateral treatment and one who required repeat treatment) were treated surgically at a mean age of fourteen years and four months. A two-stage surgical procedure was planned to correct the deformity. The first stage involved correction of the coxa vara and fixation with a hip plate. The second stage involved correction of a shepherd's crook deformity if present and definitive fixation with an interlocking cervicodiaphyseal nail. All patients were evaluated clinically and radiographically at a mean of four years and seven months after the second-stage procedure. RESULTS The femoral neck-shaft angle averaged 83° before surgery and was corrected to a mean of 130° after the first-stage procedure. In two patients, cutout of the hip screw-plate caused acute postoperative loss of correction (by 40° in one patient and 20° in the other). After the second-stage procedure, the neck-shaft angle was either fully corrected or improved and the shepherd's crook deformity was fully corrected in all patients. At the time of the latest follow-up, the mean neck-shaft angle was 124°, with a mean loss of correction of 5° relative to the angle measured at the end of the second-stage procedure. The mean estimated blood loss was 625 mL in the first stage and 979 mL in the second. CONCLUSIONS The two-stage surgical procedure designed to treat patients with polyostotic fibrous dysplasia with complex femoral deformity and severe coxa vara restored a nearly normal femoral alignment that was maintained at a mean of four years and seven months of follow-up. The clinical benefits were pain relief in all of the patients and gait normalization or improvement in most. The estimated blood loss was substantial in both stages. LEVEL OF EVIDENCE Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.