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Two-Stage Surgical Treatment of Complex Femoral Deformities with Severe Coxa Vara in Polyostotic Fibrous Dysplasia

Ippolito, Ernesto MD; Farsetti, Pasquale MD; Valentini, Matteo Benedetti MD; Potenza, Vito MD

Journal of Bone & Joint Surgery - American Volume: 21 January 2015 - Volume 97 - Issue 2 - p 119–125
doi: 10.2106/JBJS.N.00230
Scientific Articles
Supplementary Content

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.

1Department of Orthopaedic Surgery, University of Rome “Tor Vergata,” Viale Oxford, 81.00133 Rome, Italy. E-mail address for E. Ippolito:

Copyright 2015 by The Journal of Bone and Joint Surgery, Incorporated
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