Severe knee flexion contractures in patients with arthrogryposis multiplex congenita were treated by distal femoral extension osteotomy. Thirty-two operations were followed for an average of 32 months. Contractures were corrected from 49 degrees to 6 degrees. During follow-up there was a loss of correction of 22 degrees at a rate of 0.9 degrees/ month. The angle of the distal femoral physis and the shaft of the femur was 2 degrees of flexion preoperatively, and postoperatively it measured 43 degrees of extension and at late follow-up it measured 19 degrees. Remodeling occurred at a rate of 1.0 degrees/month, which correlated with recurrence. All patients increased their ambulatory ability at least one level. There was one wound infection. Distal femoral extension osteotomy is effective and safe for the correction of knee flexion contracture. Recurrence occurs in all growing children.
Weekly high-dose methotrexate with leucovorin rescue and vincristine (HDMTX) and doxorubicin was administered as adjuvant postoperative therapy to 46 patients with a diagnosis of conventional high-grade nonmetastatic osteosarcoma of an extremity between July 1976 and December 1981. The primary lesions were managed by wide or radical amputation (26 patients) or by limb-sparing resection in 20 selected patients. The margins of the resections were retrospectively classified as marginal in three, wide in 16, and radical in one. The 5-year relapse-free survival (RFS) for all patients is 59% (95% confidence interval [CI], 43%, 74%) and overall survival is 78% (95% CI, 65%, 91%). The RFS for patients initially having a limb resection procedure is 55% (95% CI, 32%, 77%) compared with 62% (95% CI, 43%, 81%) for those initially having amputations (P = .52). Using multivariate analysis, the only significant prognostic variables that predicted RFS of greater than or equal to 3 years, were the presence of moderate to marked lymphocytic infiltration of the primary tumor (P less than .002), primary site outside of the proximal humerus (P less than .005), and the absence of a predominance of osteoblastic pattern in the primary tumor (P less than .03).
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