Focal Scleroderma in Children: An Orthopaedic Perspective

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Focal scleroderma is a connective-tissue disorder manifested by fibrosis of the skin and subcutaneous tissues. Consequently, it may be associated with joint contractures, extremity deformity, and impairment of extremity function. It has a variable clinical course, with both remissions and recurrences. In resistant cases of extremity involvement, treatment considerations should include physical and occupational therapy, medical treatment, bracing, serial casting, and surgical intervention. We reviewed seven patients treated between 1960 and 1990 with significant joint contractures secondary to focal scleroderma. Serial casting was found to be useful for contractures about the wrist, knee, and ankle. Soft-tissue release was an effective treatment for contractures at the wrist, hip, and ankle. Knee disarticulation was performed on one patient with a severe knee-flexion contracture. Epiphysiodesis and femoral shortening osteotomy were effective treatments for leg-length equalization in a case of hemiatrophy.

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