FOCAL SCLERODERMA IN CHILDREN - AN ORTHOPEDIC PERSPECTIVE

Focal scleroderma is a connective-tissue disorder manifested by fibros is of the skin and subcutaneous tissues. Consequently, it may be assoc iated with joint contractures, extremity deformity, and impairment of extremity function. it has a variable clinical course, with both remis sions and recurrences. In resistant cases of extremity involvement, tr eatment considerations should include physical and occupational therap y, medical treatment, bracing, serial casting, and surgical interventi on. We reviewed seven patients treated between 1960 and 1990 with sign ificant joint contractures secondary to focal scleroderma. Serial cast ing was found to be useful for contractures about the wrist, knee, and ankle. Soft-tissue release was an effective treatment for contracture s 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.