EFFECTS OF WITHDRAWAL OF BRACING IN MATCHED PAIRS OF CHILDREN WITH OSTEOGENESIS IMPERFECTA

Objectives: To evaluate the effects of withdrawal of long-leg braces ( hip-knee-ankle-foot orthoses [HKAFO]) on activity and ambulation in ch ildren with osteogenesis imperfecta. Design: A prospective, randomized cross-over trial, that describes the effects of withdrawing HKAFO. Pa tients: Ten children who were ambulatory with the assistance of braces . All had type III or IV osteogenesis imperfecta. Children were paired for age and clinical severity. Strength testing, fractures, and indep endence in daily activity were monitored at 4-month intervals for 32 m onths (16 months each of braced and unbraced periods). Gait was analyz ed during braced and unbraced conditions. Results: Muscle strength dec lined .35 grade during unbraced and .1 grade during braced intervals. Children spent more time in upright activity during braced intervals t han during unbraced intervals (p = .17). Children were more independen t in daily activities during braced than during unbraced periods (p = .14). Seventeen fractures of lower extremities occurred during all the unbraced periods, and 8 occurred during the braced intervals (p = .08 ): the fracture rate was higher during unbraced intervals. (p = .06) B racing was associated with increased hip flexion and stride length and decreased transverse plane pelvic rotation. Conclusion: Withdrawal of HKAFO in children with osteogenesis imperfecta who had achieved uprig ht activity was not associated with significant decrease in muscle str ength or independence, but there was an associated increase in fractur e rare that nearly reached significance.