Surgical management of patients with osteogenesis imperfecta: 10 years experience

The orthopedic problems of osteogenesis imperfecta (OI) are multiple and formidable, if not untreatable. Controversy surrounds the timing and method of its management and to date, no reports have been published on the orthopedic treatment of these patients in Taiwan. This paper reports the surgical treatment of eight OI patients (average age, 3 yr 5 mo) who were unable to walk before surgery. Preoperatively, the incidence of long bone fracture ranged from once per month to twice per year. The average degree of femoral and tibial bowing as measured from leg radiographs at the first visit were 42 degrees and 32 degrees, respectively. Four patients underwent intramedullary nailing of both the femur and tibia while another four patients underwent intramedullary nailing of the femur only in the primary operation. The implants used were mostly Steinmann pins or Rush nails, and a femoral Kuntscher nail was used in one case. At the mean postoperative follow-up period of 5 years 4 months, five patients could walk independently without support. Two walked with wheel-carts and one could rise and stand with minimal support. We conclude that patients with OI can benefit from early surpical correction and stabilization and experience improvement in their ambulatory status despite the multiple procedures required.