Use of the Sheffield Telescopic Intramedullary Rod System for the Management of Osteogenesis Imperfecta: Clinical Outcomes at an Average Follow-up of Nineteen Years.

摘要

Background:Elongating intramedullary rods have been used in the management of osteogenesis imperfecta for the past fifty years. The complication rates reported in many reviews of the available techniques have been high. This study reviews the long-term functional outcomes and complications following the use of the Sheffield system of telescopic intramedullary rods.Methods:We conducted a retrospective analysis of patients with osteogenesis imperfecta who were at least eighteen years of age and who had at least thirteen years of follow-up. Complications, reoperations, and data from a disease-specific questionnaire and the Short Form-36 questionnaire were recorded.Results:Data for twenty-two patients with osteogenesis imperfecta who had been treated with Sheffield telescopic intramedullary rods were available at an average of nineteen years after the initial surgery. Reoperations involving thirty-three (50%) of the sixty-six rods were performed: ten rods (15%) were exchanged because of rod disengagement due to growth, thirteen rods (20%) were exchanged because of complications, and ten rods (15%) required further surgery other than exchange because of complications. Mobility was significantly improved at the initial postoperative visit (p = 0.0015), and this improvement was maintained into adulthood (p = 0.0077). Back pain was the most frequent symptom. Symptoms related to rod insertion across the knee and ankle were rare, but symptoms related to proximal femoral trochanteric entry were common. Physeal damage was not seen following surgery, and all rods elongated with growth. All patients were satisfied with the outcome of the surgical procedures. Short Form-36 scores for all physical domains and for social function and vitality were significantly worse than those in a normal population.Conclusions:The outcomes of this technique are satisfactory in adulthood; reoperation rates are high but are most commonly related to the patient outgrowing the rods. Concerns regarding insertion of this fixed device at the knee and ankle were unfounded, although proximal femoral fixation remains a problem.Level of Evidence:Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.