Operative Treatment of Spine Deformity in Osteogenesis Imperfecta

There has been little mention in the literature of scoliosis associated with osteogenesis imperfecta. The purpose of this study was to investigate the problem of spine deformity in a series of osteogenesis imperfecta patients and to review the operative treatment of 8 patients. A series of 49 patients (ranging in age from 8 months to 57 years, average 12 years) with osteogenesis imperfecta were studied. Thirty-five (71%) of these patients had scoliosis ranging from 7 degrees to 105 degrees (average 42 degrees). The more severely involved patients (OIC and OITI groups) demenostrated a higher incidence (80%) of scoliosis and a greater average degree of curvature (42 degrees) as compared to the mildly involved (OITII) group with a 50% incidence of scoliosis with an average curvature of 26 degrees. Attempts to control these curves by bracing have been difficult and usually had to be abandoned. Eight of these patients underwent spine stabilization in an attempt to control curve progression. All 8 patients had posterior spine fusions. One of these patients had an anterior fusion preceding the posterior fusion because of a significant kyphotic deformity. Harrington instrumentation was used in 5 patients. One patient could not be instrumented secondary to severe osteoporosis while the remaining 2 patients were treated by the Risser technique. Postoperatively, either cast or brace protection was used. Only minimal improvement in the curves was achieved. Spine stabilization rather than curve correction is the goal of spine fusion in osteogenesis imperfecta. An important finding was that there was no change in the pre- and postoperative activity and ambulatory status in these patients. Preliminary follow-up (9-12 months) in all 8