Benson D R, Newman D C
Clin Orthop Relat Res. 1981 Sep(159):147-53.
In the osteogenesis imperfecta patient, spinal deformity should be expected, particularly in the severely affected nonambulator with chest deformity. Curves develop early (age five to six) and generally progress rapidly. Early bracing, although somewhat effective, may well compress the soft osteoporotic rib cage without controlling the spinal curvature. The pulmonary compromise created by the scoliosis is compounded by the chest cage deformity secondary to bracing. In the patient with severe disease (thin bones and numerous fractures), posterior correction and fusion, with or without Harrington instrumentation, is the preferred approach. This should be done early as the osteoporotic bone does not tolerate the hook forces well; the correction is correspondingly limited. The use of methylmethacrylate bone cement around the hook provides redistribution of forces and more stable fixation. In the patient with mild disease (thick bones and few fractures) treatment should be similar to that of patients with idiopathic scoliosis. The chest cage should be carefully observed to avoid deformity from placement of the lateral or posterior pad.
对于成骨不全患者，脊柱畸形是可以预见的，尤其是在严重受累且无法行走并伴有胸廓畸形的患者中。脊柱侧弯在早期（5至6岁）就会出现，并且通常进展迅速。早期支具治疗虽然有一定效果，但很可能会压迫骨质疏松的胸廓，却无法控制脊柱侧弯。脊柱侧弯造成的肺部功能损害因支具导致的胸廓畸形而加重。对于病情严重的患者（骨骼脆弱且骨折频繁），无论是否使用哈灵顿器械，后路矫正和融合术都是首选方法。由于骨质疏松的骨骼难以承受钩子的力量，因此应尽早进行手术；相应地，矫正程度也会受到限制。在钩子周围使用甲基丙烯酸甲酯骨水泥可实现力量重新分布并提供更稳定的固定。对于病情较轻的患者（骨骼较厚且骨折较少），治疗方法应与特发性脊柱侧弯患者相似。应仔细观察胸廓，以避免因放置侧方或后方衬垫而导致畸形。
