Cristofaro R L, Hoek K J, Bonnett C A, Brown J C
Clin Orthop Relat Res. 1979 Mar-Apr(139):40-8.
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
文献中很少提及与成骨不全相关的脊柱侧弯。本研究的目的是调查一系列成骨不全患者的脊柱畸形问题，并回顾8例患者的手术治疗情况。对49例年龄从8个月至57岁（平均12岁）的成骨不全患者进行了研究。其中35例（71%）患者存在脊柱侧弯，侧弯角度从7度至105度（平均42度）。与轻度受累（OITII）组相比，受累更严重的患者（OIC和OITI组）脊柱侧弯发生率更高（80%），平均弯曲度更大（42度），而轻度受累组脊柱侧弯发生率为50%，平均弯曲度为26度。通过支具控制这些弯曲很困难，通常不得不放弃。其中8例患者接受了脊柱稳定手术以控制弯曲进展。所有8例患者均进行了后路脊柱融合术。其中1例患者由于存在明显的后凸畸形，在进行后路融合之前先进行了前路融合。5例患者使用了哈灵顿器械。1例患者因严重骨质疏松无法进行器械固定，其余2例患者采用里塞尔技术治疗。术后使用了石膏或支具保护。弯曲度仅得到了极小的改善。在成骨不全患者中，脊柱融合的目标是脊柱稳定而非弯曲矫正。一个重要的发现是，这些患者术前和术后的活动及行走状态没有变化。对所有8例患者进行了初步随访（9至12个月）
