Smith A D, Koreska J, Moseley C F
Hospital for Sick Children, Toronto, Ontario, Canada.
J Bone Joint Surg Am. 1989 Aug;71(7):1066-74.
We reviewed the clinical charts and spinal radiographs of fifty-one boys who had Duchenne muscular dystrophy, had not had surgical treatment of the spine, and had been followed until death. All had scoliosis. None of the following variables was useful in predicting which curves would become severe: age when the patient initially walked, age when he ceased walking, age at onset of spinal collapse, surgical release of the iliotibial bands, or age at the time of death. Radiographs were made within eighteen months before death for thirty-three patients; in thirty-one of them, the final curve exceeded 40 degrees and in seventeen, 90 degrees. For the remaining eighteen patients, final radiographs were made more than eighteen months before death; at that time, eight of them already had a curve of more than 90 degrees. Although there was a relationship between extension of the lumbar spine and severity of scoliosis at the time of final follow-up, early maintenance of the lumbar spine in extension rarely prevented the development of a severe curve. For most of the patients who had a severe curve, sitting was difficult and was accompanied by breakdown of the skin and pain. When a patient's curve exceeded 35 degrees, the vital capacity usually was less than 40 per cent of the predicted normal value. Therefore, when walking becomes impossible for boys who have Duchenne muscular dystrophy, routine spinal arthrodesis should be considered.
我们回顾了51例患有杜氏肌营养不良症、未接受过脊柱手术治疗且随访至死亡的男孩的临床病历和脊柱X光片。所有患者均患有脊柱侧弯。以下变量均无助于预测哪些侧弯会发展为重度：患者开始行走的年龄、停止行走的年龄、脊柱塌陷开始的年龄、髂胫束手术松解情况或死亡时的年龄。33例患者在死亡前18个月内拍摄了X光片；其中31例最终侧弯超过40度，17例超过90度。其余18例患者在死亡前18个月以上拍摄了最终X光片；当时，其中8例已有超过90度的侧弯。尽管在最后随访时腰椎伸展与脊柱侧弯严重程度之间存在关联，但早期保持腰椎伸展很少能预防严重侧弯的发展。对于大多数有严重侧弯的患者来说，坐姿困难，伴有皮肤破损和疼痛。当患者的侧弯超过35度时，肺活量通常低于预测正常值的40%。因此，当杜氏肌营养不良症男孩无法行走时，应考虑常规脊柱融合术。
