Pediatric Orthopedics Dept, La Timone Children's Hospital, 264, rue St.-Pierre, 13005 Marseille, France.
Orthop Traumatol Surg Res. 2010 Nov;96(7):741-7. doi: 10.1016/j.otsr.2010.06.002. Epub 2010 Sep 15.
Congenital scoliosis, carrying an incidence between 0.5 and 1 per 1000 births, raise the problem of their evolutive potential.
Some predictive factors for the evolution of scoliotic curvature due to congenital vertebral malformation (CVM) can be found.
This was a retrospective multicenter study of 251 patients, at least 14 years old when evaluated at end of follow-up, with CVM and spinal deformity predominating in the frontal plane.
38.8% of patients showed associated neurologic, visceral or orthopedic abnormalities. CVM was single in 60.6%, double in 20.3%, triple in 6.4% and multiple in 12.7% of cases. 34.1% of CVMs were thoracic. Congenital scoliosis curvature was single in 88.8% of patients, double in 10% and triple in 1.2%. Mean curvature angle was 31.7° at diagnosis (range, 0-105°) and 41.3° preoperatively (range, 10-105°). Sixty-one patients showed associated kyphosis. Mean change in postoperative curvature angle over follow-up was 1.6° (range, -20° to 38°) in the 73 patients managed by arthrodesis, -0.4° (-24° to 30°) in the 64 managed by epiphysiodesis, and 0.4° (-18° to 35°) in the 49 managed by hemivertebral (HV) resection. Results were found to correlate significantly with age at surgery for patients managed by epiphysiodesis, but not for those managed by HV resection or arthrodesis.
More than 30% of congenital scolioses involve associated intraspinal abnormality. All CVM patients should therefore undergo medullary and spinal MRI to assess the CVM in all three planes, and the medullary canal and its content. The evolution of scoliotic curvature induced by CVM is hard to predict. Several factors are to be taken into account: CVM type, number and location, and patient age. Curvature progression may be slow or very fast. It accelerates during the peak of puberty, stabilizing with bone maturity. Surgery is mandatory in evolutive scoliosis. Four procedures may be recommended, according to type of CVM and especially to patient age: arthrodesis, convex epiphysiodesis, HV resection or rib distraction. Surgery seeks to correct the spinal deformity induced by the CVM and prevent compensatory curvature and neurologic complications, while conserving sagittal and frontal spinal balance and sparing as many levels as possible. In case of HV involvement, the procedure of choice is CVM resection, which provides 87.5% good results in this indication; the procedure is relatively safe, conservative of spinal levels, and without age limit.
Level IV. Retrospective study.
先天性脊柱侧凸的发病率在每 1000 例出生儿中为 0.5 至 1 例，其存在潜在的进展性问题。
某些与先天性椎体畸形（CVM）相关的脊柱侧凸进展的预测因素可以被发现。
这是一项回顾性多中心研究，共纳入 251 例至少在随访结束时年龄大于 14 岁的患者，这些患者存在以冠状面为主的 CVM 和脊柱畸形。
38.8%的患者存在神经、内脏或骨科合并症。60.6%的患者 CVM 为单发，20.3%为双发，6.4%为多发，12.7%为多发。34.1%的 CVM 位于胸椎。88.8%的患者脊柱侧凸为单发，10%为双发，1.2%为多发。诊断时的平均曲率角度为 31.7°（范围，0°至 105°），术前为 41.3°（范围，10°至 105°）。61 例患者存在合并性后凸。在接受融合手术的 73 例患者中，术后曲率角度的平均变化为 1.6°（范围，-20°至 38°），在接受骺板阻滞术的 64 例患者中为-0.4°（范围，-24°至 30°），在接受半椎体切除术的 49 例患者中为 0.4°（范围，-18°至 35°）。结果发现，对于接受骺板阻滞术的患者，年龄与手术结果显著相关，但对于接受半椎体切除术或融合手术的患者则不然。
超过 30%的先天性脊柱侧凸涉及椎管内的异常。因此，所有 CVM 患者均应接受脊髓和脊柱 MRI 检查，以评估 CVM 在所有三个平面上的情况，以及脊髓管及其内容物。由 CVM 引起的脊柱侧凸进展很难预测。需要考虑多个因素：CVM 类型、数量和位置，以及患者年龄。脊柱侧凸的进展可能缓慢或非常迅速。它在青春期高峰时加速，在骨骼成熟时稳定。进展性脊柱侧凸需要手术治疗。根据 CVM 的类型，尤其是患者的年龄，可推荐四种手术方法：融合术、凸侧骺板阻滞术、半椎体切除术或肋骨撑开术。手术的目的是矫正由 CVM 引起的脊柱畸形，预防代偿性脊柱侧凸和神经并发症，同时保持矢状面和冠状面脊柱平衡，并尽可能保留更多的脊柱节段。在存在半椎体的情况下，首选的手术方法是 CVM 切除术，这种方法在该适应证中提供 87.5%的良好效果；该手术方法相对安全，保留了脊柱节段，没有年龄限制。
IV 级。回顾性研究。
