1型神经纤维瘤病合并脊柱畸形患者出现营养不良特征是否会增加手术风险？
Does the presence of dystrophic features in patients with type 1 neurofibromatosis and spinal deformities increase the risk of surgery?
作者信息
Lykissas Marios G, Schorry Elizabeth K, Crawford Alvin H, Gaines Sean, Rieley Margaret, Jain Viral V
机构信息
Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
出版信息
Spine (Phila Pa 1976). 2013 Aug 15;38(18):1595-601. doi: 10.1097/BRS.0b013e31829a7779.
STUDY DESIGN
Retrospective chart and radiographical review.
OBJECTIVE
To present the demographics of patients with scoliosis and neurofibromatosis type 1 (NF-1), to record the incidence of dystrophic features, and to determine whether the presence of dystrophic features increase the risk of surgery in patients with NF-1 and associated spinal pathology.
SUMMARY OF BACKGROUND DATA
The most common of the osseous complications of NF-1 is spinal deformity, occurring in 10% to 30% of individuals with NF-1. Many of these patients will eventually require surgery for curve progression, which makes study of demographics and identification of features predicting the need for surgery essential in this patient population.
METHODS
A retrospective review was performed in patients with NF-1 and spinal deformities, followed in a multidisciplinary neurofibromatosis center. A subset of 56 patients with complete radiographical evaluation was reviewed for identification of risk factors for spine surgery.
RESULTS
One hundred thirty-one patients from a population of 694 patients with NF-1 (19%) had scoliosis. Mean age at diagnosis of scoliosis was 9 years (range; 1-17 yr). Scoliosis and need for surgery were equally distributed between males and females. In the group of 56 patients, 63% had 3 or more dystrophic features. The presence of 3 or more dystrophic features was the strongest predictor of the need for surgery (odds ratio = 14.34; P < 0.001). Individual features most predictive of need for surgery were the presence of vertebral scalloping (odds ratio = 13.19; P < 0.001) followed by the presence of dural ectasia (odds ratio = 6.38; P = 0.005). Patients with no dystrophic features were unlikely to progress to need for surgery.
CONCLUSION
Scoliosis and need for surgery were equally distributed between males and females. The presence of 3 or more dystrophic features was highly predictive of the need for surgery, with the most significant individual predictors being vertebral scalloping and dural ectasia. A combination of radiographical and MRI features can be used to predict need for spinal surgery.
LEVEL OF EVIDENCE
研究设计
回顾性病历及影像学评估。
目的
呈现脊柱侧弯合并1型神经纤维瘤病（NF-1）患者的人口统计学特征，记录营养不良性特征的发生率，并确定营养不良性特征的存在是否会增加NF-1合并脊柱病变患者的手术风险。
背景资料总结
NF-1最常见的骨并发症是脊柱畸形，在10%至30%的NF-1患者中出现。这些患者中的许多最终将因侧弯进展而需要手术，这使得对该患者群体的人口统计学研究以及识别预测手术需求的特征至关重要。
方法
对在多学科神经纤维瘤病中心随访的NF-1合并脊柱畸形患者进行回顾性研究。对56例有完整影像学评估的患者亚组进行分析，以确定脊柱手术的危险因素。
结果
694例NF-1患者中有131例（19%）患有脊柱侧弯。脊柱侧弯诊断时的平均年龄为9岁（范围：1 - 17岁）。脊柱侧弯及手术需求在男性和女性中分布相同。在56例患者组中，63%有3种或更多营养不良性特征。存在3种或更多营养不良性特征是手术需求的最强预测因素（优势比 = 14.34；P < 0.001）。最能预测手术需求的个体特征是椎体扇贝样变（优势比 = 13.19；P < 0.001），其次是硬脊膜扩张（优势比 = 6.38；P = 0.005）。没有营养不良性特征的患者不太可能进展到需要手术。
结论
脊柱侧弯及手术需求在男性和女性中分布相同。存在3种或更多营养不良性特征高度预测手术需求，最显著的个体预测因素是椎体扇贝样变和硬脊膜扩张。影像学和MRI特征的组合可用于预测脊柱手术需求。
证据级别
3级。
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