Ⅰ型神经纤维瘤病伴萎缩性上胸段脊柱侧凸的冠状面影像学分型

A coronal imaging classification of neurofibromatosis type Ⅰ with dystrophic upper thoracic scoliosis

中文关键词:  Ⅰ型神经纤维瘤病  萎缩性脊柱侧凸  上胸段侧凸  冠状面分型  可信度

英文关键词:Neurofibromatosis type Ⅰ  Dystrophic scoliosis  Upper thoracic scoliosis  Coronal classification  Reliability

中文摘要:

  【摘要】目的：对Ⅰ型神经纤维瘤病伴萎缩性上胸段脊柱侧凸（dystrophic upper thoracic scoliosis with neurofibromatosis type 1，DUTS-NF1）进行冠状面影像学分型，验证其可信度与可重复性，探讨其临床意义。方法：回顾性分析2009年6月~2023年12月期间我院数据库中诊断为Ⅰ型神经纤维瘤病伴萎缩性脊柱侧凸患者的资料，从中筛选出主弯顶椎位于上胸椎（T1~T5）的患者，根据站立位全脊柱正位X线片上脊柱侧凸的冠状面形态分为：A型，肩颈型；B型，远端弯代偿型；C型，躯干倾斜型；测量各型患者的侧凸Cobb角、上胸段后凸角、锁骨角（clavicle angle，CA）、T1倾斜角（T1 tilt）、颈部倾斜角（neck tilt，NT）、头部偏移距离（head shift，HS）、冠状面平衡距离（coronal balanced distance，CBD），计算畸形角率（deformity angular ratio，DAR）。3位脊柱外科医师经过分型设计者专门培训后根据该冠状面分型方法独立进行两次分型，应用Kappa值对同一观察者两次分型结果进行可重复性分析，对不同观察者间分型结果进行可信度分析。结果：从367例Ⅰ型神经纤维瘤病伴萎缩性脊柱侧凸患者中共筛选出29例DUTS-NF1患者（7.9%），其萎缩性主弯Cobb角为78.7°±12.9°。分型设计者的分型结果A型16例，B型8例，C型5例。B型和C型的DAR显著性高于A型（20.6±2.2和20.0±3.0 vs 13.2±1.8，P<0.001）；C型相对于A型存在更为显著的头部及冠状面偏移距离（HS：27.6±11.7mm vs 13.5±6.7mm，P<0.001；CBD：34.8±20.5mm vs 13.9±10.9mm，P<0.001）；C型T1 tilt显著性大于A型（P<0.05）；其余影像学指标三型间无统计学差异（P>0.05）。3位观察者使用DUTS-NF1冠状位影像学分型方法共进行174次（29例×3×2次）分型，包括A型96次，B型45次，C型33次，观察者内分型一致率为（82.57±8.44）%，Kappa值为0.771～0.81，属于“基本可信”；观察者间分型一致率为（84.19±8.65）%，Kappa值为0.884～0.886，属于“完全可信”。结论：根据冠状面影像学特征可将DUTS-NF1患者分为肩颈型、远端弯代偿型、躯干倾斜型三型，该分型方法具有较高的可重复性与可信度，可为临床提供诊疗决策依据。

英文摘要:

  【Abstract】 Objectives: To propose a coronal imaging classification of neurofibromatosis type Ⅰ with dystrophic upper thoracic scoliosis(DUTS-NF1), to verify its reliability and reproducibility, and to explore its clinical significance. Methods: We retrospectively analyzed the data of patients diagnosed with neurofibromatosis type Ⅰ with dystrophic spinal scoliosis in the database of our hospital from June 2009 to December 2023. The patients whose main curve apex located in the upper thoracic vertebrae(T1-T5) were screen out and were classified according to the coronal scoliosis pattern on standing spinal X-rays: Type A, shoulder-neck type; Type B, distal thoracic compensatory curve type; Type C, trunk inclination type. The Cobb angle, upper thoracic kyphosis angle, clavicle angle(CA), T1 tilt, neck tilt(NT), head shift(HS), coronal balanced distance(CBD), and deformity angular ratio(DAR) were measured and calculated for each type of patients. Three spine surgeons, who had been specially trained, independently performed two classifications according to the method. The results of the two classifications of the same observer were analyzed for reproducibility by applying the Kappa value, and the results of the classification were analyzed for confidence between different observers. Results: 29 patients(7.9%) with DUTS-NF1 were identified from the 367 NF1 patients with dystrophic scoliosis, and the main Cobb angle was 78.7°±12.9°. The classification results of the designer were 16 patients of type A, 8 of type B, and 5 of type C. The DAR of type B and type C was significantly higher than that of type A(20.6±2.2, 20.0±3.0 vs 13.2±1.8, P<0.001); Type C had more significant head and coronal shift distances than type A(HS: 27.6±11.7mm vs 13.5±6.7mm, P<0.001; CBD: 34.8±20.5mm vs 13.9±10.9mm, P<0.001); The T1 tilt of type C was significantly higher than that of type A（P<0.05）; The other imaging parameters were not statistically different between the three types(P>0.05). 174 times of classifications were performed by the 3 observers using the DUTS-NF1 coronal imaging classification method, including 96 classifications of type A, 45 classifications of type B, and 33 classifications of type C. The intra-observer classification concordance rate was(82.57±8.44)%, with a Kappa value of 0.771-0.81, which was considered to be "Basically reliable"; The inter-observer classification concordance rate was(84.19±8.65)%, with a Kappa value of 0.884-0.886, which was considered to be "Completely reliable". Conclusions: Based on the coronal imaging features, patients with DUTS-NF1 can be classified into three types of shoulder-neck type, distal thoracic compensatory curve type, and trunk inclination type. This classification system has satisfied reproducibility and reliability, which is helpful for assessing the coronal compensation of DUTS-NF1 patients, and thus guides the clinical diagnosis and treatment.