Hale Andrew T, Adelson P David, Albert Gregory W, Aldana Philipp R, Alden Tord D, Anderson Richard C E, Bauer David F, Bonfield Christopher M, Brockmeyer Douglas L, Chern Joshua J, Couture Daniel E, Daniels David J, Durham Susan R, Ellenbogen Richard G, Eskandari Ramin, George Timothy M, Grant Gerald A, Graupman Patrick C, Greene Stephanie, Greenfield Jeffrey P, Gross Naina L, Guillaume Daniel J, Heuer Gregory G, Iantosca Mark, Iskandar Bermans J, Jackson Eric M, Johnston James M, Keating Robert F, Leonard Jeffrey R, Maher Cormac O, Mangano Francesco T, McComb J Gordon, Meehan Thanda, Menezes Arnold H, O'Neill Brent, Olavarria Greg, Park Tae Sung, Ragheb John, Selden Nathan R, Shah Manish N, Smyth Matthew D, Stone Scellig S D, Strahle Jennifer M, Wait Scott D, Wellons John C, Whitehead William E, Shannon Chevis N, Limbrick David D
1Vanderbilt University School of Medicine, Medical Scientist Training Program, Nashville, Tennessee.
2Surgical Outcomes Center for Kids, Monroe Carell Jr. Children's Hospital of Vanderbilt University, Nashville, Tennessee.
J Neurosurg Pediatr. 2020 Mar 6;25(6):629-639. doi: 10.3171/2020.1.PEDS19493. Print 2020 Jun 1.
Factors associated with syrinx size in pediatric patients undergoing posterior fossa decompression (PFD) or PFD with duraplasty (PFDD) for Chiari malformation type I (CM-I) with syringomyelia (SM; CM-I+SM) are not well established.
Using the Park-Reeves Syringomyelia Research Consortium registry, the authors analyzed variables associated with syrinx radiological outcomes in patients (< 20 years old at the time of surgery) with CM-I+SM undergoing PFD or PFDD. Syrinx resolution was defined as an anteroposterior (AP) diameter of ≤ 2 mm or ≤ 3 mm or a reduction in AP diameter of ≥ 50%. Syrinx regression or progression was defined using 1) change in syrinx AP diameter (≥ 1 mm), or 2) change in syrinx length (craniocaudal, ≥ 1 vertebral level). Syrinx stability was defined as a < 1-mm change in syrinx AP diameter and no change in syrinx length.
The authors identified 380 patients with CM-I+SM who underwent PFD or PFDD. Cox proportional hazards modeling revealed younger age at surgery and PFDD as being independently associated with syrinx resolution, defined as a ≤ 2-mm or ≤ 3-mm AP diameter or ≥ 50% reduction in AP diameter. Radiological syrinx resolution was associated with improvement in headache (p < 0.005) and neck pain (p < 0.011) after PFD or PFDD. Next, PFDD (p = 0.005), scoliosis (p = 0.007), and syrinx location across multiple spinal segments (p = 0.001) were associated with syrinx diameter regression, whereas increased preoperative frontal-occipital horn ratio (FOHR; p = 0.007) and syrinx location spanning multiple spinal segments (p = 0.04) were associated with syrinx length regression. Scoliosis (HR 0.38 [95% CI 0.16-0.91], p = 0.03) and smaller syrinx diameter (5.82 ± 3.38 vs 7.86 ± 3.05 mm; HR 0.60 [95% CI 0.34-1.03], p = 0.002) were associated with syrinx diameter stability, whereas shorter preoperative syrinx length (5.75 ± 4.01 vs 9.65 ± 4.31 levels; HR 0.21 [95% CI 0.12-0.38], p = 0.0001) and smaller pB-C2 distance (6.86 ± 1.27 vs 7.18 ± 1.38 mm; HR 1.44 [95% CI 1.02-2.05], p = 0.04) were associated with syrinx length stability. Finally, younger age at surgery (8.19 ± 5.02 vs 10.29 ± 4.25 years; HR 1.89 [95% CI 1.31-3.04], p = 0.01) was associated with syrinx diameter progression, whereas increased postoperative syrinx diameter (6.73 ± 3.64 vs 3.97 ± 3.07 mm; HR 3.10 [95% CI 1.67-5.76], p = 0.003), was associated with syrinx length progression. PFD versus PFDD was not associated with syrinx progression or reoperation rate.
These data suggest that PFDD and age are independently associated with radiological syrinx improvement, although forthcoming results from the PFDD versus PFD randomized controlled trial (NCT02669836, clinicaltrials.gov) will best answer this question.
对于患有脊髓空洞症（SM；Chiari I型畸形合并脊髓空洞症，即CM-I+SM）且接受后颅窝减压术（PFD）或后颅窝减压联合硬脑膜成形术（PFDD）的儿科患者，与空洞大小相关的因素尚未完全明确。
作者利用帕克-里夫斯脊髓空洞症研究联盟登记系统，分析了接受PFD或PFDD治疗的CM-I+SM患者（手术时年龄<20岁）中与空洞影像学结果相关的变量。空洞消失定义为前后径（AP）≤2mm或≤3mm，或AP直径缩小≥50%。空洞缩小或进展的定义为：1）空洞AP直径变化（≥1mm），或2）空洞长度变化（头尾方向，≥1个椎体节段）。空洞稳定定义为空洞AP直径变化<1mm且空洞长度无变化。
作者确定了380例接受PFD或PFDD治疗的CM-I+SM患者。Cox比例风险模型显示，手术时年龄较小和PFDD与空洞消失独立相关，空洞消失定义为AP直径≤2mm或≤3mm，或AP直径缩小≥50%。PFD或PFDD后，影像学上的空洞消失与头痛改善（p<0.005）和颈部疼痛改善（p<0.011）相关。其次，PFDD（p=0.005）、脊柱侧弯（p=0.007）和空洞跨多个脊髓节段的位置（p=0.001）与空洞直径缩小相关，而术前额枕角比值（FOHR）增加（p=0.007）和空洞跨多个脊髓节段的位置（p=0.04）与空洞长度缩小相关。脊柱侧弯（风险比[HR]0.38[95%置信区间0.16-0.91]，p=0.03）和较小的空洞直径（5.82±3.38 vs 7.86±3.05mm；HR 0.60[95%置信区间0.34-1.03]，p=0.002）与空洞直径稳定相关，而术前较短的空洞长度（5.75±4.01 vs 9.65±4.31个节段；HR 0.21[95%置信区间0.12-0.38]，p=0.0001）和较小的pB-C2距离（6.86±1.27 vs 7.18±1.38mm；HR 1.44[95%置信区间1.02-2.05]，p=0.04）与空洞长度稳定相关。最后，手术时年龄较小（8.19±5.02 vs 10.29±4.25岁；HR 1.89[95%置信区间1.31-3.04]，p=0.01）与空洞直径进展相关，而术后空洞直径增加（6.73±3.64 vs 3.97±3.07mm；HR 3.10[95%置信区间1.67-5.76]，p=0.003）与空洞长度进展相关。PFD与PFDD与空洞进展或再次手术率无关。
这些数据表明，PFDD和年龄与影像学上空洞改善独立相关，尽管PFDD与PFD随机对照试验（NCT02669836，clinicaltrials.gov）即将得出的结果将最能回答这个问题。
