伴有脊髓空洞症和脊柱侧弯的Ⅰ型阿诺德-基亚里畸形：扁桃体下移、脊髓空洞形态与侧弯特征之间的影像学相关性：一项回顾性研究
Type I Arnold Chiari Malformation with Syringomyelia and Scoliosis: Radiological Correlations between Tonsillar Descent, Syrinx Morphology and Curve Characteristics: A Retrospective Study.
作者信息
Shanmugasundaram Sivaraj, Viswanathan Vibhu Krishnan, Shetty Ajoy Prasad, Rai Nimish, Hajare Swapnil, Kanna Rishi Mukesh, Rajasekaran Shanmuganathan
机构信息
Department of Orthopaedic Spine, Manipal Hospital, Salem, India.
Department of Spine Surgery, Ganga Medical Center and Hospital, Coimbatore, India.
出版信息
Asian Spine J. 2023 Feb;17(1):156-165. doi: 10.31616/asj.2021.0483. Epub 2022 Jul 4.
STUDY DESIGN
Retrospective cohort.
PURPOSE
The current study was planned to evaluate deformity characteristics, assess relationship between morphology of syrinx/Arnold Chiari malformation (ACM) and deformity, analyze effect of posterior fossa decompression (PFD), and evaluate outcome.
OVERVIEW OF LITERATURE
Scoliosis in ACM-I and syringomyelia (SM) is uncommon, and deformity characteristics differ from those seen in idiopathic scoliosis.
METHODS
Data regarding patients, who underwent PFD for ACM-I presenting with SM and scoliosis between January 2009 and December 2018, were retrospectively collected. Only patients with 2-year follow-up were included. Sagittal/coronal deformity and sagittal spinopelvic parameters were examined. Symmetry and extent of tonsillar descent, as well as morphology (configuration/variation) and extent of syrinx were determined.
RESULTS
A total of 42 patients (20 females; age: 14.2±5.8 years) were included; 35 patients (83.3%) had atypical curves. Mean preoperative coronal Cobb was 57.7°±20.9°; and 12 (28.6%) had significant coronal imbalance. Tonsillar descent was classified as grade 1, 2, and 3 in 16 (38.1%), 11 (26.2%), and 15 (35.7%) patients; 35 patients (83.3%) had asymmetric tonsillar descent; 17 (40.4%), 3 (7.1%), 16 (38.1%), and 6 (14.4%) had circumscribed, moniliform, dilated, and slender syrinx patterns; and 9 (21.4%), 12 (28.6%), and 21 (50%) of syrinx were right-sided, left-sided, and centric. There was no significant relationship between side of tonsillar dominance (p =0.31), grade of descent (p =0.30), and convexity of deformity. There was significant association between side of syrinx and convexity of scoliosis (p =0.01). PFD was performed in all, and deformity correction was performed in 23 patients. In curves ≤40°, PFD alone could stabilize scoliosis progression (p =0.02). There was significant reduction in syrinx/cord ratio following PFD (p <0.001).
CONCLUSIONS
ACM-I+SM patients had atypical curve patterns in 83% of cases, and the side of syrinx deviation correlates with scoliosis convexity. Syrinx shrinks significantly following PFD. PFD may not stabilize scoliosis in curves >40°.
研究设计
回顾性队列研究。
目的
本研究旨在评估畸形特征，评估脊髓空洞症/阿诺德-基亚里畸形（ACM）形态与畸形之间的关系，分析后颅窝减压术（PFD）的效果，并评估预后。
文献综述
I型阿诺德-基亚里畸形合并脊髓空洞症（SM）中的脊柱侧弯并不常见，其畸形特征与特发性脊柱侧弯不同。
方法
回顾性收集2009年1月至2018年12月期间因I型阿诺德-基亚里畸形合并脊髓空洞症和脊柱侧弯而接受后颅窝减压术的患者数据。仅纳入有2年随访资料的患者。检查矢状面/冠状面畸形和矢状面脊柱骨盆参数。确定扁桃体下疝的对称性和程度，以及脊髓空洞症的形态（形态/变异）和范围。
结果
共纳入42例患者（20例女性；年龄：14.2±5.8岁）；35例患者（83.3%）有非典型曲线。术前平均冠状面Cobb角为57.7°±20.9°；12例（28.6%）有明显的冠状面失平衡。16例（38.1%）患者的扁桃体下疝分为1级、11例（26.2%）为2级、15例（35.7%）为3级；35例患者（83.3%）有不对称的扁桃体下疝；17例（40.4%）、3例（7.1%）、16例（38.1%）和6例（14.4%）分别有局限性、串珠状、扩张型和细长型脊髓空洞症模式；脊髓空洞症9例（21.4%）、12例（28.6%）和21例（50%）分别位于右侧、左侧和中央。扁桃体优势侧（p =0.31）、下疝分级（p =0.30）与畸形凸度之间无显著关系。脊髓空洞症侧与脊柱侧弯凸度之间存在显著关联（p =0.01）。所有患者均行后颅窝减压术，23例患者进行了畸形矫正。在曲线≤40°的患者中，单纯后颅窝减压术可稳定脊柱侧弯进展（p =0.02）。后颅窝减压术后脊髓空洞症/脊髓比值显著降低（p <0.001）。
结论
83%的I型阿诺德-基亚里畸形合并脊髓空洞症患者有非典型曲线模式，脊髓空洞症偏离侧与脊柱侧弯凸度相关。后颅窝减压术后脊髓空洞症明显缩小。后颅窝减压术可能无法稳定曲线>40°的脊柱侧弯。
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