Ciaramitaro Palma, Migliaretti Giuseppe, Ferraris Marilena, Garnero Andrea, Morana Giovanni, Carucci Paolo, Stura Ilaria, Massaro Fulvio, Garbossa Diego
Neuroscience Department, University of Torino, 10126 Torino, Italy.
CRESSC, AOU Città della Salute e della Scienza di Torino, 10126 Torino, Italy.
J Clin Med. 2023 Apr 21;12(8):3019. doi: 10.3390/jcm12083019.
Syringomyelia (Syr) in patients with Chiari 1 malformation (CM1) may be attributable to abnormal dynamics of cerebrospinal fluid (CSF) in the upper cervical segment; fourth ventricle enlargement has been reported in association with a worse clinical and radiological presentation, independently of the posterior fossa volume. In this study, we analyzed presurgery hydrodynamic markers to evaluate if their changes could be associated with clinical and radiological improvement after posterior fossa decompression and duraplasty (PFDD). As a primary endpoint, we aimed to correlate improvement in the fourth ventricle area with positive clinical outcomes.
In total, in this study, we enrolled 36 consecutive adults with Syr and CM1 who were followed by a multidisciplinary team. All the patients were prospectively evaluated with clinical scales and neuroimaging, including CSF flow, the fourth ventricle area, and the Vaquero Index by using a phase-contrast MRI before (T0) and after surgical treatment (T1-Tlast, with a range of 12-108 months). The CSF flow at the craniocervical junction (CCJ), the fourth ventricle area, and the Vaquero Index changes were statistically analyzed and compared to the clinical and quality of life improvement after surgery. The good outcome prediction ability of presurgical radiological variables was tested.
Surgery was associated with positive clinical and radiological outcomes in more than 90% of cases. The fourth ventricle area significantly reduced after surgery (T0-Tlast, = 0.0093), but no significant associations with clinical improvement were found. The presurgical presence of CSF flow at the CCJ was able to predict a good outcome (AUC = 0.68, 95% CI 0.50-0.87 and LH+ = 2.1, IC 95% 1.16-3.07) and was also significantly associated with post-surgical pain relief (rho = 0.61 and = 0.0144).
Presurgery CSF flow at the CCJ is proposed as a radiological marker with the ability to predict a positive outcome after PFDD in adults with syringomyelia and CM1. Measurements of the fourth ventricle area could be useful additional information for evaluating surgical long-term follow-up; further experience on larger cohorts is required to better define the prognostic yield of this radiological parameter.
Chiari 1型畸形（CM1）患者的脊髓空洞症（Syr）可能归因于上颈段脑脊液（CSF）动力学异常；有报道称第四脑室扩大与更差的临床和影像学表现相关，且与后颅窝容积无关。在本研究中，我们分析了术前流体动力学标志物，以评估其变化是否与后颅窝减压及硬脑膜成形术（PFDD）后的临床和影像学改善相关。作为主要终点，我们旨在将第四脑室面积的改善与积极的临床结果相关联。
在本研究中，我们共纳入了36例连续的患有Syr和CM1的成年人，由多学科团队进行随访。所有患者在手术治疗前（T0）和后（T1 - T末次，范围为12 - 108个月）均通过临床量表和神经影像学进行前瞻性评估，包括CSF流动、第四脑室面积以及使用相位对比MRI测量的Vaquero指数。对颅颈交界区（CCJ）的CSF流动、第四脑室面积和Vaquero指数变化进行统计学分析，并与术后临床和生活质量改善情况进行比较。测试术前放射学变量的良好结局预测能力。
超过90%的病例手术与积极的临床和影像学结果相关。术后第四脑室面积显著减小（T0 - T末次， = 0.0093），但未发现与临床改善有显著关联。术前CCJ处存在CSF流动能够预测良好结局（AUC = 0.68，95% CI 0.50 - 0.87，LH + = 2.1，IC 95% 1.16 - 3.07），并且还与术后疼痛缓解显著相关（rho = 0.61， = 0.0144）。
术前CCJ处的CSF流动被提议作为一种放射学标志物，能够预测患有脊髓空洞症和CM1的成年人PFDD术后的积极结局。第四脑室面积的测量对于评估手术长期随访可能是有用的额外信息；需要在更大队列中积累更多经验，以更好地确定该放射学参数的预后价值。
