Troussier Sébastien, Milleron Olivier, Rannou François, Eliahou Ludivine, Jondeau Guillaume, Nguyen Christelle
AP-HP.Centre-Université Paris Cité, Service de Rééducation et de Réadaptation de l'Appareil Locomoteur et des Pathologies du Rachis, Hôpital Cochin, Paris, 75014, France.
AP-HP.Nord-Université Paris Cité, Service de Cardiologie, Hôpital Bichat, Paris, 75018, France.
Eur Spine J. 2025 May 31. doi: 10.1007/s00586-025-08911-z.
To compare frequencies and characteristics of painful symptoms, spine-specific activity limitations and health-related quality of life, between individuals with Marfan syndrome with dural ectasia, and those without.
We conducted a cross-sectional comparative study. All individuals with Marfan syndrome followed in a single center were screened. The presence of dural ectasia was assessed using CT-scan or magnetic resonance imaging. Endpoints were the frequencies and characteristics of painful symptoms, spine-specific activity limitations and health-related quality of life.
Overall, 90 individuals were included: 86 (96%) had FBN1 mutations, 55 (61%) had dural ectasia and 35 (39%) had not. Mean age was 39.3 (9.4) years, 45 (50%) were women and 80 (89%) had back pain. The frequencies of headache with upright posture and of pain in the lower back when coughing, laughing and/or sneezing were numerically higher in participants with dural ectasia (49% vs. 26% and 13% vs. 0%, respectively). We found no difference between the 2 groups for health-related quality of life.
Individuals with Marfan syndrome and dural ectasia, as compared to individuals with Marfan syndrome without dural ectasia, could display a specific pattern of painful symptoms, including lower back pain and headache with upright posture. However, none of the comparisons performed reached statistically significant difference.
比较患有硬脊膜扩张的马凡综合征患者与未患该病症的患者之间疼痛症状的频率和特征、脊柱特定活动受限情况以及健康相关生活质量。
我们进行了一项横断面比较研究。对在单一中心随访的所有马凡综合征患者进行筛查。使用CT扫描或磁共振成像评估硬脊膜扩张的存在情况。研究终点为疼痛症状的频率和特征、脊柱特定活动受限情况以及健康相关生活质量。
总体而言，共纳入90名患者：86名（96%）有FBN1基因突变，55名（61%）有硬脊膜扩张，35名（39%）没有。平均年龄为39.3（9.�）岁，45名（50%）为女性，80名（89%）有背痛。硬脊膜扩张患者出现直立位头痛以及咳嗽、大笑和/或打喷嚏时下背痛的频率在数值上更高（分别为49%对26%和13%对0%）。我们发现两组在健康相关生活质量方面没有差异。
与没有硬脊膜扩张的马凡综合征患者相比，患有硬脊膜扩张患者可能表现出特定模式的疼痛症状包括下背痛以及直立位头痛。然而，所进行的所有比较均未达到统计学显著差异。
