40. ehers - danlos综合征颈椎融合术后结果：较低的不愈合，但较高的吞咽困难和脑脊液漏率

IF 2.5 Q3 Medicine

North American Spine Society Journal Pub Date : 2025-07-01 DOI:10.1016/j.xnsj.2025.100734

Logan Karlen BS , Liliane Luu BS , Kushagra Verma MD, MS

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引用次数: 0

摘要

tehlers - danlos综合征（EDS）是一种遗传性结缔组织疾病，表现为关节过度活动、皮肤过度弹性和血管脆弱。这种全身性病理可能会影响手术结果，尤其是颈椎融合，可能会增加术后不愈合、吞咽困难和脑脊液（CSF）泄漏等并发症的风险。颈椎融合术是治疗脊柱退行性病变的常用手术；然而，关于EDS患者术后预后的数据很少。目的：本研究探讨EDS患者和非EDS患者行颈椎融合术后不愈合、吞咽困难和脑脊液漏的发生率。研究设计/设置n /患者样本/结果测量测量的结果是EHS和非EHS患者之间不愈合、吞咽困难和CSF的可能性，其百分比与风险差异和p值相关。方法采用TriNetX进行回顾性队列研究，研究术后不愈合、吞咽困难和脑脊液泄漏的发生率。年龄在18-65岁的患者被分为EDS （ICD-10: Q79.6, n=527）和非EDS （n=73,130）两组。患有糖尿病、吸烟、癌症、慢性类固醇使用和其他结缔组织疾病的患者被排除在外。数据收集参数采用骨不连、吞咽困难和脑脊液渗漏的ICD-10编码（分别为M96.0、R13.10和G96.0）。ICD-10代码也必须添加到患者术后3年内的遭遇中，才能纳入研究。结果EDS患者的骨不连率（11.195%）明显低于非EDS患者（16.663%）（p=0.0008）。此外，EDS患者的吞咽困难发生率（11.006%）高于非EDS患者（7.201%）（p<0.0008）。最后，EDS患者脑脊液漏发生率（3.605%）高于非EDS患者（0.655%）（p<0.0001）。结论：无论结缔组织是否异常，接受颈椎融合手术的eds患者的不愈合率明显低于非eds患者。这可能是外部影响的结果，如外科医生选择偏差、创新的固定方法或与关节过度活动相关的骨代谢先天差异。相比之下，EDS患者表现出明显更高的吞咽困难和脑脊液泄漏率，强调EDS病理对手术结果的影响。EDS患者吞咽困难的增加可能与自主神经功能障碍有关，其关节活动过度容易表现为迷走神经收缩，导致吞咽和胃肠运动障碍。有趣的是，尽管吞咽困难通常与颈椎融合手术相关，但EDS患者的吞咽困难率与颈椎和腰椎融合手术相似。这表明一个系统性因素可能与自主神经功能障碍有关，而不是颈椎手术的机械后果。由于胶原合成缺陷引起的硬脑膜脆性，EDS患者脑脊液泄漏的可能性增加。本研究的一个弱点是ICD-10在区分颈椎前路融合和后路融合患者百分比方面的局限性。EDS不同手术入路的分层可以进一步研究。根据所讨论的结果，对EDS患者进行颈椎融合手术入路、术前咨询和术后监测提供特殊的考虑是有益的，以优化结果并减少并发症。FDA器械/药物状态本摘要不讨论或包括任何适用的器械或药物。

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40. Postoperative outcomes in cervical fusion for Ehlers-Danlos syndrome: lower nonunion but higher dysphagia and CSF leak rates

BACKGROUND CONTEXT

Ehlers-Danlos syndrome (EDS) is a genetic connective tissue disorder that manifests as joint hypermobility, skin hyperelasticity, and vascular fragility. This systemic pathology may affect surgical outcomes, most notably cervical fusions, by potentially increasing the risks of complications namely nonunion, dysphagia and cerebrospinal fluid (CSF) leaks postoperatively. Cervical fusions are a common procedure to address degenerative spine pathologies; however, there is minimal data on postoperative outcomes in EDS patients.

PURPOSE

This study investigates the rates of nonunion, dysphagia, and CSF leak in EDS patients and non-EDS patients who underwent cervical fusion procedures.

STUDY DESIGN/SETTING

N/A

PATIENT SAMPLE

N/A

OUTCOME MEASURES

The outcomes measured were the possibility of nonunion, dysphagia, and CSF between EHS and non-EHS patients in percentages associated with the risk difference and p-value.

METHODS

Using TriNetX, a retrospective cohort study was performed to study the rates of postoperative nonunion, dysphagia, and CSF leak. Patients aged 18-65 were categorized into EDS (ICD-10: Q79.6, n=527) and non-EDS (n=73,130) cohorts. Those with diabetes, tobacco use, cancer, chronic steroid use, and other connective tissue disorders were excluded. ICD-10 codes for nonunion, dysphagia, and CSF leaks (M96.0, R13.10, and G96.0, respectively) were utilized as data collection parameters. ICD-10 codes must also be added to the patient’s encounter within 3 years postoperative to be included in the study.

RESULTS

The nonunion rate is notably lower in EDS patients (11.195%) compared to non-EDS patients (16.663%) (p=0.0008). Additionally, the occurrence of dysphagia was seen to be higher in EDS patients (11.006%) when compared to non-EDS patients (7.201%) (p<0.0008). Finally, CSF leak in EDS patients (3.605%) is higher than non-EDS patients (0.655%) (p<0.0001).

CONCLUSIONS

EDS patients who underwent cervical fusion procedures had notably lower nonunion rates than non-EDS patients, regardless of the abnormalities within their connective tissues. This is potentially a result of external influences akin to surgeon selection bias, innovative fixation approaches, or innate differences in bone metabolism associated with joint hypermobility. In contrast, EDS patients exhibited significantly higher rates of dysphagia and CSF leaks, emphasizing the impact EDS pathology has on surgical outcomes. Increased dysphagia in EDS patients may be connected to their autonomic dysfunction, as their joint hypermobility tends to manifest vagus nerve constriction, resulting in swallowing and gastrointestinal motility deficits. Interestingly, dysphagia rates among EDS patients appear comparable between cervical and lumbar fusions, despite dysphagia traditionally being more associated with cervical fusion procedures. This suggests a systemic factor potentially linked to autonomic dysfunction, rather than a mechanical consequence of cervical surgery. EDS patients may have an increased likelihood of CSF leaks due to dura mater fragility caused by defective collagen synthesis. A weakness of this study is the ICD-10 limitations in distinguishing between the percentage of patients with anterior versus posterior cervical fusion. The stratification of different surgical approaches in EDS can be further studied. Based on the findings discussed, there is a benefit to providing special considerations to surgical approaches, preoperative counseling, and postoperative monitoring for EDS patients undergoing cervical fusions to optimize outcomes and minimize complications.