PDF(Pubmed)
Abstract：
UNASSIGNED: Ehlers-Danlos syndrome (EDS) is a connective tissue disorder that has been linked to several neurological problems including Chiari malformations, atlantoaxial instability (AAI), craniocervical instability (CCI), and tethered cord syndrome. However, neurosurgical management strategies for this unique population have not been well-explored to date. The purpose of this study is to explore cases of EDS patients who required neurosurgical intervention to better characterize the neurological conditions they face and to better understand how neurosurgeons should approach the management of these patients.
UNASSIGNED: A retrospective review was done on all patients with a diagnosis of EDS who underwent a neurosurgical operation with the senior author (FAS) between January 2014 and December 2020. Demographic, clinical, operative, and outcome data were collected, with additional radiographic data collected on patients chosen as case illustrations.
UNASSIGNED: Sixty-seven patients were identified who met the criteria for this study. The patients experienced a wide array of preoperative diagnoses, with Chiari malformation, AAI, CCI, and tethered cord syndrome representing the majority. The patients underwent a heterogeneous group of operations with the majority including a combination of the following procedures- suboccipital craniectomy, occipitocervical fusion, cervical fusion, odontoidectomy, and tethered cord release. The vast majority of patients experienced subjective symptomatic relief from their series of procedures.
UNASSIGNED: EDS patients are prone to instability, especially in the occipital-cervical region, which may predispose these patients to require a higher rate of revision procedures and may require modifications in neurosurgical management that should be further explored.
UNASSIGNED: A retrospective review was done on all patients with a diagnosis of EDS who underwent a neurosurgical operation with the senior author (FAS) between January 2014 and December 2020. Demographic, clinical, operative, and outcome data were collected, with additional radiographic data collected on patients chosen as case illustrations.
UNASSIGNED: Sixty-seven patients were identified who met the criteria for this study. The patients experienced a wide array of preoperative diagnoses, with Chiari malformation, AAI, CCI, and tethered cord syndrome representing the majority. The patients underwent a heterogeneous group of operations with the majority including a combination of the following procedures- suboccipital craniectomy, occipitocervical fusion, cervical fusion, odontoidectomy, and tethered cord release. The vast majority of patients experienced subjective symptomatic relief from their series of procedures.
UNASSIGNED: EDS patients are prone to instability, especially in the occipital-cervical region, which may predispose these patients to require a higher rate of revision procedures and may require modifications in neurosurgical management that should be further explored.
摘要：
■Ehlers-Danlos综合征（EDS）是一种结缔组织疾病，与包括Chiari畸形在内的几种神经系统疾病有关，寰枢椎不稳定性（AAI），颅颈不稳定（CCI），脊髓栓系综合征.然而,迄今为止,针对这一独特人群的神经外科治疗策略尚未得到很好的探讨.这项研究的目的是探索需要神经外科干预的EDS患者的病例，以更好地表征他们所面临的神经系统疾病，并更好地了解神经外科医生应如何处理这些患者的管理。
在2014年1月至2020年12月期间，所有诊断为EDS的患者均接受了高级作者（FAS）的神经外科手术。人口统计,临床,Operative,并收集了结果数据，并收集了作为病例说明的患者的其他影像学数据。
■确定了符合本研究标准的67名患者。患者经历了各种各样的术前诊断，Chiari畸形,AAI,CCI和脊髓栓系综合征占大多数。患者接受了一组异质性手术，其中大多数包括以下手术的组合-枕下颅骨切除术，枕颈融合术,颈椎融合术,齿状突切除术,和系绳释放。绝大多数患者经历了一系列手术的主观症状缓解。
■EDS患者容易出现不稳定，尤其是在枕颈区域，这可能使这些患者需要较高的翻修率，并且可能需要对神经外科治疗进行修改，应进一步探讨。
在2014年1月至2020年12月期间，所有诊断为EDS的患者均接受了高级作者（FAS）的神经外科手术。人口统计,临床,Operative,并收集了结果数据，并收集了作为病例说明的患者的其他影像学数据。
■确定了符合本研究标准的67名患者。患者经历了各种各样的术前诊断，Chiari畸形,AAI,CCI和脊髓栓系综合征占大多数。患者接受了一组异质性手术，其中大多数包括以下手术的组合-枕下颅骨切除术，枕颈融合术,颈椎融合术,齿状突切除术,和系绳释放。绝大多数患者经历了一系列手术的主观症状缓解。
■EDS患者容易出现不稳定，尤其是在枕颈区域，这可能使这些患者需要较高的翻修率，并且可能需要对神经外科治疗进行修改，应进一步探讨。
