Clarke Jamie E, Reyes Joshua M, Luther Evan, Govindarajan Vaidya, Leuchter Jessica D, Niazi Toba, Ragheb John, Wang Shelly
Leonard M. Miller School of Medicine, University of Miami, Miami, FL, USA.
Department of Neurosurgery, University of Miami, Miami, FL, USA.
World Neurosurg X. 2023 Mar 5;18:100173. doi: 10.1016/j.wnsx.2023.100173. eCollection 2023 Apr.
Chiari malformation type I (CMI) is relatively common neurosurgical condition typically treated with posterior fossa decompression. However, the management of CMI in patients with heritable connective tissue disorders (CTDs), such as Ehlers-Danlos Syndrome, Marfan Syndrome, or Osteogenesis Imperfecta, involves a unique set of perioperative challenges.
This study aims to define the demographic information, comorbidities, and perioperative course of patients with concomitant CMI and CTD.
Patients with CMI admitted for surgical decompression from 2008 to 2015 were captured using the National Inpatient Sample (NIS). Information was collected based on ICD-9 codes. Descriptive and regression analyses were performed in SPSS (version 26).
38,169 CMI patients, 353 of whom had CTD (0.92%), were identified. CMI patients with CTD were more likely to be female ( < 0.001) and present during teenage ( = 0.033) or young adult years ( < 0.001). They had more chronic issues ( < 0.001): systemic comorbidities include postural orthostatic tachycardia syndrome, cardiac dysrhythmias, and gastroparesis (all < 0.001). CNS comorbidities include migraine, tethered spinal cord, and epilepsy (all < 0.001). They have increased joint instability (both < 0.001), as well as craniocervical instability (CCI). More posterior cervical fusion surgeries and application of cervical halo devices were seen during the same inpatient stay (both < 0.001).
Patients with concurrent CTD and CMI were more likely to present with complex Chiari and associated CCI. They were also younger, more often female, and had more systemic, CNS, and joint abnormalities. As such, preoperative recognition of an underlying CTD is imperative to achieve optimal outcomes in this patient population.
I型Chiari畸形（CMI）是一种相对常见的神经外科疾病，通常采用后颅窝减压术治疗。然而，对于患有遗传性结缔组织疾病（CTD）的患者，如埃勒斯-当洛综合征、马凡综合征或成骨不全症，CMI的治疗在围手术期面临一系列独特的挑战。
本研究旨在明确合并CMI和CTD患者的人口统计学信息、合并症及围手术期病程。
利用国家住院患者样本（NIS）收集2008年至2015年因手术减压入院的CMI患者。基于ICD-9编码收集信息。在SPSS（版本26）中进行描述性和回归分析。
共识别出38169例CMI患者，其中353例患有CTD（0.92%）。合并CTD的CMI患者更可能为女性（P<0.001），且在青少年期（P = 0.033）或青年期（P<0.001）发病。他们有更多慢性问题（P<0.001）：全身合并症包括体位性直立性心动过速综合征、心律失常和胃轻瘫（均P<0.001）。中枢神经系统合并症包括偏头痛、脊髓拴系和癫痫（均P<0.001）。他们的关节不稳定性增加（均P<0.001），以及颅颈交界区不稳定（CCI）。在同一住院期间，进行后路颈椎融合手术和应用颈椎 Halo 装置的情况更多见（均P<0.001）。
合并CTD和CMI的患者更易出现复杂的Chiari畸形及相关的CCI。他们年龄更小，女性更常见，且有更多的全身、中枢神经系统和关节异常。因此，术前识别潜在CTD对于在该患者群体中取得最佳治疗效果至关重要。
