Ohio State University Wexner Medical Center, Department of Neurosurgery, N1014 Doan Hall, 410 West 10th Avenue, Columbus, OH 43210, USA.
Ohio State University Wexner Medical Center, Department of Neurosurgery, N1014 Doan Hall, 410 West 10th Avenue, Columbus, OH 43210, USA.
J Clin Neurosci. 2014 Apr;21(4):685-8. doi: 10.1016/j.jocn.2013.07.007. Epub 2013 Oct 21.
The association between Klippel-Feil syndrome and vertebral artery dissection is quite rare. We report an adult patient with vertebral artery dissection and Klippel-Feil syndrome, to our knowledge only the third reported case of its kind. A 45-year-old woman with a known history of Klippel-Feil syndrome presented with occipital head and neck pain following forced neck extension. Diagnostic cerebral angiography revealed a high grade vertebral artery stenosis, consistent with vertebral artery dissection. Following 6 months of medical management, a repeat diagnostic angiogram revealed complete healing of the vessel. While cervical fusion, as seen in Klippel-Feil syndrome, has previously been shown to cause neurologic injury secondary to hypermobility, the association with vertebral artery dissection is incredibly rare. We hypothesize that this hypermobility places abnormal shear force on the vessel, causing intimal injury and dissection. Patients with seemingly spontaneous vertebral artery dissection may benefit from cervical spine radiography, and this predisposition to cerebrovascular injury strongly suggests further evaluation of vascular injury following trauma in patients with Klippel-Feil syndrome or other cervical fusion as clinically warranted.
Klippel-Feil 综合征与椎动脉夹层之间的关联非常罕见。我们报告了一例成人椎动脉夹层合并 Klippel-Feil 综合征患者，据我们所知，这是第三例此类病例。一名 45 岁女性，已知患有 Klippel-Feil 综合征，在强行伸展颈部后出现枕部头部和颈部疼痛。诊断性脑血管造影显示高度狭窄的椎动脉，符合椎动脉夹层。经过 6 个月的药物治疗，重复诊断性血管造影显示血管完全愈合。虽然 Klippel-Feil 综合征中所见的颈椎融合先前已被证明会因过度活动而导致神经损伤，但与椎动脉夹层的关联却非常罕见。我们假设这种过度活动会导致血管壁产生异常的剪切力，从而导致内膜损伤和夹层。看似自发性椎动脉夹层的患者可能受益于颈椎 X 线摄影，而这种对脑血管损伤的易感性强烈提示 Klippel-Feil 综合征或其他颈椎融合患者在有临床指征时，应进一步评估创伤后的血管损伤。
