克-费综合征中齿状突与第三颈椎的先天性融合
Congenital Fusion of Dens to T3 Vertebra in Klippel-Feil Syndrome.
作者信息
Verla Terence, Prablek Marc, Ropper Alexander E, Xu David S, Raber Michael
机构信息
Department of Neurosurgery, Baylor College of Medicine, Houston, Texas, USA.
Department of Neurosurgery, Baylor College of Medicine, Houston, Texas, USA.
出版信息
World Neurosurg. 2020 Nov;143:18-22. doi: 10.1016/j.wneu.2020.06.232. Epub 2020 Jul 9.
BACKGROUND
Patients with Klippel-Feil syndrome may present with neurologic complaints such as neck pain, radiculopathy and gait instability. Here we describe surgical management of a patient with congenital fusion of the occipital-cervical region and also block circumferential fusion of dens to T3 with spinal cord compression. This report is the first of its kind with such extensive fusion.
CASE DESCRIPTION
Our patient was a 56 year-old female, who presented with neck pain and tingling in all extremities. On exam, she had a short neck, prominent jaw with extremely limited range of motion in neck and features of myelopathy. CT showed fusion of the dens to T3 vertebrae. Patient underwent sub-occipital craniectomy, C1 laminectomy and Occiput to T5 posterior fixation and fusion with neurologic improvement.
CONCLUSION
This is the first reported case of Klippel-Feil syndrome with fusion of all cervical vertebrae down to T3. We recommend surgery for advanced cases of myelopathy or radiculopathy due to stenosis and spinal instability.
背景
克-费综合征患者可能会出现如颈部疼痛、神经根病和步态不稳等神经学症状。在此，我们描述了一名枕颈区域先天性融合且伴有齿状突至T3椎体环形融合并脊髓受压患者的手术治疗情况。本报告是首例如此广泛融合的病例。
病例描述
我们的患者是一名56岁女性，表现为颈部疼痛及四肢麻木。检查时，她颈部短，下颌突出，颈部活动范围极度受限，并有脊髓病特征。CT显示齿状突至T3椎体融合。患者接受了枕下颅骨切除术、C1椎板切除术以及枕骨至T5后路固定融合术，术后神经功能得到改善。
结论
这是首例报道的颈椎椎体全部融合至T3的克-费综合征病例。对于因狭窄和脊柱不稳定导致的晚期脊髓病或神经根病病例，我们建议进行手术治疗。
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