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颈椎前路脊膜脊髓膨出:一种罕见的脊髓畸形。

Anterior cervical myelomeningocele: a rare malformation of the spinal cord.

机构信息

Spine Surgery, Affiliated Drum Tower Hospital of Nanjing University Medical School, Nanjing, China.

出版信息

J Neurosurg Spine. 2012 Mar;16(3):257-60. doi: 10.3171/2011.10.SPINE11484. Epub 2011 Dec 9.

DOI:10.3171/2011.10.SPINE11484
PMID:22150240
Abstract

Cervical myelomeningocele (MMC) is an uncommon congenital malformation of the spinal cord and accounts for a small proportion of neural tube defects. These lesions mostly occur in the dorsal part of the body. Only a single case of an anterior cervical MMC has been previously reported. The authors report a second case of anterior cervical MMC diagnosed when the patient began to experience symptoms of bilateral hand weakness in adulthood. In this patient, MR imaging of the cervical spine showed an anterior cervical MMC at the C6-7 level with hydrocephalus, thinning of the genu and trunk of the corpus callosum, maldevelopment of the cerebellar tonsils, and expansion of the fourth ventricle, posterior cranial fossa, and subarachnoid space. A CT scan and a 3D CT reconstruction of the cervical spine clearly demonstrated contiguous fusions of multiple lower-cervical vertebrae and neural arches, which was consistent with Type III Klippel-Feil syndrome. The patient was advised to undergo operative treatment to prevent the progression of her neurological deficit. However, after being notified of the potential neurological risks, the patient declined surgery and opted for conservative treatment with a hard neck collar. At 4 months' follow-up, the patient's neurological deficit remains stable with the MMC left untreated. The authors presume that the possible pathogenesis of anterior cervical MMC may greatly differ from that of posterior lesions. This lesion could also be associated with multiple other spinal abnormalities, which highlights the importance of comprehensive preoperative radiological examinations.

摘要

颈椎脊膜脊髓膨出(MMC)是一种少见的脊髓先天性畸形,占神经管缺陷的一小部分。这些病变主要发生在身体的背部。此前仅报道过一例颈椎前 MMC。作者报告了第二例颈椎前 MMC 病例,该患者成年后开始出现双侧手部无力症状。该患者颈椎 MRI 显示 C6-7 水平的颈椎前 MMC 合并脑积水、胼胝体膝部和干部变薄、小脑扁桃体发育不良以及第四脑室、颅后窝和蛛网膜下腔扩张。颈椎 CT 扫描和 3D CT 重建清楚地显示多个下颈椎和神经弓的连续融合,符合 III 型 Klippel-Feil 综合征。建议患者进行手术治疗以防止神经功能缺损进展。然而,在告知患者潜在的神经风险后,患者拒绝手术,选择了硬颈领保守治疗。在 4 个月的随访中,MMC 未治疗,患者的神经功能缺损仍保持稳定。作者推测,颈椎前 MMC 的可能发病机制与后病变有很大不同。该病变也可能与其他多种脊柱异常相关,这凸显了全面术前影像学检查的重要性。

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引用本文的文献

1
Cervical vertebral fusion with anterior meningocele.颈椎融合伴前侧脑脊膜膨出。
Neuroradiol J. 2015 Apr;28(2):205-8. doi: 10.1177/1971400915576308. Epub 2015 Apr 13.
2
Surgical treatment in a patient with Klippel-Feil syndrome and anterior cervical meningomyelocele: a case report and review of literature.Klippel-Feil 综合征合并颈前脊髓脊膜膨出患者的手术治疗:病例报告及文献复习。
Eur Spine J. 2013 May;22 Suppl 3(Suppl 3):S517-20. doi: 10.1007/s00586-013-2769-6. Epub 2013 Apr 12.