Anterior cervical myelomeningocele: a rare malformation of the spinal cord

Case report

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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.

Abbreviation used in this paper: MMC = myelomeningocele.

Article Information

Address correspondence to: Yong Qiu, M.D., Spine Surgery, Affiliated Drum Tower Hospital of Nanjing University Medical School, Zhongshan Road 321, Nanjing 210008, China. email: scoliosis2002@sina.com.cn.

Please include this information when citing this paper: published online December 9, 2011; DOI: 10.3171/2011.10.SPINE11484.

© AANS, except where prohibited by US copyright law.

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Figures

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    Midsagittal T1-weighted (left) and T2-weighted (right) MR images showing hydrocephalus and an anterior cystic swelling of the meninges with the kinked cervical spinal cord prolapsed into the meningeal cyst at the C6–7 level. 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 were also demonstrated.

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    Axial T2-weighted MR image demonstrating anterior herniation of the meninges and spinal cord through the defect of the C-7 vertebral body. Part of the cervical cord was located in the spinal canal while part was located outside the anterior vertebral body.

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    Sagittal reconstruction of the CT scan (A) and anterior (B) and posterior (C) views of the 3D CT reconstruction showing contiguous fusions of multiple lower-cervical vertebrae and neural arches with anterior defects of C-6 and C-7 bodies.

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