Adult Diastematomyelia

Abstract

With modern imaging techniques, various types of spinal dysraphism are being diagnosed in adults with increasing frequency. We report a 42-year-old woman with diastematomyelia whose symptoms characteristically were precipated by trauma. She exhibited other typical features such as a posterior midline hair patch and vetebral malformations. Metrizamide myelography with computerized tomography showed a bony septum at Tl 1 with a cleft at T9-T11 separating two dural tubes each of which contained a single spinal hemicord. The septum was excised with complete relief of symptoms.Resume: Diastematomyelie adulte. Depuis l'avenement des techniques modemes d'imagerie, differents types de dysraphie medullaire sont diagnostiques de plus enplus souvent chez I'aulte. Nous rapportons le cas d'une femme de 42 ans, porteuse d'une diastematomyelie, dont les symptomes ont ete precipites typiquement par un traumatisme. Elle presentait d'autres manifestations caracteeristiques, somme une touffe de poils en position mediane posterieure et des malformations vertebrales. Une myelographie au meetrizamide avec tomographie assistee par ordinateur a montree un septum osseux a D l l avec une fente a D9-D11 separant deux tubes duraux dont chacun contenait une demi-moelle epiniere. Le sptum a ete excise entrainant une disparition complete des symptomes.Can. J. Neurol. Sci. 1994; 21: 72-74 Diastematomyelia (DM) is a relatively uncommon form of spinal dysraphism, in which the spinal cord or conus is split in the sagittal plane, over a number of segments, into two separate parts or hemicords. The cleft usually contains an osseocartilagenous septum that projects backwards from the posterior surface of the vetebral body to create two distinct arachnoidaldural sheaths, each containing a separate hemicord.' In another type of DM, both hemicords are enclosed within a single meningeal sheath without a septum, but usually with fibrous bands attaching the neural elements to the posterior dura or to the laminae.2 Theories of the embryogenesis of DM are usually based upon a primary abnormality or a secondary defect in the neural ectoderm which causes it to split and results in the formation of two hemicords. The gap between them becomes filled with mesodermal tissue which creates the septum.1 DM is associated with a well-recognized syndrome or lower spinal cord or conus medullaris dysfunction, the features of which include weakness and atrophy of the distal lower limb musculature, impairment of sphincter function and sacral sensory loss. Associated cutaneous abnormalities, especially a mid-line hair patch and vertebral malformations are common.1 -3 Although adult cases have been reported 4 -5 DM has been considered to be almost exclusively a condition of childhood. Now, with the modern imaging techniques, it is being diagnosed in older patients with increasing frequency. 68In this report we describe the case of a 41-year-old woman with DM who became symptomatic after an accident. Computerized tomographic (CT) scanning after metrizamide myelogr...

Split cord malformation in children undergoing neurosurgical intervetion in India: a descriptive study

“…Split cord malformations (SCM) are spinal cord defects, having double neural tube due to vertical split in the cord (1). SCM is one of the commonest subset of occult spinal dysraphism.…”