Spinal Dysraphism

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TETHERED CORD: in some patients the conus medullaris lies well below its normal level (LI), 'tethered' by the filum terminale. Since vertebral growth proceeds more rapidly than growth of the spinal cord, tethering may produce progressive back pain or neurological impairment as the cord is stretched.

DIASTOMATOMYELIA: A congenital splitting of part of the spinal cord by a bony, fibrous or cartilaginous spur. This usually lies at the upper lumbar region and extends directly across the spinal canal in an antero-posterior direction. The split cord does not always reunite distal to the spur (diplomyelia).

Investigation: MRI is the investigation of choice in spinal dysraphism, but straight X-ray may reveal associated congenital abnormalities: spina bifida occulta, fused or hemivertebrae. CT scanning may help demonstrate the presence of a bony spur.

T1 weighted MRI

Butterfly vertebra (congenital anomaly)

Conus medullaris extending down to L4 due to tethering

Spur

'Tight' filum — terminale

T1 weighted MRI

Butterfly vertebra (congenital anomaly)

Conus medullaris extending down to L4 due to tethering

Management: Although some recommend prophylactic division of the tethered filum terminale in the absence of neurological impairment, most reserve operative treatment for those who present with a neurological deficit, especially if there is evidence of progression, or prior to correction of any spinal deformity. In contrast, prophylactic removal of the spur in patients with diastomatomyelia is usually performed, even in the absence of neurological impairment.

LIPOMENINGOCELE

Lipomas may occur in association with spinal dysraphism and range from purely intraspinal lesions to very large masses extending along with neural tissues through the bony defect. All are adherent to the conus and closely related to the lumbosacral roots, preventing complete removal and increasing operative hazards.

CONGENITAL DERMAL SINUS TRACT/DERMOID CYST

This congenital defect results from a failure of separation of neuronal from epithelial ectoderm and may occur with other midline fusion defects, e.g. diastomatomyelia and a tethered cord. A tiny sinus in the lumbosacral region may represent the opening of a / blind ending duct or may extend into the spinal canal. Dermoid cysts arise at any point ' along the sinus tract and often lie adjacent to the conus.

Clinical presentation varies from repeated attacks of unexplained meningitis to neurological deficits arising from the presence of an intraspinal mass. Treatment involves excision of the whole tract and any associated cyst (after 412 treating any meningitic infection).

SECTION IV

LOCALISED NEUROLOGICAL DISEASE

AND ITS MANAGEMENT C. PERIPHERAL NERVE AND MUSCLE

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