Cutaneous stigmata of spina bifida occulta include dimples, dermal sinuses, subcutaneous lipomas, tufts of hair, or hemangiomas. Cutaneous lesions may occur in isolation, or herald an underlying tethered cord due to a low-lying conus or fat-infiltrated filum. Only a small subset of cutaneous lesions is associated with an intraspinal anomaly, and MRI performed within a few months of age can often exclude the diagnosis without radiation or sedation. The tethered spinal cord syndrome occurs when a hypertrophied filum terminale is too inflexible and causes progressive traction and relative caudal displacement of the conus medullaris as the spine grows. This traction can produce progressive ischemia in the conus medullaris and lead to symptoms of sphincter dysfunction and gait abnormalities. One third of infants with a congenital tethered cord are likely to eventually develop neurologic dysfunction if the tethered cord is not treated. Prophylactic detethering by microsurgical sectioning of the filum terminale, ideally before 1 year of age, allows immediate ascent of the conus medullaris toward a more normal location within the spinal canal and minimizes the chance of development of neurologic deficits as the spine grows. For older children who have a late symptomatic presentation, treatment may minimize the further progression of neurologic deficits. If spina bifida occulta occurs in conjunction with a dermal sinus (an epithelium-lined tract linking the dural sac with the skin surface), there is a potential for communication between the skin and intraspinal contents and subsequent infection. Dermal sinuses located above the sacrococcygeal region should be removed surgically after MRI imaging to evaluate for other associated lesions.

SPINA BIFIDA APERTA

Dysraphic conditions in which there are overt manifestations of the underlying bony defect are referred to as “spina bifida aperta” (see Plate 1-8). Within this group, the progression of neurologic sequelae is defined, to a large extent, by the degree to which the contents of the spinal canal are displaced from their normal location. In the case of a meningocele, the most benign form of spina bifida aperta, a meningeal cyst free of neural elements is extruded. Often, a meningocele can be completely removed surgically and the defect closed.

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