SPINA BIFIDA

I. Description
Myelodysplasia refers broadly to any malformation of the spinal canal and cord.
Midline defects involving failure of the osseous (bony) spine to close are called spina
bifida, the most common defect of the CNS. SB is categorized into two types: SB
occulta and SB cystica.
! SB occulta refers to a defect that is not visible externally. It occurs most
commonly in the lumbosacral area (L5 and S1). Routine radiographic
examinations indicate that the disorder may occur in as many as 10% to 30% of
the general population. However, it may not be apparent unless there are
associated cutaneous manifestations or neuromuscular disturbances. Superficial
cutaneous indications include a skin depression or dimple (which may also mark
the outlet of a dermal sinus tract that extends to the subarachnoid space);
port-wine angiomatous nevi; dark tufts of hair; and soft, subcutaneous lipomas.
These signs may be absent, appear singly, or be present in combination.
If associated neurologic involvement is present, the defect is known as occult
spinal dysraphism. Fibrous bands and adhesions, an intraspinal lipoma (fatty tumor)
or subcutaneous lipoma (lipomyelomeningocele), a dermoid or epidermoid cyst,
diastematomyelia (spinal cord split in two), or a tethered cord can distort the spinal
cord or roots. The usual cause is abnormal adhesion, or tethering, to a bony or fixed
structure, resulting in traction on the spinal cord and cauda equina.
Neuromuscular disturbances usually consist of progressive or static changes in
gait with foot weakness, foot deformity, or bowel and bladder sphincter disturbances.
Some manifestations may not be evident until the child walks or is toilet trained.
Plain radiography is employed to disclose the precise bony defect in the
symptomatic lesion and to establish the diagnosis in the suspected, nonsymptomatic
occult variety. Magnetic resonance imaging (MRI) is the most sensitive tool for
evaluating the defect. Computed tomography (CT), ultrasonography, and
myelography are also used to differentiate between SB occulta and other spinal
disorders.

! SB cystica refers to a visible defect with an external saclike protrusion. The two
major forms of SB cystica are meningocele, which encases meninges and spinal
fluid but no neural elements, and myelomeningocele (or meningomyelocele),
which contains meninges, spinal fluid, and nerves. Neurologic deficit is not
associated with meningocele but occurs in varying, often serious, degrees in
myelomeningocele.

Children with spina bifida are high risk for developing later allergies of frequent
exposure to latex during catheterizations, shunt placements, and other operations.
Later allergy is estimated to occur in approximately 73% of children with spina bifida.
Allergic reactions can range from mild signs and symptoms to anaphylactic shock.

A saclike protrusion on the infant’s back indicates meningocele or myelomeningocele.