Spina Bifida is one of the most serious neural tube
defects that is also compatible with prolonged
life. Its severity varies from the occult type
(with no clinical findings) to a completely open spine
(rachischisis) with severe neurological disability and
death. In Spina Bifida Cystica, the protruding
sac can contain meninges (meningocele), Spinal Cord
(myelocele), or both (myelomeningocele). Spina
Bifida is most common in the lower thoracic, lumbar, or
sacral region and usually extends for 3 to 6 vertebral
segments. The sac in a myelomeningocele usually
consists of meninges with a central neural plaque. If not well
covered with skin, the sac can easily rupture, increasing the risk of
When the Spinal Cord or lumbosacral nerve roots are
involved in the Spina Bifida, as is usual,
varying degrees of paralysis occur below the affected
level. Since this paralysis occurs in the fetus,
orthopedic problems are often present at birth
(i.e. clubfoot, arthrogryposis, dislocated hip and other extremity
issues.) The paralysis frequently affects the urinary bladder
and rectal functions, and the resulting genitourinary disorder
can eventually lead to severely damaged kidneys.
Kyphosis can be associated with Spina Bifida and can
hinder surgical closure preventing the infant from lying on
their back. Hydrocephalus is commonly associated with
Spina Bifida and may be related to Aqueduct of Sylvius
stenosis or an
Other congenital anomalies may be present as well.
Prenatally, open Spina Bifida can be diagnosed by finding
elevated a-fetoprotein levels in maternal serum
and in amniotic fluid. Ultrasound can show bony spinal
defects and soft tissue masses.
In cases of Myelomeningocoele, X-rays of the Spine, Skull, hips
and lower extremities (if they are malformed) are usually
done soon after birth. Further testing depends on the associated
defects and may include intracranial studies (CT or ultrasound).
Spina Bifida may never cause any symptoms. In some cases,
symptoms become apparent in adolescence or later (including
adulthood). In these instances, CT and/or MRI Scans are
helpful. Occasionally Myelography and post-myelographic
CT scans are used to more definitively identify the
critical anatomical details of the anomaly (See Figure 1).
Figure 1: Post-myelographic CT Scan of Spina Bifida|
This Lumbar Vertebra's Spinous Process is "BIFID" (meaning
"split in two") indicated by the Double-headed Arrow. The
Lamina (on either side) are abnormal (Horizontal Arrows) as
well. In this case the Spinal Cord is split into 2 separate
segments (Vertical Arrows).
This represents a case that was not identified until later
life when as an adult the patient developed urinary
Urinary tract evaluation is essential and includes urinalysis,
urine culture, Blood chemistry analysis (BUN and creatinine), IVP
(intravenous pyelogram) and ultrasound.
Treatment requires a united effort by specialists from several
disciplines. Initially important are neurosurgical,
urologic, orthopedic, pediatric, and social service evaluations.
Thorough evaluation of the infant and counseling of the
family should generally precede intervention.
It is important to assess the type, level, and extent of the
lesion; the infant's general health status and
associated deficits; the family's strengths, desires,
and resources; and the community resources, including
ongoing care. Once evaluation is complete, a decision can be made on
how aggressive treatment should be.
If the defect is leaking CSF, antibiotics and urgent
neurosurgical evaluation and repair will reduce the risk of meningeal
or ventricular infection. Hydrocephalus may require a
This is a large Myelomeningocoele in a
1 day-old infant. The defect extends over the majority of the
lumbar spine. The Neural Plaque (Curved Arrow indicating the
exposed dark area) represents Spinal Cord tissue that is
markedly abnormal. The Meningeal portion of the defect is seen
on either side of the Neural Plaque (Vertical Arrows). There
is some abnormal skin (Horizontal Arrow) associated with
defect at the periphery of the Myelomeningocoele at the lower
end and asymmetrically more on the Left than the right.
Surgical closure can be challenging. The associated
Hydrocephalus must be treated lest the repair of the
Myelomeningocoele disintegrate due to the CSF pressure on
This Myelomeningocoele involves 3
Lumbar segments. The Neural Plaque (Horizontal Arrow) can be
seen through the intact, thin meningeal envelope.
Figure 4: Operative Photo. Myelomeningocoele Repair
(same patient as Figure 3)
A multilayer repair is standard for
these development defects. The first layer (Arrow indicates
the sutures) has meticulously reconstructed as it covers the
Neural Plaque. The surgeons (Drs. Lazar & Bland) are preparing
to close the 2nd layer.
Figure 5: Operative Photo. Myelomeningocoele Repair (Same
Patient as Figure 3 & 4)
There are several reliable techniques
that are used to close most of the skin defects. Occasionally
the defects are large enough to require special plastic
surgery skin flap techniques.
Kidney function must be monitored carefully and
urinary tract infection (UTI) should be treated promptly. Obstructive
kidney disease at either the bladder outlet or ureteral level must be
treated vigorously, especially when infection occurs.
Orthopedic care should begin early. If a
clubfoot is present, it should initially be
managed with a cast. The hip joints should be checked
closely for dislocation. Other continuing orthopedic concerns are
scoliosis, pathologic fractures, development of pressure sores, and
muscle weakness and spasm, which may cause further deformities.
Prognosis is determined by the number and severity of
abnormalities and is poorest for patients with total paralysis below
the lesion, kyphosis, hydrocephalus, early hydronephrosis, and
associated congenital defects. With proper care, however, many
children do well. Loss of renal function and shunt complications are
the usual causes of death in older patients with Spina Bifida.
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This page last edited on 2/22