SPINA BIFIDA
Definition:
Spina bifida, also called spinal dysraphia, is a malformation of the spine inwhich the posterior portion of the laminae of the vertebrae fails to close. It occurs inapproximately 1 per 1,000 live births in the United States and is the most commondevelopmental defect of the central nervous system (CNS). It is more common inCaucasian than in nonCaucasian people.(A) Normal spine. (B) Spina bifida occulta. (C) Spina bifida with meningocele. (D)Spina bifida with myelomeningocele.
Spina Bifida Occulta
The defect is only in the vertebrae. The spinal cord and meninges are normal.
Meningocele
The meninges protrude through the opening in the spinal canal. This forms a cyst filledwith CSF and covered with skin.
Myelomeningocele (or Meningomyelocele)
The spinal cord and cord membranes protrude through the defect in the laminae of thevertebral column. Myelomeningoceles are covered by a thin membrane.
PATHOPHYSIOLOGY AND ETIOLOGY
1.Unknown etiology but generally thought to result from genetic predisposition triggered by something in the environment
A.
Certain drugs, including valproic acid, have been known to cause neural tubedefects if administered during pregnancy.
B.
Women who have spina bifida and parents who have one affected child have anincreased risk of producing children with neural tube defects.2.Involves an arrest in the orderly formation of the vertebral arches and spinal cord thatoccurs between the fourth and sixth weeks of embryogenesis.3.Theories of causation include:
A.
There is incomplete closure of the neural tube during the fourth week of embryonic life.
B.
The neural tube forms adequately, then ruptures.4.In spina bifida occulta, the bony defect may range from a very thin slit separating onelamina from the spinous process to a complete absence of the spine and laminae.
A.
A thin, fibrous membrane sometimes covers the defect.
B.
The spinal cord and its meninges may be connected with a fistulous tractextending to and opening onto the surface of the skin.5.In meningocele, the defect may occur anywhere on the cord. Higher defects (fromthorax and upward) are usually meningoceles.
A.
Surgical correction is necessary to prevent rupture of the sac and subsequentinfection.
B.
Prognosis is good with surgical correction.6.In myelomeningocele (meningomyelocele), the lesion contains both the spinal cord andcord membranes.
A.
A bluish area may be evident on the top because of exposed neural tissue.
B.
The sac may leak in utero or may rupture after birth, allowing free drainage of CSF. This renders the child highly susceptible to meningitis.
C.
Occurs four to five times more frequently than meningocele.
SPINA BIFIDA
In utero fetal developmentFailure of spinous process to join lumbosacral areaDefect in closure of neural tube
Drug Alert
Maternal periconceptional use of folic acid supplementation reduces by 50% or more theincidence of neural tube defects in pregnancies at risk.
CLINICAL MANIFESTATIONSSpina bifida occultaSkin depression or simpleport wine angioma tufts of hair subcutaneous lipomasBowel and bladder sphincterweakness (persistent or intermittentenuresis) foot weakness, gaitdisturbance (late walking)Protruding sac throughdefect-containing meningesComplete nonclosure of neuraltube with protruding sacthrough defect containing partsof spinal cordMeningoceleMyelomeningoceleSurgical repaid and V-P shuntbased on type and extent of defectand deficitResolution of defect and minordeficitsImmobility or mobility withbraces and crutches
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