SPINA BIFIDA
NEUROSURGICAL ISSUES

What is spina bifida?

Spina bifida is one of the most common birth defects compatible with life and can involve several organ systems. It literally means "cleft spine" and results from incomplete or faulty closure of the spinal cord and surrounding tissues. There are several types of defects including spina bifida occulta, which is a defect in the bone overlying the spinal cord without an abnormality in the cord itself. This is very common, affecting up to 40% of the adult population and is of no concern. The more severe forms are the open, or aperta, defects which include meningocele and myelomeningocele in which the coverings over the spinal cord or the malformed cord protrudes through an opening in the bone. In some cases the defects may be covered by skin, in others the nerve roots or malformed spinal cord may be exposed. This is the most severe of the common forms of spina bifida. Other neurologic abnormalities associated with spina bifida include Chiari malformations, hydrocephalus, and syringohydromyelia.

What causes spina bifida?

Causes of spina bifida include environmental and genetic factors. Although a family history of spina bifida increases the risk of having a baby with spina bifida it does not follow simple inheritance patterns. Folate deficiency is a contributing factor and insuring adequate folate intake will help to prevent this disability. The U.S. Public Health Service issued a recommendation that all women of childbearing age (15-44 years) who are capable of becoming pregnant should consume 0.4 milligram (400 micrograms) of folic acid per day to reduce the risk of having a baby affected by spina bifida. This is because the spinal cord forms very early in the fetus and women often don’t know they are pregnant until after the cord has formed.

How is spina bifida diagnosed prenatally?

Spina bifida may be diagnosed prenatally by high-resolution ultrasound or by testing the mother for high levels of alpha-fetoprotein. Prenatal diagnosis gives the parents a chance to plan for the medical or surgical treatment the baby may need after birth. Cesarean delivery may also help to reduce the severity of paralysis in these infants.

How is spina bifida treated?

The current standard of treatment is early closure of the open spine defect. This is primarily to prevent infection which can be life threatening. Hydrocephalus, which occurs in up to 90% of children with myelomeningoceles also needs to be treated and the timing of that treatment is decided by the neurosurgeon. Because spina bifida affects a number of organ systems multiple specialists are involved with the care of these patients including urologists, orthopedic surgeons, physical therapists, occupational therapists and orthotists. Genetic counseling for the parents is also in order.

What is the long-term outlook for children with spina bifida?

Although most children with spina bifida are not able to walk independently they can be mobile with braces or with wheelchairs. Most children are incontinent but are able to achieve social continence with self-catheterization. Thus many children and adults with spina bifida live productive, fulfilling lives.

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