Spinal Cord Defects
What is Spinal Cord Defects ?
Spinal cord defects are caused by defective embryonic neural tube closure during the first trimester of pregnancy. These defects usually occur in the lumbosacral area, but they're occasionally found in the sacral, thoracic, and cervical areas. The meninges and brain tissue can protrude if the skull doesn't fuse properly.
Spina bifida occulta is an incomplete closure of one or more vertebrae without protrusion of the spinal cord or meninges. It's the most common and least severe spinal cord defect. In more severe forms of spina bifida such as spina bifida cystica, incomplete closure of one or more vertebrae causes protrusion of the spinal contents in an external sac or a cystic lesion.
Spina bifida cystica has two classifications: myelomeningocele (meningomyelocele) and meningocele. In myelomeningocele, the external sac contains meninges, cerebrospinal fluid (CSF), and a portion of the spinal cord or nerve roots distal to the conus medullaris. When the spinal nerve roots end at the sac, motor and sensory functions below the sac are terminated. In meningocele, less severe than myelomeningocele, the sac contains only meninges and CSF. Meningocele may produce no neurologic symptoms.
Spina bifida is relatively common: Each year, in the United States, about 12,000 infants are born with some form of spina bifida. Incidence is highest in people of Welsh or Irish ancestry.
Causes of Spinal Cord Defects
The exact cause of spinal cord defects isn't known. Viruses, radiation, and other environmental factors may be responsible for such defects. Spinal cord defects occur in offspring of women who have previously had children with similar defects, so genetic factors may also be responsible.Normally, about 20 days after conception, the embryo develops a neural groove in the dorsal ectoderm. This groove rapidly deepens as the two edges fuse to form the neural tube. By about day 23, this tube is completely closed except for an opening at each end. Theoretically, if the posterior portion of the neural tube fails to close by the 4th week of gestation, or if it closes and then splits open from a cause such as an abnormal increase in CSF later in the first trimester, a spinal cord defect results.
Signs & Symptoms of Spinal Cord Defects
Some of the symptoms commonly associated with spinal cord injury include:
In addition to the aforementioned primary symptoms associated with spinal cord injury, there are a variety of secondary medical problems that can occur, some of which include:
Care of the patient with a severe spinal defect requires a team approach, including the neurosurgeon, orthopedist, urologist, pediatrician, nurse, social worker, occupational and physical therapists, and family members. Specific measures depend on the severity of the neurologic deficit.
Spina bifida occulta requires little or no treatment. If indicated, surgery is performed soon after birth to release the tethered spinal cord and prevent further neurologic deterioration.
Initial treatment for spina bifida cystica includes surgical closure of the defect as soon as possible after birth, if possible within 48 hours. Then the child's growth and development are continually assessed throughout life. If the protruding sac is large, plastic surgery is required for skin grafting over the lesion. Surgery doesn't reverse the neurologic deficit. Usually, a shunt is necessary to relieve associated hydrocephalus. If hydrocephalus isn't apparent at the time of the initial surgery, the child must be frequently reassessed for its occurrence because hydrocephalus occurs in about 80% of children with myelomeningocele.
After surgery, supportive measures are required to promote independence and prevent further complications. Orthopedic, rehabilitation, and urologic consultations help determine the extent of the child's disabilities. Rehabilitation measures, which may be necessary throughout the child's life, may include:
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