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Secondary Medical Conditions Associated with Spina Bifida: Hydrocephalus -- SHNIC Educators Information

Hydrocephalus (hydro-water, cephalus-brain) occurs when there is an increase in the amount of cerebrospinal fluid (CSF) in the brain. The CSF normally drains from the brain, down the spinal cord and back up to the brain. Most students with hydrocephalus have an Arnold Chiari Type II malformation, which involves the brain stem.

The abnormal positioning of the brain stem causes disruption of the flow of CSF. The majority of students will not have symptoms, but those that do may experience mild to severe manifestation of those symptoms. Since the brain stem is involved, there may be critical areas of cranial nerve dysfunction. The student may have weak eye muscles resulting from oculomotor dysfunction, putting them at risk for amblyopia if not treated. There may be gagging and swallowing problems associated with pharyngeal dysfunction. There may be changes in the quality of a child's voice, such as hoarseness, that suggests a laryngeal dysfunction. Respiratory centers are also located in the brain stem. In severe situations, an individual may experience apnea (mainly associated with sleep).

In addition to the obvious health implications, there are questions of the impact of chronic sleep apnea on alertness and overall cognitive functioning. There may be subtle changes in upper extremity functioning over time. Some students may demonstrate changes in writing and increased tone in their upper extremities. The main role of the school is to be aware of the student's baseline status and alert the family of any changes. The changes are usually gradual, such as a gradual decrease in handwriting legibility before spasticity becomes very evident. Certainly the involvement of the school occupational therapist would be helpful in assessing for early changes in upper extremity functioning. This therapist should be in communication with the student's spina bifida team. Treatment ranges from monitoring to neurosurgical intervention in severe situations where there has been demonstrated neurological deterioration.

Students with hydrocephalus may have a shunt surgically placed under the skin to help drain excess fluid from the brain. At times, the shunt may malfunction (tube clogged, break, outgrown). When this happens, it usually needs to be replaced. It is important as an educator to know the signs of shunt failure. They include:

  • Personality changes
  • Headache
  • Changes in vision
  • Deterioration in school performance
  • Decrease in sensory or motor function
  • Swelling along shunt tract
  • Irritability
  • Increased head size
  • Vomiting
  • Seizures
  • Lethargy

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