Syringomyelia

syringomyelia-2x

Overview

Normally, the spinal cord and brain are bathed in a fluid called cerebrospinal fluid. Syringomyelia is an accumulation of spinal fluid that actually forms within the spinal cord. This accumulation is called a syrinx. Most of the time, syringomyelia is caused by Chiari malformations, which is a birth defect of the back and lower part of the brain. Sometimes syringomyelia is caused by hydrocephalus, tethered spinal cord, tumor or other rare causes.

Signs & symptoms

The symptoms of syringomyelia are very variable, and often progress slowly over years. Classic findings include:

  • Scoliosis
  • Sensory loss in a “cape” distribution (over the tops of the shoulders); loss of pain and temperature sensation without loss of touch and position sense
  • Neck pain and pain in the back of the head
  • Pain in legs and arms, and hands and feet
  • Hand and arm weakness and loss of muscle function
  • High muscle tone in the arms and legs (spasticity)
  • Sleep apnea

Diagnosis

Syringomyelia is diagnosed with MRI. Small syrinxes or hydromyelia in the spinal cord can be a normal finding.

Treatment

Large syringomyelia requires surgical treatment. The treatment is directed at the cause of the syrinx, such as Chiari I malformation, or Tethered cord, rather than the syrinx itself.

UCSF Benioff Children's Hospitals medical specialists have reviewed this information. It is for educational purposes only and is not intended to replace the advice of your child's doctor or other health care provider. We encourage you to discuss any questions or concerns you may have with your child's provider.

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Neurosurgery Program

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Cerebral Palsy & Movement Disorders Center

Cerebral Palsy & Movement Disorders Center

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