Chiari malformation

Signs and Symptoms

Symptoms most often begin during infancy, although in many cases symptoms don't emerge until adolescence or adulthood. Symptoms may include:

• Headache
• Neck pain
• Fatigue
• Vomiting
• Muscle weakness in the head and face
• Difficulty swallowing
• Some mental impairment
• Paralysis of the arms and legs
• Rapid involuntary downward eye movements
• Dizziness
• Double vision
• Deafness
• Difficulty coordinating movements
• Occasional sharp pain in or near the eyes

Diagnosis

During your child's visit, our doctors and other health professionals will evaluate your child to provide a precise diagnosis. Our staff will ask you about your child's medical history, as well as your family medical history. If your child's pediatrician has sent any medical records, we will review this information. Be sure to tell your child's neurologist about any medications your child is using, including over-the-counter medications, vitamins, nutritional supplements or herbal remedies. Our doctors will evaluate your child's nervous system, and look for symptoms of Chiari malformation. We also will rule out other conditions that might be causing problems.

Your child's evaluation probably will include magnetic resonance imaging (MRI), which makes images of the brain using a magnetic field and radio waves instead of X-rays, as well as other imaging techniques to produce a detailed picture of the brain and spinal cord. Other tests may be conducted to measure possible impaired coordination. Sensory loss or other physical problems also may be assessed.

Treatment

Your neurologists, along with your primary care physician, will design a treatment plan tailored to your child's medical condition, state of health and individual needs. Your child may need more than one kind of treatment, including surgery. You also may be referred to additional doctors or other medical professionals.

Infants with very severe malformations may have life-threatening complications. Some patients may have prolonged periods of relative stability. Untreated, however, the chronic crowding of the brainstem and spinal cord can lead to very serious consequences including paralysis. They can lead to the development of a cyst, called a syrinx, that expands and over time destroys the center of the spinal cord.

Chiari malformation often is treated with the following surgeries:

• Surgery to repair a myelomeningocele, or protruding sac of membrane.
• Implantation of a shunt, a small plastic tube that drains fluid from the brain when hydrocephalus develops or is part of the condition.
• "Chiari decompression" to enlarge the opening in the back of the skull. This can help relieve pressure within the skull.

Your medical team will explain any possible risks or complications from the treatments. Don't be afraid to ask your doctors, nurses or therapists about the treatment. If treatment includes medication, be sure that it's taken exactly as the doctor has instructed. Remember to keep all follow-up appointments with our doctors and other medical staff.