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Chiari Malformation

Chiari malformation is a structural defect in cerebellum, the area in the back of the head where the brain and the spinal cord connect. The congenital (present at birth) defect occurs when the indented space at the base of the skull is too small to hold the lower part of the cerebellum, forcing it down into the spinal column. As a result, the brain and spinal cord are compressed, blocking flow of spinal fluid.

There are different types of Chiari malformation. Type I is the most common type observed in children, but it can also develop in adults. Many people with Type I Chiari malformations have no symptoms, whereas others develop symptoms in their 20s or 30s, and may include headache, dizziness, neck pain, impaired balance, nausea, and vision problems.

The diagnosis of Chiari malformation includes a physical examination, a complete neurological evaluation to check functions controlled by the cerebellum and spinal cord such as balance, motor skills, and reflexes, and a magnetic resonance imaging (MRI) test to examine structures at the base of the skull.

The Temple neurologists at Jeanes Hospital are highly experienced in the diagnosis, management and treatment of Type I Chiari malformation.

Chiari Malformation Treatment at Jeanes Hospital

Treatment for Chiari malformation depends on symptoms and severity of the patient's condition. If there are no symptoms or they are mild and not affecting day-to-day activities, the Temple neurologist may recommend no treatment and monitor the condition on a regular basis. If symptoms such as headache, dizziness, or other types of pain are present, medication may be the recommended treatment option.

If the symptoms are more severe and are having a serious impact the patient's quality of life, the Temple neurologist may recommend surgery to reduce pressure on the cerebellum and spinal cord and restore circulation of the spinal fluid.

The most common type of surgery for Chiari malformation is posterior fossa decompression, which involves removing one or more small pieces of the bone at the base of the patient's skull (craniectomy). This helps relieve the pressure, creates more room for the cerebellum and spinal cord, and allows the fluid to circulate. Other procedures that the Temple neurosurgeon may perform during this surgery may include:

  • Laminectomy – the removal of the top of one or vertebra of the spinal column to make more room for the brain
  • Intradural – the opening and widening of the protective membrane of the brain (dura mater) and replacing it with a patch to allow for more room for the brain
  • Electrocaultery – the reducing in size of the bottom of the cerebellum with a tool heated by electric current to make more room for spinal fluid

If there is a build-up of fluid in the brain, a small tube called a shunt may be inserted to drain the excess fluid and relieve pressure.

To schedule an appointment with a Temple neurosciences team member at the Jeanes Hospital Neurosciences Center, click here or call 215-728-CARE (2273).

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