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Chiari Malformation

Beaumont neuroscience experts determine a Chiari patient's optimal treatment based on the individual's unique history and symptoms, not just a test result. Our team's comprehensive approach combines in-depth patient history, neurophysiological assessment and thorough decompressive surgical technique to ensure long-standing relief of Chiari symptoms. These symptoms can range from the most basic headache to complex, multi-system problems.

Chiari malformation (kee-AHR-ee) is a disease of the brain in which brain tissue is pushed down into the spinal canal. It occurs when the hole at the base of the skull (foramen magnum) is abnormally small and misshapen, pressing on the brain and forcing it downward. The part of the brain that is pushed down is called the cerebellar tonsils. Chiari malformation can cause a blockage in the flow of cerebrospinal fluid (CSF), which can cause the normal fluid spaces in your brain to enlarge, a condition known as hydrocephalus.

Most cases of Chiari are congenital, meaning it was present since before birth.

Types of Chiari Malformation

There are four types of Chiari malformation:

Chiari I is the most common type of Chiari malformation. It is often associated with syringomyelia (SM) and scoliosis. SM is a disease in which there is a syrinx, or fluid-filled cyst, in the spinal cord. Scoliosis is a curved spinal column, or backbone. CM I usually does not cause any problems during childhood. It typically begins to become a problem in the teen and adult years. When problems do begin, the first is usually a bad headache.

Chiari II also is called Arnold-Chiari syndrome. It is associated with myelomeningocele, a birth defect in which the spinal column does not close before birth. Myelomeningocele is a kind of spina bifida. CM II also is associated with hydrocephalus. It causes shifting of the brain stem and is commonly diagnosed when the patient is still a child.

Chiari III is a severe nervous system disease. CM III usually is connected with a disease in which a baby's skull does not close completely before birth. Part of the baby's brain can then come through the openings in the skull. CM III is usually a terminal condition unless surgically treated.

Chiari IV is the least common type of Chiari malformation and involves a lack of development of a portion of the base of the brain called the cerebellum.

Associated Conditions

Some conditions that can be associated with or be a result of Chiari malformation include:

  • Arachnoiditis
  • Autism Spectrum
  • Basilar Invagination
  • Connective Tissue Disorder
  • Hydrocephalus
  • Neuropathic Pain Syndrome
  • Pseudo Tumor Cerebri
  • Scoliosis (Spinal Curvature)
  • Spina Bifida
  • Syringomyelia
  • Tethered Cord Syndrome