Chiari Malformation

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What is Chiari malformation?

Chiari malformation refers to a deformity of the cerebellum. The cerebellum is the part of the brain that controls coordination and movement execution. It is located at the back and lower portion of the brain. In Chiari malformation, the cerebellum and brainstem are compressed, which can lead to fluid buildup and other neurological symptoms. Several types of Chiari malformations exist, most of which are due to birth defects.

Common symptoms that occur with Chiari malformation include neck pain, dizziness or vertigo, difficulty with vision, coordination problems, and muscle weakness. Symptoms of Chiari malformation may get worse when you cough, sneeze, or stretch your neck. In some cases, Chiari malformation may not ever cause symptoms.

For mild, symptomatic Chiari malformations, treatment may be as simple as pain medication and physical therapy. In more serious cases, surgical intervention may be required. Surgery for Chiari malformation is designed to open up more space around the cerebellum by taking out small pieces of the skull.

While Chiari malformation is known to be congenital (present at birth), it is unknown whether it is hereditary. Some research suggests that certain types of Chiari malformation may be genetic in origin, although the genes responsible have yet to be identified.

Seek immediate medical care (call 911) if you have a change in level of consciousness or alertness, such as passing out or unresponsiveness; or respiratory or breathing problems, such as shortness of breath, difficulty breathing or inability to breathe, labored breathing, wheezing, or choking.

Seek prompt medical care if you experience impaired balance or neck pain, especially pain that worsens with sneezing, coughing, and stretching.

What other symptoms might occur with Chiari malformation?

Symptoms that occur with Chiari malformation typically result from the excess pressure on the brain caused by the malformation.

Neurological symptoms that may occur along with Chiari malformation

Chiari malformation may accompany other symptoms affecting the brain including:

Symptoms that might indicate a serious condition

In some cases, Chiari malformation may occur with other symptoms that might indicate a serious condition that should be evaluated immediately in an emergency setting. Seek immediate medical care if you, or someone you are with, have Chiari malformation along with other serious symptoms including:

  • Change in level of consciousness or alertness, such as passing out or unresponsiveness

  • Respiratory or breathing problems, such as shortness of breath, difficulty breathing or inability to breathe, labored breathing, wheezing, or choking

What causes Chiari malformation?

Chiari malformation is a congenital disorder (present at birth). The underlying cause of Chiari malformation is not known. It is suspected that some forms of Chiari malformation may be hereditary, but genetic variations responsible for the condition have not been identified.

What are the potential complications of Chiari malformation?

Chiari malformation is often asymptomatic or very mild in nature, and may not lead to complications. In progressive cases, however, Chiari malformation may lead to the development of complications. Failure to seek treatment for symptomatic cases of Chiari malformation can result in serious complications and permanent damage. Once the underlying cause is diagnosed, it is important for you to follow the treatment plan that you and your health care professional design specifically for you to reduce the risk of potential complications including:

  • Brain damage
  • Impaired balance and coordination
  • Pain that does not respond to treatment (intractable pain)
  • Paralysis
  • Permanent loss of sensation
  • Permanent vision loss
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Medical Reviewer: William C. Lloyd III, MD, FACS
Last Review Date: 2021 Jan 8
THIS TOOL DOES NOT PROVIDE MEDICAL ADVICE. It is intended for informational purposes only. It is not a substitute for professional medical advice, diagnosis or treatment. Never ignore professional medical advice in seeking treatment because of something you have read on the site. If you think you may have a medical emergency, immediately call your doctor or dial 911.
  1. NINDS Chiari malformation information page. National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/disorders/Chiari/Chiari.htm.
  2. Chiari malformation. American Syringomyelia & Chiari Alliance Project. http://www.asap.org/index.php/disorders/Chiari-malformation/.
  3. Fernández AA, Guerrero AI, Martínez MI, et al. Malformations of the craniocervical junction (Chiari type I and syringomyelia: classification, diagnosis and treatment). BMC Musculoskelet Disord 2009; 10 Suppl 1:S1.