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Chiari Malformation

(Arnold-Chiari Malformation; Arnold-Chiari Syndrome; Type II Chiari Malformation; Cerebellomedullary Malformation Syndrome)

Definition

Chiari malformation is a problem with the structures at the back of the brain. An area of the brain called the cerebellum normally sits inside the back of the skull. In Chiari malformation, the cerebellum sits partially or fully below the skull. This position can put pressure on the cerebellum, nearby brainstem, and upper spinal cord. It can also block the flow of cerebrospinal fluid (CSF) around the brain and spinal cord. CSF normally cushions the brain and spine but when it becomes blocked it can cause a buildup of pressure in the brain and spinal cord.

There are 4 types of Chiari malformations. The types are based on the severity of the disorder and the parts of the brain that protrude into the spinal canal.

Causes    TOP

Chiari malformation is most often caused by a problem in the formation of the skull before birth. It is not clear why the skull does not develop normally, but it may be due to genetics or the health of the mother during pregnancy. The back of the skull normally has a curved area for the cerebellum and brainstem to sit in. In some babies, the space is too small for the brain to properly sit in and the cerebellum and brain stem are pushed downward into the spinal canal.

Type I Chiari malformation, the most common type, usually presents in older children, adolescents, and sometimes adults.

Type II is present at birth and is usually associated with spina bifida and occasionally with hydrocephalus.

Type III is usually present at birth and is usually associated with hydrocephalus.

Brain Stem and Lower Brain

GM00010_97870_brainstem.jpg
Copyright © Nucleus Medical Media, Inc.

Risk Factors    TOP

There are no known risk factors for Chiari malformation. There may be a genetic connection in some families.

Symptoms    TOP

Both the position of the brain and problems with CSF flow can cause a range of neurological symptoms that vary based on type. Those with type I may never have symptoms. When symptoms do occur, headaches are a hallmark symptom.

Symptoms may include:

  • Headaches—may be made worse with coughing
  • Lightheadedness and fainting
  • Weakness in the legs
  • Inability to hear or ringing in the ears
  • Eye problems, such as difficulty seeing, eye pain, and rapid eye movement
  • Poor coordination
  • Uncontrolled shaking or trembling
  • Difficulty walking
  • Numbness or tingling in the arms or legs

Symptoms in infants with type II or III may include:

  • Gagging or vomiting
  • Paralysis of the limbs
  • Difficulty swallowing
  • Difficulty breathing
  • Inability to gain weight
  • Chronic cough or hoarseness
  • Irritability, especially during feeding

Diagnosis    TOP

Type I may be suspected based on symptoms or discovered accidentally during tests for other conditions or injuries. Type II and III may be suspected when associated defects are present or symptoms are present.

If someone is experiencing symptoms, a doctor will ask about symptoms and medical history. A physical exam will be done which may include cognitive tests of memory, reflexes, or motor skills. Imaging tests may be done to evaluate the brain and skull. Tests may include:

Special studies may also be done to evaluate the flow of fluid around the brain and spinal cord.

Treatment    TOP

Chiari I malformations that do not cause symptoms do not require treatment. Those that do cause symptoms will be treated based on individual symptoms and the degree of pressure on the brain. Medication may be recommended to help manage headaches and pain.

Treatments to help manage symptoms that interfere with everyday life may include:

  • Physical or occupational therapy can help improve muscular coordination and trembling. Braces or a wheelchair may also be needed.
  • Speech therapy for speech or swallowing problems.

Severe compression of brain tissue, a buildup of pressure in the brain or spinal cord, and types II and III will require surgery to prevent further damage. Surgical options may include:

  • Sections of bone from the skull or vertebrae may be removed. The removal of bone sections may help relieve pressure on the affected tissue.
  • Tissue that surrounds the brain and spinal cord may be opened to examine the brain and spinal tissue. The tissue may then be expanded to improve the flow of CSF.
  • A shunt may be placed that allows extra CSF to drain from the brain or spine into the chest or abdomen when hydrocephalus is present.
  • Repair of any associated neurologic abnormalities, if possible.

Multiple surgeries may be needed.

Prevention    TOP

There is no known way to prevent Chiari malformation. Genetic counseling may help parents of a child with this condition to determine the risk in future children.

RESOURCES:

American Syringomyelia and Chiari Alliance Project
http://www.asap.org
National Institute of Neurological Disorders and Stroke
https://www.ninds.nih.gov

CANADIAN RESOURCES:

Canadian Neurological Sciences Federation
http://www.cnsfederation.org

References:

Chiari malformations. EBSCO DynaMed Plus website. Available at:
...(Click grey area to select URL)
Updated October 24, 2016. Accessed February 20, 2017.
Chiari malformation information page. National Institute of Neurological Disorders and Stroke website. Available at:
...(Click grey area to select URL)
Accessed February 20, 2017.
Chiari malformation. American Association of Neurological Surgeons website. Available at:
...(Click grey area to select URL)
Updated March 2006. Accessed February 20, 2017.
Chiari malformation. Columbia University Medical Center website. Available at:
...(Click grey area to select URL)
Accessed February 20, 2017.
Hekman KE, Aliaga L, et al. Positive and negative predictors for good outcome after decompressive surgery for Chiari malformation type 1 as scored on the Chicago Chiari Outcome Scale. Nuerol Res. 2012;34(7):694-700.
Last reviewed March 2017 by EBSCO Medical Review BoardRimas Lukas, MD
Last Updated: 2/20/2017

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