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Chiari Malformation

(Arnold-Chiari Malformation; Arnold-Chiari Syndrome; Type 2 Chiari Malformation; Cerebellomedullary Malformation Syndrome)

Definition

Chiari malformations are problems with the structures at the back of the brain. A part of the brain called the cerebellum normally sits inside the back of the skull. This problem causes it to sit partially or fully below the skull. This can put pressure on the cerebellum, brainstem, and spinal cord. It can also block the flow of cerebrospinal fluid (CSF) and cause pressure around the brain and spinal cord.
There are four types:
  • Type 1 is often found in older children and young adults (most common)
  • Type 2 is a more severe form of Type 1 that is often found at birth in children with spina bifida
  • Type 3 is a rare form that is found at birth and causes severe problems with the nervous system
  • Type 4 is a rare form that happens when the cerebellum has not developed the right way

Causes

Chiari malformation is most often caused by a problem in the way the skull forms before birth. It is not known why this happens. Genes may play a role.
Brain Stem and Lower Brain
GM00010 97870 brainstem.jpg
Copyright © Nucleus Medical Media, Inc.

Risk Factors

There are no known risk factors for this health problem. It may run in some families.

Symptoms

People with type 1 may not have symptoms. Others may have symptoms based on the position of the brain and problems with CSF flow.
Babies may have:
  • Problems swallowing
  • Irritability, especially during feeding
  • A lot of drooling
  • A weak cry
  • Gagging or vomiting
  • Weak arms
  • A stiff neck
  • Problems breathing
  • Problems gaining weight
Older children and adults may have:
  • Headaches —may be worse when coughing
  • Neck pain
  • Hearing or balance problems
  • Weak or numb muscles
  • Lightheadedness and fainting
  • Problems speaking or swallowing
  • Vomiting
  • A curved spine
  • Problems sleeping
  • Problems with coordination and movement

Diagnosis

The doctor may ask about your symptoms and health history. A physical exam will be done. This may be enough to suspect Type 1. It may also be found during tests for other health problems.
Type 2 and 3 may be suspected in a child who has other birth defects and symptoms.
Images may be taken to confirm the diagnosis. This can be done with an MRI scan.

Treatment

People with type 1 may not need to be treated unless they have problems. People who do have problems will be treated based on their symptoms and the amount of pressure on their brain.
The goal of treatment is to manage problems that get in the way of daily life. Options are:
  • Physical or occupational therapy to improve movement. Braces or a wheelchair may also be needed.
  • Speech therapy for speech or swallowing problems.
People with severe problems and those with types 2 and 3 will need surgery. More than one surgery may be needed. Some options are:
  • Removing sections of bone from the skull or vertebrae to ease pressure on tissue
  • Opening the tissue around the brain and spinal cord to improve the flow of CSF
  • Placing a shunt to let extra CSF drain from the brain or spine and into the chest or belly

Prevention

There are no current guidelines to lower the risk of this health problem.

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
Health Canada
https://www.canada.ca

References

Chiari malformation. American Association of Neurological Surgeons website. Available at: https://www.aans.org/Patients/Neurosurgical-Conditions-and-Treatments/Chiari-Malformation. Accessed April 7, 2020.
Chiari malformation. Columbia University Medical Center website. Available at: http://www.columbianeurosurgery.org/conditions/chiari-malformation. Accessed April 7, 2020.
Chiari malformation. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/chiari-malformation/ . Updated May 10, 2017. Accessed April 7, 2020.
Chiari malformation fact sheet. National Institute of Neurological Disorders and Stroke website. Available at: https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Chiari-Malformation-Fact-Sheet. Updated March 13, 2020. Accessed April 7, 2020.

Revision Information

  • Reviewer: EBSCO Medical Review Board Rimas Lukas, MD
  • Review Date: 02/2020
  • Update Date: 04/07/2020
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