KidsMD Health Topics

Chiari Malformation in Children

  • At Boston Children’s Hospital, we have already helped many infants, children and adolescents who have been diagnosed with a Chiari malformation, an abnormality in the meeting of the brain and the spinal cord at the base of the child’s skull.

    There are four types of Chiari malformation. Type I is by far the most common form of the condition in children, while type II is typically only seen in children with spina bifida. The other types of Chiari malformation are extremely rare.

    For the purposes of this webpage, the information you will read below focuses only on type I and type II Chiari malformation.

    Here are some of the basics about Chiari malformation:

    Type I:

    • Type I Chiari malformation occurs in around 3 percent of the general population. However, since individuals with this condition often don’t show symptoms and go undiagnosed, it’s difficult to say exactly how many people actually have it.
    • Most children with type I Chiari malformations have no signs or symptoms and are only diagnosed when they get a magnetic resonance imaging (MRI) scan for another problem.
    • When symptoms occur, they typically present in late childhood or early adulthood and usually include neck pain. Other symptoms may include balance problems, sleep apnea (a common disorder that causes pauses in breathing during sleep) and swallowing difficulties.
    • The Chiari malformation can also cause fluid to accumulate in the spinal cord – a condition called syringomyelia – which can cause scoliosis in children. Syringomyelia can also make it harder for the brain to process and organize sensory information that comes from the surrounding environment.
    • If the Chiari malformation is not causing problems for the child, no treatment is needed. When symptoms do occur, treatment generally involves surgery to relieve pressure on the cerebellum, brain stem and spinal cord.

    Type II:

    • Type II Chiari malformation occurs when the tissue from the cerebellum, along with the brain stem itself, protrude downward through the base of the skull into the upper spinal canal.
    • Type II Chiari malformation almost always affects children who have spina bifida. It is present at birth (congenital).
    • Most children with this form of the condition require treatment for buildup of fluid in the brain (hydrocephalus).

    How Boston Children’s approaches Chiari malformation

    Since a child with a type I Chiari malformation may not have symptoms, the safest approach is to leave it untreated and perform a follow-up evaluation by MRI and examination if necessary. However, for symptomatic patients, or those who have developed an accumulation of fluid in the spinal cord, we typically recommend surgical treatment.

    Chiari malformation: Reviewed by Benjamin C. Warf, MD
    © Children’s Hospital Boston; posted in 2012

Request an Appointment

If this is a medical emergency, please dial 9-1-1. This form should not be used in an emergency.

Patient Information
Date of Birth:
Contact Information
Appointment Details
Send RequestIf you do not see the specialty you are looking for, please call us at: 617-355-6000.International visitors should call International Health Services at +1-617-355-5209.
Please complete all required fields

This department is currently not accepting appointment requests online. Please call us at: 617-355-6000. International +1-617-355-6000.

This department is currently not accepting appointment requests online. Please call us at: 617-355-6000. International +1-617-355-6000.

Thank you.

Your request has been successfully submitted

You will be contacted within 1 business day.

If you have questions or would like more information, please call:

617-355-6000 +1-617-355-6000
Find a Doctor
Search by Clinician's Last Name or Specialty:
Select by Location:
Search by First Letter of Clinician's Last Name: *ABCDEFGHIJKLMNOPQRSTUVWXYZ
Condition & Treatments
Search for a Condition or Treatment:
View allSearch

Contact the Department of Neurology

  • 1-617-355-6388
The future of pediatrics will be forged by thinking differently, breaking paradigms and joining together in a shared vision of tackling the toughest challenges before us.”
- Sandra L. Fenwick, President and CEO