Chiari malformation type 2

Is Chiari malformation type 2 inherited?

Chiari malformation type 2 usually occurs sporadically (in people with no family history of the condition). However, the exact cause is not known. Genes may play a role in predisposing a person to the condition, but environmental factors (such as lack of proper vitamins or nutrients in the maternal diet during pregnancy) may also contribute to the condition.

There have been reports in the medical literature of families in which more than one family member had a Chiari malformation. However, limited information specific to familial cases of Chiari malformation type 2 is available.

Last updated on 05-01-20

What is the long-term outlook for people with Chiari malformation type 2?

The long-term outlook depends on the nature of the malformation and the symptoms present in each person. Although some people experience a reduction of symptoms, there is no guarantee that surgery will help every person. Nerve damage that has already occurred usually cannot be reversed with surgery, and pain from nerve damage can be difficult to treat. Some people may need repeat surgeries.

Last updated on 05-01-20

How might Chiari malformation type 2 be treated?

People with Chiari malformation type II are typically treated with surgery. Surgery may include closure of open neural tube defects shortly after birth, treatment for hydrocephalus (most often by use of a shunt), and posterior fossa decompression (creating more space for the cerebellum and relieving pressure on the spinal cord). Medical issues may involve management of neurogenic bowel and bladder, neonatal feeding difficulties, respiratory failure, and apnea.

As with any form of surgery, there are risks associated with surgery to treat Chiari malformations. Sometimes, surgery leads to no improvement or even worsening of symptoms. For example, if nerve injury in the spinal canal has already occurred, surgery will not reverse the damage. However, most people who have surgery have improvement of symptoms afterwards. Even if symptoms do not improve significantly, surgery might prevent existing symptoms from worsening.

Last updated on 05-01-20

Name: American Syringomyelia & Chiari Alliance Project ASAP PO Box 1586
Longview, TX, 75606-1586 , United States
Phone: 903-236-7079 Toll Free: 800-272-7282 Fax : 903-757-7456 Email: Url:
Name: Bobby Jones Chiari & Syringomyelia Foundation Bobby Jones CSF c/o Dorothy Poppe 29 Crest Loop
Staten Island, NY, 10312, United States
Phone: +1-718-966-2593 Fax : +1-718-966-2593 (please call first) Email: Url:
Name: Worldwide Syringomyelia & Chiari Task Force Inc. PO Box 491975
Lawrenceville, GA, 30049,
Phone: +1-914-510-CURE (2873) Email: Url:

Connect with other users with Chiari malformation type 2 on the RareGuru app

Do you have information about a disease, disorder, or syndrome? Want to suggest a symptom?
Please send suggestions to RareGuru!

The RareGuru disease database is regularly updated using data generously provided by GARD, the United States Genetic and Rare Disease Information Center.

People Using the App

Join the RareGuru Community

To connect, share, empower and heal today.

People Using the App