TREATMENTS &
RESEARCH

Search the
latest
treatment
information
here.

Dr. Huntley's
Diagnosis
Checklist

Have a symptom?
See what questions
a doctor would ask.

Next Prev

Diseases » Chiari Malformation » Summary

What is Chiari Malformation?

Contents

What is Chiari Malformation?

Chiari Malformation: Protrusion of the brain down the spinal column.
Chiari Malformation: A group of congenital malformations involving the brainstem, cerebellum, upper spinal cord, and surrounding bony structures. Type II is the most common, and features compression of the medulla and cerebellar tonsils into the upper cervical spinal canal and an associated MENINGOMYELOCELE. Type I features similar, but less severe malformations and is without an associated meningomyelocele. Type III has the features of type II with an additional herniation of the entire cerebellum through the bony defect involving the foramen magnum, forming an ENCEPHALOCELE. Type IV is a form a cerebellar hypoplasia. Clinical manifestations of types I-III include TORTICOLLIS; opisthotonus; HEADACHE; VERTIGO; VOCAL CORD PARALYSIS; APNEA; NYSTAGMUS; swallowing difficulties; and ATAXIA. (From Menkes, Textbook of Child Neurology, 5th ed, p261; Davis, Textbook of Neuropathology, 2nd ed, pp236-46)
Source - Diseases Database

Chiari Malformation is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Chiari Malformation, or a subtype of Chiari Malformation, affects less than 200,000 people in the US population.
Source - National Institutes of Health (NIH)

Ophanet, a consortium of European partners, currently defines a condition rare when it affects 1 person per 2,000. They list Chiari Malformation as a "rare disease".
Source - Orphanet

Chiari Malformation: Introduction

Types of Chiari Malformation:

Broader types of Chiari Malformation:

How serious is Chiari Malformation?

Complications of Chiari Malformation: see complications of Chiari Malformation
Prognosis of Chiari Malformation: Most patients who have surgery experience a reduction in their symptoms. Some patients may experience prolonged periods of relative stability. Infants with very severe malformations may have life-threatening complications. (Source: excerpt from NINDS Chiari Malformation Information Page: NINDS)