Chiari malformation is a disorder that affects the Chiari I malformation (CIM) and the Chiari II malformation (CIM). Chiari malformation is a birth defect that can cause problems with the brain, spinal cord, and nerves. Chiari malformation is most commonly found in infants and young children. Chiari malformation can cause problems with brain development, including problems with movement, vision, and hearing. Chiari malformation also can cause problems with swallowing and breathing. Chiari malformation is most commonly found in children between the ages of one and five. Chiari malformation is a birth defect that can cause problems with the brain


Many people with Chiari malformation have no signs or symptoms and don’t need treatment. Their condition is detected only when tests are performed for unrelated disorders. However, depending on the type and severity, Chiari malformation can cause a number of problems.

The more common types of Chiari malformation are:

  • Type 1
  • Type 2

Although these types are less serious than the rarer pediatric form, type 3, signs and symptoms still can be life disrupting.

Chiari malformation type 1

In Chiari malformation type 1, signs and symptoms usually appear during late childhood or adulthood.

Headaches, often severe, are the classic symptom of Chiari malformation. They generally occur after sudden coughing, sneezing or straining. People with Chiari malformation type 1 can also experience:

  • Neck pain
  • Unsteady gait (problems with balance)
  • Poor hand coordination (fine motor skills)
  • Numbness and tingling of the hands and feet
  • Dizziness
  • Difficulty swallowing, sometimes accompanied by gagging, choking and vomiting
  • Speech problems, such as hoarseness

Less often, people with Chiari malformation may experience:

  • Ringing or buzzing in the ears (tinnitus)
  • Weakness
  • Slow heart rhythm
  • Curvature of the spine (scoliosis) related to spinal cord impairment
  • Breathing problems, such as central sleep apnea, which is when a person stops breathing during sleep

Chiari malformation type 2

In Chiari malformation type 2, a greater amount of tissue extends into the spinal canal compared with that in Chiari malformation type 1.

The signs and symptoms can include those related to a form of spina bifida called myelomeningocele that nearly always accompanies Chiari malformation type 2. In myelomeningocele, the backbone and the spinal canal don’t close properly before birth.

Signs and symptoms may include:

  • Changes in breathing pattern
  • Swallowing problems, such as gagging
  • Quick downward eye movements
  • Weakness in the arms

Chiari malformation type 2 is usually noted with ultrasound during pregnancy. It may also be diagnosed after birth or in early infancy.

Chiari malformation type 3

In the most severe type of the condition, Chiari malformation type 3, a portion of the lower back part of the brain (cerebellum) or the brainstem extends through an opening in the back of the skull. This form of Chiari malformation is diagnosed at birth or with an ultrasound during pregnancy.

This type of Chiari malformation has a higher mortality rate and may also cause neurological problems.


To diagnose your condition, your doctor will review your medical history and symptoms and conduct a physical examination.

Tests may include:

  1. a)Magnetic resonance imaging (MRI). An MRI is often used to diagnose Chiari malformation

This safe, painless test produces detailed 3D images of structural differences in the brain that may be contributing to symptoms. It can also provide images of the cerebellum and determine whether it extends into the spinal canal.

  1. b)Computerized tomography (CT) scan. A CT scan uses X-rays to obtain cross-sectional images of the body. This can help to reveal brain tumors, brain damage, bone and blood vessel problems, and other conditions.


There are a few different treatment options for chiari malformation. Depending on the severity of the malformation, different treatments may be necessary. Some treatments include surgery, medication, and physical therapy. Surgery is the most common treatment for chiari malformation. Surgery may include the removal of part of the brain or the skull. Medication is often used to treat chiari malformation. Medications can help to reduce the symptoms of the malformation, such as headaches, neck pain, and difficulty speaking. Physical therapy may also be helpful in treating chiari malformation.





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