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Chiari malformation

What is a Chiari malformation?

Chiari malformation diagram

With a Chiari malformation, the space for the lower part of your brain (called the cerebellum) is either too small or unusually shaped. This can cause part of the cerebellum to push into the funnel-like opening at the bottom of the skull (called the foramen magnum) and into the space occupied by the spinal cord.

There are several types of Chiari malformations 

The most common type in the children we serve at CHoR’s center for craniofacial care is a Chiari 1 malformation. The development of a Chiari 1 malformation can be related to abnormal growth of the facial bones or skull (craniofacial conditions).

A Chiari 2 malformation is a specific type found most often in children with spina bifida.

Chiari 3’s are more severe malformations and may not support life.

Children with complex craniosynostosis or a craniofacial syndrome (abnormalities of the face and skull and possibly other areas) are at risk for developing a Chiari malformation.

What causes a Chiari malformation?

The exact cause is not known. Suspected causes include early joining together of the bones of the skull that enclose the brain and the joints that form the skull and raised pressure within the skull. Blood flow and heart valve problems and hydrocephalus may also be a cause.

For children with craniofacial conditions, the Chiari malformation may develop in the first months of life and worsen over time. This may be due to the lower part of the brain growing too much while that part of the lower part of the skull remains unusually small.

What are the symptoms of a Chiari malformation?

Symptoms of Chiari malformation in infants include:

  • Difficulty swallowing
  • Irritability when being fed
  • Excessive drooling
  • A weak cry
  • Gagging or vomiting
  • Arm weakness
  • A stiff neck
  • Breathing problems
  • Developmental delays
  • Problems gaining weight

For toddlers and older children, symptoms may include:

  • Breathing problems
  • Neck pain
  • Balance problems
  • Muscle weakness
  • Numbness or other abnormal feelings in the arms or legs
  • Dizziness
  • Vision problems
  • Difficulty swallowing
  • Ringing or buzzing in the ears
  • Hearing loss
  • Vomiting
  • Difficulty sleeping
  • Depression
  • Headaches made worse by coughing or straining
  • Hand coordination and fine motor skills may also be affected

It’s important to note that not all children with Chiari malformations develop symptoms. Children with a high risk of developing a Chiari malformation are monitored with MRI imaging studies. Sleep studies may also be used to determine if breathing is affected.

How do you treat a Chiari malformation?

In some instances, surgery to enlarge the space within the back of the skull may be needed to treat a Chiari malformation. This may involve surgery to reshape the bones of the back of the skull or remove bone at the back of the skull.

Why choose CHoR for Chiari malformation treatment?

  • Our center is made up of providers from over 15 specialties: Our craniofacial center is made up of health care providers who are part of specialized, multidisciplinary care teams.
  • Our pediatric neurosurgeons are the most experienced in the region: Our dedicated and compassionate surgeons utilize state-of-the-art technology and minimally invasive procedures to address the needs of those we serve. In addition, we provide the only dedicated 24/7 pediatric anesthesia team in the community. Each member of the team has specialized training in pediatric anesthesiology, and all have considerable experience in pediatric surgery.
  • A unique treatment plan is created for your child: Our teams meet weekly to evaluate and develop a treatment plan for each child. Team members see the child and family during multidisciplinary clinic appointments, then meet face to face to develop a plan based on all the needs of the child. They are available to follow a child between appointments as needed or to work with a child’s local health care providers.



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