Institute for Brain Protection Sciences

Chiari Malformation

Chiari (kee-AR-ee) malformation occurs when the bony area covering the lower part of the brain is smaller than normal. This can cause part of the brain to push through the skull into the spinal canal and a syrinx, or a collection of fluid, may form. Commonly present at birth, this condition is related to family history in about 10 to 12 percent of cases but no identified genes.

Our team is led by the most experienced pediatric Chiari malformation neurosurgeon in Florida and works closely with other specialties within the Johns Hopkins All Children’s Institute for Brain Protection Sciences to address any associated conditions.

Conditions We Treat

  • Type 0: Identified by an accumulation of fluid within the spinal cord without a blockage of the spinal canal.
  • Type 1 (most common): A blockage greater than 5-7 millimeters is present.
  • Type 2: Associated with myelomeningocele, a form of spina bifida. These patients rarely need treatment of their Chiari. 

A classic symptom of Chiari malformation is a headache that worsens with sneezing, coughing or straining. Other symptoms may include neck pain, difficulty swallowing, gagging, scoliosis which may or may not be associated with a syrinx, and sleep disturbances. While most Chiari 1 patients have no other associated conditions, kids with other types may also have:

What to Expect

To confirm a Chiari malformation your child will need an imaging scan, typically an MRI. In some cases there may also be a sleep study.

For patients who are not showing symptoms, unless a syrinx is present, surgery is usually not required. When surgery is necessary, one of two approaches may be taken:

  • Bony decompression–The procedure relieves pressure by removing small pieces of bone. Recovery time is about two to four days on the surgical unit.
  • Bony decompression plus duraplasty–This procedure provides more extensive decompression by opening the covering of the brain and spinal cord and placing a patch to expand the area. Post-operative recovery involves an overnight stay in the pediatric intensive care unit followed by a two- to three-day stay in the surgical unit.

After surgery children can usually return to school within a week, but should avoid strenuous physical activity for several weeks. Follow up visits are scheduled around two weeks and six months post-procedure. An updated MRI scan may also be needed.

Resources

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