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The four types of Chiari malformations are named according to their severity. Type I is the least severe and often not found until adulthood. Type II causes more severe brain malformations, but with early treatment, most children live a long, healthy life.
Whether your child has been diagnosed with a Chiari malformation type II (Chiari II) or you’re worried about their symptoms, you can turn to the team at THINK Neurology for Kids for support, compassionate care, and expertise in diagnosing and treating Chiari II.
Here, they provide the information you need to know about Chiari II malformations and their treatment.
Chiari II malformations develop before birth when the upper spine and base of the skull don’t develop properly. As a result, parts of the brain, including some of the cerebellum and brain stem, protrude down into the spinal cord.
The cerebellum is located on the lower back of your brain, while the brain stem is an elongated structure in front of the cerebellum. In Chiari II, parts of both structures push down through the opening that lets the spinal cord pass through the skull.
Most children with a Type II Chiari malformation also have another congenital disability called myelomeningocele. Myelomeningocele, a spina bifida, also occurs during fetal development when part of the spine doesn’t close properly.
This congenital disability can occur anywhere along the spine but is visible (even before birth) and often appears in the lower back. The gap in the spinal bones allows a sac-like structure (containing spinal fluid and part of the spinal cord) to bulge out from the spine.
The presence of myelomeningocele is the first sign of a Chiari II malformation. Parents may also notice symptoms such as:
Some infants with Chiari II develop hydrocephalus, a buildup of fluids in the brain. Signs of hydrocephalus include:
Older children may have other symptoms of Chiari II and hydrocephalus:
Chiari malformations may also cause an accumulation of fluid in the spinal cord. If this develops, your child may have weakness in their arms and legs, numbness or decreased sensitivity to pain, and loss of bowel and bladder control.
If your child has mild symptoms, we may recommend carefully monitoring their condition while providing medications to ease headaches or pain. Depending on your child’s age, they may also improve with treatments such as speech therapy (for swallowing problems) and vestibular rehabilitation (for balance problems).
Otherwise, surgery is the only treatment for Chiari II. The good news is that surgery often stops symptoms and prevents progressive damage to the central nervous system.
The most common type of surgery, posterior fossa decompression, creates more space for the cerebellum and brainstem by removing a small piece of bone at the base of the skull. We may also need to remove the back side of the vertebra; a procedure called a spinal laminectomy.
If needed, we can also safely remove two small areas of the cerebellum, called the cerebellar tonsils, to create additional room for the brain. These two tonsils don’t serve essential functions and can be eliminated without causing neurological problems.
Infants and children with myelomeningocele need surgery to put the spinal cord back in its normal position and close the opening in the spine. In many cases, this procedure is done while the baby is still in the womb.
If your child develops hydrocephalus, we insert a tube (shunt) that drains the excess fluid, relieves pressure, and eases symptoms.
If you have questions about your child’s health, call THINK Neurology for Kids or book an appointment online today.
