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 What Problems Can Chiari Malformation Type I Cause?

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Many children with Chiari malformation type I don’t have symptoms or develop problems. But the very nature of Chiari I — a structural problem that lets part of the brain protrude into the spinal canal — means that problems can be serious if they appear.

Our THINK Neurology for Kids team — which includes Shaun S. Varghese, MD, Cristina R. Marchesano, MD, Lorena Herbert, MD, Barbara Kiersz-Mueller, DO, Lauren Weaver, MD, Alicia Walls, MD, Sundeep Mandava, MD, Patricia DeForest DO, Jennifer E. Martin, CPNP-PC, Tammy DeLaGarza, FNP-C, Heather King, CPNP-PC, and Robby Korah, FNP-C — specializes in identifying and treating Chiari malformations.

Their personalized care supports each child's healthy, thriving life by lowering their risk for the following Chiari I problems.

Headaches

Headaches are the most common sign of Chiari I. These headaches primarily affect the back of the head or neck and may radiate to the shoulders. Your child's pain could be severe, sharp, or throbbing.

You may notice that your child's headache worsens when they're active or laugh, sneeze, or cough.

Eye and vision problems

Visual symptoms, another relatively common sign of Chiari I, include blurry vision, sensitivity to light, and eye pain. Some children develop strabismus or nystagmus.

Strabismus occurs when both eyes don’t look in the same direction, causing crossed eyes or other problems. For example, both eyes may turn inward or outward instead of moving in sync.

Syrinx

A syrinx is a fluid-filled cyst that develops in the spinal cord. Though several conditions can cause syrinxes, Chiari malformations are the most common cause.

The cyst usually starts in the upper cervical spine (neck) as Chiari I blocks the flow of cerebrospinal fluid, and the fluid accumulates in one area.

If the cyst enlarges, it presses against the nerves and can extend down into the spinal cord. The cyst may involve a ventricle (a hollow area in the brain) or the brainstem, the stalk-shaped part of the brain that connects with the spinal cord.

A syrinx doesn’t cause symptoms unless it compresses the spinal nerves. If that happens, your child may have problems like:

  • Neck, back, shoulder, arm, and leg pain
  • Difficulty using their arms or legs
  • Muscle weakness
  • Loss of sensation

Being unable to feel pain, changes in temperature, pressure, and other sensations is a sign of advanced nerve damage.

Scoliosis

Chiari I is associated with a higher risk of developing scoliosis, especially in children who develop syringomyelia (a cyst at the top of their spinal cord). Up to 36% of kids with Chiari malformation and syringomyelia also have scoliosis.

Scoliosis causes an unusual side-to-side curve in the spine. Children seldom experience pain or discomfort due to scoliosis. Instead, you may notice that their shoulders or waistline are uneven, or one hip may be higher.

Permanent nerve damage

Any health condition that pushes against nerves in your brain and spine can result in permanent nerve damage. A syrinx is among the top causes of nerve damage. However, the structural changes associated with Chiari I can also compress the nerves.

Chiari malformation type I occurs when the lower part of the brain drops down through the opening where the brain meets the spinal cord. These changes put unusual pressure on the brain and spinal cord.

Additionally, between 30-50% of people with Chiari I have structural problems that can affect nerves. For example, the spine may fuse to the skull, or the first two cervical vertebrae may partially grow together.

Central sleep apnea

There are two types of sleep apnea: obstructive (OSA) and central (CSA). Both temporarily stop breathing many times while your child sleeps, but have different causes.

When doctors (and most people) refer to sleep apnea, they mean OSA. OSA stops air flowing from your nose or mouth to your lungs when tissues like your tongue and throat muscles fall over the airway.

CSA occurs when your brain doesn’t send nerve signals to the muscles that control breathing. Brain tumors, traumatic brain injuries, and Chiari malformations can all cause central sleep apnea. 

Children may wake up during the night, have morning headaches, lack focus, and be unusually tired, irritable, and oppositional the next day. Your child may also snore, but that’s not a key symptom of central sleep apnea.

Call THINK Neurology for Kids if you notice signs of Chiari I problems or have concerns about your child’s health. You can also connect online to request an appointment at their offices in The Woodlands, Katy, Sugar Land, Lakeway, San Antonio, and Lake Jackson, Texas.