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Chiari Malformation in Children

What is pediatric chiari malformation?

Chiari malformation in children is a condition in which brain tissue extends into the spinal canal. The brain can be displaced downward by a small or misshapen skull. It’s an uncommon condition, but it is diagnosed more frequently because of advanced imaging technology. Type 1 chiari malformation, the most common form, usually develops in late childhood or adulthood, but types II and III are present at birth. Type III is a rare pediatric form that can be life disrupting.

Causes of chiari malformation

Chiari malformations are usually caused by structural defects in the brain and spinal cord during fetal development. In some cases, a lack of nutrients can result in the skull being abnormally small. The condition is also linked to a genetic condition and to contact with a harmful substance during pregnancy. An injury or infection later in life may cause excess leaking of spinal fluid, leading to acquired chiari malformation.

Signs of chiari malformation

The most common symptom is headaches. With chiari malformation type I, headaches are more common after coughing, sneezing or straining. Children might experience dizziness, weakness, neck pain, poor coordination, and difficulty swallowing. Sleep apnea is also a common symptom, causing brief interruptions in sleep. Some chiari malformations can cause hydrocephalus, or a buildup of fluid in the brain, which puts pressure inside of the head and can expand a child’s skull bones. Chiari malformation also can cause symptoms related to a type of spina bifida – a birth defect that occurs when the neural tube doesn’t close all the way in the first month of pregnancy, resulting in damage to the surrounding spinal cord and nerves.

Chiari malformation may cause breathing and swallowing problems, weakness in the arms, and downward eye movement. In the most severe form, chiari malformation type III, a portion of the cerebellum extends through an abnormal opening in the back of the skull. It is usually diagnosed during a prenatal ultrasound or diagnosed at birth. This type may also cause other neurological issues.

Diagnosis

Depending on the severity, it Is possible for chiari malformation to go undetected for years. In those cases, treatment may not be needed. In other cases, it will be identified on a prenatal ultrasound or shortly after birth. An MRI scan will several an abnormal profusion of the cerebellum into the spinal cord.

Treatment

If the condition is not interfering with daily life, your doctor may recommend monitoring it with regular MRIs. More intense monitoring can be conducted with a cine phase contrast MRI, which tracks the flow of spinal fluid and can identify any blockages. Medication is available to help with any pain. Surgery is the most common treatment for chiari malformations. Most commonly, a posterior fossa decompression will be performed to create more space for the cerebellum and to relieve pressure on the spinal cord. In a posterior fossa decompression, the neurosurgeon will make an incision at the back of the head and remove a small portion of bone. More than one surgery may be needed to correct the condition. Hydrocephalus, if it occurs, would also need to be corrected with a procedure in which a surgeon drains extra cerebrospinal fluid to help the fluid leave the brain, such as the insertion of a shunt or third ventriculostomy.

What You Can Expect at UTHealth Neurosciences

Our dedicated team uses advanced technology to accurately diagnose and treat neurological diseases and conditions impacting babies and children. We work in multidisciplinary teams of specialists and pediatric neurosurgeons who share insights, leading to better treatment decision-making and outcomes, as well as lower costs and time savings. Throughout treatment, we will work closely with the doctor who referred your family to ensure a smooth transition back to your child’s regular care. While your family is with us, they will receive expert care, excellent communication, and genuine compassion.


Contact Us

At UTHealth Neurosciences, we offer patients access to specialized neurological care at clinics across the greater Houston area. To ask us a question, schedule an appointment, or learn more about us, please call (713) 486-8000, or click below to send us a message. In the event of an emergency, call 911 or go to the nearest Emergency Room.


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