About Chiari Malformations
Chiari malformations are structural defects that affect tissue in the brainstem, cerebellum (the area of the brain at the back of the skull), and spinal cord. They can cause symptoms ranging from headaches and neck pain to issues with sleep and swallowing.
Types of Chiari Malformations
Chiari malformations can be categorized into different types, with Chiari malformation type I being the most common.
Type I symptoms can develop during childhood, adolescence, or even into adulthood. With Chiari malformation type I, the brain bulges through an opening at the base of the skull, putting pressure on the brain and spinal cord. This can block the normal flow of cerebrospinal fluid (CSF), causing hydrocephalus (a buildup of fluid in the brain) or a syrinx (a fluid-filled cyst within the spinal cord). A syrinx can lead to numbness, tingling, weakness, back or extremity pain, scoliosis, or changes in bowel or bladder function.
Type II typically affects children born with spina bifida, specifically infants with a myelomeningocele -- spinal cord tissue that protrudes through an opening in the spine.
Type III is a rare and very serious form of Chiari malformation. Some parts of the brain may stick out, or herniate, through an abnormal opening in the back of the skull.
Type IV is very rare. In this type, part of the brain is underdeveloped or missing.
Diagnosing Chiari Malformations
Duke neurosurgeons combine clinical evaluation with advanced testing to diagnose and better understand your or your child’s condition. We take time to listen and understand your medical history, symptoms, prior treatments, and their impact on your quality of life.
Physical and Neurological Exam
Your doctor will conduct a thorough physical and neurological examination to identify signs and symptoms.
Imaging
Your doctor may recommend imaging tests, such as MRI or CT scans, to confirm a diagnosis or evaluate changes over time. Other images, such as X-rays, can be beneficial if you have associated medical conditions, such as scoliosis.
Treatments for Chiari Malformations
Our neurosurgeons collaborate with neurologists, sleep medicine specialists, therapists (physical, occupational, and speech), neuro-ophthalmologists, neuro-radiologists, pain medicine specialists, and others to provide you or your child with the best care.
Monitoring
For people with less severe symptoms, we may recommend monitoring the Chiari malformation over time. In adults, this usually means tracking symptoms and updating our team if concerns come up. In children, this can mean periodic repeat imaging as they grow. In younger children, a Chiari malformation may improve on its own without surgery.
Headache Medication
Medications can help manage headache pain.
Endoscopic Third Ventriculostomy (ETV)
For people who have hydrocephalus or enlarged ventricles (open structures in the brain that hold cerebrospinal fluid) related to their Chiari malformation, endoscopic third ventriculostomy (ETV) creates an alternate route for cerebrospinal fluid to circulate in the brain. ETV is less invasive than placing a shunt or undergoing traditional Chiari decompression surgery, because surgeons create a very small opening in the skull and use an endoscope -- a thin, hollow tube with a high-resolution camera on the end -- to reach deep areas within the brain. Surgeons create a small hole in the base of the third ventricle to divert cerebrospinal fluid into a space underneath the brain. Because this procedure is minimally invasive, you or your child may be able to go home in as little as 24 hours after the procedure.
