Chiari Malformation

What is Chiari Malformation?

Chiari malformation is a neurological disorder that occurs from an abnormality in the brain’s cerebellum which causes it to push through the opening in the skull into the spinal area. This malformation causes pressure on both the brain and spinal canal which can cause headaches, neck pain and other symptoms.

Although Chiari malformation is rare, there are different sub-types. The most common is identified as type I and is congenital (present at birth). Symptoms for this type typically develop in adolescence or early adulthood as the brain and skull grow. Other sub-types such as type II are also congenital but are detected in vitro or at birth and most commonly affect children with neural tube defects such as spina bifida. Chiari malformation is more common in females than males.

What Causes Chiari Malformation?

Chiari malformation is caused by the defective development of the brain during pregnancy. While present at birth, it often goes undetected because symptoms do not usually present themselves until later developmental stages of growth in adolescence. In rare instances, acquired Chiari malformation can develop in adults as a result of trauma, injury or infection.

Symptoms

Many patients with Chiari malformation type I never experience symptoms and the condition is discovered during diagnostic imaging tests. For those with more severe malformation, symptoms include:

Headache, neck and lower back pain which intensifies during physical exertion
Sleep apnea
Hoarseness and difficulty swallowing
Dizziness, loss of balance and limb weakness
Presence of a syrinx – a fluid-filled sac on the spinal cord that is closely associated with Chiari malformation

Diagnosis

At the Face Pain and Headache Clinic, we believe the best physical and emotional outcomes for our patients begin with our expert diagnostic team. The board-certified neurological specialists at UTHealth Houston Neurosciences are trained across multiple medical disciplines, including neurology and neurosurgery, and are highly skilled in quickly and thoroughly diagnosing patients with Chiari malformation through a variety of tests and procedures which may include:

Neurological exams of the eyes, vision, muscle strength and reflexes
MRI (magnetic resonance imaging – a test that uses magnetic waves to create images of structures inside the head)
CT (computed tomography – a series of x-ray images taken from different angles to produce cross-sectional images)

Advanced Treatment for Chiari Malformation

Each patient’s treatment options are unique, and will vary based upon their overall health, age and other factors such as the severity of the symptoms and the degree to which they affect quality of life. Many patients with Chiari malformation do not experience any symptoms, and no treatment will be required other than watchful monitoring through regular exams.

Common treatments for Chiari malformation include:

Posterior fossa decompression- a surgical procedure to relieve pressure
Electrocautery to narrow the lower cerebellum
Spinal laminectomy to increase the size of the spinal canal
Medications to manage pain

Related Conditions and Treatments

Contact Us

At UTHealth Houston 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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