Chiari malformation is a structural issue in the cerebellum, in which brain tissue extends downward into your spinal canal.
This rare condition occurs when the space at the bottom rear of your skull is smaller than it should be. This can cause the cerebellum and brain stem to be pushed downward, blocking cerebrospinal fluid, resulting in excess pressure on the cerebellum and brain.
The malformation may be present at birth or develop while the brain is growing and not show symptoms until adulthood.
Symptoms of Chiari Malformation
Many people never experience symptoms of Chiari malformation and never need treatment. However, the most common symptom is a severe headache that starts in the back of your head and radiates forward, usually after coughing and/or sneezing.
Other symptoms include:
- Neck pain
- Loss of muscle strength in hands and arms
- Dizziness or balance problems
- Double or blurred vision
Diagnosing Chiari Malformation
Most often, a diagnosis comes by ruling out all other possibilities. Your doctor will review your symptoms and medical history and perform a physical exam. You will also have a range of imaging tests, such as CT and MRI scans.
Because the condition is rare, it’s important to see a doctor with experience. Experts at the UPMC Chiari Center take a comprehensive approach to evaluating, diagnosing, and treating Chiari malformations.
Chiari Malformation Surgery
The primary treatment for Chiari malformation is surgery.
- The most common surgery is called posterior fossa decompression. A neurosurgeon removes a small section of bone in the back of the skull to make more space for your brain.
- The surgeon may also open the covering of your brain (the dura) and sew a patch in place to enlarge the covering. This makes more space and relieves the pressure of blocked cerebrospinal fluid, which is what causes symptoms.
- In some cases, your doctor may be able to perform the surgery using the Endoscopic Endonasal Approach (EEA). This technique accesses the malformation through the nose with no incisions.
Chiari symptoms can overlap with other neurological conditions, so it’s important to get a thorough evaluation from an experienced neurosurgery team.