Chiari malformation is a condition that causes brain tissue to extend out and settle into the spinal canal. It protrudes out where the skull and neck meet. It occurs because part of the skull is too small or shaped in a unique way that allows part of the brain to settle into the foramen magnum. The foramen magnum is a large opening at the bottom of the skull. The brain's nerves go through into the spinal canal and join the spinal cord. However, only the nerves should be present. The brain should not be able to push/press/leak through. When it does this is Chiari malformation.
Causes
Chiari malformation can be caused by structural problem/s with the:
- Brain
- Skull
- Spinal canal
Structural conditions/issues can be present at birth, which are congenital defects. This is also called primary Chiari malformation and is not caused by any other condition. Secondary Chiari malformations are caused by something else, most often through surgery. This is extremely rare, but it is possible to develop after having surgery to remove a tumor in the skull or neck region. A surgeon could have removed too much bone while removing the tumor. This allowed the brain to settle into the open space.
Types
There are 4 types and are categorized by how much brain tissue protrudes into the spinal canal.
Type I
This is the adult version and is also the most common.
It is usually first noticed and discovered from an examination for something else. Most individuals don't realize that they have Chiari malformation unless the symptoms are severe. With Type I a part of the brain, specifically the cerebellar tonsils settle into the foramen magnum.
Type II
Type II is also known as Arnold-Chiari malformation. This is the pediatric version. Symptoms are more severe with Type II because more brain tissue comes through. With this type, the cerebellar tonsils and some of the brainstem protrude. With Type II myelomeningocele, which is a form of spina bifida is a concern. What happens is the vertebrae and spinal canal do not close correctly before birth, so the spinal cord has no protection.
Type III
This type also affects children and is more severe than types 1 or 2. Here a significant portion of the brain, including the cerebellum and the brainstem protrude all the way through the foramen into the spinal canal.
Type IV
Type IV is the most severe form. With this type, the brain does not develop properly.
Symptoms
The symptoms vary based on the type and severity. The most common symptom is a headache. Individuals with a Chiari malformation usually have headaches in the occipital region of the brain. This is the back of the head, right where the skull joins the cervical spine/neck. The headaches can be aggravated being in certain positions and actions, tilting the head back, and coughing. Typical symptoms include:
- Balance problems
- Dizziness
- Difficulty with fine motor skills
- Trouble swallowing
- Vision problems
- Weakness
However, Chiari malformation can interrupt the flow of cerebrospinal fluid. Cerebrospinal fluid is necessary to protect the brain and spinal cord. If the normal flow is disrupted it becomes more difficult for the brain and spinal cord to send/receive nerve messages. The pressure built up can also cause nerve issues/problems. For some individuals, symptoms can come and go. This depends on how much cerebrospinal fluid has built up. Individuals with Type I sometimes don't have any symptoms. It all depends on the severity.
Diagnosis
Diagnosis is done with a magnetic resonance imaging test or MRI. The MRI will show the various parts of the brain, skull, spinal cord, and spinal canal. They will be able to see abnormalities that could point to Chiari malformation.
Treatment
The recommended treatment depends on the severity. If pain is presenting a doctor could recommend pain medications to help manage the pain. Non-steroidal anti-inflammatory meds could also be recommended to reduce inflammation. Analgesics or pain killers can be recommended. Often both non-steroidal anti-inflammatories and analgesics are available in over-the-counter and prescription. The doctor will figure out the best medication treatment plan.
Surgery can be used to relieve symptoms and is the only way to relieve the pressure on the spinal cord and nerves. The goal is to stop the malformation from worsening. Surgeons use a posterior fossa decompression procedure. The surgeon removes part of the skull to make more room for the brain to sit in. This takes the pressure off the brain and spinal cord, and should reduce the neurological symptoms and problems. The surgeon may increase the size of the dura or the sac around the brain.
Laminectomies at C1 and C2, which are the first and second levels of the neck and are utilized to make more room for the brain. The surgeon will place a patch that is made from animal or synthetic tissue that will grow into the dura. The patch makes the dura bigger, which allows more room for the brain. Not all surgeries involve the dural patch.
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