Arnold Chiari malformation occurs when brain tissue extends into the spinal canal. Yes, it sounds complex and it is, that’s why I always try to be very clear and understanding with concerned parents who consult about the health of their little ones.
So let’s talk in simpler words. Chiari malformation is an anomaly in which the cerebellum (coordinator of muscle movement) protrudes and occupies part of the space of the spinal cord. It is usually congenital and could be hereditary.
Basically, it happens because part of the skull is smaller than normal or misshapen, pressing on the brain and forcing it down.
This condition is also called tonsillar hernia or tonsillar ectopia, since the part of the cerebellum that descends is called the “cerebellar tonsils.”
Chiari malformation is diagnosed from:
- Medical history
- Symptom assessment
- Physical examination
- Magnetic Resonance Imaging or Computed Tomography
Now, this condition is classified into 3 types, depending on the anatomy of the brain tissue that moves into the spinal canal:
Chiari Malformation Type 1: Signs and symptoms usually appear during late childhood because it develops as the skull and brain grow. It is the most common, and sometimes people do not know they have it until adulthood.
You have symptoms such as:
- Severe headaches.
- Numbness and tingling in the hands and feet.
- Dizziness.
- Difficulty swallowing, nausea, choking, and vomiting.
- Insomnia and depression.
- Neck Pain.
- Problems with balance.
- Poor hand coordination.
People with this condition may also have ringing in the ears, scoliosis, a slow heart rate, and breathing problems.
For its part, Chiari malformations of type 2 and 3 are pediatric forms and are present at birth (they develop inside the mother’s womb).
Chiari Malformation Type 2: Occurs when more tissue extends into the spinal canal. Your symptoms are:
Abrupt changes in breathing.
- Swallowing problems, such as gagging or nausea.
- Rapid, downward eye movements.
- Weakness in the arms.
An important fact to consider is that the signs of this disease can be added to those related to myelomeningocele (a form of spina bifida that almost always accompanies Chiari type 2 malformation).
This condition is usually detected with an ultrasound during pregnancy. It can also be diagnosed after birth or in early childhood. The same goes for Chiari type 3 malformation.
Chiari malformation type 3: It is rare and serious. It occurs when a portion of the cerebellum or brain stem protrudes or slides through an opening (tiny pit) in the back of the skull.
This type of Chiari malformation has a higher mortality rate and can also cause neurological problems.
Now, what is the treatment for Chiari malformation?
There is no cure for this condition, but there are mechanisms to relieve symptoms. In fact, when the severity and characteristics of the injury warrant, surgery is chosen to stop the progression of changes in the anatomy of the brain and spinal canal.
Surgery usually reduces pressure in the cerebellum and spinal cord (decompression), and restores the flow of cerebrospinal fluid, giving the patient quality of life.
What risks does surgery involve? For a matter of transparency and professional ethics, I always tell my patients that after this type of surgery there is the possibility of suffering from infections, fluid in the brain, fluid leakage or difficulties with wound healing.
Finally, and after surgery, follow-up tests, such as diagnostic imaging tests, are required. If you have questions or need more information about Chiari malformation, contact me, I am a specialist in Neurosurgery and I will be honored to advise you.
