My Daughter, Kelly, suffers from a “disease” for which she has had 2 brain surgeries in the past 2 years. This is a very uncommon and little known syndrome, so I wanted to talk a little bit about it!

Arnold-Chiari Malformation or ACM, is an uncommon structural condition affecting the cerebellum. Essentially there is extra cerebellum crowding the outlet of the brainstem/spinal cord from the skull on its way to the spinal canal. This crowding will commonly lead to severe headaches, neck pain, loss of balance, tingling in the arms and/or legs, stiffness, and less often will cause problems such as chronic coughing, difficulties with swallowing, and choking. Often the symptoms are made worse with straining.

Untreated, the chronic crowding of the brainstem and spinal cord can lead to very serious consequences including paralysis. In addition they can lead to the development of syrinxes (Cysts in the spinal cord) which may further injure the patients spinal cord and function. When the diagnosis is suspected the study of choice is an MRI scan. These malformations are very difficult to see on CT scans and impossible to see on plain x-rays.

There are many ways to treat Chiari malformations, but all require surgery. The basic operation is one of uncrowding the area at the base of the cerebellum where it is pushing against the brainstem and spinal cord. This is done by removing a portion of bone at the base of the skull deep to the neck muscles as well as often removing a part of the back of the first and occasionally additional spinal column segments.

in layman’s terms ….
The procedure involves removing a small section of skull bone at the rim of the foramen magnum (the hole in the base of your skull where your brainstem/spinal cord goes through) , and then they also (not always) will put a dural graft in the area of the Chiari malformation to allow for more room back there. Also (and again.. not always) they will remove the back section of the c1 vertebra and possibly the C2 also to give the cerebellar tonsils even more room to co-exist down in the foramen magnum with your brain stem/spinal cord. This allows for the free flow of cerebral spinal fluid (and is a great insurance policy against getting a syrinx (cyst on the spine) (or syringomyelia). If you already have syringomyelia, the new free flow of spinal fluid will often allow the syrinx to dissipate on it’s own. In some cases a shunt is installed.

Kelly has had 2 surgeries as I said earlier, but she is a fighter and most times you would never know anything was wrong with her physically. I just wanted to talk a bit about this syndrome so that more people know about it as well as in hopes that there can be more education and research out there!