Chairman, Department of Neurological Surgery
Chiari malformation is a congenital anomaly of the posterior part of the skull and the brain and what happens is that in the back of the skull there is a hole called the foramen magnum through which the spinal cord comes out. However in some cases part of the cerebellum which is the back of the brain which it’s back here protrudes, it herniates down that, that opening and it obstructs the flow of cerebral spinal fluid creating a high pressure system and the patients get symptoms from that.
There are symptoms with Chiari malformation that are more typical of the syndrome, for example a headache that begins in the back of the head and radiates forward, most often associated with coughing or sneezing or lifting a heavy object. That’s the most typical symptom of a Chiari malformation. There are other symptoms that can also present, for example numbness and tingling of the hands and shoulders, trouble swallowing, ringing in the ears and a variety of different symptoms.
It is important when the diagnosis of a symptomatic Chiari malformation is made that a number of different issues are identified. First is that their imaging is characteristic of a Chiari malformation and the important part of that is that the posterior part of the brain called the cerebellum is herniating down through the opening of the base of the skull called the foramen magnum, that causes a compression of the spinal cord for example and the fluid of the brain cannot flow freely through that area. Other than the imaging which is critical is also the symptoms; it’s important that the patient has appropriate imaging characteristics of a Chiari malformation that goes together with the symptoms, and that’s the best way of making the diagnosis.
The most critical part of managing a patient with Chiari malformation is having the accurate diagnosis. Once the diagnosis is made and one is confident of the diagnosis the treatment is surgical. The problem with a Chiari malformation is a mechanical problem; there is too much brain tissue essentially in the opening of the base of the skull, so it requires a mechanical solution. We need to open it up with surgery, and what is done is to open up the bone in the back of the head, opening up the coverings of the brain and then going in carefully with a microscope and making sure that there is no scarring where the fluid of the brain is supposed to flow. Once we are assured there is no blockage there then we place a patch to provide more space in that area and close the wound.
What’s most critical in evaluating a patient with headaches and a number of other neurologic symptoms is to determine if the patient’s symptoms are related to the Chiari malformation observed on an MRI for example. Here at UPMC, our team has seen many, many patients with these kinds of symptoms and we are able to make the determination if the symptoms are or are not related to the Chiari malformation and therefore the patients that are treated have the best chances of getting better from the procedure.
For more information, call us at (412) 647-6358, or visit us at neurosurgery.pitt.edu.