Dan Heffez, MD, Neurosurgery and Medical Director, Columbia St.
Mary’s Wisconsin Chiari Center
Often misdiagnosed and misunderstood, Chiari malformation is a relatively rare structural abnormality at the base of the skull that affects the brain and spinal cord. While not life-threatening, it can cause a wide range of debilitating symptoms.
Patients often suffer from Chairi malformation for years without ever receiving an official diagnosis. I have treated patients who have been living with headache, pain, dizziness and a host of other symptoms sometimes for decades. They may have seen countless doctors who were unable to provide any long-term relief. Often, the diagnosis just goes unrecognized. That's where the Wisconsin Chiari Center comes in.
Many describe Chiari malformation as an abnormality of the brain, but that is not accurate. The brain is entirely normal. The malformation involves the base of the skull, which is too small to accommodate the lower portion of the brain. As a result, the lower part of the cerebellum, called the tonsil, protrudes into the upper spinal column. This results in compression of the brain stem and spinal cord, and an interference with the circulation of the spinal fluid.
What makes Chiari malformation so difficult to properly diagnose is the wide range of vague symptoms that affect so many different parts of the body. Also, despite the fact the Chiari malformation is present at birth, symptoms typically don’t appear until early adulthood.
The most common symptom is chronic, unexplained headaches, often described as intense pressure that begins at the back of the head and radiates behind the eyes. Chiari headaches can easily be confused with migraines, which further delays diagnosis.
Other symptoms include neck pain that radiates down the spine and across the shoulders, dizziness/vertigo, vague pains throughout the body, impaired balance/clumsiness, chronic nausea, foggy thinking, poor memory and concentration, frequent or urgent urination, irritable bowel syndrome, auditory symptoms (such as ringing in the ears, decrease in the ability to hear or sensitivity to sounds), difficulty swallowing, hoarseness and visual symptoms (including double vision, sensitivity to lights and blind spots). If you are suffering from any combination of the above symptoms, I recommend you talk to your doctor about the possibility of Chiari malformation.
The diagnostic process begins with a complete medical history followed by a detailed neurological examination. An MRI scan is the best diagnostic tool for detecting Chiari malformation. This test provides us with detailed anatomical information regarding the structures at the base of the brain and allows us to determine if the brain stem is being compressed.
If it is determined you have a Chiari malformation, there are several treatment options. Some people with a Chiari malformation experience no symptoms – in these cases no treatment is required. Some symptoms of Chiari malformation such as headache and dizziness may be effectively treated by medication. Medication alone, however, will not correct the root cause of the symptoms: the brain stem compression. As a rule, when the Chiari malformation causes symptoms that seriously impair the patient’s quality of life, surgery should be considered.
The goal of surgery for Chiari malformation is to make more room for the brain. A surgeon will remove a small section of bone at the back of the skull and spine. A patch of tissue is then sewn into the lining of the brain that expands the space and relieves compression of the brain stem and spinal cord. Most people who have had decompression surgery report immediate relief of many symptoms, and once surgery is performed the problem does not recur.
If your life has been challenged by a host of mysterious symptoms without any clear cause, you may be suffering from a Chiari malformation. The Wisconsin Chiari Center might just be able to help.
Dr. Dan Heffez is a neurosurgeon and medical director of Columbia St. Mary’s Wisconsin Chiari Center. For more information, call 414-585-1078.
This article appeared in the Thursday, September 10, issue of The Ozaukee News Graphic.