Thursday, September 23, 2010

Living with Chiari

I first took notice of Chiari Malformation when I found out a friend was living with Chiari. She had been diagnosed before I met her, and I would have never guessed that she suffered from anything other than a bright and cheerful attitude. Working in the medical field I knew of the condition but as far as I know I had never before encountered anyone with Chiari. Like so many other bits and pieces of information I had picked up in school it got shuffled into the background.

For those who do not know Chiari Malformation is a congenital condition where a part of the brain protrudes through a opening in the base of the skull called the foramen magnum and into the spinal canal. This part of the brain, known as the cerebellar tonsils, which normally lie above the foramen, puts pressure on the spinal canal. This pressure can cause pain in the back of the neck and radiating upward. Other symptoms of Chiari vary widely and can include blurred vision, muscle weakness, unsteady gait, facial numbness and trouble swallowing.

A related disorder found often, but not always in people with Chiari is Syringomyelia. Syringomyelia is when cerebrospinal fluid, also known as CSF, enters the interior of the spinal cord. There it forms a syrnix or cavity. The syrnix can expand over time which destroys the center of the spinal cord. In most cases Syringomyelia, like Chiari, is congenital. However a second form of Syringomyelia can be brought on by trauma. In people with Chiari the protruding part of the brain blocks normal CSF flow and can cause a syrnix. However not everyone with Chiari will develop Syringomyelia.

Treatment for Chiari Malformation can range from pain management with medication in mild cases to surgery. The surgery is known as posterior fossa decompression. In this surgery small portions of bone form the back of the skull are removed. This allows the tonsils to move back into their normal position in the skull and reduces compression of the brain stem. This surgical procedure gained notoriety in the horror movie Saw. The villain Jigsaw, though not suffering from Chiari had the surgery to remove pressure from a brain tumor.

Surgery is the only viable treatment for Syringomyelia. While in mild cases  continued at
My friend Alicia who is living with Chiari

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