Aronold-Chiari malformation is a form of hydrocephalus that is usually not discovered until adulthood. The reason for this is though it is present from birth, as a rule individuals will not have issues or symptoms until they are grown. Before birth, the posterior fossa does not form properly. Currently this is believed to be a genetic condition, however a few documented cases of birth and head trauma also point to a possible cause of Chiari development in some cases.
Because the posterior fossa is small, the brain stem, cerebellum, or cerebellar brain tissue becomes pressed downward through an opening at the bottom of the skull. The cerebellum or the brain stem may extend beyond the skull in some cases pushing into the spinal column. The displaced tissues may obstruct the flow of cerebrospinal fluid CSF, the substance or “water” that flows around the brain and spinal cord nourishing them and removing waste. This is the same fluid blocked in more commonly known and diagnosed forms of hydrocephalus.
When a patient develops symptoms of Chiari malformation, they often resemble symptoms of other medical issues, so diagnosis may be difficult to catch at first. The most common symptom is a headache that begins at the base of the skull or neck and usually radiates though the back of the head. The simple act of sneezing, coughing, or leaning over to pick something up may set these headaches off. They can me simple lasting on a few minutes to hours and are sometimes linked with nausea.
Patients report more pain on one side, a weakness in the arm or hand. There may be a period of prickly feeling in the arm and hand, a numbness or burning sensation. They may also report spells of dizziness or being unsteady on their feet. Difficulty swallowing or the feeling that food catches in their throat may be reported. Vision issues such as blind spots, double vision, or a drop of one or both eyes.
When Chiari malformation is suspected an MRI scan of the brain will show the crowing of the space between the spinal cord and brain. Treatment is usually surgery to relieve pressure on the cerbellar area. This is done by removing a small part fo the bone at the base of the sckull. By opening up the space decompression of the posterior fossa allows for a normal flow of CFS. During surgery the doctor will check for any brain tissue blocking the flow of CFS, and will shrink the brain tissue if this is found to be an issue.
Once the surgery is over prognosis in excellent. During the recovery time, some symptoms of Chiari may still be experienced, but will diminish as healing takes place.
Chiari may be present with other disabilities, and in these cases may be worse or have more extreme symptoms. As with any disorder in your child or yourself, if you suspect there is something more going on it never hurts to investigate. Keep a list of issues and when they occur, this will help in diagnosing any new or uncovered issue.
Chiari is not as uncommon as once thought, occurring in about one in every 1,000 births. If you suspect Chiari it’s a good idea to bring up the subject with your physician. The best cure for any illness is prevention, and while Chiari could not be prevented, it certainly can be detected, treated and dealt with.
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