Controlling Chiari Malformation

The pain has returned again, another round of headaches. You feel like you must throw up. The pain is so bad that you cannot see. You could have Chiari malformation.

Chiari malformations develop when the bony space at the lower rear of the skull that holds the cerebellum and parts of the brain stem is smaller than normal. That causes the cerebellum and brain stem to be pushed downward into the upper spinal canal. There are classic types of Chiari malformation. Chiari malformation Type 1 (CM-1) is normally diagnosed in adolescence or young adulthood. The other types are rarer and diagnosed at an early age.

The pressure that results may affect the functions controlled by that portion of the brain. It may also cause a disruption in the flow of cerebrospinal fluid that cushions the brain and the spinal cord.

Chiari malformation can cause severe headaches, neck and shoulder pain, trouble swallowing and speaking, respiratory problems, balance problems and other neurological issues. Untreated, it can cause nerve damage and paralysis. Symptoms may change depending on the pressure caused by built-up cerebrospinal fluid.

Chiari malformation is also associated with other conditions, one of which is syringomyelia, a cerebrospinal fluid-filled cyst that forms within the central canal of the spinal cord. As it enlarges, the cyst can cause pain and weakness and stiffness of the back, legs, shoulders or arms.

Another condition is the tethered cord syndrome, a condition where patients are born with that is related to Chiari malformation. The spinal cord is attached to the bone by a band called the filum terminale, but in certain people that structure becomes too tense, and instead of just loosely anchoring the cord, it’s kind of pulling it down.

The condition is poorly understood and often misdiagnosed. On average, it can take more than five years of symptoms before a patient is properly diagnosed.

One of the more troubling aspects is that some Chiari patients have been told at one time or another that they were suffering from a mental or emotional problem and that there was nothing wrong with them physically, which of course later turned out not to be true.

According to the Cleveland Clinic, very rarely, a Chiari malformation can develop in someone who wasn’t born with the condition. In those cases, the skull or spinal cord may change shape due to:

·       A brain tumor.

·       A cyst.

·       Hematoma (blood accumulation).

·       Hydrocephalus.

·       Intracranial hypertension or pseudotumor cerebri.

The malformations are classified by the severity of the disorder.

Type 1-the prevalence of this type of malformation is the most common among children, where only the lower part of the cerebellum (excluding the brain stem) extends into the opening at the base of the skull, which usually has opening only for the spinal cord. This is the only type which can be acquired.

Type 2-this type is usually a consequence in children born with spina bifida (split spine defect). Also known as the classic Chiari malformation, in this both, the cerebellum and the brain stem extend into the opening.

Type 3-this, despite being a rare case, is the most serious form, as it involves herniation and protrusion of the cerebellum and the brain stem into the opening which has a great potential to cause severe neurological defects.

Type 4-being the rarest of all the other types, this type of malformation involves an incomplete/underdeveloped cerebellum and is sometimes associated with exposed parts of the skull.

Upon suspicion of the malformation, the treating doctors require a physical examination to confirm the condition which involves inspecting the proper functioning of the spinal cord and cerebellum. If the person has proper and normal body balance, motor skills, sensation, reflexes, this condition may be ruled out for further diagnostics. Other diagnostics like CT scans and MRI may be used to identify the condition with precision.

If the symptoms are not persistent and do not hinder with the quality of life, then treatment may not be necessary. While in severe cases where medication provided to subside pain, do not provide relief and interfere with daily activities, the patient may require a surgical procedure.

While surgery remains the last resort to correct the functional outcomes or curb down the progression of the damage rate to the nervous system, the surgery aims to relieve the pressure on the brain and spine, and re-establish the normal fluid circulation.