Can You Repeat That Again

It was Tuesday April 6, 1999 and there were very few times in my life when a person could walk in the room say one sentence and catch my attention. But let someone step in and say “I am doing your surgery” and you are on full listen mode after all its not every day you get brain surgery.

I introduced myself and told the doctor that we knew nothing about Chiari and, with the advice from our doctor, had chosen to not research online because we did not want to get bad information. Dr. Boggin told us that the advice we were given was strong advice as to often people post information that is incorrect.

Editors Note:

The following information is the information that Dr Boggin gave us that day about my surgery. I have referred to the Mayo Clinic and Mayfield Chiari Center websites for accuracy. It is important to remember that each Chiari patient is as different as the disease itself and each doctor, hospital and clinic varies in the way they treat Chiari.

The information that follows is how I was treated and the information that I was given. ALSO!!!!! My surgery was in 1999 many things have changed in the medical world with medical treatment and technology. Therefore, some treatments and information are different now than they were then.

Dr. Boggin:

Chiari malformation (CM)—also commonly referred to as cerebellar ectopia or Arnold Chiari malformation—is a structural defect in the lower-rear portion of the brain (cerebellum). This is the part of the brain that controls balance.  CM is most commonly caused by a congenital defect. Many doctors were sure that developments before and directly after birth were the only possible causes of the malformation. However, studies have shown a correlation between a traumatic incident (i.e., auto accident or slip-and-fall) and symptomatic CM.

 In other words, a Chiari malformation can be asymptomatic for an indefinite period of time until a traumatic episode instigates its symptoms. For lots of injury victims, this comes as surprise since most individuals with an underlying Chiari malformation have no idea they even have it. Now, research is coming out that may suggest that trauma like auto accidents, whiplash and sports injuries can not only awaken CM symptoms, but actually cause the malformation altogether. I feel your Chiari is a direct result from your brain injuries and the fact that your brain is larger than your skull.

Chiari malformation is a structural defect, in this case an enlargement, of the cerebellum. Normally, the cerebellum and the upper portion of the brain stem sit in an indented space at the lower rear of the skull. When part of the cerebellum is too large to sit in this area and so begins to extrude below the opening, it is called a Chiari malformation. Since the bony space is smaller than normal, it causes the cerebellum and brain stem to be pushed downward into an area called the foramen magnum and into the upper spinal canal. The resulting pressure on the cerebellum and brain stem may affect functions controlled by these areas and block the flow of cerebrospinal fluid—the clear liquid that surrounds and cushions the brain and spinal cord. This can have serious repercussions in the way the brain operates and the symptoms a patient experiences.

Symptoms

Many people with Chiari malformation have no signs or symptoms and don't need treatment. Their condition is detected only when tests are performed for unrelated disorders. However, depending on the type and severity, Chiari malformation can cause a number of problems.

You have Type 1 of Chiari malformation:

·        

      Type 1

In Chiari malformation type I, signs and symptoms usually appear during late childhood or adulthood. Headaches, often severe, are the classic symptom of Chiari malformation. They generally occur after sudden coughing, sneezing or straining. People with Chiari malformation type I can also experience: (Bolded signify my symptoms)

·         Neck pain  

·         Unsteady gait (problems with balance)

·         Poor hand coordination (fine motor skills)

·         Numbness and tingling of the hands and feet

·         Dizziness

·         Difficulty swallowing, sometimes accompanied by gagging, choking and vomiting

·         Vision problems (blurred or double vision)

·         Speech problems, such as hoarseness

Less often, people with Chiari malformation may experience:

•    Ringing or buzzing in the ears (tinnitus)
•    Weakness
•   Slow heart rhythm
•   Curvature of the spine (scoliosis) related to spinal cord impairment
•   Abnormal breathing, such as central sleep apnea.
•    Fainting

·          Weakness of the legs

•   Double vision
•   Deafness
•   Swelling of the optic nerve region
•    Lack of muscular coordination
•   Uncontrolled shaking or trembling 

The complications associated with this condition include:

·      Hydrocephalus:   An accumulation of excess fluid within your brain (hydrocephalus)may require placement of a flexible tube (shunt) to divert and drain the cerebrospinal fluid to another area of your body.

·       Spina bifida:  Spina bifida, a condition in which your spinal cord or its covering isn't fully developed, may occur in Chiari malformation. Part of the spinal cord is exposed, which can cause serious conditions such as paralysis. People with Chiari malformation type II usually have a form of spina bifida called myelomeningocele.

·       Syringomyelia:  Some people with Chiari malformation also develop a condition called syringomyelia, in which a cavity or cyst  forms within the spinal column.

·      Tethered cord syndrome: In this condition, your spinal cord attaches to your spine and causes your spinal cord to stretch. This can cause serious nerve and muscle damage in your lower body.

Surgery

Doctors usually treat symptomatic Chiari malformation with surgery. The goal is to stop the progression of changes in the anatomy of your brain and spinal canal, as well as ease or stabilize your symptoms. When successful, surgery can reduce pressure on your cerebellum and spinal cord, and restore the normal flow of spinal fluid.

What happens during surgery

Step 1: Prepare the patient:
You will lie on the operating table and be given anesthesia. Once asleep, your head will be placed in a 3-pin skull-fixation device, which attaches to the table and holds your head in position during surgery. An inch wide strip of hair is shaved along the planned incision. The scalp is prepped with an antiseptic.

Step 2: Make a skin incision:
An incision is made in the skin, down the middle of the neck, allowing the surgeon to gently spread the muscles apart. The skin incision is about 3 inches long (Fig. 14). The skin and muscles are lifted off the bone and folded back, giving the surgeon a clear view of the skull and the top of the spine.

Figure 14. A 3 inch incision is made down the middle of the neck. Shaded areas represent bone to be removed.	Figure 15. A small portion of the skull is removed (craniectomy). The dura covering the brain is seen.

Step 3: Remove bone:
The surgeon removes a small section of skull at the back of your head (suboccipital craniectomy). In my cases the bony arch of the C1 vertebra may be removed (laminectomy). These steps expose the protective covering of the brain and spinal cord called the dura (Fig. 15). Bone removal relieves compression of the tonsils.

Step 4: Open the dura:
Next, the surgeon opens the dura to view the tonsils and cisterna magna (Fig. 16Sometimes bone removal alone may restore normal CSF flow.

Step 5: Reduce the tonsils: (optional)
Depending on the size of herniation, the stretched and damaged tonsils may be shrunk with electrocautery. This shrinkage ensures that there is no blockage of CSF flow out of the 4th ventricle. I did not get this.

Step 6: Attach dura patch:
A patch of synthetic material or the patient’s pericardium (a piece of deep scalp tissue just outside the skull) is sutured into place (Fig. 17). This patch enlarges the dura opening and the space around the tonsils. The dural patch is sutured in a watertight fashion. The suture line is covered with a dural sealant to prevent CSF leak (Fig. 18). My patch is made of Bovine Pericardium.

Figure 16. The dura is opened. Figure 17. A patch is sewn to the dura to enlarge the CSF space. Figure 18. A dural sealant applied to the suture line prevents CSF leak.

Step 7: Close the Incision
The strong neck muscles and skin are sutured together. A dressing is placed over the incision.

RISKS

General complications of the surgery include bleeding, infection, blood clots, stroke, reactions to anesthesia, and death . You must remember that the brain and spine are sensitive areas so there are risks. Specific complications related to a Chiari decompression craniotomy and duraplasty may include: Risk of head and neck pain is variable.

Results

Exceptional headache and neck pain respond well to decompressive surgery as do most of the brainstem signs (e.g., swallowing problems, facial pain/numbness, voice changes, tinnitus, eye problems, dizziness). Recovery of sleep problems, memory, and spinal cord signs (e.g., numbness or tingling in hands and feet, muscle weakness) take longer and may not completely return to normal.

  

I was spinning in my mind and had to absorb it all in. He then said “Your brain has set on your spine for some time, while the surgery will relieve pressure the symptoms may remain or take many years to heal. I want to do the surgery on April 20, 1999. I said why are we in such a hurry? He stated that the reason my legs were starting to fade was because of the restricted flow of my spine and that it could lead to paralysis and in some cases death.  

Were we scared? Maybe a little but we knew that we would not let fear control us as we served an awesome God. WE STILL DO!

Isaiah 41:10

So do not fear, for I am with you: do not be dismayed, for I am your God. I will strengthen you and help you; I will uphold you with my righteous right hand.

So we headed home. It would be a short few days and we would return. During those days we would be loved and prayed for by family and friends and we would be reminded time and time again that we were not walking alone. Now, more than ever, we were being carried as we made our way on “My Journey.”