Along with my SCI I also have to be aware that some of my symptoms may be occurring because of Chiari. For me, two of the most common symptoms I deal with is Spinal headaches and vision changes. I always have to be aware that if my symptoms stay at a heightened level that decompression surgery may be necessary.
In the past few months
In April after a long discussion with my Neurosurgeon and husband I decided to go ahead with the surgery to remove the Arachnoid cysts which were growing in size and put more pressure on my spinal cord. The two known cysts had formed in the Arachnoid space in my thoracic spine. My symptoms of numbness in my legs, gait disturbances, feeling of spiders crawling down my back and legs, and over-all pain had increased as well making the decision for surgery fairly easy.
The procedure was explained. A small 2-3 inch incision would be made down the middle of my back, then the spiny processes would be removed at T4-T7, and then the cysts would be drained and removed. The spiny processes would then be put back into place with small screws. The procedure would last about three hours in length. I would then have an overnight stay in the hospital. I would have some physical limitation of no lifting anything over 5 lbs for at least six weeks, no strenuous exercise for 6 weeks and no driving for at least a week.
On May 3rd the surgery was performed. The surgery took about 3.5 hours. There was one complication in that I bled more than they thought and two pints of plasma were required. The doctor stated that the surgery went well. He stated that the cysts were a bit more tangled than expected, but despite that all went well. I was up and walking the next morning. I felt pretty could minus the soreness at the incision site.
I was discharged home later the day after my surgery. My discharge instructions were to walk as much as possible, no lifting anything over 5 lbs., no bending or reaching due to the fall risk, and to rest as much as possible. I was also discharged with several medications Neurontin 600 mgs twice a day, Amitriptyline 20 mgs at bedtime, Flexeril three times per day, and Hydrocodone 10-325mg every 4 hours for pain. Over the course of the next 7 days I appeared to be healing well and felt pretty good. I was doing everything I was told.
On May 10th I felt more sore than I had the previous day. I was still feeling pretty good. My family even celebrated Mother’s Day on Saturday May 11th with a nice dinner. It was wonderful to see everyone. I continued to become more sore over the next few days. On May 12th, Mother’s Day, I got up and took a shower anticipating going to church and celebrating Mother’s Day. The cramping in my back became so severe that I knew something was wrong. My husband agreed that we should go to the ER.
I was taken back after waiting almost two hours in pain. The pain increased with each passing moment. I was immediately connected to an I.V. and given the pain medication Dilaudid. The pain was reduced, but the nausea that this med created was horrible. I was given Zofran which helped with the nausea. Another nurse came to check on me later and my pain had increased. She stated she could give me more Dilaudid. I told her that it made me very nauseous. She said she could dilute it and push it slower over a 2-3 minute time frame. I agreed. It worked and no nausea.
After several tests including an MRI it was determined that the cysts had reoccurred and a second surgery would have to be performed. I was admitted to the hospital again. The next morning they ran several more tests and attempted to get my pain under control with using a combination of oral pain meds and Dilaudid. My husband and I both made sure that the nurses diluted the Dilaudid and pushed into the IV slowly. My doctor came in later that day and stated that I was definitely going to need a second surgery. I was back in surgery the next morning. The doctors explained that the he used a microscope to make sure the cysts cleaned out. My incision was larger now and closed with staples instead of sutures.
After spending 5 days in the hospital. I was discharged home with the same limitations as before along with a long list of medications. I was now taking Neurontin 600 mgs twice a day, Amitriptyline 20 mgs at bedtime, Somma 350mg every 6 hours, Robaxin 750 mg three times per day, and Hydrocodone 10-325mg every 4 hours for pain. Although when I arrived home I found that I could not lay on our bed because it was painful to lie on the staples. Also, it was very hard to roll in either direction. I could not sit nor stand for two long because my back would begin to spasm. But as time passed, the incision continued to heal and sleeping in bed became much easier and the use of pain medication was reduced. But it was not over yet.
I began to really feel much better. I even went to the local market night with my family. I walked around and was able to go out to dinner with them. It had been 11 days since my last surgery. But the next morning that all changed. I began having severe cramping in both my legs as well as spasms in my back. Again, I returned to the ER. I was immediately admitted. This time a mylogram was done to see if they could determine exactly where the problem was. At the same time a spinal drain was placed. It was the hope of the doctors that the drain would relieve the pressure by removing the excess spinal fluid from around the area. This was not a painless procedure.
For the next two days spinal was drained every 4 to 6 hours. The pain it caused when it was drained was incredible. It caused my legs and back to spasm as well as light-headedness. On the third day it was finally decided that the drain was not working so it was removed and the muscle relaxants and pain meds were increased to control the spasms and pain.
After much discussion the only option was surgery. The doctor stated he needed to place a drain. The only option was not a good one. He stated that a shunt would be placed in the spinal cord and drain into my stomach via a tube. He was not keen on this as there is a high failure rate as well as another surgical incision had to be made into my abdomen. He left my husband and myself feeling scared and unsure of what this really meant.
Later that afternoon my physician returned. He stated he came up with something had never been done but he was confident it was the best of action. He stated he was going to place two pieces of titanium tubing in the arachnoid space allowing for better flow and reducing the risk of the cysts returning.
It was a much better option than having a shunt drain into my stomach. So I was prepped for surgery the next morning. The surgery was a success. I spent two more days in the hospital. I returned home with much soreness, but better use of my legs and noticed a reduction of spasms.
It has now been 3 months since this last surgery. I am getting back to a more normal life. I still have pain and spasms but they are far less. I would consider this success. I can at least now enjoy more things in life such as hiking, playing with my family and kids, swimming and hopefully more things to come.
I am sharing the below information with others to help raise awareness of Chiari Malformation. Although it is a rare condition more people are being diagnosed as a result of better technology. I was diagnosed several years ago, but as of late the condition has progressed and I am in the process of seeking a second opinion before proceeding with surgery. Learning to live with this condition along with a spinal cord injury has made daily life a challenge.
What are Chiari Malformations
Chiari malformation is a condition in which the cerebellum and part of the brain stem push through the lower part of the skull. Normally the cerebellum, part of the brain that controls balance, and parts of the brain stem sit in a designated space at the base of the skull above the foramen magnum.
Chiari malformations may occur when the space where the cerebellum and part of the brain stem is smaller than normal, thus causing them to protrude downward into the upper spinal canal. As a result the flow of cerebrospinal fluid (CSF) may become blocked. Cerebrospinal fluid is the clear liquid that surrounds and cushions the brain and spinal cord. When the pathways are blocked is can cause a buildup of fluid on the brain called hydrocephalus or in the spinal cord called syringomyelia.
These malformations are uncommon, yet with improved modern imaging techniques and comprehensive exams physicians are beginning to make more accurate diagnoses.
Chiari Malformation type 1 is the most common type of the four possible types of the condition. Type 1 is also the only type that can be acquired. All other types are congenital.
Patients with type 1 normally do not have symptoms until late childhood or adulthood. This is because the malformation does not develop until the skull and brain have developed. On the other hand, patients with types 2-4 are often diagnosed in utero (during pregnancy), during birth, or during the first stages of infancy.
Patients that have Chiari malformation type I may complain of neck pain, headaches, balance problems, muscle weakness, vision problems, numbness or abnormal feelings in the legs and/or arms, ringing of the ears (tinnitus),hearing loss, dizziness, nausea, difficulty swallowing, facial pain, insomnia, and depression. Fine motor skills and hand coordination may also be affected.
Symptoms may vary from person to person and some may have no symptoms at all. The difference in symptoms depends on the buildup of CSF and the change in pressure on the tissues and nerves. For example, adolescent and adults may have no symptoms initially then later in life may develop signs of the disorder. Infants may show symptoms of any type of malformations. Their symptoms maybe difficulty swallowing, irritability during feedings, gagging or vomiting, excessive drooling, arm weakness, breathing problems, developmental delay and inability to gain weight.
If Chiari malformation type 1 if untreated may also result in the development of fluid filled cavities in the brain (hydrocephalus) or spinal cord (syrinx) which may eventually lead to syringomyelia. This can lead to irreversible damage to the brain or spinal cord.
How common are Chiari malformations?
In the past it has been estimated the condition happens one in every 1,000 to 2,00 births. However, the development of better technology and the increase use of diagnostic imaging has shown that the malformations may be more common. The estimates of actual cases is difficult as people born with the condition do not display symptoms at all or may not display them until they are in adolescence or adulthood.
The most common method of diagnosis is the use of an MRI (Magnetic Resonance Imaging) in conjunction with the patient history, and a neurological examination. Sometimes a special MRI scan called CINE studies are needed to make the diagnosis. Additional imaging techniques may be used. Early diagnosis of Chiari malformation type 1 can lead to treatment before the formation of syrinx.
The treatment of Chiari type 1 varies on several factors such as the severity of symptoms, whether or not syrinx (CSF fluid filled areas on the spinal cord) exists. For example, if a patient has no symptoms or neurological findings, the doctor ill often just observe the patient and schedule periodic visits to manage the condition. If the patient displays mild symptoms that are manageable, the physician may prescribe medications to help reduce the pain.
If the patient has symptoms that progress in severity, such as a decline in neurological function and/or they develop an enlarging syrinx, surgery may become necessary.
If surgery is recommended, it typically begins by making an incision is the middle of the back of the skull , and extends to about midway down the back of the neck. All the muscles are separated from the base of the skull to the fist cervical vertebra.
Next, a surgical removal of a portion of the cranium called a craniectomy is performed. This is done to enlarge the opening at the base of the skull and create additional space next to the brain to allow better flow of CSF. The neurosurgeon uses a specialized surgical instrument to carefully shave a small piece of bone about the size of a silver dollar. Next, the first cervical vertebra is removed. This called a laminectomy. At times, removal of additional cervical vertebra may be necessary.
Most patients with Chiari malformation type 1 often have no symptoms. The patient only finds out they have the condition after it is found during an evaluation for other non-related conditions.
Once a Chiari malformation is diagnosed, it is important to get an evaluation by a neurologist who then can refer you to a neurosurgeon if further treatment is needed. Early diagnosis and treatment of this condition is critical as surgical intervention for symptomatic Chiari malformation type 1 is most often successful when done early on. Many patients experience significant reduction of their symptoms after surgery and are able to return to daily life without activity restrictions.