Weird topic...right??? Well, this topic is why I have not posted in a couple weeks. The Tuesday after we got home from Disney World we had a routine check up with Micah's Orthopaedic surgeons, Dr. Didelot. Long story short, he saw some things with Micah's walking, ie...He saw that his left foot was turning in more again. He also looked at his back and I told him that Micah has told me lately, "that his back 'feels funny.' And, he has not been staying dry between cathing... So, in less than a week Ryan took Micah back down to St. Vincent's for a lower lumbar MRI, and 4 days later, this past Tuesday, we were sitting in the Neurosurgeon's office being told that Micah does have a tethered Spinal Cord, and that he needs surgery soon. So, June 22nd, Micah will have a surgery to de-tether his Spinal Cord. I will 'bold' the parts below that pertain specifically to Micah.
Please pray for our little guy!!! He has signs of 'Post Traumatic Stress Disorder' before any surgery, and the signs of PTSD are already showing. The past 2 nights he has woken up in the middle of the night and comes in to our room and sleeps on our floor. We wake up in the morning to Micah sleeping right beside our bed. He also gets over fixated with safety issues... He was a wreck today when Luca was playing around in the car and tried to un-buckle his 5 point harness. Things will get noticeably get worse over the next week, and then after surgery his symptoms will subside.
I hate that he has to be so brave and live a life like this. I talk to him everyday about small aspects of the surgery just to keep the "lines" of communication open.
I cracked up yesterday in the car when he asked when the "restaurant" would open at the hospital and when he would be able to eat. That is my boy!!!!!!!!!!!! :-)
Below is some info on Tethered spinal Cord.. I know I have some SB moms that read our blog, so this is probably more important and interesting to them... Sorry everybody else!!! :-)
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What is tethered spinal cord?The term tethered spinal cord refers to any condition in which the spinal cord is attached to an immovable structure. Normally the bottom of the spinal cord, called the conus medullaris, floats freely within a pool of spinal fluid. There are, however, an array of pathologic conditions that cause tethering of the cord. This leads to abnormal stretching with growth and movement, which over time can lead to the characteristic symptoms of tethered spinal cord, including muscle weakness, sensory loss, change in bowel or bladder control, and orthopedic deformity.
Myelomeningocele or spina bifida is the most common cause of tethered cord. Because of the open exposed end of the spinal cord at the time of birth, there is considerable scar tissue that develops at the end of the spinal cord and the area of the myelomeningocele known as the neural placode. Thus all children with myelomeningocele have tethering of the spinal cord from the time of birth. Because of this, these patients are watched closely as they grow for signs of neurological deterioration.
How many children with myelomeningocele will require untethering as they grow? We don't know the final answer to that question. It is apparent, however, that the percentage is relatively high. If you follow children with spinal bifida from birth through adolescence and teenage years, current data would suggest that approximately 25 percent will have symptoms suggestive of tethered cord and benefit from surgical intervention. Although children with myelomeningocele can have multiple anomalies and problems, their problems are related to the malformations that occur prior to birth and, in general they should not worsen or deteriorate as time passes. Obviously there are circumstances where deterioration may occur, but the natural history of a person with myelomeningocele should not be loss of function with advancing age. If neurological function is being lost a search for a treatable cause is required. Deterioration in bladder control, such as decreasing ability to maintain some continence with medication and intermittent catheterization, is a common sign of tethering. Increasing weakness in the legs, changes in the strength of the arms and hands, and progressive curvature of the spine are symptomatic of tethered cord.
It is unusual for a child with myelomeningocele to need tethered cord release at an early age. Some children have required untethering by 2 years of age, but this is quite uncommon. The more typical circumstance is for the child to be in the later childhood years before symptoms occur. However, if a child is operated at an early age and there is a significant growth of the spinal column yet to occur, the chance of needing a repeat procedure later in life is increased. A few children have required untethering two or three times because of the growth of their spine and due to their sensitivity to neurological changes caused by the tethering.
Once spine growth has been achieved (this is usually achieved at approximately age 13 in girls and age 16-17 in boys) there is less likelihood of symptomatic spinal cord tethering. If a child has to be untethered at 10-12 years of age they are much less likely to need repeat untethering because there is not as much spine growth left to occur. So the question of the number of surgeries and the interval between surgeries is dependent upon many factors. It is dependent upon the sensitivity of the given individual to neurological changes that may be occurring, it is related to spine growth and it is related to the potential for scar tissue formation that the individual may manifest. The overwhelming numbers of children with myelomeningocele who require untethering require only one procedure.
There is another point that is important to make. In the myelomeningocele population, when we speak of tethering, we are really speaking of symptomatic tethering. All children with myelomeningocele have a tethered cord caused by the scar tissue that forms due to the open myelomeningocele at birth and the surgery to close the opening. However, because the cord looks tethered, as seen on MRI, this does not necessarily mean that there will be symptomatic changes related to the tethering. In children with myelomeningocele the untethering operation is reserved for those who show deterioration. Surgery is not performed just because the MRI suggests that tethering is present. With this in mind, an untethering procedure will again produce tethering of the spinal cord because scar tissue naturally forms. Once the untethering has been accomplished the healing process starts and is associated with scarring. This leads to repeat tethering. However, it may not lead to symptomatic re-tethering. This is a very important distinction. The spinal cord will always look tethered on the MRI in patients with a myelomeningocele. An MRI is obtained prior to untethering operations to look for associated conditions. There may be evidence of a syrinx (loculated fluid within the central cavity of the spinal cord), cyst formation outside the spinal cord or small retained dermoid tumors that can occur from elements of the skin.
Once tethering symptoms have begun in the patient with a myelomeningocele, it still remains a judgment call as to how soon to perform surgery. If the primary symptom is pain and the patient is relatively stable and not requiring significant medication, then it is certainly reasonable to wait. If there are bladder changes, i.e. the child is no longer able to have any periods of continence with intermittent catheterization, this is a much more urgent situation and surgery should be scheduled relatively soon. Any loss of strength can be devastating to a patient who already has weakness and this is a signal to proceed with untethering. As a general rule any neurological function that is lost may not recover.
In patients who have shunted hydrocephalus, it is not uncommon for a shunt malfunction to mimic symptoms of a tethered cord. Patients with myelomeningocele may be very sensitive to any type of change affecting the lower spinal cord. In the case of a shunt malfunction, the pressure may build up in the spinal canal affecting the distal spinal cord and symptoms can mimic tethering. It is important to check shunt function before making a decision to proceed with untethering.
There is no technique for closure of the myelomeningocele at the time of the original surgery that will prevent tethering. However, there are some techniques that may minimize the amount of tethering that occurs. The open neural placode at the distal end of the spinal cord can be folded over and anatomically made into a tube by suturing the edges of the open placode together. This makes the distal portion of the spinal cord tubular in nature, shaped like the rest of the spinal cord and tends to minimize the amount of scarring that occurs. It is easier for the flat, open neural placode to form more dense scar to the overlying tissue of the lower back than a closed tubular-shaped distal spinal cord. It doesn't prevent tethering, but it seems to make the surgery for untethering easier and perhaps less risky.
Scar tissue formation is a normal response of the body to tissue injury. There is no way at present to prevent scar formation. Many different types of tissue have been tried in an effort to minimize the scarring as a result of tethered cord surgery. Silastic, Gore-Tex™, artificial dura, and many others have been tried as closure over the area of the surgery. None have been found to prevent scar tissue formation. There is nothing a present that can prevent this process.
What is the usual treatment for tethering of the spinal cord?Once signs and symptoms of spinal cord tethering are present, especially if progressive, conservative (non-surgical) management is rarely effective. Rest and physical therapy may be effective in relieving some of the early symptoms but the young patient is essentially doomed to worsen as time passes.
The only effective treatment is surgical untethering of the underlying cause. The surgery for untethering of a spinal cord has two goals: first, to stop any further neurological deterioration, second, to hopefully see some improvement in any lost function. Once neurological function is lost it may never recover. This is why surgery is indicated when there are early signs of neurological change.Although the causes of tethering vary, the general principles of the surgery are similar.
The overwhelming majority of tethering is in the lumbar or sacral regions of the spine so the surgery is usually performed on the low portion of the back. A midline incision is made in the skin. The muscles are retracted away from the midline and exposure of the bones of the posterior spine is accomplished. The spinous process and arch of bone covering the posterior or back portion of the spinal canal (lamina) is removed in order to gain exposure to the cause of the tethering. The dura, the covering over the spinal cord and nerves) is opened, exposing the tethering process. The operation is usually done through the operating microscope or with the surgeon wearing special magnifying glasses so that vision is enhanced. Lasers or other specialized surgical equipment may be used. Depending on the cause of the tethering, the surgery may last many hours. Six to eight hours is not uncommon in complex cases. This operation is not one that usually has a lot of bleeding. Blood transfusions are rarely required.The patient may be kept flat in bed for 24-48 hours. After that there is usually no need for prolonged bed rest. Depending on the neurological condition of the patient and the extent of the surgery, the patient may be restricted from vigorous activity for a few weeks.
