What is Syringomyelia by Angela Lamb

Given we are currently studying the nervous system, I thought it would be fitting to write about a neurological spinal cord disorder called Syringomyelia.  Syringomyelia is a disorder that few are aware of unless they have had it or know a family member or friend affected by the disorder.  Prevalence of the disorder is uncommon estimated at roughly 8.4 cases per 100,000 people.

Syringomyelia in general refers to a cyst or cavity that develops inside the spinal cord.  The fluid-filled cyst called a syrinx can grow and destroy nerve fibers.  As a result, neuropathic pain or loss of sensation may occur.  Other potential symptoms are bowel and bladder functional problems; muscle weakness and atrophy; and loss of reflexes.

There are several causes of this disorder but the most common is due to a Chiari malformation.  A Chiari malformation is a result of an abnormal extension of brain tissue in the spinal canal, which disrupts the natural flow of cerebrospinal fluid.  Other potential causes are meningitis, spinal cord injury, spinal cord tumor, spinal scar tissue, or tethered spinal cord syndrome.  Tethered spinal cord syndrome is the tissue that is attached to your spinal cord that limits movement as a result.

For the lucky few, no problems result and no intervention is needed.  However, for others mild to severe problems can occur and be progressively damaging.  Additionally, if the disorder was caused by a spinal injury or trauma it may take many years for the disorder to develop.  If aware of the disorder, regular MRIs and assessment of symptoms by a neurologist may be necessary.

If the symptoms of the disorder continue to worsen or persist, surgery may be needed.  Given the rarity of this disorder, searching for a syringomyelia neurosurgeon specialist who has completed many successful surgeries is advised.  Depending on the cause of the disorder, there are several different surgical treatments.  If the disorder is a result of a Chiari malformation, the neurosurgeon and his or her team may enlarge the opening at the base of the skull and expand the covering of the brain which in most incidences normalizes cerebrospinal fluid flow and reduces pressure on your brain and spinal cord.  If a syrinx was created at some point due to trauma or injury, it will be drained during surgery utilizing a small catheter.  In some cases, a shunt is also inserted into the syrinx, which drains from the syrinx to the outside of the spinal cord and remains in the body after surgery to ensure the syrinx remains drained.  Although normal activity can resume after recovery with the shunt, it is recommended that scuba diving and parachuting out of a plane for pleasure be avoided due to pressure concerns and the shunt inside your body.  If the disorder is a result of a tumor or another obstruction and symptoms worsen, surgical removal of the obstruction with the goal of returning cerebrospinal fluid to normal occurs.  If the disorder is a result of spinal abnormality due to a tethered spinal cord, surgery to correct the problem is completed.

Typically after surgery, periodic MRIs to ensure surgical success and symptom assessment by a neurologist will likely occur.  Although surgery may ensure no additional damage occurs, there may still be symptoms of syringomyelia as a result of the damage that was done prior to surgery.  Therefore early detection typically warrants the best outcome.

 

References:

http://www.ninds.nih.gov/disorders/syringomyelia/detail_syringomyelia.htm

http://en.wikipedia.org/wiki/Syringomyelia

http://www.mayoclinic.com/health/syringomyelia/DS01127