What is Syringomyelia?
Syringomyelia is a pathological condition in which there is development of cysts filled with fluid in the spinal cord. With time, these cysts start to enlarge causing significant damage to the spinal cord resulting in pain, weakness, and stiffness along with other symptoms. There may be many causes resulting in development of Syringomyelia, although majority of the cases are due to a medical condition called as Arnold-Chiari Malformation in which the cerebellum protrudes into the top part of the spinal cord due to misshapen and extremely small skull. Some of the other causes of Syringomyelia are tumors of the spinal cord, any type of injury or trauma to the spinal cord, or inflammation around the spinal cord. If Syringomyelia is asymptomatic then no treatment is rendered and observation is the preferred choice, but in case if the patient is experiencing symptoms that are quite bothering then the most preferred route of treatment is surgery.
What Causes Syringomyelia?
As of now the exact cause of Syringomyelia is not clear, but when this condition develops there is accumulation of the cerebrospinal fluid around the spinal cord forming these fluid filled cysts. There are also certain medical conditions which may cause Syringomyelia to include:
- As stated above Arnold-Chiari malformation can be a cause of Syringomyelia.
- Meningitis in which there is inflammation of the membranes which surround the brain and spinal cord
- Tumors of the spinal cord affecting the normal flow of the cerebrospinal fluid
- Certain congenital conditions like tethering of the spinal cord which is a condition in which the tissue of the spinal cord limit its movements can also cause Syringomyelia
- Any trauma or injury to the spinal cord like in motor vehicle accidents may also result in Syringomyelia.
What are the Symptoms of Syringomyelia?
In some cases Syringomyelia, remains completely asymptomatic but in some cases there may be certain symptoms which usually develop after some time has passed post the development of Syringomyelia. If Syringomyelia is caused due to Arnold-Chiari Malformation then the onset of symptoms may be around the age of 20-40. Early symptoms of Syringomyelia may affect the back part of the neck, shoulders, and hands. As and when the condition advances the following symptoms may be observed:
- Muscle weakness along with atrophy
- Sensation loss
- Back stiffness
- Pain in the neck and hands
- Bowel and bladder dysfunction
- Muscle weakness
- Facial pain
- Development of scoliosis
How is Syringomyelia Diagnosed?
To begin with, the physician will take a detailed history asking as to where the patient has suffered any trauma to the spinal cord. The physician will then ask as to the duration of the symptoms. A complete physical examination will then be performed to look for signs of any areflexia, muscle weakness or other classic features of Syringomyelia. In some cases, when this condition is completely asymptomatic then it may be diagnosed incidentally when imaging studies are being conducted for some other reason. If the physician suspects, Syringomyelia then the following tests will be conducted. MRI scan of the brain and spinal cord to look at the internal structures of the brain and spinal cord which will virtually confirm the presence of cyst in the spinal cord confirming the diagnosis of Syringomyelia. This MRI scan may be done with or without contrast dye being injected, but MRI with contrast is preferred so as to pinpoint the position of the cysts. Once Syringomyelia is confirmed serial MRIs may be conducted to track down the disease process and check for any progression of the disease.
How is Syringomyelia Treated?
Treating Syringomyelia will depend on the severity and progression of the disease and what is the severity of the symptoms. In case if the disease is completely asymptomatic, then the physician may just recommend observing the condition with serial MRIs to look for any progression of the disease and no treatment is recommended.
In case if Syringomyelia is causing symptoms which are quite bothersome to the patient, then the most preferred route of treatment is surgery. The main aim of the surgical procedure for Syringomyelia is to relieve the pressure the cysts are putting on the spinal cord and restore normal flow of the CSF. The surgical procedure required will depend on what is causing Syringomyelia. Some of the surgical procedures done are as follows:
Surgery for Syringomyelia Caused due to Arnold-Chiari Malformation: If Syringomyelia is caused by this condition then surgery called posterior fossa decompression is performed in which the surgeon will take out a bit of bone from behind the skull to create more room for the brain to expand and hence reduce the pressure being put on the brain and spinal cord and thus treating this condition along with Syringomyelia.
Draining the Cyst: In order to drain the fluid form the cyst the surgeon inserts a shunt containing a tube and a valve with one end of the tube being attached to the cyst and the other end being attached to other part of the body which is usually the abdomen and the fluid is drained from the cyst using this shunt.
Removal of Obstruction: In case if there is a tumor or an abnormal growth restricting the slow of the CSF then removal of the tumor is done so as to restore normal flow of the CSF and allowing the fluid from the cysts to drain.
In case if Syringomyelia is being caused due to a tethered cord then correcting the abnormality is useful in restoring normal flow of the CSF and draining the fluid from the cyst and resolving the symptoms which arises from Syringomyelia.
- Cleveland Clinic. (2021). Syringomyelia. https://my.clevelandclinic.org/health/diseases/17692-syringomyelia
- Cedars-Sinai. (2021). Syringomyelia. https://www.cedars-sinai.org/health-library/diseases-and-conditions/s/syringomyelia.html
- Rare Diseases. (2021). Syringomyelia. https://rarediseases.org/rare-diseases/syringomyelia/
- University of Michigan Medicine. (2021). Syringomyelia. https://www.uofmhealth.org/health-library/tv7033