Syringomyelia (SM) is the development of a cyst or a fluid-filled cavity in the spinal cord. In medical terms this cyst is referred to as syrinx. This cyst, over a period of time, elongates and expands causing damage to the center of the cord. As the nerves in the extremities are connected to the spinal cord, any damage to the spinal cord may result in weakness, pain, stiffness in the back, legs, arms and shoulders of the individual. An individual may also complain of headaches and is sometimes unable to feel hot or cold in the extremities, especially in his or her hands. People who suffer from syringomyelia find that it sometimes adversely affects their bowel and bladder control, sexual function, and sweating.
Syringomyelia symptoms if not treated, very often get worse and in some cases patients have to go in for surgery. If syringomyelia does not cause you any problems as such, simply monitor the condition. If the symptoms bother you, then you may need to go in for surgery. Complications such as scoliosis (where your spine is abnormally curved), Horner syndrome (a disorder that causes damage to the nerves that go from your brain to your face and eye), and chronic pain may bother you and warrant surgery.
What are the different forms of Syringomyelia?
There is no clarity as to what exactly causes syringomyelia. However, some of the main syringomyelia causes include congenial development problems related to the brain and/or spinal cord. The majority of syringomyelia cases are related to a condition called Chiari-I malformation where the brain tissue juts out into your spinal cord. Due to this, a syrinx sometimes develops in the cervical area of the spinal cord. This type of syringomyelia is sometimes referred to by doctors as communicating syringomyelia. In this form, the symptoms usually start sometime around the age of 25 to 40 years and generally worsen when there is some activity that causes a sudden fluctuation in the cerebrospinal fluid pressure. In some patients the periods of stability are long. In some cases it is found that there is an accumulation of cerebrospinal fluid in the skull. This is known as hydrocephalus. In a few cases it is found that the arachnoid membrane, which is a covering of the spinal cord, is inflamed. This condition is known as arachnoiditis.
Any trauma caused to the spinal cord such as a severe fall or accident might manifest a couple of years later as another form of syringomyelia. This form of syringomyelia occurs due to complications caused by conditions such as arachnoiditis, meningitis, and a tumor. Such conditions cause damage to a certain segment of the spinal cord and it is here that a cyst or syrinx develops. Doctors sometimes refer to this condition as noncommunicating syringomyelia. The symptoms here include weakness, pain, and sensory impairment which originate at the point of trauma.
In both cases of syringomyelia, we find that there are chances that the problem is undetected for years until you may get bothered by a symptom that requires medical attention. Syringomyelia sometimes remains undetected till you reach midlife. Very often some medical conditions may obstruct the regular flow of cerebrospinal fluid and it gets redirected to the spinal cord. This leads to the formation of a cyst, the further development of which causes damage to your spinal cord.
Pain is the primary symptom of post-traumatic syringomyelia, and it is found that this pain moves upward from the point where it originates. It is found that symptoms such as weakness, pain, and numbness are usually limited to one side of the body. In rare cases, it is found that certain cases of syringomyelia are hereditary. In one particular form of syringomyelia, a section of the brain is involved. This section, called the brainstem, is responsible for controlling many crucial functions such as heartbeat and respiration. When the brainstem gets affected by syrinxes the condition is known as syringobulbia.
To be able to diagnose syringomyelia your doctor will start by asking you details about your medical history and then follow this with a thorough physical examination. Your health care provider generally uses a tool known as MRI (Magnetic Resonance Imaging) to determine if you have syringomyelia. An imager is used to take pictures of your body structures. These images are 3-D and of a very high resolution. The doctor takes vivid images of your brain and spinal cord. In some cases a dye is injected during the test. This dye travels through your spine and enhances the images. The results help your doctor figure out if you have any tumor and also helps check for other conditions including the syrinx in the spine. In many cases your doctor accidentally comes across the fact that you have syringomyelia when he/she orders tests for other reasons that are not related to syringomyelia.
Sometimes additional tests are ordered by your doctor to confirm if you have syringomyelia. This includes an electromyography (EMG) which helps to measure the weakness of muscles. A lumbar puncture is also ordered to analyze and check the pressure levels of the cerebrospinal fluid. A computed tomography (CT) helps indicate the incidence of hydrocephalus and other abnormalities.
Syringomyelia treatment depends on how severe and how far your symptoms and signs have progressed. If your doctor discovers that you have syringomyelia during some other unrelated examination, and you do not have any symptoms or signs that indicate the problem, regular monitoring is important. This could be done with the help of a neurological exam or a periodic MRI. In very few cases it is found that a syrinx is resolved without the help of any treatment.
Syringomyelia Surgery: Doctors usually recommend surgery for patients suffering from syringomyelia. Through surgery, extra space is provided for the malformation or cerebellum that is present at the upper neck and the base of the syringomyelia. This has to be done without entering the spinal cord or brain. As a result of this, the primary cavity flattens or disappears. In case syringomyelia is caused by a tumor, the best course of treatment is to remove the tumor and it always results in the elimination of the syrinx.
Most patients find that there is some modest improvement and some stabilization as a result of surgery. It is important that you treat syringomyelia as soon as possible as any delay might cause permanent injury to your spinal cord. If syringomyelia recurs after surgery you may have to go in for additional operations. These additional operations may not be fully successful over a long term.
In some cases doctors have to use drainage tubes, a catheter, or valves to drain fluids from the syrinx. This method is called a shunt. A shunt is used in both noncommunicating syringomyelia and communicating syringomyelia. The process requires that the surgeon first find out where the syrinx is located. The doctor places the shunt into the syrinx and the other end of the shunt drains the fluid into a cavity. This is known as a ventriculoperitoneal shunt. A shunt helps arrest the progress of symptoms and to a great extent relieves pain, tightness, and headaches.
An extensive discussion is necessary between the doctor and patient before the decision to make use of a shunt is taken. This is because there are chances of injuries to the spinal cord. In addition, there are increased chances of blockage, infection, and hemorrhage and this procedure may not work well for all patients.
If syringomyelia is caused by trauma, surgery is usually performed at the level where the initial injury was. With the help of surgery the cyst usually collapses but it becomes necessary to use a shunt or a tube to stop any re-expansion.
In the case of syringomyelia, it is found that drugs do not have any curative value. Radiation is beneficial if there is a tumor present, else it is of very little benefit. Radiation, when used for a tumor, helps in the alleviation of any pain and also halts any extension of a cavity.
If you do not have any symptoms, syringomyelia is generally not treated. Doctors usually do not recommend that patients who are of advanced age treat the condition. Treatment is also not recommended in cases where there is no change or progress in the symptoms. Irrespective of whether you are treated or not, doctors advise most patients who are suffering from syringomyelia to avoid activities that cause strain.
Recent developments
Doctors have yet to find out the exact causes of syringomyelia. A lot of research is being carried out to find out what leads to the development of syrinxes. The neurosurgical community is also working at refining the surgical techniques that are used to treat syringomyelia. There is continuous development in diagnostic technology as well. Scientists today, with the help of an MRI are able to check the conditions in your spine long before the symptoms appear. Patients can, in the future, expect even better methods to treat and diagnose syringomyelia.
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