Syringomyelia

Syringomyelia (SM) is a disorder in which a cyst forms within the spinal cord. This cyst, called a syrinx, expands and elongates over time, destroying the center of the cord. Since the spinal cord connects the brain to the nerves in the extremities, this damage may result in pain, weakness, and stiffness in the back, shoulders, arms or legs. Other symptoms may include headaches and loss of the ability to feel extremes of hot or cold, especially in the hands and disruption in body temperature. SM may also adversely affect sweating, sexual function and bladder and bowel control.

There are two major types of SM. In most cases it is related to a congenital malformation involving the hindbrain (cerebellum) called a Chiari I Malformation. This malformation occurs during fetal development and is characterized by downward displacement of the lower part of the brain (cerebellar tonsils) beneath the foramen magnum, into the cervical spinal canal. This displacement blocks the normal flow of cerebrospinal fluid. When normal flow is obstructed, a syrinx can form in the spinal cord. Not all patients with Chiari Malformations will develop a syrinx, however.

SM can also occur as a complication of trauma, meningitis, tumor, arachnoiditis, or a tethered spinal cord. In these cases the syrinx forms in the section of the spinal cord damaged by these conditions. As more people are surviving spinal cord injuries, more cases of post-traumatic SM are being diagnosed as the syrinx can form years after the trauma.

What Causes Syringomyelia?

What Are The Symptoms?

Symptoms develop slowly over time, but can come on suddenly after a fall or minor trauma. Sensation may be affected first. Some common symptoms include: -

 

Scoliosis may be the only symptom in children. Often people with scoliosis undergo spinal MR imaging because of an atypical left-sided thoracic curve.

Many individuals suffer from chronic pain and some will develop neuropathic pain syndromes. This type of pain is difficult to treat. A large percentage of people have headaches which can be severe.

What Is The Prognosis?

Symptoms usually begin between the ages of 25 and 40 and may worsen with straining or any activity that causes cerebrospinal fluid pressure to fluctuate. Some patients, however, may have long periods of stability. Surgery results in stabilization or modest improvement in symptoms for most patients. Delay in treatment may result in irreversible spinal cord injury.

How Is Syringomyelia Diagnosed?

Diagnostic imaging has significantly increased the number of syringomyelia cases detected in the beginning stages of the disorder. Physicians primarily use magnetic resonance imaging (MRI) to diagnose syringomyelia. Computer-generated radio waves and a powerful magnetic field produce images of body structures, such as the brain and spinal cord, in vivid detail. This test will show the syrinx in the spine as well as other conditions, such as the presence of a tumor. Images taken in rapid succession can be used for “dynamic imaging” (in “cine mode”) to observe the fluid flowing around the spinal cord and within the syrinx. Computed tomography (CT), which uses x-rays and a computerized scanner to produce cross-sectional images of the body or an organ structure, may reveal the presence of tumors and other abnormalities such as hydrocephalus. Another test, called a myelogram, takes x-ray-like pictures and requires a contrast medium or dye to do so. Since the introduction of MRI this test is rarely necessary to diagnose syringomyelia.

The physician may order additional tests to help confirm the diagnosis. One of these is called electromyography, which measures muscle weakness. The doctor may also wish to test CSF pressure levels and to analyze the CSF by performing a lumbar puncture, in which a hollow needle is inserted into the spinal canal and a small sample of the fluid is removed.

How IS SYRINGOMYELIA TREATED?

Surgery is usually recommended for individuals with syringomyelia. The type of surgery and its location depend on the type of syringomyelia. In Chiari malformation, the main goal of surgery is to provide more space at the base of the skull and upper neck, without entering the brain or spinal cord. This results in the primary cyst becoming much smaller. Surgery results in stabilization or modest improvement in symptoms for most individuals with Chiari malformation. Delay in treatment may result in irreversible spinal cord injury. Recurrence of syringomyelia after surgery may make additional operations necessary; these may not be completely successful over the long term.

In some individuals it may be necessary to drain the syrinx, which can be accomplished using a catheter, drainage tubes, and valves. This system is known as a shunt. Shunts are used in both the communicating and noncommunicating forms of the disorder. First, the surgeon must locate the syrinx. Then, the shunt is placed into it with the other end draining the syrinx fluid into a cavity, usually the abdomen. This type of shunt is called a syringoperitoneal shunt. A shunt of CSF from the brain to the abdomen is called a ventriculoperitoneal shunt and is used in cases involving hydrocephalus. By draining syrinx fluid or CSF, a shunt can halt the progression of symptoms and relieve pain, headache, and tightness. Without correction, symptoms generally continue.

The decision to use a shunt requires extensive discussion between the surgeon and the individual, as this procedure carries with it the risk of injury to the spinal cord, infection, blockage, or hemorrhage and may not necessarily work for all people.

If a tumor is causing syringomyelia, removing the tumor is the treatment of choice and almost always eliminates the syrinx.

In the case of trauma-related syringomyelia, the preferred surgical approach is to operate at the level of the initial injury to expand the space around the spinal cord and decrease fluid volume.

In the case of trauma-related syringomyelia, the preferred surgical procedure is to operate at the level of the initial injury and expand the space around the spinal cord. This operation is performed outside the spinal cord. An alternate operation is to place a shunt in the syrinx, which requires a hole to be made in the spinal cord. Shunts may injure the spinal cord and may require replacement if they clog over time. Many surgeons now consider shunt placement only as a last resort. Instead, surgeons usually choose to expand the space around the spinal cord. This is done by removing scar tissue that “tethers” the cord in place and prevents the free flow of CSF around it is removed, and adding a patch to expand the “dura,” the membrane that surrounds the spinal cord and contains the CSF (a procedure called expansive duraplasty). In some cases, the vertebrae may need to be realigned to correct spinal deformity that is narrowing the spinal column.

Many spinal cord-injured individuals have a cyst at the site of the original injury. These cysts do not always require treatment, although treatment may be warranted if a cyst grows larger or begins to cause symptoms.

Drugs have no curative value as a treatment for syringomyelia but may be used to ease pain. Radiation is used rarely and is of little benefit except in the presence of a tumor. In these cases, it can halt the extension of a cavity and may help to alleviate pain.

In the absence of symptoms, syringomyelia is usually not treated. In addition, a physician may recommend not treating the condition in individuals of advanced age or in cases where there is no progression of symptoms. Whether treated or not, many individuals will be told to avoid activities that involve straining.

 

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