Syringomyelia

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at October 10, 2016
StartDiseasesSyringomyelia

Syringomyelia is diagnosed when a fluid-filled cyst forms in the spine. Over time, the cyst can enlarge, leading to spinal cord damage. Although syringomyelia has many possible causes, the most common cause of it is Chiari malformation. Once symptoms occur, surgical treatment is generally recommended. 

Contents

Definition & Facts

Syringomyelia occurs when a fluid-filled cyst forms in the spinal cord. The cyst is known as a syrinx. It destroys the spinal cord from the center and expands outward. As the cyst enlarges, it compresses and damages nerve fibers that carry messages from the brain to the body's extremities

Symptoms & Complaints

In most cases, symptoms of syringomyelia develop slowly over time. However, in some people, sudden symptoms of the condition can appear after coughing or straining, though neither of these cause the disorder itself.

Early signs and symptoms of the condition may initially affect the back of one's neck, shoulders, arms, or hands. Early signs of syringomyelia include muscle weakness or wasting (muscle atrophy), loss of sensitivity to pain, an inability to feel temperature, and loss of reflexes

Other symptoms of the condition may include bladder problems and bowel problems, scoliosis (spinal curvature), muscle spasms and weakness in the legs, headaches, and facial numbness or facial pain.

Someone with syringomyelia may also experience shoulder stiffness, back stiffness, leg stiffness, and arm stiffness. One may also experience neck pain, back pain, and arm pain. Individuals will experience different symptoms based on where the syrinx is located within the spinal cord and how much the cyst has expanded. 

While some individuals with syringomyelia have no symptoms, for others, the condition becomes progressive, causing complications. One's ability to walk may be impaired due to stiffness of limbs. Additionally, damage to the spinal cord can lead to severe chronic pain.

Causes

It isn't known exactly how and why syringomyelia occurs. When the condition occurs, cerebrospinal fluid, the fluid that surrounds, protects, and cushions the brain and spinal cord, gathers within the spinal cord itself, creating a fluid-filled cyst called a syrinx. 

There are two major forms of syringomyelia. The first form is related to a congenital malformation involving the cerebellum called Chiari malformation. Chiari malformation happens during fetal development. It is characterized by a downward displacement of the cerebellar tonsils, the lower part of the brain, into the cervical spinal canal.

Chiari malformation inhibits the normal flow of cerebrospinal fluid. When the flow of cerebrospinal fluid is inhibited, a syrinx can develop within the spinal cord. This type of syringomyelia is sometimes called communicating syringomyelia. Individuals with this type of syringomyelia typically develop symptoms between the ages of 25 and 40 years of age. 

In the other type of syringomyelia, sometimes called noncommunicating syringomyelia, the condition is related to a spinal cord tumor, trauma, hemorrhage, tethered spinal cord, arachnoiditis, or meningitis. A spinal cord tumor may prevent the normal flow of cerebrospinal fluid. A tethered spinal cord is present at birth (it's a congenital disorder), and it is caused when tissue attached to the spinal cord interferes with its movement.

Meningitis is an inflammation in the meninges (membranes) surrounding the spinal cord and brain. The inflammation often causes a stiff neck, headaches, and fever. Arachnoiditis is a pain condition that is caused by inflammation of one of the meninges that surrounds and protects the nerves in the spinal cord called the arachnoid mater. Arachnoiditis is characterized by neurological problems and stinging, burning pain. In the case of trauma, symptoms of syringomyelia can develop months or years after the injury occurs.

Diagnosis & Tests

When someone goes to a physician with symptoms of syringomyelia, the physician will ask the patient about her symptoms and medical history. The physician will also perform a physical examination. If the physician suspects syringomyelia, he will send the patient for an imaging test, such as a magnetic resonance imaging (MRI) scan or a computed tomography (CT) scan.

In a magnetic resonance imaging (MRI) scan, a strong magnetic field and radio waves produce images of the spine and spinal cord. A contrast dye may be injected into the groin in order to make any tumors or other abnormalities visible on the scan as well. An MRI is the most reliable tool a physician can utilize in the diagnosis of syringomyelia. A CT scan utilizes X-rays to form detailed images of the spine and spinal cord. A CT scan can also show if there are any tumors or other abnormalities present in the spine and spinal cord as well. 

Treatment & Therapy

If syringomyelia isn't causing symptoms, a physician may choose to monitor a patient's condition with periodic MRI scans. When signs and symptoms of the condition rapidly worsen or interfere with a patient's life, treatment is required.

Surgery is the primary means of treatment for syringomyelia, and the goal of surgery is to relieve the pressure on the spinal cord and restore the normal flow of cerebrospinal fluid. The type of surgery a patient requires will depend on the cause of the condition. 

In one type of surgery, a shunt is placed in the body to drain the syrinx. A shunt is a flexible tube with a valve that drains fluid from the syrinx. One end of the tube is put into the syrinx while the other end is placed into another part of the body, such as the abdomen

If a spinal abnormality, such as a tethered spinal cord, is causing the obstruction of cerebrospinal fluid, surgery to correct the abnormality may restore the normal flow of cerebrospinal fluid, allowing the cyst to drain. If a tumor is obstructing the flow of cerebrospinal fluid, removing it may lead to the restoration of normal cerebrospinal fluid flow, which may allow the syrinx to drain. 

If a patient has Chiari malformation, a physician may suggest a surgery to enlarge the base of the skull and expand the covering of the brain. This surgery may restore the normal flow of cerebrospinal fluid, decrease or eliminate symptoms, and reduce pressure on the spinal cord and brain. 

Even after treatment for syringomyelia, symptoms of the condition may persist because the cyst can do permanent damage to the nerves and spinal cord. Additionally, a patient will need to be monitored with periodic MRIs following surgery because syringomyelia may reoccur. 

It is recommended that a patient who has been diagnosed with syringomyelia avoid doing anything that puts excessive force on the spinal cord, such as straining and heavy lifting. Patients who have symptoms of the condition, such as fatigue, stiffness, pain, and muscle weakness may benefit from a physical therapy program.

Prevention & Prophylaxis

To the extent that syringomyelia can be caused by a spinal cord injury, precautions that mitigate the likelihood of common causes of spinal cord injury such as motor vehicle accidents and accidents pertaining to alcohol abuse can be considered preventative measures against the development of syringomyelia and should be taken in any event.

Tuberculous meningitis, which is a type of bacterial meningitis that can cause syringomyelia, may be prevented by adopting hygiene protocols such as regular hand washing. There is also a vaccine called the bacille Calmette-Guerin (BCG) vaccine that can help prevent TB meningitis.

Because there appear to be genetic factors that cause Chiari malformation, which in turn causes syringomyelia, there isn't a known way to prevent this underlying cause of syringomyelia.