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The term syringomyelia refers to a collection of differing conditions characterized by damage to the spinal cord that is caused by a formation of abnormal fluid-filled cavities (syrinx) within the cord. In 1827, French physician Charles-Prosper Ollivier d'Angers (1796–1845) suggested the term syringomyelia after the Greek syrinx, meaning pipe or tube, and myelos, meaning marrow. Later, the term hydromyelia was used to indicate a dilatation of the central canal, and syringomyelia referred to cystic cavities separate from the central spinal canal.
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Diagnosis
Physical exam: The most prominent early signs include decreased reflexes in the arm, although increased reflexes and spasticity may occur in the lower extremities. There may be a lack of sensation (dissociated anesthesia) below the level of the syrinx often in the pattern of a cape or shawl. Symptoms are nearly always present on both sides of the body (bilateral) but may be asymmetrical or affect one side only (unilateral). Due to the loss of pain and temperature sensation in the hands and inner forearms, painless ulcers of the extremities may follow trauma.
Tests: CT, photographs of the spinal cord taken after the injection of a contrast material (myelography), MRI, and x-rays of the skull and cervical spine are utilized in making a definitive diagnosis. Nerve conduction velocity testing or electromyography may be used to demonstrate nerve damage; more sensitive electrodiagnostic testing examining evoked potentials (somatosensory evoked potentials, or SSEPs) may also be utilized. A lumbar puncture, in which a needle is inserted between the individual’s vertebrae to obtain cerebrospinal fluid (CSF), should be performed. Angiography can rule out neurologic causes of symptoms.
The first step after diagnosis is finding a neurosurgeon who is experienced in the treatment of syringomyelia. Surgery is the only viable treatment for syringomyelia. Not all patients will advance to the stage where surgery is needed. Evaluation of the condition is often difficult because syringomyelia can remain stationary for long periods of time, and in some cases progress rapidly.
Surgery of the spinal cord has certain, characteristic risks associated with it and the benefits of a surgical procedure on the spine have to be weighed up against the possible complications associated with any procedure. Surgical treatment is aimed at correcting the condition that allowed the syrinx to form. It is vital to bear in mind that the drainage of a syrinx does not necessarily mean the elimination of the syrinx-related symptoms, but rather is aimed at stopping progression. In cases involving an Arnold-Chiari malformation, the main goal of surgery is to provide more space for the cerebellum at the base of the skull and upper cervical spine without entering the brain or spinal cord. This often results in flattening or disappearance of the primary syrinx or cavity, over time, as the normal flow of cerebrospinal fluid is restored. If a tumor is causing syringomyelia, removal of the tumor is the treatment of choice and almost always eliminates the syrinx.
Surgery results in stabilization or modest improvement in symptoms for most patients. 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.
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In some patients it may also be necessary to drain the syrinx, which can be accomplished using a catheter, drainage tubes, and valves. This system is also 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 cerebrospinal fluid (CSF) into a cavity, usually the abdomen. This type of shunt is called a ventriculoperitoneal shunt and is particularly useful in cases involving hydrocephalus. By draining syrinx fluid, a shunt can arrest the progression of symptoms and relieve pain, headache, and tightness. Without correction, symptoms generally continue.
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