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Syrinx/Syringomyelia

TREATMENT OPTIONS

Surgery is usually recommended for syringomyelia patients. The main goal of surgery is to provide more space for the cerebellum (Chiari malformation) at the base of the skull and upper neck, without entering the brain or spinal cord. This results in flattening or disappearance of the primary cavity. 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.

In some patients it may 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 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 arrest 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 doctor and patient, 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 patients.

In the case of trauma-related syringomyelia, the surgeon operates at the level of the initial injury. Until the 1990s, the most common approach was to collapse the cyst in surgery and insert a tube or shunt to prevent its re-expansion.   Because shunts routinely become clogged and require multiple operations, many surgeons now consider this option only as a last resort.   Instead, surgeons now expand the space around the spinal cord by realigning the vertebrae or discs that are narrowing the spinal column.  They then add a patch to expand the “dura,â€? the membrane that surrounds the spinal cord and contains the CSF (a procedure called “expansive duraplastyâ€?).   It is also considered important to remove scar tissue attached to the membranes that “tetherâ€? the cord in place and prevent the free flow of CSF around it.



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