The treatment of syringomyelia is directed toward the specific symptoms that are apparent in each individual. Treatment may require the coordinated efforts of a team of specialists. Pediatricians, neurologists, neurosurgeons, surgeons, eye specialists (ophthalmologists) and other healthcare professionals may need to systematically and comprehensively plan an affected child’s treatment.
Specific therapeutic procedures and interventions may vary, depending upon numerous factors, such as disease progression; the presence or absence of certain symptoms; the underlying cause; the impact of symptoms on quality of life; an individual’s age and general health; and/or other elements. Decisions concerning the use of particular drug regimens and/or other treatments should be made by physicians and other members of the health care team in careful consultation with the patient based upon the specifics of his or her case; a thorough discussion of the potential benefits and risks, including possible side effects and long-term effects; patient preference; and other appropriate factors.
Some individuals with syringomyelia who do not have any symptoms may not require treatment, but should be regularly monitored to see whether the disorder progresses.
General therapeutic options include pain medications (analgesics), physical therapy, and a reduction in activities, especially those that require straining such as heavy lifting. The goal of treatment for syringomyelia is to restore the proper flow of cerebrospinal fluid and to remove the pressure that a syrinx places on the spinal cord. Initial treatment is usually targeted at the underlying cause of syringomyelia. There is no specific, agreed-upon therapy or treatment regimen for the most common cause of a syrinx, a Chiari malformation. Neurosurgeons and other physicians may disagree as to the best approach to treat a Chiari malformation. Different neurosurgeons may recommend different surgical techniques or treatment regimens.
Like syringomyelia, individuals with a Chiari malformation who do not have symptoms are generally not treated, but monitored to see whether the disorder progresses. If mild or nonspecific symptoms are present, such as neck pain or headaches, physicians may recommend conservative treatment. Symptomatic Chiari malformations are most often treated by surgery. There are no specific criteria or objective tests that can be used to determine when to undergo surgery or the best procedures to choose. The most common surgery is known as posterior fossa decompression. With this procedure, a surgeon creates room by removing small pieces of bone in the back of the skull, thereby enlarging the foramen magnum. This relieves pressure and reduces compression of the brainstem. The surgeon may also choose to open the covering (dura mater) of the brain in this region and explore the herniated tissue and then sew in a graft (duraplasty).
If hydrocephalus is associated with the Chiari malformation-related syrinx, then the surgeon will usually treat this first. The most common surgical treatment is to place a shunt that connects the dilated ventricles of the brain to another cavity of the body. The most common cavity chosen is the abdomen. Once the hydrocephalus is treated, then the Chiari malformation is addressed.
Surgery may be used to treat other conditions that cause syringomyelia including surgery to remove a tumor. Tethered spinal cord may require surgery to release the tension of the cord.
In some cases, a tiny tube called a shunt/stent may be placed into the syrinx. The shunt allows the fluid within the syrinx to drain to an area outside of the spinal column. Shunting can stop the progression of the disorder and relieve some symptoms such as pain and headaches. However, shunts can be associated with significant side effects including spinal cord injury or infection, bleeding (hemorrhaging), and blockage.
Posttraumatic syringomyelia may be difficult to treat. Surgery is recommended for individuals with neurological deterioration and/or intractable pain. Surgery is aimed at expanding the area around the spinal cord by the site of trauma or injury and to decrease fluid volume. The insertion of shunt may also be used to treat posttraumatic syringomyelia. Shunting in posttraumatic syringomyelia carries risks including further spinal cord injury and may need to be replaced if the shunt becomes clogged or defective. Many physicians consider shunts a last resort for individuals with posttraumatic syringomyelia.
Surgery for syringomyelia can often lead to an improvement of symptoms and stabilization of the disorder in many individuals. However, syringomyelia can recur after successful treatment, thereby necessitating more operations. After surgery, the physician will evaluate the syrinx to make sure that it stabilizes or decreases in size. These evaluations will entail obtaining an MRI.