Spina Bifida Family Support
"Families Helping Families"
Scoliosis and Syringomyelia
By Robert P. Stanton, M.D.
Pediatric Orthopaedic Surgeon
Adolescent Idiopathic Scoliosis comprises about 80% of the cases of scoliosis that we see in our usual practice of pediatric orthopaedic surgery. Although the cause of this type of curvature of the spine is not completely understood, it is clear that the tendency to develop this problem is inherited. The scoliosis is more likely to be seen in the females in any one family, and usually begins to be noticeable on physical exam around age ten. In virtually all cases, the curves are concave to the right side of the chest ( that is the spine deviates toward the right side of the patients body.) From early in my residency in orthopaedic surgery, I was taught to be especially wary of left sided thoracic curves. At the time, we did not have M.R.I. as an imaging tool. Syringomyelia was a very poorly understood condition that was almost never diagnosed in a child. Usually the diagnosis was made only in adult patients following years of vague progressive and non-specific complaints. Patients required a pneumoencephalogram to properly make the diagnosis. For those who may be unfamiliar with this outdated test, let me assure you that you would not want to go through one, and I do not remember ever ordering one on a child. Thus we recognized that the left sided thoracic curve behaved in a strange way ( did not respond very well to brace treatment) but we did not appreciate why. I suspect that many of these children had the Arnold-Chiari malformation and syringomyelia that went undiagnosed.
Now that we are aware of the association between syringomyelia and unusual scoliosis patterns, we order M.R.I. testing to look for syringomyelia. At our children's hospital, we find several cases of syringomyelia every year, and few of the patients have any findings on neurologic exam, and almost none of these children have any complaints. We are left with many unresolved questions concerning the treatment that should be recommended for the management of these children.
I try to inform the parents that there is very much that we do not know about the natural history of syringomyelia and its associated scoliosis (natural history = what will happen to the patient if nothing at all is done.) We do not know the way in which syringomyelia produces the scoliosis, since many patients with syringomyelia do not have any scoliosis and most patients with scoliosis do not have syringomyelia. In the absence of strong scientific proof, treatment is guided by general principles that are used for Idiopathic Scoliosis.
My colleagues and I generally attempt to control scoliosis with braces if the curve exceeds 25 degrees in a growing child. If an Arnold-Chiari malformation is identified in association with syringomyelia and scoliosis, we recommend decompression before bracing, as many of our patients have shown either reduction or stabilization of the curve after this procedure. Most often the decompression will also produce a significant reduction in the size of the syringomyelia. If the scoliosis continues to be progressive, we institute brace management. The success of brace management is not as high for these patients as it is for patients with idiopathic Scoliosis. There are those who feel that braces are not indicated for the treatment of scoliosis associated with syringomyelia. I would say that although the success rate is lower, until a large series of patients is studied closely and the long term results are published, it is still probably a wise conservative approach to use a brace in an attempt to arrest the progression of the curvature. Regardless of whether bracing is used, the patient should be followed closely (X-Rays approximately every four months) for progression. If the child is still growing and the curvature exceeds 45 degrees, we would recommend spinal fusion with instrumentation. This presents the surgeon and the family with yet another dilemma. Instrumentation dramatically increases the healing rates for this surgery, and can produce dramatic improvements in the cosmetic appearance of the spine, especially in the young, flexible patient. Contrary to some very early published reports, we have found no increased risk of neurologic damage associated with the use of internal fixation devices in patients with syringomyelia. The usual fixation devices are made of stainless steel, and therefore are not ideal for use in patients who would likely be in need of M.R.I. testing to follow the course of their syringomyelia. Titanium versions of most standard instrumentation are available, and with these in place, M.R.I. testing can safely be performed, however, even though the testing can be done, the quality of the images will be inferior due to artifact produced by the metal implants.
In conclusion, since the number of cases of patients with scoliosis associated with syringomyelia is small, no hard evidence exists to completely support any recommendations concerning management. I believe that bracing should be attempted in curves that are progressing, although braces are probably less effective in these patients than in patients with adolescent idiopathic scoliosis. I have seen enough of my patients benefit from decompression of their Arnold-Chiari malformations (decreased curvatures, shrinking of the size of their syringomyelia) that I have recommended that procedure to others. I am less certain about whether decompression of a stable syringomyelia in an asymptomatic patient is indicated. It is clear that this disease is rare enough that definitive recommendations cannot be made until multi-center studies are used to accumulate enough data to produce reliable statistics concerning the benefit of various treatments. Until then, each patient and their physicians will continue to make their decisions based on careful counseling in light of the scant information available.
This information does not constitute medical advice for any individual. As specific cases may vary from the general information presented here, Spina Bifida Family Support advises readers to consult a qualified medical or other professional on an individual basis.