Syrinx with Scoliosis

DEFINITION

Scoliosis is a Greek term that is defined as a lateral curvature of the spine. When looking at the spine from the back, it should be relatively straight. When looking at the spine from the side, it has a normal curvature including a mild kyphosis (round back) in the upper spine and a compensatory lordosis in the lower or lumbar spine.

ASSOCIATION WITH SYRINGOMYELIA

Most forms of scoliosis are idiopathic in nature, meaning that we do not have an exact etiology (cause). Scoliosis is associated with syringomyelia quite commonly. According to two studies, a little more than half the time, children with syringomyelia develop scoliosis. This does appear to be related to the age at which the child becomes symptomatic from the syringomyelia. If the child develops symptoms prior to age 16, the incidence of scoliosis is much higher than if the symptoms develop later.

CLINICAL EVALUATION

Clinically, one can detect scoliosis by carefully examining the child in a standing position. If the shoulders are uneven or if, when upon bending forward, there is rib asymmetry (rib prominence), this could be consistent with scoliosis. Although scoliosis is commonly idiopathic (no identifiable cause), there are many causes for scoliosis, such as syringomyelia, tumor, arachnoid cyst, etc. Therefore, if scoliosis is suspected, an evaluation by a specialist should be sought. Uncommonly, scoliosis can actually be the first sign of an underlying syringomyelia. If the scoliosis is progressive, has an uncommon shape, or it occurs at a younger age, one should be concerned about syringomyelia and an MRI should be obtained.

TREATMENT

In general, non-surgical treatments for scoliosis, such as bracing, have not been shown to be effective. Treating the syrinx may benefit the scoliosis and typically only after the syrinx has been treated would one consider surgical treatment for the scoliosis. The exact link between syringomyelia and scoliosis is unknown. If the scoliosis is progressive despite successful syrinx treatment surgical treatment can be implemented. This is especially true if the scoliosis angle reaches 50 degrees. Surgical treatment typically involves fusing the segments that have curved significantly.

CONTROVERSIES

When the scoliosis is severe, some surgeons elect to treat the syrinx and scoliosis at the same surgery. This is a controversial issue. Advantages of sequential treatment (syrinx treatment first, and scoliosis treatment a few months later) include: 1) waiting to see if the scoliosis stabilizes or improves after syrinx treatment; 2) Ensuring that the syrinx is improving before fusing the spine and making further spinal cord surgery very difficult. New treatment options for scoliosis including non-fusion techniques are now being studied, and it is likely that more options will become available as technology develops and our understanding of these disorders deepens.

REFERENCES

  1. Eule J, Erickson MA, O’Brien MF, Handler, M. Chiari I Malformation Associated with Syringomyelia & Scoliosis. Spine 2002;22(6):771-779.
  2. Kontio K, Davidson D, Letts M. The Management of Scoliosis & Syringomyelia in Children. The Journal of Pediatric Orthopedics 2002;27(13):1451-14

 

Reviewed on 9/2019