Patients with Chiari malformation without syringomyelia show less severe scoliosis curves

A recent study compared the physical symptoms between the two groups of patients.

SAN FRANCISCO — Patients with Chiari malformation I-associated scoliosis in the absence of syringomyelia have less severe scoliotic curves, fewer left-facing curves and present with less physical symptoms compared to patients with Chiari malformation I and syringomyelia, according to a study presented at the American Association of Neurological Surgeons Annual Meeting.

“Given these results and the sagittal profile, we still continue to believe that those patients who have Chiari malformation and scoliosis that the scoliosis results from the underlying neurological disease, not necessarily representing the basal level of scoliosis in the population,” Jakub Godzik, BA, said, here.

A retrospective study

Godzik presented results of a retrospective study in which researchers reviewed the medical records at Washington University in St. Louis from 2000 and 2013. They identified patients younger than 18 years old who had Chiari I malformation (CIM) with scoliosis in the absence of syringomyelia. Godzik and colleagues identified patients who had a CIM of 5 mm or more and patients that had scoliosis of more than 10° on radiographic imaging. The group used a threshold of 2 mm to identify those patients who had syringomyelia.

The study excluded patients with any genetic chromosomal issues and those who lacked imaging.

Patients were matched by age and gender to patients who have CIM with scoliosis and syringomyelia. The investigators compared the groups’ initial curve patterns, imaging characteristics and physical findings.

Overall, Godzik and colleagues identified 524 patients with CIM, of which 18 patients were found to have CIM-associated scoliosis in the absence of syringomyelia, for a prevalence of 3.3%, as compared with a prevalence of 38% of patients with syringomyelia. Four patients were male with a mean age of 11.5 years.

Godzik said in his presentation there were no differences in age or gender between the two groups of patients.

Less severe curves without syringomyelia

Patients without syringomyelia were found to have less severe scoliotic curves (30.2°) compared to the patients with syringomyelia (50.3°). However, the researchers noted both groups had a comparable amount of hyperkyphosis (46° vs. 51°). Two patients without syringomyelia had left-facing deformities compared with left-facing deformities in 50% of the patients with CIM and syringomyelia. The researchers found 33% of the patients with CIM and syringomyelia had neurological deficits, however, no patients without syringomyelia showed similar neurological deficits.

In terms of surgical management, Godzik said 50% of patients with CIM and without syringomyelia underwent scoliosis surgery as compared to 78% of patients with CIM and syringomyelia.

There are limitations to the study, Godzik said. It is a retrospective study and also patients with CIM and no syrinx typically do not undergo radiographic imaging of the spine, so there is a chance the study under represented the number of patients in that group, he said.

More research and additional studies are needed to determine the basis for the formation of this spine deformity in this particular population, according to Godzik. – by Kristine Houck, MA, ELS, and Robert Linnehan.

Reference:

Godzik J. Paper #654. Presented at: American Association of Neurological Surgeons Annual Meeting; April 5-9, 2014; San Francisco.

For more information:

Jakub Godzik, BA, can be reached at the Department of Orthopaedic Surgery, Washington University School of Medicine, 660 S. Euclid Ave., Campus Box 8233, St. Louis, MO 63110; email: godzikj@wusm.wustl.edu.
Disclosure: Godzik has no relevant financial disclosures.