Radiological and clinical associations with scoliosis outcomes after posterior fossa decompression in patients with Chiari malformation and syrinx from the Park-Reeves Syringomyelia Research Consortium

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  • 1 Department of Neurological Surgery, Washington University School of Medicine, St. Louis, Missouri;
  • 2 Department of Epidemiology, University of Iowa, Iowa City, Iowa;
  • 3 Department of Neurological Surgery, Vanderbilt University Medical Center, Nashville, Tennessee;
  • 4 Department of Neurosurgery, Neuroscience Institute, Johns Hopkins All Children’s Hospital, St. Petersburg, Florida;
  • 5 Department of Radiology, Washington University School of Medicine, St. Louis, Missouri;
  • 6 Department of Neurological Surgery, Columbia University College of Physicians and Surgeons, New York, New York; and
  • 7 Department of Orthopedic Surgery, Washington University School of Medicine, St. Louis, Missouri
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OBJECTIVE

In patients with Chiari malformation type I (CM-I) and a syrinx who also have scoliosis, clinical and radiological predictors of curve regression after posterior fossa decompression are not well known. Prior reports indicate that age younger than 10 years and a curve magnitude < 35° are favorable predictors of curve regression following surgery. The aim of this study was to determine baseline radiological factors, including craniocervical junction alignment, that might predict curve stability or improvement after posterior fossa decompression.

METHODS

A large multicenter retrospective and prospective registry of pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and a syrinx (≥ 3 mm in width) was reviewed for clinical and radiological characteristics of CM-I, syrinx, and scoliosis (coronal curve ≥ 10°) in patients who underwent posterior fossa decompression and who also had follow-up imaging.

RESULTS

Of 825 patients with CM-I and a syrinx, 251 (30.4%) were noted to have scoliosis present at the time of diagnosis. Forty-one (16.3%) of these patients underwent posterior fossa decompression and had follow-up imaging to assess for scoliosis. Twenty-three patients (56%) were female, the mean age at time of CM-I decompression was 10.0 years, and the mean follow-up duration was 1.3 years. Nine patients (22%) had stable curves, 16 (39%) showed improvement (> 5°), and 16 (39%) displayed curve progression (> 5°) during the follow-up period. Younger age at the time of decompression was associated with improvement in curve magnitude; for those with curves of ≤ 35°, 17% of patients younger than 10 years of age had curve progression compared with 64% of those 10 years of age or older (p = 0.008). There was no difference by age for those with curves > 35°. Tonsil position, baseline syrinx dimensions, and change in syrinx size were not associated with the change in curve magnitude. There was no difference in progression after surgery in patients who were also treated with a brace compared to those who were not treated with a brace for scoliosis.

CONCLUSIONS

In this cohort of patients with CM-I, a syrinx, and scoliosis, younger age at the time of decompression was associated with improvement in curve magnitude following surgery, especially in patients younger than 10 years of age with curves of ≤ 35°. Baseline tonsil position, syrinx dimensions, frontooccipital horn ratio, and craniocervical junction morphology were not associated with changes in curve magnitude after surgery.

ABBREVIATIONS CM-I = Chiari malformation type I; pB = posterior basion; PFD = posterior fossa decompression.

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Contributor Notes

Correspondence Jennifer M. Strahle: Washington University School of Medicine, St. Louis Children’s Hospital, St. Louis, MO. strahlej@wustl.edu.

INCLUDE WHEN CITING Published online April 10, 2020; DOI: 10.3171/2020.1.PEDS18755.

Disclosures Dr. Kelly reports receiving support for a non–study-related clinical or research effort that he oversees from DePuy Synthes. Dr. Limbrick reports receiving support for a non–study-related clinical or research effort that he oversees from Microbot, Inc., and from Medtronic.

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