Child's Nervous System

, Volume 35, Issue 10, pp 1853–1862 | Cite as

Scoliosis in patients with Chiari malformation type I

  • Mohammad Hassan A. Noureldine
  • Nir Shimony
  • George I. JalloEmail author
  • Mari L. Groves
Special Annual Issue


The literature about the association between Chiari malformations (CMs) and scoliosis has been growing over the last three decades; yet, no consensus on the optimal management approach in this patient population has been reached. Spinal anomalies such as isolated syrinxes, isolated CM, and CM with a syrinx are relatively common among patients with presumed idiopathic scoliosis (IS), a rule that also applies to scoliosis among CM patients as well. In CM patients, scoliosis presents with atypical features such as early onset, left apical or kyphotic curvature, and neurological deficits. While spinal X-rays are essential to confirm the diagnosis of scoliosis among CM patients, a magnetic resonance imaging (MRI) is also recommended in IS patients with atypical presentations. Hypotheses attempting to explain the occurrence of scoliosis in CM patients include cerebellar tonsillar compression of the cervicomedullary junction and uneven expansion of a syrinx in the horizontal plane of the spinal cord. Early detection of scoliosis on routine spinal examination and close follow-up on curve stability and progression are essential initial steps in the management of scoliosis, especially in patients with CM, who may require full spine MRI to screen for associated neuro-axial anomalies; bracing and spinal fusion may be subsequently pursued in high-risk patients.


Chiari malformation Scoliosis Syrinx Syringomyelia Suboccipital decompression Spinal fusion Left apical curve Cobb angle Posterior fossa Curve progression 



The authors acknowledge the Hough Family Foundation for support in writing this manuscript.


This research was funded by the Hough Family Foundation.

Compliance with ethical standards

Conflict of interest

None of the authors have potential conflicts of interest to be disclosed.


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© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Johns Hopkins University School of Medicine, Institute for Brain Protection SciencesJohns Hopkins All Children’s Hospital, Saint PetersburgSaint PetersburgUSA
  2. 2.Geisinger Medical CenterNeuroscience InstituteDanvilleUSA
  3. 3.Geisinger Commonwealth School of MedicineScrantonUSA
  4. 4.Johns Hopkins University Department of NeurosurgeryJohns Hopkins HospitalBaltimoreUSA

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