Comparison of spinal deformity in children with Chiari I malformation with and without syringomyelia: matched cohort

J Godzik1, A Dardas2, M P Kelly3

  • 1Department of Neurosurgery, Barrow Neurological Institute, 350 W. Thomas Road, Phoenix, AZ, 85013, USA. jakub.godzik@bnaneuro.net.

Insights

Patients with Chiari malformation I (CIM) and scoliosis without syringomyelia present with less severe curves and fewer neurological issues compared to those with syringomyelia. This study highlights key differences in spinal deformity presentation in CIM patients.

Area of Science:

  • Neurosurgery
  • Orthopedic Surgery
  • Radiology

Background:

  • Chiari malformation type I (CIM) is a condition where brain tissue extends into the spinal canal.
  • Scoliosis is a common spinal deformity, and its association with CIM is increasingly recognized.
  • Understanding the specific curve patterns in CIM patients with and without syringomyelia (SM) is crucial for diagnosis and management.

Purpose of the Study:

  • To delineate the characteristics of spinal curve patterns in patients diagnosed with Chiari malformation I (CIM) but lacking syringomyelia (SM).
  • To compare these curve patterns with those observed in patients who have both CIM and SM.
  • To investigate associated craniovertebral junction parameters and neurological signs in both patient groups.

Main Methods:

  • A retrospective review of medical records from 2000 to 2013 at a single institution identified patients with CIM and scoliosis.
  • Patients were categorized into two groups: CIM without SM and CIM with SM (CIM + SM), matched for age and gender.
  • Radiographic data on spinal curves, MRI findings of the craniovertebral junction, and neurological examinations were analyzed and compared between the cohorts.

Main Results:

  • Eighteen patients with CIM and scoliosis but no SM were identified, predominantly female (78%) with a mean age of 11.5 years.
  • Patients with CIM alone showed significantly less severe scoliotic curves (32.1°) compared to the CIM + SM group (46.1°, p=0.04).
  • Fewer patients in the CIM group (11%) had left-sided thoracic apex curves compared to the CIM + SM group (50%, p=0.01), and neurological abnormalities were only present in the CIM + SM cohort (33%, p=0.007).

Conclusions:

  • Patients with CIM and scoliosis without SM exhibit distinct spinal deformity patterns, characterized by less severe curves and a lower incidence of left-sided thoracic apex deformities.
  • Comparable thoracic kyphosis was noted in both CIM and CIM + SM groups, suggesting specific pathogenetic mechanisms for scoliosis in CIM.
  • Further research is warranted to elucidate the pathogenesis of spinal deformities in CIM, both with and without syringomyelia, to guide clinical practice.
Abstract