Fourth Ventricle Enlargement in Chiari Malformation Type I

World Neurosurg. 2020 Jan:133:e259-e266. doi: 10.1016/j.wneu.2019.08.230. Epub 2019 Sep 9.

Abstract

Objective: How Chiari malformation type I (CM-I) affects posterior fossa brain structures and produces various symptoms remains unclear. The fourth ventricle is surrounded by critical structures required for normal function. The foramen of Magendie can be obstructed in CM-I; therefore, fourth ventricle changes may occur. To test this hypothesis, we assessed fourth ventricle volume in CM-I compared with healthy controls.

Methods: Using our database from 2007-2016, we studied 72 patients with CM-I and 30 age-matched healthy control subjects. Fourth and lateral ventricle volumes and posterior fossa volumes (PFV) were assessed and correlated with clinical signs and symptoms. Statistical analysis was performed.

Results: Patients with CM-I had larger fourth ventricle volumes compared with control subjects (1.31 vs. 0.95 mL; P = 0.012). There were no differences in lateral ventricle volume or PFV. CM-I fourth ventricle volume was associated with tonsillar descent (P = 0.030). CM-I fourth ventricle volume variance was larger than healthy controls (F71,29 = 8.33; P < 0.0001). Patients with CM-I with severe signs and symptoms had a significantly larger fourth ventricle than patients with CM-I with mild signs and symptoms (1.565 vs. 1.015 mL; P = 0.0002).

Conclusions: The fourth ventricle can be enlarged in CM-I independent of lateral ventricle size and is associated with greater tonsillar descent. Most importantly, fourth ventricle enlargement was associated with a worse clinical and radiographic presentation independent of PFV. Fourth ventricle enlargement can affect critical structures and may be a mechanism contributing to symptoms unexplained by tonsil descent. Fourth ventricle enlargement is a useful adjunct in assessing CM-I.

Keywords: Arachnoid veil; Cerebellum; Chiari I malformation; Cognitive function; Dentate nucleus; Fourth ventricle; Tonsillar herniation.

MeSH terms

  • Adolescent
  • Adult
  • Arnold-Chiari Malformation / complications
  • Arnold-Chiari Malformation / diagnostic imaging
  • Arnold-Chiari Malformation / pathology*
  • Child
  • Child, Preschool
  • Cranial Fossa, Posterior / diagnostic imaging
  • Cranial Fossa, Posterior / pathology*
  • Dilatation, Pathologic / diagnostic imaging
  • Dilatation, Pathologic / pathology
  • Encephalocele / diagnostic imaging
  • Encephalocele / etiology
  • Female
  • Fourth Ventricle / diagnostic imaging
  • Fourth Ventricle / pathology*
  • Humans
  • Imaging, Three-Dimensional
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Neuroimaging
  • Organ Size
  • Prospective Studies
  • Young Adult