Ectopic peripontine arcuate fibres, a novel finding in pontine tegmental cap dysplasia

Eur J Paediatr Neurol. 2014 May;18(3):434-8. doi: 10.1016/j.ejpn.2013.12.007. Epub 2014 Jan 11.


Background: Pontine Tegmental Cap Dysplasia (PTCD) is a recently described hindbrain malformation presenting hypoplasia of the ventral pons, and a "pontine tegmental cap". Previous DTI studies identified ectopic transversely oriented nerve fibres in the cap, and absence of transverse fibre bundles in the ventral pons, characterizing PTCD as an embryonic axon guidance defect. A new case with relatively mild symptoms was investigated to identify fibre tracts in the tegmental cap by tracking their connections. In the process a new bilateral ectopic fibre tract was found.

Methods: Routine T1- and T2 weighted images and Diffusion Tensor Imaging (DTI) data were obtained on a 3 T MR scanner. Fractional Anisotropy maps colour coded for orientation were generated. High Angular Resolution Diffusion Imaging (HARDI) data were used for reconstructing maps denoting multiple fibre orientations (i.e. fibre crossings) per voxel through which accurate fibre tracking was performed.

Results interpretation: Peripontine arcuate fibres were identified, representing a second structural abnormality not previously recorded in PTCD.

Keywords: Axon guidance; Brainstem malformation; DTI; Fibretracking; MRI; Radiology.

Publication types

  • Case Reports

MeSH terms

  • Axons / pathology*
  • Child
  • Diffusion Magnetic Resonance Imaging / methods
  • Humans
  • Image Processing, Computer-Assisted / methods
  • Male
  • Malformations of Cortical Development / diagnosis
  • Malformations of Cortical Development / pathology*
  • Pons / pathology*