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Defining optic nerve tortuosity.

G T Armstrong1, A R Localio, T Feygin

  • 1Division of Oncology, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine Philadelphia, PA, USA. greg.armstrong@stjude.org

AJNR. American Journal of Neuroradiology
|April 10, 2007
PubMed
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Optic nerve tortuosity in neurofibromatosis type 1 can be operationally defined by specific MR imaging findings. Lack of optic nerve congruity and subarachnoid space dilation together reliably diagnose tortuosity.

Area of Science:

  • Neuroradiology
  • Ophthalmology
  • Genetics

Background:

  • Optic nerve tortuosity is a nonmalignant finding in neurofibromatosis type 1 (NF1).
  • It may be associated with optic pathway gliomas in NF1 patients.
  • An operational definition for optic nerve tortuosity is needed.

Purpose of the Study:

  • To establish an operational definition for optic nerve tortuosity on MR imaging.
  • To identify specific radiographic factors indicative of optic nerve tortuosity.

Main Methods:

  • A focus group of pediatric neuroradiologists identified 6 radiographic factors from 20 MR images.
  • 28 MR images were assessed for tortuosity using a global assessment and the 6 identified factors.
  • Statistical analysis identified factors predicting tortuosity.

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Main Results:

  • Lack of optic nerve congruity in >1 coronal section and optic nerve subarachnoid space dilation were sensitive (89%) and specific (93%) for tortuosity.
  • Perfect inter-rater agreement was achieved in 75% of cases.
  • Absence of these factors and axial plane deviation reliably exclude tortuosity.

Conclusions:

  • Lack of optic nerve congruity and optic nerve subarachnoid space dilation provide an operational definition for optic nerve tortuosity.
  • This definition aids in diagnosing optic nerve tortuosity in NF1 patients.