Ophthalmic manifestations in neurofibromatosis type 1

Surv Ophthalmol. 2018 Jul-Aug;63(4):518-533. doi: 10.1016/j.survophthal.2017.10.007. Epub 2017 Nov 16.

Abstract

Neurofibromatosis type 1 (NF1) is a relatively common multisystemic inherited disease and has been extensively studied by multiple disciplines. Although genetic testing and confirmation are available, NF1 remains a clinical diagnosis. Many manifestations of NF1 involve the eye and orbit, and the ophthalmologist, therefore, plays a significant role in the diagnosis and treatment of NF1 patients. Improvements in diagnostic and imaging instruments have provided new insight to study the ophthalmic manifestations of the disease. We provide a comprehensive and up-to-date overview of the ocular and orbital manifestations of NF1.

Keywords: Lisch nodules; neurofibromatosis type 1; optic pathway gliomas; plexiform neurofibromas; von Recklinghausen's disease.

Publication types

  • Review

MeSH terms

  • Anterior Eye Segment / pathology
  • Antineoplastic Agents / therapeutic use
  • Eye Diseases* / diagnosis
  • Eye Diseases* / etiology
  • Eye Diseases* / pathology
  • Humans
  • Neurofibromatosis 1 / complications*
  • Neurofibromatosis 1 / diagnosis
  • Neurofibromatosis 1 / therapy
  • Ophthalmologic Surgical Procedures / methods
  • Orbital Diseases* / etiology
  • Orbital Diseases* / pathology
  • Orbital Diseases* / therapy
  • Radiotherapy / methods

Substances

  • Antineoplastic Agents