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Plexiform Neurofibroma

  • Shantha AmrithEmail author
  • Stephanie Ming Young
  • Eric Ting
  • Bingcheng Wu
  • Min En Nga
  • Gangadhara Sundar
Chapter

Abstract

The manifestations of neurofibromatosis (NF) in the ocular adnexa consist of plexiform neurofibroma in the eyelids, often with orbital extension, and sphenoid wing hypoplasia. NF may be associated with tumours such as optic nerve gliomas, meningiomas or schwannomas. It is the most common peripheral neural tumour of the ocular adnexa causing hypertrophy of periocular tissues, resulting in mechanical ptosis with a characteristic S-shaped deformity in the upper eyelid. Individualized treatment plan must be drawn after proper preoperative assessment, taking into consideration the disfigurement and functionality that a patient.

Further Reading

  1. 1.
    Avery RA, Katowitz JA, Fisher MJ, Heidary G, Dombi E, Packer RJ, Widemann BC. Orbital/peri-orbital plexiform neurofibromas in children with neurofibromatosis type 1: multi-disciplinary recommendations for care. Ophthalmology. 2017;124(1):123–32.CrossRefGoogle Scholar
  2. 2.
    Chaudhry IA, Morales J, Shamsi FA, Al-Rashed W, Elzaridi E, Arat YO, Jacquemin C, Oystreck DT, Bosley TM. Orbitofacial neurofibromatosis: clinical characteristics and treatment outcome. Eye (Lond). 2012;26(4):583–59.CrossRefGoogle Scholar
  3. 3.
    National Institute of Health Consensus Development Conference. Neurofibromatosis Conference statement. Arch Neurol. 1988;45:575–8.Google Scholar

Copyright information

© Springer Nature Singapore Pte Ltd. 2019

Authors and Affiliations

  • Shantha Amrith
    • 1
    • 2
    Email author
  • Stephanie Ming Young
    • 1
    • 2
  • Eric Ting
    • 3
    • 4
    • 5
  • Bingcheng Wu
    • 6
  • Min En Nga
    • 6
    • 7
  • Gangadhara Sundar
    • 1
    • 2
  1. 1.Department of OphthalmologyNational University HospitalSingaporeSingapore
  2. 2.Department of OphthalmologyYong Loo Lin School of Medicine, National University of SingaporeSingaporeSingapore
  3. 3.Department of Diagnostic ImagingNational University HospitalSingaporeSingapore
  4. 4.Department of Diagnostic ImagingYong Loo Lin School of Medicine, National University of SingaporeSingaporeSingapore
  5. 5.Advanced Medicine ImagingSingaporeSingapore
  6. 6.Department of PathologyNational University HospitalSingaporeSingapore
  7. 7.Department of PathologyYong Loo Lin School of Medicine, National University of SingaporeSingaporeSingapore

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