Plexiform Neurofibroma as an Uncommon Cause of Periorbital Swelling

JOURNAL TITLE: International Journal of Advanced and Integrated Medical Sciences

Author
1. Yusuf Rizvi
2. Abhinav Srivastava
3. Vaibhav Yadav
ISSN
DOI
10.5005/jp-journals-10050-10120
Volume
3
Issue
1
Publishing Year
2018
Pages
3
Author Affiliations
    1. Department of ENT, Rohilkhand Medical College and Hospital Bareilly, Uttar Pradesh, India
    1. Depatment of Ophthalmology, Rohilkhand Medical College & Hospital, Bareilly, Uttar Pradesh, India
    1. Department of Ophthalmology, Rohilkhand Medical College and Hospital, Bareilly, Uttar Pradesh, India
  • Article keywords
    Plexiform neurofibroma, Proptosis, Surgical excision

    Abstract

    Introduction: Plexiform neurofibromas are benign tumors originating from subcutaneous or visceral peripheral nerves, which are usually associated with neurofibromatosis type I. Orbital neurofibromas are relatively rare and usually present with high infiltration into the periocular structures making the management extremely difficult. Purpose: To r eport a c ase o f p lexiform n eurofibroma w ith neurofibromatosis type I in a young boy managed satisfactorily with surgical excision. Materials and methods: A 14-year-old boy presenting with a soft, painless, nontender, gradually enlarging swelling of the left upper eyelid and adjacent temporal region and of 6 years duration was managed with complete surgical excision. Histopathological examination of excised mass confirmed the diagnosis of neurofibroma. Results: Satisfactory functional and cosmetic result was achieved following surgical excision of the subcutaneous fibromatous mass. A 1-year follow-up did not reveal any functional deficit or recurrence. Conclusion: Plexiform neurofibroma may present as an uncommon cause of periorbital swelling even in the absence of typical features of neurofibromatosis type I and can be surgically managed to achieve acceptable cosmetic and functional results.

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