Multiple intraorbital neurofibromas: a rare cause of proptosis

Details

Serval ID
serval:BIB_1666BA848F13
Type
Article: article from journal or magazin.
Publication sub-type
Case report (case report): feedback on an observation with a short commentary.
Collection
Publications
Institution
Title
Multiple intraorbital neurofibromas: a rare cause of proptosis
Journal
Klinische Monatsblatter fur Augenheilkunde
Author(s)
Berney  C., Spahn  B., Oberhansli  C., Uffer  S., Borruat  F. X.
ISSN
0023-2165 (Print)
Publication state
Published
Issued date
05/2004
Volume
221
Number
5
Pages
418-20
Notes
Case Reports
Journal Article --- Old month value: May
Abstract
BACKGROUND: Orbital neurofibromas are rare, accounting for 0.5 to 2.4 % of all orbital tumors. Generally, they manifest as slowly progressive proptosis, in a young adult or middle-aged person, and are usually solitary lesions. Sometimes, they can be associated with type 1 neurofibromatosis. We present a case of proptosis related to multiple intraorbital neurofibromas in an 82-year-old woman without type 1 neurofibromatosis. HISTORY AND SIGNS: An 82-year-old woman was referred for slowly progressive left proptosis associated with an ocular burning sensation. Neuro-ophthalmic examination revealed 9.5 mm of left exophthalmos, signs of minimal left optic neuropathy but normal extraocular movements. Magnetic resonance imaging revealed the presence of 4 intraorbital lesions. THERAPY AND OUTCOME: The two most anterior tumors were removed. Pathological studies showed these tumors to be neurofibromas. Post-operative evolution was favorable with reduction of left proptosis to 7 mm and disappearance of the burning sensation of the left eye. No other signs of neurofibromatosis were found. CONCLUSIONS: Multiple circumscribed intraorbital tumors are rare. Slowly progressive proptosis with radiological imaging of multiple round lesions should evoke the diagnosis of orbital neurofibromas, even in patients outside the typical age range or without neurofibromatosis.
Keywords
Aged Aged, 80 and over Exophthalmos/*etiology/pathology/surgery Female Humans Magnetic Resonance Imaging Neurofibromatoses/*complications/diagnosis/pathology/surgery Optic Nerve Diseases/diagnosis/etiology/pathology Orbit/pathology/surgery Orbital Neoplasms/*complications/diagnosis/pathology/surgery
Pubmed
Web of science
Create date
28/01/2008 12:37
Last modification date
20/08/2019 12:45
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