Multiple intraorbital neurofibromas: a rare cause of proptosis

Détails

ID Serval
serval:BIB_1666BA848F13
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Etude de cas (case report): rapporte une observation et la commente brièvement.
Collection
Publications
Titre
Multiple intraorbital neurofibromas: a rare cause of proptosis
Périodique
Klinische Monatsblatter fur Augenheilkunde
Auteur(s)
Berney  C., Spahn  B., Oberhansli  C., Uffer  S., Borruat  F. X.
ISSN
0023-2165 (Print)
Statut éditorial
Publié
Date de publication
05/2004
Volume
221
Numéro
5
Pages
418-20
Notes
Case Reports
Journal Article --- Old month value: May
Résumé
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.
Mots-clé
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
Création de la notice
28/01/2008 12:37
Dernière modification de la notice
20/08/2019 12:45
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