Erdheim-Chester disease of the orbit with compressive optic neuropathy

ISSN
0941-293X
Date Issued
2010
Author(s)
Manousaridis, K.
Casper, Jochen
Nizze, H.
Guthoff, R. F.
DOI
10.1007/s00347-009-2041-y
Abstract
A 60-year-old man presented with left exophthalmos and deterioration in visual acuity of slow evolution. Bilateral orbital Erdheim-Chester disease was diagnosed. Systemic evaluation revealed a retroperitoneal fibrosis. Treatment with interferon-alpha followed, but bilateral compressive optic neuropathy with visual acuity deterioration and visual field defects evolved. Bilateral orbital decompression was performed.