[Erdheim-Chester disease of the orbit with compressive optic neuropathy].
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.
Klinik und Poliklinik für Augenheilkunde, Universität Rostock, Deutschland. klemanousaridis@kabelmail.de
K
K
Manousaridis
J
J
Casper
M P
MP
Schittkowski
H
H
Nizze
R F
RF
Guthoff
D003937:Diagnosis, Differential; D031249:Erdheim-Chester Disease / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009408:Nerve Compression Syndromes / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D009900:Optic Nerve / Q000473:pathology; D009901:Optic Nerve Diseases / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D009915:Orbit / Q000473:pathology / Q000601:surgery; D012185:Retroperitoneal Fibrosis / Q000175:diagnosis; D012187:Retroperitoneal Space; D014057:Tomography, X-Ray Computed