Hoffmann E., Pitz S., Pfeiffer N.

Dept. of Ophthalmology, University of Mainz, Langenbeckstr.1, D-55101 Mainz

Objective: The Erdheim-Chester Disease is a rare histiocytosis histological characterized by xanthogranulomatous histocytes. It affects bones, heart, lung, liver, kidneys, retroperitoneum, cerebrum and orbita. The orbital infiltration is normally bilateral. Today 23 cases with orbital involvement have been reported in literature
Case: We present a 61 year old man with progedient decrease of visual acuity in both eyes because of retinal and choroidal folds and optic atrophy since six years following exophthalmus. The computed tomogramm of the abdomen shows a multifocal fibrosclerosis. These findings led to the diagnosis of a M. Ormond. A biopsy of the orbita revealed a chronic fibrous infiltration, consistent with the first diagnosis of a M. Ormond with orbital involvement. A treatment with radiotherapy and long-term corticosteroids followed. Under therapy the retroperitoneal infiltration decreased but the orbital involvement showed no response on the streoids. A repetition of the orbital biopsy was performed outwards and histologic examination exhibited the diagnosis of Erdheim-chester disease because of xanthogranulomatous infiltration and Touton giant cells. The diffuse orbital masses induced a progressive decrease of visual acuity, why an endonasal decompression operation was performed. The visual acuity and the patient`s condition is stable under therapy with low-dose corticosteroids.( 10mg per day)
Conclusion: M. Ormond as well as Erdheim- Chester disease are characterized of orbital infiltration and multiorgan involvement. Patients with Erdheim- Chester disease have a worse prognosis than those with M. Ormond.

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