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Erdheim-Chester-Erkrankung der Orbita mit Kompressionsneuropathie

Erdheim-Chester disease of the orbit with compressive optic neuropathy

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Zusammenfassung

Ein 60-jähriger Mann stellte sich mit einem zunehmenden Exophthalmus und einer allmählichen Sehverschlechterung des linken Auges vor. Es wurde die Diagnose einer bilateralen Erdheim-Chester-Erkrankung (ECH) der Orbita gestellt. Die weitere Diagnostik ergab eine retroperitoneale Fibrose. Eine Therapie mit Interferon-α wurde durchgeführt, aber im Verlauf kam es zu einer bilateralen Kompressionsneuropathie mit Visusabfall und Gesichtsfeldausfällen. Eine bilaterale orbitale Dekompression wurde durchgeführt.

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-α followed, but bilateral compressive optic neuropathy with visual acuity deterioration and visual field defects evolved. Bilateral orbital decompression was performed.

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Authors and Affiliations

  1. Klinik und Poliklinik für Augenheilkunde, Universität Rostock, Doberanerstraße 140, 18055, Rostock, Deutschland

    K. Manousaridis & R.F. Guthoff

  2. Klinik für Hämatologie und Onkologie, Universität Rostock, Rostock, Deutschland

    J. Casper

  3. Klinik für Augenheilkunde, Universität Göttingen, Göttingen, Deutschland

    M.P. Schittkowski

  4. Institut für Pathologie, Universität Rostock, Rostock, Deutschland

    H. Nizze

Authors
  1. K. Manousaridis
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  2. J. Casper
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  3. M.P. Schittkowski
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  4. H. Nizze
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  5. R.F. Guthoff
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Correspondence to K. Manousaridis.

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Manousaridis, K., Casper, J., Schittkowski, M. et al. Erdheim-Chester-Erkrankung der Orbita mit Kompressionsneuropathie. Ophthalmologe 107, 266–269 (2010). https://doi.org/10.1007/s00347-009-2041-y

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  • DOI: https://doi.org/10.1007/s00347-009-2041-y

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