gms | German Medical Science

76th Annual Meeting of the German Society of Oto-Rhino-Laryngology, Head and Neck Surgery

German Society of Oto-Rhino-Laryngology, Head and Neck Surgery

04.05. - 08.05.2005, Erfurt

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Orbital manifestation of Ormond’s disease

Meeting Abstract

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  • corresponding author Wolfram Aust - HNO-Klinik, MLU Halle-Wittenberg, Halle
  • Stephan Knipping - HNO-Klinik, MLU Halle-Wittenberg, Halle
  • Hans-Jürgen Holzhausen - Institut für Pathologie, MLU Halle-Wittenberg, Halle
  • Marc Bloching - HNO-Klinik, MLU Halle-Wittenberg, Halle

Deutsche Gesellschaft für Hals-Nasen-Ohren-Heilkunde, Kopf- und Hals-Chirurgie. 76. Jahresversammlung der Deutschen Gesellschaft für Hals-Nasen-Ohren-Heilkunde, Kopf- und Hals-Chirurgie e.V.. Erfurt, 04.-08.05.2005. Düsseldorf, Köln: German Medical Science; 2005. Doc05hno365

The electronic version of this article is the complete one and can be found online at: http://www.egms.de/en/meetings/hno2005/05hno245.shtml

Published: September 22, 2005

© 2005 Aust et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.en). You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.

Outline

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Typically the Ormond’s disease appears as idiopathic retroperitoneal fibrosis. It is characterized by proliferation of fibrous tissue replacing the normal tissue in the central retroperitoneum. Usually the fibrosis involves the area between the renal arteries and sacrum, and causes encasement of the aorta, inferior vena cava and ureters, resulting in obstructive hydronephrosis.

We present a case of a 42-years-old female patient with a pronounced exophthalmus, ptosis, a reduced visual acuity and ophthalmoplegia of the right eye. Magnetic resonance (MR) images showed a soft tissue mass on contrast-enhanced T2-weighted images of the right orbita with inclusion of the muscles and the optical nerve. The lateral orbitotomy with biopsies, demonstrated fibrous tissue with infiltration of lymphocytes and plasma cells without any evidence of malignancy, confirming the diagnosis of Ormond’s disease. A retroperitoneal participation of the disease could be excluded.

Complaints were declining with a reduction of the exophthalmus, improved eye movement and an improved visual acuity. The further anti-inflammatory therapy was a combination of prednisolone and methotrexate.