Aims: It was the aim of this study to perform a review of the clinical features, treatments and outcomes of patients with idiopathic sclerosing orbital inflammation (ISOI) in Taiwan. Methods: All patients with histologically confirmed ISOI from 1995 to 2007 at the National Taiwan University Hospital were included. We reviewed the medical charts to see the clinical presentation, treatment modalities, treatment efficacy and final clinical status of these patients. Results: Six male and 3 female patients ranging in age from 33 to 82 years were included. The most common symptoms were pain and blurred vision (both 77.8%). Reduced visual acuity and proptosis were the most common signs (both 89%). Five patients received oral steroid treatment with/without pulse therapy combined with tissue removal, the response to which was good in 1 patient and partially effective in the other 4 patients. One patient received oral steroids and surgery, followed by radiotherapy, with only partial response. Patients with oral prednisolone or surgery only all had poor responses. Conclusion: ISOI is rare and also difficult to diagnose and manage. Early intervention with steroids/immunosuppressants combined with surgical debulking procedures may result in control or regression of this recalcitrant disease.

Keywords:
Debulking surgery, Idiopathic sclerosing orbital inflammation, Proptosis, Reduced visual acuity
1.
Rootman J, McCarthy M, White V, Harris G, Kennerdell J: Idiopathic sclerosing inflammation of the orbit: a distinct clinicopathologic entity. Ophthalmology 1994;101:570–584.
2.
Abramovitz JN, Kasdon DL, Sutula F, Post KD, Chong FK: Sclerosing orbital pseudotumor. Neurosurgery 1983;12:463–468.
3.
Levine MR, Kaye L, Mair S, Bates J: Multifocal fibrosclerosis: report of a case of bilateral idiopathic sclerosing pseudotumor and retroperitoneal fibrosis. Arch Ophthalmol 1993;111:841–843.
4.
Hsuan JD, Selva D, McNab AA, Sullivan TJ, Saeed P, O’Donnell BA: Idiopathic sclerosing orbital inflammation. Arch Ophthalmol 2006;124:1244–1250.
5.
Fujii H, Fujisada H, Kondo T, Takahasi T, Okada S: Orbital pseudotumor: histopathological classification and treatment. Ophthalmologica 1985;190:230–242.
6.
Weissler MC, Miller E, Fortune MA: Sclerosing orbital pseudotumor: a unique clinicopathologic entity. Ann Otol Rhinol Laryngol 1989;98:496–501.
7.
Kennerdell JS: The management of sclerosing nonspecific orbital inflammation. Ophthalmic Surg 1991;22:512–518.
8.
Mombaerts I, Schlingemann RO, Goldschmeding R, Noorduyn LA, Koornneef L: The surgical management of lacrimal gland pseudotumors. Ophthalmology 1996;103:1619–1627.
9.
Zborowska B, Ghabrial R, Selva D, McCluskey P: Idiopathic orbital inflammation with extraobital extension: case series and review. Eye 2006;20:107–113.
10.
On AV, Hirschbein MJ, Williams HJ, Karesh JW: CyberKnife radiosurgery and rituximab in the successful management of sclerosing idiopathic orbital inflammatory disease. Ophthal Plast Reconstr Surg 2006;22:395–397.
11.
Brannan PA: A review of sclerosing idiopathic orbital inflammation. Curr Opin Ophthalmol 2007;18:402–404.
12.
Sahlin S, Lignell B, Williams M, Dastmalchi M, Orrego A: Treatment of idiopathic sclerosing inflammation of the orbit (myositis) with infliximab. Acta Ophthalmol 2008, Epub ahead of print.
© 2009 S. Karger AG, Basel
2009
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.