Hostname: page-component-cd9895bd7-jn8rn Total loading time: 0 Render date: 2024-12-22T16:49:53.386Z Has data issue: false hasContentIssue false
  • English
  • Français

Wegener's granulomatosis of the orbit: two cases requiring endoscopic surgical decompression

Published online by Cambridge University Press:  09 April 2008

J M Fishman ,
A Slovick  and
C A East
J M Fishman*
Affiliation:
Department of Otolaryngology, The Royal Free Hospital, London, UK
A Slovick
Affiliation:
Department of Otolaryngology, The Royal Free Hospital, London, UK
C A East
Affiliation:
Royal National Throat, Nose and Ear Hospital, London, UK
*
Address for correspondence: Mr Jonathan Fishman, Department of Otolaryngology, The Royal Free Hospital, Pond Street, London NW3 2QG, UK. Fax: 0207 8302422 E-mail: jfishman@doctors.org.uk
Get access Rights & Permissions [Opens in a new window]

Abstract

Objectives:

We report two unusual and contrasting cases of Wegener's granulomatosis involving the orbit, requiring urgent endoscopic orbital decompression surgery.

Case series:

Both patients presented with an acute deterioration in visual function which failed to improve with medical management. Case one presented with systemic features of the condition, in contrast with case two, who presented with a more localised form of the disease. Whereas case one exhibited primary orbital disease with maxillary sinus extension (i.e. focal disease), case two illustrates orbital involvement secondary to sinus disease extension (i.e. contiguous disease). Prompt diagnosis, assisted by the presence of systemic features, led to a good visual outcome in case one. In case two, in which diagnosis was difficult and surgery delayed, the outcome was poor.

Conclusions:

These cases of orbital Wegener's granulomatosis illustrate the diagnostic challenge, the requirement for early intervention following acute visual deterioration and the importance of heightened awareness of the rarer ENT manifestations of this disease.

Keywords

Wegener's GranulomatosisOrbitParanasal SinusesVisual Loss
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 122 , Issue 11 , November 2008 , pp. 1257 - 1259
Copyright
Copyright © JLO (1984) Limited 2008

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1 Srouji, IA, Andrews, P, Edwards, C, Lund, VJ. Patterns of presentation and diagnosis of patients with Wegener's granulomatosis: ENT aspects. J Laryngol Otol 2007;121:653–8CrossRefGoogle ScholarPubMed
2 Pakrou, N, Selva, D, Leibovitch, I. Wegener's granulomatosis: ophthalmic manifestations and management. Semin Arthritis Rheum 2006;35:284–92CrossRefGoogle Scholar
3 Rao, JK, Weinberger, M, Oddone, EZ, Allen, NB, Landsman, P, Feussner, JR. The role of antineutrophil cytoplasmic antibody (c-ANCA) testing in the diagnosis of Wegener granulomatosis. Ann Intern Med 1995;123:925–32CrossRefGoogle ScholarPubMed
4 Kalina, PH, Lie, JT, Campbell, RJ, Garrity, JA. Diagnostic value and limitations of orbital biopsy in Wegener's granulomatosis. Ophthalmology 1992;99:120–4CrossRefGoogle ScholarPubMed