Objective: To report a retrospective case series of 29 Australian and New Z
ealand patients with orbital and adnexal Wegener's granulomatosis (WG).
Design: Retrospective case series.
Participants: Twenty-nine cases of orbital and adnexal WG were identified.
Methods. A number of oculoplastic surgeons and other clinicians in Australi
a and New Zealand was asked about their experience with orbital and adnexal
WG. Clinical data regarding these cases were conveyed by means of a questi
onnaire. Cases of ophthalmic WG without features of orbital or adnexal dise
ase were excluded.
Main Outcome Measures: Data obtained from the questionnaire includes age, g
ender, limited or generalized disease, antineutrophil cytoplasmic antibody
(ANCA) status, symptoms and signs: nasolacrimal obstruction, sinusitis, fis
tula/orbital bone erosion, orbital mass/proptosis, extraocular muscle/diplo
pia, visual acuity reduction caused by optic nerve compression, orbital pai
n, lid edema/erythema, biopsy status, and treatment status.
Results: Twenty-nine patients with orbital and adnexal WG were identified a
nd described. Symptoms included awareness of an orbital mass, epiphora, orb
ital pain and diplopia. Signs included an orbital mass or proptosis (69%),
nasolacrimal duct obstruction (52%), limited ocular rotations (52%), lid er
ythema and edema (31%), bony destruction (21%), and reduced visual acuity (
17%). Two patients had a persistent nasolacrimocanthal fistula. Cytoplasmic
pattern antineutrophil cytoplasmic antibodies (c-ANCA) were present in 52%
of patients, and in 9 of 10 patients with generalized disease. However, c-
ANCA was positive in only 32% (6 of 19) of patients with limited WG. Perinu
clear pattern antineutrophil cytoplasmic antibodies (p-ANCA) was positive i
n 10% of cases.
Conclusions: To diagnose and treat ophthalmic WG effectively, the clinician
must be aware of its protean orbital and adnexal manifestations. WG may oc
cur with or without systemic involvement, and c-ANCA was negative in approx
imately half our cases. Our cases also demonstrated two orbital fistulae, a
n observation previously believed to be rare. (C) 2001 by the American Acad
emy of Ophthalmology.