Australasian orbital and adnexal Wegener's granulomatosis

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.