[Orbital exenteration in therapy-resistant painful scleritis-associated inflammation of orbital soft tissues]

Presentation of a 67-year-old female with persisting left hemifacial pain - very intense in the periorbital area, and an ipsilateral red eye. The patient had received analgesics and corticosteroids systemically, but no significant improvement was achieved. She had a history of anterior nodular scleritis in her left eye three years ago. Immunologic screening, especially for Wegener's granulomatosis, was negative. The ophthalmic evaluation revealed a left blind eye with diffuse anterior scleritis, an intercalar staphyloma nasally and superiorly, absolute angle closure glaucoma with rubeotic iris and mature cataract. With a 3 mm proptosis with a totally immurable globe, the MRI demonstrated a diffuse orbital tumour with involvement of intraconal space and extraconal muscles. A lid-sparing exenteration of the left orbit was performed. Postoperatively, the patient recovered rapidly and had no more pain. Macroscopically, the orbital fat was replaced by dense scar tissue. Microscopically, a focal necrotising granulomatous vasculitis of small vessels and scleritis with zonal granulomatous reaction on scleral collagen was observed.

Unilateral proptosis with anterior and posterior scleritis combined with granulomatous inflammation of orbital soft tissues may be the first symptom of Wegener's granulomatosis. In addition to systemic immunosuppression, the lidsparing orbital exenteration may be one option of therapy.