P 369
Orbital pseudotumor leading to orbital exenteration clinico-pathologic correlation including MRI featuresF. Colombo, A. Viestenz, L. M. Holbach
Introduction: Orbital pseudotumor is one of the most common causes of proptosis. It is a benign entity that usually shows an excellent response to corticosteroid treatment. Orbital exenteration is only rarely indicated.
Patient and Methods: A 67-year-old female was referred for left hemifacial pain - more intense in the periorbital area - and an ipsilateral red eye. The pain persisted in spite of the administration of analgesics and topical and systemic steroids. The patient had a history of anterior nodular scleritis 3 years ago and a left facial palsy because of a diabetic mononeuropathy 4 years ago.
Results: The ophthalmic evaluation showed a left blind eye with diffuse anterior scleritis, an intercalar staphyloma nasally and superiorly as well as a total lens opacity. The eye movements were totally restricted and there was a 3 mm proptosis of the left eye. MRI demonstrated a diffuse intraconal lesion in the left orbit, hypointense in T1- and T2-weighted images, with postcontrast enhancement. Pertinent systemic diseases were ruled out. A lid-sparing exenteration of the left orbit was performed. Macroscopically, the orbital fat was replaced intraconally by dense fibrous tissue. Microscopically, this abnormal tissue showed a diffuse, lymphoplasmacytic inflammatory infiltrate, as well as collagen deposit. The globe displayed an intercalar staphyloma, a secondary angle closure glaucoma with rubeosis iridis and marked uveitis. Postoperatively, the patient recovered rapidly and had no more pain.
Conclusion: Orbital pseudotumor may be corticosteroid-resistant and show an aggressive clinical behavior. In cases associated with persisting pain and a blind eye, a lid-sparing orbital exenteration may be indicated.
Augenklinik mit Poliklinik, Universität Erlangen-Nürnberg, Schwabachanlage 6, D-91054 Erlangen