Abstract
Abstract: :
Purpose:No consensus exists regarding the treatment algorithm for cases of sclerosing orbital pseudotumor (SOP). Generally, steroid therapy is initiated and followed by other modalities (radiotherapy, immunotherapy, debulking surgery) for treatment failures. We describe our experience with this subtype of orbital inflammatory disease, and discuss factors that may warrant aggressive secondary therapy. Methods:Retrospective chart review of cases of biopsy–proven SOP, comparing results in cortiosteroid (CS) or corticosteroids with adjuvant radiation therapy (CS+RT) subgroups. Results: Eight patients (5 men, 3 women, mean age = 48.1 years) with biopsy–proven SOP were followed for a mean of 12.6 months (range = 3–41 months). After biopsy, five received only CS and three required RT (2,550 cGy, 13 sessions) after steroid therapy failed to control the disease process. The CS and CS+RT groups had similar mean age at presentation and gender (47 years, 3 men, 2 women vs. 50 years, 2 males, 1 woman). The CS group had an initial mean proptosis of 4.6 mm (vs. 5.0 mm in the CS+RT group). One patient (33%) in the CS+RT group had no light perception at presentation. All other CS+RT patients had acuities of 20/30 or better. In the CS group, four patients (80%) had acuity of 20/30 or better, and the fifth had 20/400 vision. One CS patient (20%) had a greater than 25% reduction in extra–ocular movements. One CS+RT (33%) patient had less than 25% motility. Both groups had similar mean initial oral prednisone doses (CS=52 mg/day, CS+RT=60 mg/day) and similar periods of oral steroid therapy (7.6 weeks vs. 7 weeks). The CS group experienced an 83% reduction in proptosis, and the CS+RT group a 34% reduction. The CS and CS+RT groups presented at a mean of 1.7 months and 9.0 months after the development of symptoms, repectively. No patient in the CS group presented after three months, and no CS+RT patient presented before seven months. Conclusions: Neither age nor gender predicted the need for adjuvant radiation therapy (vs. corticosteroid therapy alone) in the treatment of our cases of sclerosing orbital pseudotumor. Prolonged duration of symptoms prior to initiation of therapy appears to be associated with increased disease severity, decreased responsiveness to corticosteroids and a greater likelihood for requiring radiation therapy. Early recognition and initiation of therapy for SOP may allow disease control using corticosteroids alone. Long–standing SOP may be less responsive to corticosteroids, and the earlier initiation of radiation therapy may be appropriate in cases of longer duration.
Keywords: orbit • radiation therapy • clinical (human) or epidemiologic studies: risk factor assessment