Idiopathic orbital inflammatory disease.

The purpose of this study was to review our clinical experience with 132 idiopathic orbital inflammation cases seen between 1971 and 1994. Clinical charts of the patients were evaluated retrospectively. Patient age ranged from 5 to 80 (mean 46.5) years. The follow-up period was between 6 to 172 (mean 42.8) months. Proptosis (82%), motility restriction (54%), visual acuity loss (38%) were the three more common presenting signs. The diagnosis was made by open biopsy in 96 cases and clinically in 36 cases. Radiologic patterns of involvement were available for 84 cases. Diffuse inflammatory disease (40 cases) was the most frequent radiologic pattern followed by myositis (21 cases) and dacryoadenitis (14 cases). Focal encapsulated mass (5 cases), Tolosa-Hunt syndrome (2 cases) perineuritis (1 case) and periscleritis (1 case) were the other types of orbital pseudotumors. The response to treatment was known for 60 of the 84 radiologically classified cases. High-dose oral corticosteroid treatment was successful in 35 out of 60 (58.3%) cases and radiotherapy in 9 out of 14 (64.3%) cases resistant to corticosteroids. One resistant case responded to cyclophosphamide and 2 cases with focal mass lesions were treated with orbitotomy. Four additional cases had spontaneous remission. Overall, 51 out of 64 patients (79.7%) had an eventual good outcome. In conclusion, we found open biopsy to be a safe and reliable procedure in orbital pseudotumors with the exclusion of acute phase cases. The success rate of treatment for this group of orbital disorders is high with the exception of certain disease patterns.