PURPOSE: To describe the clinical and histopathologic findings of eight cases of idiophatic sclerosing orbital inflammation. METHODS: A retrospective study from the file of the Ophthalmic Pathology Laboratory of the Hospital Universitário Professor Edgard Santos, Federal University of Bahia, during the period from 1974 to 2010. The clinical data, computed tomography and histopathologic sections obtained by biopsy of all cases were reviewed. RESULTS: From 10312 specimens submitted to the Ophthalmic Pathology Laboratory, eight (0,08%) had a final diagnosis of idiopathic sclerosing orbital inflammation. All cases were unilateral and in all of them vision acuity decreased or was lost despite of treatment, except for one case that remained with light perception as in the first examination. A predominance of diffuse orbital involvement was observed by computed tomography. Histopathologic evaluation disclosed dense fibrosis and collagen deposition with paucicellular inflammation in all cases. All patients were treated by immunosupression with steroids and one case had an association of anti-inflammatory radiotherapy. Two patients were also treated with ciclophosphamide. Partial orbital exenteration was necessary in two cases due to uncontrollable pain. CONCLUSION: Idiopathic sclerosing orbital inflammation remains as a disease with poor response to treatment ans a very bad visual prognosis.
Inflammation; Orbital diseases; Orbit; Prognosis; Diagnosis, differential
