Clinical Characteristics of Sclerosing Pseudotumor of the Orbit

ABSTRACT

Sclerosing pseudotumor of the orbit is a unique clinico-pathological entity which is characterized by an insidious, chronic and progressive fibrosing process damaging orbital structures.As this entity forms immunologically mediated fibrosis even in early phase and frequently involves orbital apex and adjacent intracranial structures, it can cause severe ocular symptoms, visual impairments and even can threaten the life. We report clinical characteristics of nine biopsy-proven cases of sclerosing pseudotumor of the orbit.Five cases out of them have shown clinical improvement with combined therapeutic approach of surgical debulking, systemic steroids therapy, radiation therapy, and immunosuppressive therapy. We believe that prompt diagnosis through biopsy, early introduction of radiation therapy and immunosuppressive therapy are the most rational, currently available treatment for sclerosing pseudotumor of the orbit.