Idiopathic orbital inflammation with extraorbital extension: case series and review

Idiopathic orbital inflammation (IOI) is a well-recognised pathological process usually confined to the orbit. It is an orbital mass lesion characterised by infiltration of soft tissues by inflammatory cells and fibrous tissue. It is essentially a diagnosis of exclusion. Extraorbital extension (EOE) is rare and, to the best of the authors' knowledge, inclusive of our series, there have been 22 cases of IOI with EOE reported in the literature. We describe four patients with IOI with EOE-their presentation, histopathological findings and management strategies. Histopathology of three patients revealed sclerosing and one had a nonspecific variant of IOI. In our series, all four patients required additional immunosuppression. Hence, corticosteroid therapy often needs to be supplemented by radiotherapy and/or immunosuppressive therapy.