I feel it in my bones: A rare presentation of idiopathic sclerosing orbital inflammation with hyperostosis

摘要

Idiopathic sclerosing orbital inflammation (ISOI) is a unique clinicopathological subset of orbital inflammation that is poorly understood and is described in the literature only in case reports or small series. The incidence has been estimated at 5–7.8% of inflammatory orbital lesions, and it is characterized by an insidious, chronic and progressive fibrosing process that damages the orbit and surrounding structures.[1] ISOI has been associated with bone destruction; however, there are no reports with an association of hyperostosis. An 80-year-old African American man presented with orbital pain, diplopia and proptosis of the left eye for several months. On examination, visual acuity was 6/9 in both eyes. Extraocular movements were intact, and slit-lamp and fundus examinations were normal bilaterally. Exophthalmometry showed 2-mm left proptosis, with no other abnormalities except for age-related inferior orbital fat and lacrimal gland prolapse bilaterally (Fig.?1a). A computed tomography of the orbits showed an ill-defined left superolateral extraconal mass, along with thickening of the left greater wing of the sphenoid (Fig.?1b).