Idiopathic sclerosing orbital inflammation with intranasal extension

Abstract

Introduction: To report a case of idiopathic orbital sclerosing inflammation (ISOI) with intranasal extension.

Material and methods: The patient presented with a 6-month history of epiphora, upper eyelid swelling, ptosis and mild orbital pain. Ophthalmologic examination, CT, MRI and biopsy with surgical debulking were performed.

Results: MRI revealed a homogeneously enhancing diffuse right orbital mass in the inferonasal quadrant of the orbit, which extended to the nasal cavity up to inferior nasal concha, maxillary and ethmoid sinuses. Histological analysis showed dense collagenous tissue with sparse infiltration of mixed inflammatory cells. Inmunohistochemical analysis confirmed polyclonality. The diagnosis of idiopathic sclerosing orbital inflammation was made and 80 mg/day of oral prednisolone was prescribed. At last follow up, one year later, there was no clinical evidence of recurrent orbital disease.

Conclusion: ISOI can present with extraorbital extension. Corticosteroids are a reasonable first-line treatment, until the pathogenesis is better understood.