To report an unusual manifestation of the rare condition, idiopathic sclerosing orbital inflammation.

A 66 year old female presented with right medial canthal inflammation preceded by epiphora. Oral co-amoxiclav for presumed acute dacryocystitis was unsuccessful. Worsening symptoms plus proptosis prompted CT orbits and sinuses which demonstrated a medial anterior orbital mass extending into the nasolacrimal duct, nasal cavity and frontal, ethmoidal and sphenoid sinuses.

Anterior orbital biopsy revealed a firm white lesion over the lacrimal fossa extending superiorly and inferiorly. Histology showed fulminant sclerosing orbital inflammation. No evidence of malignancy , micro-organisms, vasculitis or IgG4 was found. Biopsies via a right endoscopic ethmoidectomy, sphenoidectomy and middle meatal antrostomy by Otorhinolaryngology confirmed the same histological diagnosis with the sphenoid site demonstrating a separate area of the same disease. A slow reducing course of daily high-dose oral prednisolone provided improvement until reduced to 20mg with resultant increasing proptosis. Cyclophosphamide infusions with steroid cover by Rheumatology gave good effect.

Idiopathic sclerosing orbital inflammation usually presents in the 4th decade with proptosis. The unusual anterior location with extra-orbital components in our case caused a presentation more typical of acute dacryocystitis. There is currently no formal consensus on best management for this rare condition. Multidisciplinary management to tailor investigation and treatment is therefore essential to promote patient recovery.