Department Of Pathology and Ophthalmology
CT showing a mass involving extra orbital muscles and probably lacrimal gland
Had two decompression orbitectomies- Biopsies from the same follows
Biopsy showing areas of necrotizing granulomas, hematoxylin eosin 200x
Dense lymphoplasmacytic infiltrate and multinucleate giant cells, HE 400x
Concentric fibrosis around small vessels,HE 200x
Foci of vasculitis with inflammatory cells around and infiltrating small vessels, HE 200x
Diagnosis : Idiopathic orbital pseudotumour ( Idiopathic orbital inflammatory syndrome)
- Heterogeneous group of disorders characterised by orbital inflammation without any identifiable local or systemic causes.
- Classical cases show cellular inflammatory infiltrate with varying degrees of fibrosis
- Lesions with atypical histopathological patterns such as extensive sclerosis, vasculitis, granulomatous inflammation and tissue eosinophilia represent subgroups within the umbrella diagnosis of IOIS.
- Most require treatment with oral steroids and additional immunosuppressant drugs or radiotherapy.
- The clinical and pathological features do not correlate with treatment outcomes.
Br J Ophthalmol. 2007; 91(12): 1667–1670. Idiopathic orbital inflammatory syndrome: Clinical features and treatment outcomes