Orbital disorder revealing idiopathic inflammatory pseudotumor

Author: amel ben abid (Tunisia)

Co-authors: mona rekik, takwa sammouda, salma gargouri, sonda kammoun, amira trigui

Purpose

to report a case of exophthalmos with painful ophtalmoplegia revealing idiopathic inflammatory pseudotumor.

Setting/Venue

Department of Ophthalmology, Habib Bourguiba University Hospital, Faculty of Medicine, University of Sfax, Tunisia.

Methods

case report

Results

A 45-year-old female patient presented with complains of rapidly progressive loss of vision in the left eye. Her personal medical history included diabetes treated with oral hypoglycemic agents. Examination revealed left ophthalmoplegia, exophthalmos with an afferent pupillary defect. Visual acuity was limited to count of fingers in the left eye, 10/10 in the right eye. The fundus examination showed venous tortuosity in the left eye with normal optic disc. The remainder of the neurologic examination was unremarkable. Brain magnetic resonance imaging revealed left intra-orbital mass that engages the optic nerve suggestive of orbital inflammatory pseudo tumor. The immunological and infectious assessments were negative except for a positive intradermal tuberculin test at 18mm. A biopsy was performed. The histopathological examination of the biopsy showed a moderate chronic inflammatory changes in the suborbital fat anteriorly and posteriorly without tuberculoid granuloma. The etiologic survey was negative, then the diagnosis of idiopathic orbital inflammatory pseudo tumor was kept. The patient received corticosteroids and empirical anti-tubercular therapy. Outcome was favorable with complete regression of oculomotor disorders, exophthalmos after few days of treatment but she kept as sequels a low visual acuity

Conlusions

Idiopathic inflammatory pseudo tumor is a benign inflammatory condition of the orbit without identifiable local or systemic causes. Consider idiopathic orbital inflammatory pseudotumor in cases of painful ophthalmoplegia; the disease is typically unilateral and may be recurrent. The presentation may vary according to the specific location and degree of inflammation, fibrosis, and mass effect. Patients may present with proptosis, diplopia, conjunctival chemosis, visual disability, and restriction of extra ocular muscle movement. He may also have associated optic neuropathy. The differential diagnosis includes orbital cellulitis, thyroid ophthalmopathy, sarcoidosis, lymphoid tumor, lymphangioma and metastatic carcinoma.

Financial Disclosure

no financial relations