Idiopathic orbital inflammatory pseudotumor (IOIP) accompanied with panuveitis: a case report and review of the literature

Idiopathic orbital inflammatory pseudotumors (IOIP) are non-granulomatous inflammatory process of the orbit for unknown etiology, and are associated with diagnostic challenge and high recurrence rate. In this report, we present an unusual case of 10IP initially accompanied with panuveitis. A 57-year-old female presented with a two-month history of decreased vision of the right eye for no obvious reason at her first visit. She had intermittent attacks of ophthalmalgia, photophobia, foreign body sensation and headache. Clinical examination of the right eye revealed a diminished visual acuity of the right eye (VOD), a moderately deep anterior chamber, opisthosynechia at Pars iridica, slight opacification and pigmentation of crystalline lens, and vitreous opacification. Hematological investigations revealed an increased number of leukocyte, cell -hemoglobin concentration. Immunologic test showed higher blood sedimentation and complement C4. She was initially diagnosed with panuveitis and improved after the systemic administration of Methylprednisolone Na succinate by intravenous drip (0.75 g/d) for 5 d and local application of I-Prednicet, atropine ophthalmic gel and so on. However, she had twice relapsed thereafter, and further diagnosis of 10IP was made. Finally she was given enucleation of eyeball and orbital exenteration. The diagnosis of 10IP should be strongly considered in the presence of non-granulomatous inflammatory process and a suggestive MRI image. Treatment of 10IP accompanied with panuveitis should be started immediately to improve visual acuity of the eye, reduce inflammatory symptoms and preserve eyeball functions. The preferred treatment of choice was systemic treatment combined with local hormone therapy.