A 23-year-old Indian female presented to a tertiary institute of Northeast India with the chief complaints of dimness of vision, pain and redness in the right eye (OD) for 3 months. The patient was previously treated elsewhere with two posterior sub-Tenon injections of triamcinolone acetonide given 6 weeks apart. She was also on topical steroids and cycloplegic. She did not report any benefit from the previous treatment. There was no history of trauma to the eye or elsewhere. Other medical, surgical, and family histories were unremarkable.\nOn examination, ocular alignment was normal with a visual acuity of counting finger at 3 m in the OD. There was minimal sclerouveitis noted in the inferior-temporal quadrant of OD. On slit-lamp examination, OD showed 360° posterior synechiae with anterior chamber flare and cells (+++) with 1 mm hypopyon []. There was a suspected mass in the inferior-temporal quadrant behind the iris, pushing it anteriorly. The left eye examination was unremarkable.\nB-scan ultrasound (B-scan USG) of OD was suggestive of ciliary body mass with adjoining exudative retinal detachment (ERD). USG bio-microscopy findings were consistent with the findings of B-scan USG. The patient was advised with complete blood count with peripheral blood smear which was within normal limit. Syphilis screening (venereal disease research laboratory) was nonreactive with normal thyroid function test and negative Mantoux test (5TU) reading. Based on the clinical examination and baseline investigations, we came to the differential diagnosis of ciliary body melanoma or similar tumors. Magnetic resonance imaging suggested the possibility of ciliary body melanoma in OD [Fig. and ]. After obtaining informed consent, enucleation was carried out in OD, and eyeball specimen was sent to ocular pathology laboratory for histopathological analysis. In grossing, there was a grayish-white-colored tumor seen in the inferior-temporal part of the ciliary body measuring (10.99 × 7.98) mm. Adjoining the grayish-white ciliary body mass, there was an ERD noted [].\nMicroscopic description of enucleated eyeball showed peripheral corneal vascularization. Few inflammatory cells were seen in the anterior chamber []. Part of the iris tissue showed inflammation. An eosinophilic mass was seen in the ciliary body with diffuse plasmacytoid cells, with reactive lymphoid follicle and other inflammatory cells []. Episcleral and scleral tissue were inflamed and infiltrated by various inflammatory cells [Fig. and ]. Occasional vascularization of the sclera was noted []. Cut end of the optic nerve did not show any tumor involvement. Immunohistochemistry (IHC) was advised to explore the possibility of various inflammatory lesions. The steps for IHC were carried by kit methods available commercially.\nIHC for both Kappa and Lambda was positive. Cluster differentiation (CD20, CD3, and CD45) was focal-positive. CD138 was positive in the specimen and HMB45 was negative. IHC for IgG4 was positive in occasional cells which also expressed CD138 (plasma cells). All positive controls showed appropriate positive immunostaining. Negative control slide did not show immunostaining. IgG4-positive plasma cells were seen in high-power field in the documented slides. Nearly, 30% of all the CD138 positive plasma cells were found to be IgG4-positive [].\nProtein chemistry revealed hypergammaglobulinemia with higher levels of total protein and gamma fraction. No myeloma band or Bence Jones protein was detected []. Serum IgG4 levels were also elevated. A hematologist was consulted, and bone marrow examination revealed mild anemia, for which she was given supportive treatment. In the postoperative period, the patient was given a course of oral steroids. On follow-up at 6 weeks, she was given prosthesis in OD, and the patient was doing well till her last review.
A 43-year-old woman with a history of recurrent headaches and associated vision disturbances for the past 4 years presented to our emergency department with a 4-week history of progressive left-eye pain that radiated to the back of her head, swelling around the left eye, double vision, and a unilateral left-sided headache. There were no factors that worsened or improved her symptoms. She first developed this 4 years ago while living in another state. At that time, she was admitted to a hospital, given intravenous (IV) then oral steroids which helped suppress the pain and swelling, but she did not recall a specific diagnosis. Since that time, she has had three separate flare-ups, each resolving after steroid treatment, with the current episode being the most recent and severe. On physical exam she had left periorbital swelling and edema, 20/25 vision in her right eye and 20/30 in her left eye, and extra ocular muscles were intact, but she reported left-eye pain when at extreme medial and lateral gaze. She did not have temporal lobe tenderness on examination. There was normal muscle tone, range of motion, and sensation in all four extremities bilaterally. Laboratory results at admission were as follows: erythrocyte sedimentation rate (ESR) of 30 mm/h and normal complete blood count, renal function, hepatic function, and transaminases. Computed tomography (CT) of the head without contrast, CT of the orbits with contrast (), CT angiogram of the head, and an electrocardiogram (EKG) were performed. All were normal aside from the CT angiogram of the head which suggested a varix versus aneurysm involving the anterior cerebral arteries in the region of the inferior sagittal sinus (). Ophthalmology was consulted, excluded acute angle closure glaucoma, retinal disorders, diagnosed her with recurrent idiopathic orbital inflammation syndrome and possibly Tolosa-Hunt syndrome, and recommended treatment with methylprednisolone 80 mg IV daily for 3 days. Within 24 h she noted a 50% reduction in pain and had complete resolution of her symptoms within 48 h. Magnetic resonance imaging (MRI) of the brain with and without contrast conducted the next day was normal and did not confirm the presence of a vascular anomaly suggested by the CT scan. At this point she was discharged on 60 mg oral prednisone daily with a taper over the course of 2 weeks. One week after discharge she was seen at her ophthalmologist’s office and remained asymptomatic.