A 39-year-old Caucasian man presented to the department of ophthalmology with severe loss of vision in the left eye (LE) over the last month. His past ocular history was relevant for an accidental penetrating injury to the right eye (RE) 9 years before. He was an intravenous drug addict diagnosed with HIV and chronic hepatitis C 20 years previously. He was on treatment with the highly active antiretroviral therapy (HAART) combination emtricitabine, tenofovir, and nevirapine. Unfortunately, previous CD4 counts and medical records could not be obtained.
On examination, visual acuity (VA) was no light perception in the RE and hand movements in the LE. The RE was in phthisis bulbi. LE showed moderate ciliary injection and mutton fat keratic precipitates. There were +3 cells and +3 flare in the anterior chamber with multiple posterior synechiae. There was severe vitritis with no fundus view. The patient was admitted for further work-up and treatment. Blood samples analyses taken upon admission showed a CD4+ T lymphocyte count of 549 with undetectable HIV viral load. Our working differential diagnoses were SO, acute retinal necrosis, toxoplasma panuveitis, syphilitic or tuberculous panveitis, sarcoidosis, endogenous endophthalmitis and less likely, immune reconstitution inflammatory syndrome (IRIS). Chest x-ray and head CT scan were reported as normal. Treatment was initiated with topical dexamethasone 0.1 % and atropine 1 %. Systemic treatment was started with oral valacyclovir, intravenous (IV) 1 g methylprednisolone daily for 3 days, followed by oral prednisone 1 mg/kg daily. Syphilis and toxoplasma serology were negative. QuantiFERON®-TB Gold (Cellestis Ltd. Carnegie, Victoria, Australia) was negative. Polymerase chain reaction (PCR) of aqueous and vitreous taps for herpes simplex virus 1 and 2 and varicella zoster virus was negative. PCR for CMV was not done. In view of the blood analyses and PCR results, valacyclovir was discontinued.
Enucleation of the phthisical RE was performed. Microscopically, the choroid presented foci of lymphocytes and plasma cells as well as epithelioid granulomata with some multinucleated giant cells (Fig. 1a–c). These multinucleated giant cells contained phagocytosed melanin granules (Fig. 1d). There was fibrosis between the choroid and retina with large cholesterol deposition and groups of epithelioid cells interpreted as Dalen-Fuchs nodules. After 3 months of therapy with oral prednisone, the alkylating agent cyclophosphamide at a dose of 25 mg daily was added as steroid-sparing medication.
As the inflammation subsided, fundus examination of the LE showed moderate vitritis with widespread retinal pigment epithelium patches of depigmentation and a small intraretinal hemorrhage along the supero-temporal vascular arcade. There were no signs of vasculitis (Fig. 2a, b). Fluorescein angiography (FA) revealed multiple diffuse hypofluorescent choroidal lesions (Dalen-Fuchs nodules) in early frames that became hyperfluorescent in late phases (Fig. 2c, d). Indocyanine green angiography (ICG) depicted multiple hypofluorescent choroidal lesions in early and late frames widespread over the fundus (Fig. 2e, f).
At 15-month follow-up, VA was 20/200 with ongoing recalcitrant intraocular inflammation. Cyclophosphamide was discontinued, and a new 3-day pulse of 1 g methylprednisolone IV was administered followed by oral prednisone 1 mg/kg daily and cyclosporine A at a dose of 7 mg/kg/daily.