A 45-year-old Greek female patient presented in the 2nd Department of Ophthalmology, Aristotle University of Thessaloniki, Papageorgiou General Hospital, Thessaloniki, Greece, with a 3-day history of bilateral blurry vision and mild ocular discomfort. Past medical history included a hysterectomy for polycystic ovary syndrome and a recent presumed diagnosis of intracranial hypertension, set on the grounds of recurrent headaches and borderline raised intracranial pressure on lumbar puncture. No recent travel abroad or relevant family history was reported. Upon presentation, best-corrected visual acuity (BCVA) was 6/6 bilaterally. Anterior segment examination revealed anterior chamber granulomatous inflammation of 2+ cells with faint flare (SUN working group grading system) bilaterally, mutton-fat keratic precipitates, visible iris Koeppe nodules, and posterior synechiae in 2 clock-h RE. Intraocular pressure was 14 mm Hg bilaterally. Dilated fundus examination of RE revealed moderate vitritis of 2+ vitreous haze, a RE peripheral solitary granuloma at 6 o'clock, an optic nerve head granuloma, and choroidal neovascular membrane (PCNM) in the LE with hemorrhage localized in the papillomacular bundle, extending into the macula. Fluorescein angiography and optical coherence tomography (OCT) confirmed biomicroscopy findings; RE: hot disc, LE early staining peripapillary and in the papillomacular bundle (Fig. 1a-d). Laboratory testing (full blood count, clotting, liver screen, viral screen, autoimmune screen, and QuantiferonTB gold) revealed no significant findings with the exception of angiotensin converting enzyme (ACE:148). Thoracic high-resolution computerized tomography revealed irregular nodular thickening predominantly in the middle and upper lung zones bilaterally with no lymphadenopathy, while MRI scans revealed no findings. Blind conjunctival biopsy confirmed sarcoidosis as systemic diagnosis. The patient was followed up by dilated fundoscopy, fluorescein angiography, OCT, and OCT angiography. Unfortunately, indocyanine green angiography was not available in our department during the follow-up period for this patient.
Within 4 weeks, PCNM showed rapid enlargement (BCVA: 6/60) with foveal involvement. The patient was managed with oral steroids (prednisolone 0.5 mg/kg), topical steroid eye drops (dexamethasone 0.1%), and cycloplegia in addition to 3 aflibercept injections that were administered in monthly intervals resulting in significant neovascular membrane regression and VA improvement (Fig. 1e-g).
Two more aflibercept injections in monthly intervals followed, due to rapid NV recurrence (Fig. 1h-j). Following the 2nd aflibercept injection, oral steroids were tapered by 5 mg every 2 weeks as per rheumatologist's advice while oral methotrexate was introduced (starting at 7.5 mg/week, increased by 5 mg increments every 2 weeks until a dose of 20 mg/week was reached) with regular monitoring for hepatic and hematologic toxicity. Throughout this 39-month period after oral steroid tapering, there were no significant systemic occurrences that required treatment.
Six months after the onset of treatment and 5 injections, lack of aflibercept availability prompted the use of ranibizumab: 5 injections were administered over 6 months with 5-8-week intervals, each resulting in total NV regression and 6/6 VA. Following a 15-month period of stability, NV recurrence coupled with VA deterioration was observed; over the subsequent 8 months, 4 ranibizumab injections were administered. Finally, the following 16 months were characterized by lack of NV recurrence and 6/6 VA. Overall, during a 50-month follow-up period up to April 2020, on every occasion, an anti-VEGF injection was followed by total NV regression, as depicted by OCT and OCT angiography (Fig. 2) and 6/6 VA.