A 10-year-old male consulted our ophthalmology department due to bilateral blurred vision for one week. He had experienced a 1-week episode of flu-like symptoms, including fever and general malaise, 1 month before the ocular presentation. The best-corrected visual acuity (BCVA) was 0.025 (both eyes, BE). The intraocular pressure was within the normal range. Slit lamp examinations revealed 0.5+ anterior chamber cells (BE) with otherwise normal anterior segment structures. Indirect ophthalmoscopy revealed bilateral diffuse perivascular sheathing of the arteries and veins, Purtscher fleckens, and mild optic papillitis. Necrotizing retinitis was not observed (Figures 1A, B). OCT revealed profound macular edema with intraretinal fluid, subretinal fluid, and inner to middle retinal layer hyperreflectiveness (Figure 2A). Ultrawide-field fluorescein angiography revealed bilateral segmental phlebitis, occlusive arteritis, and retinal non-perfusion with macular ischemia. Due to previous systemic prodromes, a pediatrician was consulted for multidisciplinary care.
Physical examination results aside from the ocular findings were unremarkable. Abdominal ultrasonography revealed mild hepatomegaly. Initial hematology and serology investigations revealed an elevated erythrocyte sedimentation rate at 58 mm/h, alanine aminotransferase at 160 U/L, and positive antinuclear antibody (1:640). A systemic infection survey targeting retinal vasculitis and hepatitis revealed negative results for HSV-1, CMV, VZV, EBV, HTLV, HBV, HCV, Quantiferon-TB Gold, syphilis, and toxoplasma serology. However, a highly positive titer of HSV 2 IgM (5.10) was noted. An aqueous tap was not performed because of its invasiveness considering the age of the patient. In addition, the associated autoimmune profiles were positive for anti-smooth muscle antibody (Ab), but negative for anti-dsDNA, anti-mitochondria Ab, or anti-liver kidney microsome Ab (anti-LKM Ab). Therefore, this patient was diagnosed with post-HSV-2 infection-related FBA, PuR, and concurrent autoimmune hepatitis. The patient was initially prescribed prednisolone (40 mg/day), valaciclovir (500 mg every 12 h), and acetylsalicylic acid (100 mg every other day) (Figure 3).
Although the bilateral FBA and macular edema gradually alleviated (Figures 1C, 2B) after several weeks of medical treatment, the BCVA improvement was minimal (0.05, right eye; counting finger, left eye) (Table 1). Persistent macular ischemia, with clinical presentations of profound PuR and compatible ischemic hyperreflective changes on OCT (Figures 1C, 2C), was identified to be the main cause of poor vision. Therefore, HBOT was initiated with 2.0 atmosphere absolute (ATA) for 75 min per session, 1 session per day, 5 days per week. After a total of 39 sessions of HBOT, the visual acuity steadily improved to 0.16 (RE) and 0.2 (LE), with gradual resolution of Purtscher fleckens (Table 1). Subsequent immunosuppressive medications, including methotrexate (10 mg/week) and cyclosporine (50 mg/day), were also initiated for FBA treatment and corticosteroid tapering (Figure 3). Follow-up fundus photograph and fluorescein angiography performed 21 weeks after the initial visit and 8 weeks following HBOT revealed almost completely resolution of FBA and decrease in size of PuR, but extensive macular and mid-peripheral retinal ischemia (Figures 1D, E). Therefore, retinal photocoagulation on the non-perfusion area was performed 13 weeks after the initiation of HBOT to prevent further neovascular complications, such as vitreous hemorrhage, tractional retinal detachment, and neovascular glaucoma (Figure 1F).