A 31-year-old healthy female with a history of myopia (-6 diopter) and prior Laser Assisted in Situ Keratomileusis surgery, was noted to have a torpedo maculopathy temporal parafoveally in the right eye (Fig. 1a).
Fig. 1Fundus photography of both eyes. a: Fundus photography 5 years ago before the Laser Assisted in Situ Keratomileusis (LASIK), showing a torpedo maculopathy adjacent to the right fovea. b: Fundus photography of right eye at the first visit. Greyish-white dots are noted around the torpedo maculopathy area (white arrowhead). c: Fundus photography of left eye at the first visit
She noticed a decline in best corrected visual acuity (BCVA), intermittent flashes and a new focal central scotoma in the right eye three days following her second recombinant mRNA vaccine (Moderna). Three months later, she was referred for medical aid. No other ocular symptoms such as tearing, pain, redness, or eye movement problem were noted. The patient was a non-smoker and had no allergy history. She denied having any systemic diseases, including diabetes mellitus, hypertension and cardiovascular diseases. No night sweats, cough, headache, skin rashes, prior viral prodromes, or family history of autoimmune diseases was reported.
On examination, her BCVA was hand motion in the right eye and 30/50 in the left eye. Fundus examination revealed a few greyish-white dots confluent at the level of retinal pigment epithelium (RPE) around the torpedo maculopathy in the right eye (Fig. 1b), and normal in the left eye (Fig. 1c). The spectral-domain optical coherence tomography (SD-OCT) showed normal retinal layers of the left eye (Fig. 2a-c), whilst the right eye illustrated inner and outer segment (IS/OS) line disruption with hyperreflective deposits in the outer nuclear layer (ONL), hazy and disrupted external limiting membrane (ELM), and undetectable interdigitation zone (IDZ) (Fig. 2d-f). Fluorescein angiography (FAG) showed RPE staining with subtle mid-phase leakage at torpedo maculopathy site in the right eye (Fig. 3a), and it was surrounded by hypo-fluorescent spots in late phase on indocyanine green angiography (ICGA) exam (Fig. 3b). Fundus autofluorescence (FAF) illustrated speckled hyper-AF lesions around the torpedo maculopathy in the right eye (Fig. 3c) and normal in the left (Fig. 3d).
Fig. 2Infrared (IR) fundus image and Spectral-domain optical coherence tomographic (SD-OCT) exam of left eye (a-c) and right eye (d-f). a, d: IR fundus images at the first visit. b: Magnified view of the area in the image of (c) outlined by yellowish dashed line. c.f: Spectral-domain optical coherence tomographic (SD-OCT) exam in the left (c) and right eye (f) at the first visit. e: Magnified view of the area in the image of (f) outlined by yellowish dashed line, revealing columnar inflammatory deposits in outer retinal area (red arrow), fuzzy and disrupted External limiting membrane (ELM, yellow arrow), and disrupted ellipsoid zone (EZ) and interdigitation zone (IDZ), blue arrow
Fig. 3Fundus fluorescein angiography (FAG), indocyanine green angiography (ICGA) and fundus autofluorescence (FAF) images at initial visit (a, b, c, d) and at 7-month follow-up (e.f). a, f: FAG at 3 min illustrated a window defect and subtle leakage at the old macular scar (a) which resolved 7 months later (f). b: ICGA at 15 min at the first visit showed hypocyanine spots around torpedo maculopathy site. c: FAF image of the right eye at the first visit (c), and at seven months later (e). The hypo-autofluorescence lesions at torpedo maculopathy site were surrounded by blue arrowhead. The speckled hyper-autofluorescence around the torpedo maculopathy area (surrounded by yellow arrowhead) shrunk 7 months later (e). However, the hypo-AF areas were slightly enlarged (blue arrowhead). d: FAF image of the left eye at the first visit (d)
Laboratory surveys including hematology test, biochemistry examination, autoimmune workup (ANA and rheumatic factor), QuantiFERON gold test and rapid plasma regain (RPR), ESR and CRP were within normal range. Thus, syphilis, Mycobacterium tuberculosis uveitis, and autoimmune retinopathy were ruled out. Although the image didn’t present the typical trizonal appearance of AZOOR, there were no wreath-like hyperfluorescence dots characteristic of MEWDS. In addition, there were also no punched-out multifocal choroiditis lesions presented on ICGA images. The diagnosis of AZOOR complex was established by exclusion. Initially, we kept the patient under observation. However, after three months of observation, the patient reported a further decline in visual acuity. Thus, she was treated with 8-month of oral prednisolone and mycophenolate mofetil. Her BCVA recovered to 40/50 in the right eye. The speckled hyper-AF area was shrunk by 79%, which was calculated with ImageJ (Fig. 2e). FAG at 3 min showed resolved window defect (Fig. 3f). On FAF images, the hyper-AF lesions faded, but the hypo-AF spots persisted with slightly enlarged in size and encroaching fovea in the right eye (Fig. 2d). The hyper-reflective dots in ONL resolved. The ELM and IDZ were partially recovered, but slightly hazy (Fig. 4a-c).
Fig. 4Infrared (IR) fundus image and Spectral-domain optical coherence tomographic (SD-OCT) exam (a, b, c) in the right eye at seven months of follow-up and Humphrey visual field 30 − 2 examination (d, e). a: Infrared (IR) fundus image at seven months later. b: Magnified view of the area in the image of (c) outlined by yellowish dashed line. At 7 months of follow-up, the ELM was continuous and prominent, the EZ line was slightly disrupted (blue arrow), and the IDZ line was still not prominent. c: SD-OCT image at seven months later. d: Humphrey visual field 30 − 2 examination at the first visit. e: Humphrey visual field 30 − 2 examination at 7 months later. The mean visual field loss improved from − 4.42 ± 5.87 dB at initial visits (d) to -3.38 ± 2.36 dB at the final visit (e). The nasal superior visual defect was improved at 7 months later
Despite BCVA recovery, the patient described a persistent central scotoma in her right eye (Fig. 4d-e). The initial visual field defect demonstrated on Humphrey visual field (HVF) 30 − 2 test was presented over the superior nasal quadrant with central scotoma in the right eye (Fig. 4d). After treatment, the visual field defect was improved but some central VF defect remained (Fig. 4e), which was compatible to the FAF findings (Fig. 3e). The mean visual field loss improved from − 4.42 ± 5.87 dB at the initial visit to -3.38 ± 2.36 dB at the final visit.
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