Histocompatibility Leukocyte Antigen-A29-Associated Retinal Vasculitis without Choroidal Lesions: A Report of 4 Cases.

This study describes a cohort of patients presenting with histocompatibility leukocyte antigen (HLA)-A29-associated retinal vasculitis without choroidal lesions that may share clinical features with birdshot retinochoroiditis. The methods include a retrospective chart review of patients presenting with HLA-A29-associated retinal vasculitis without choroidal lesions. The data on the patients were entered retrospectively into a new database and analyzed. Four patients who had HLA-A29-associated retinal vasculitis without choroidal lesions were identified. The median age at presentation was 40 years (range: 14-71); 75% were female. At presentation, all four patients had a visual acuity of 20/50 or better in both eyes. All the eyes had mild vitritis, three eyes (37.5%) had cystoid macular edema, and two eyes (25%) had optic disc edema. All the patients required treatment with systemic steroids and immunosuppressive therapy. HLA-A29-associated retinal vasculitis without choroidal lesions appears to share many clinical features with birdshot chorioretinitis, including the need for systemic immunosuppressive therapy. Whether this entity represents an early form of birdshot retinochoroiditis or a more localized variant of the disease is a topic for additional studies.

Bilateral Acute Iris Transillumination Associated with Moxifloxacin Antibiotic Use.

PURPOSE: To describe the clinical and demographic characteristics and associated factors leading to bilateral acute iris transillumination (BAIT) syndrome. METHODS: A retrospective review of patients with BAIT syndrome was performed. RESULTS: Thirty-five patients with a diagnosis of BAIT were identified. The median age at presentation was 53 years; 80% of the patients were female. Twenty-six patients (74%) had recent histories of systemic antibiotic treatment. Of those with such a history, 24 patients (92%) had been receiving moxifloxacin. Two patients within our cohort were prescribed moxifloxacin prophylactically prior to a systemic surgical procedure and had no evidence of systemic illness or recent viral illness. CONCLUSIONS: Our data support the notion that moxifloxacin might be associated with the onset of BAIT syndrome. Notably, within our cohort, two patients received moxifloxacin as surgical prophylaxis and subsequently developed BAIT syndrome. This could suggest a potential association between moxifloxacin and the onset of BAIT, though further studies are needed to confirm this finding.

Atypical Unilateral Birdshot Chorioretinitis in a Hispanic Female.

A 62-year-old female patient was evaluated for gradual vision loss, floaters, and photopsia in her left eye. A left fundus examination revealed vitreous cells, hypopigmented lesions, and retinal vasculitis, and a workup revealed a positive HLA-A29 serology, all of which findings were consistent with birdshot chorioretinopathy. The patient was treated with oral prednisone and mycophenolate mofetil, which resulted in the adequate control of her uveitis. Even though unilateral cases are exceedingly rare and do not meet the established research criteria, this case highlights the importance of not minimizing the significance of birdshot lesions in the differential of patients with unilateral multifocal chorioretinitis.

Systemic Disease Associations in a Cohort of Hispanic Patients with Scleritis.

(1) Purpose: A patient with scleritis may have an associated systemic disease, which is often autoimmunological and seldom infectious in origin. The data regarding such associations in Hispanic populations are scarce. Therefore, we evaluated the clinical characteristics and systemic-disease associations of a cohort of Hispanic patients with scleritis. (2) Methods: A retrospective review of the medical records (January 1990-July 2021) of two private uveitis practices in Puerto Rico was performed. Clinical characteristics and systemic-disease associations observed either at presentation or diagnosed as a consequence of the initial workup were recorded. (3) Results: A total of 178 eyes of 141 patients diagnosed with scleritis were identified. An associated autoimmune disease was present in 33.3% of the patients (rheumatoid arthritis, 22.7%; Sjögren's syndrome, 3.5%; relapsing polychondritis, 2.8%; sarcoidosis, 1.4%; systemic lupus erythematosus, 1.4%; and systemic vasculitis, 0.7%). An associated infectious disease was present in 5.7% of the patients (2.13%, syphilis; 1.41%, herpes simplex; 1.14%, herpes zoster; and 0.71%, Lyme disease). One patient had all-trans retinoic-acid-associated scleritis. Statistical analysis revealed that patients with nodular anterior scleritis were less likely to have an associated immune-mediated disease (OR: 0.21; p = 0.011). (4) Conclusion: Rheumatoid arthritis was the most common systemic autoimmune disease association, while syphilis was the most common infectious disease associated with scleritis patients. Our study suggests that patients with nodular scleritis have a lower risk of having an associated immune-mediated disease.

Aflibercept as a Treatment for Secondary Central Serous Chorioretinopathy in a Patient With Myasthenia Gravis.

We report on a case of central serous chorioretinopathy (CSCR) secondary to chronic steroid use that showed sustained improvement when treated with an aflibercept intravitreal injection. A 44-year-old woman presented with decreased visual acuity of the left eye (OS). The patient had a recent history of myasthenia gravis and was being treated with systemic corticosteroids and immunosuppressants. At presentation, her visual acuity was 20/80 OS; an examination (using fluorescein angiography) of the left fundus revealed a serous retinal detachment of the posterior pole that extended to the mid-periphery and multiple areas of leakage, which findings were consistent with CSCR. The patient also had a history of unresolved strabismic amblyopia in her right eye. The patient's CSCR was managed with one injection of intravitreal aflibercept (2 mg/0.05 mL). One month following treatment, her visual acuity improved to 20/20 OS, and the serous retinal detachment had resolved. Ten months following treatment, an examination revealed a sustained improvement, with a visual acuity of 20/20 OS. Concomitantly, the patient's amblyopic eye revealed an improved visual acuity of 20/20. Our case suggests that some cases of secondary CSCR may respond to treatment with intravitreal aflibercept. This case also suggests that the CSCR imposed a unique form of occlusion therapy that helped improve the amblyopia of the contralateral eye in this adult patient.

Unilateral Focal Retinitis as an Initial Manifestation of Cat-Scratch Disease.

We report on a case of focal retinitis as the initial manifestation of cat-scratch disease. A 56-year-old Hispanic woman presented for a routine follow-up examination. A fundus examination of the right eye revealed a white retinal lesion along the inferotemporal artery; this lesion was noted to have progressed after one week of observation. On further inquiry, the patient reported she had seven cats at home, some of which were less than six months old and had recently scratched her. She received empirical treatment for focal retinitis with azithromycin (500 mg daily) and valacyclovir (1 g three times daily), which would cover the most common parasitic, viral, and bacterial etiologies. She was lost to a follow-up examination. However, she continued the same dose of antibiotic and antiviral treatment. Upon her eventual follow-up, three months later, it was noted that the lesion had resolved. The initial work-up revealed that she was positive for Bartonella henselae IgM (1:20) and IgG (1:512), as well as for B. quintana IgG (1:256); however, she was negative for B. quintana IgM. At a four-month follow-up appointment, the B. henselae IgM was negative, the IgG had decreased from 1:512 to 1:64, and the B. quintana antibody test was negative for IgM and IgG, all of which are consistent with an adequately treated case of cat-scratch disease. Focal retinitis can be a rare initial manifestation of cat-scratch disease, which should be considered part of the differential diagnosis in cases of focal retinitis, especially in patients with a history of close contact with young cats. Additionally, oral azithromycin may be considered as a treatment for some cases of cat-scratch-associated focal retinitis.

Bilateral Granulomatous Iridocyclitis Associated with Early-Onset Juvenile Psoriatic Arthritis.

Purpose: The purpose of this study is to report on a case of bilateral granulomatous iridocyclitis in a patient with early-onset juvenile psoriatic arthritis (JPsA). Methods: The method used is an observational case report. Observations. A 3-year-old Hispanic girl was sent to our uveitis service for further evaluation of her granulomatous uveitis. The initial ophthalmologic examination revealed bilateral band keratopathy, large mutton-fat keratic precipitates, multiple posterior synechiae, and 4+ anterior chamber cells. The physical exam was notable for left knee edema and right axillary rash. Laboratory testing was remarkable for an erythrocyte sedimentation rate of 80 mm/h, positive antinuclear antibodies (1 : 1, 280), and negative human leukocyte antigen B27. A cutaneous biopsy was obtained, which confirmed the diagnosis of a psoriatic rash. Treatment with oral prednisolone and topical prednisolone acetate with atropine sulfate resulted in the complete resolution of the uveitis. Conclusion and Importance. Bilateral granulomatous iridocyclitis may be a rare presentation of ocular involvement in patients with early-onset JPsA.

Characteristics Upon Presentation of Patients with Type 2 Macular Telangiectasia in Puerto Rico.

OBJECTIVE: To describe the systemic disease associations and clinical features upon initial presentation of a cohort of patients with type 2 macular telangiectasia who live in Puerto Rico. METHODS: A retrospective review of patients with macular telangiectasia was performed in 4 private retina practices in Puerto Rico. The demographic and clinical characteristics were recorded. RESULTS: Twenty-one patients who were diagnosed with macular telangiectasia were included in the analysis. The median age of presentation was 62 years; 86% were female, and all patients were Hispanics. The median visual acuity at presentation was 20/50. A prior medical diagnosis of type II diabetes mellitus was found in 15 (71.4%) patients, essential hypertension in 12 (57.1%), and dyslipidemia in 9 (42.9%). All patients had bilateral disease. The most common ocular findings were the presence of right-angle vessels in 32 (76.2%) eyes and angiographic hyperfluorescence temporal to the fovea, found in 22 (52.4%) of the affected eyes. One eye had evidence of choroidal neovascularization. CONCLUSION: Our cohort showed a higher prevalence of type 2 diabetes in patients with type 2 macular telangiectasia than in other cohorts. It also supports the findings of other studies showing that macular telangiectasia patients are more likely to have type 2 diabetes and hyperlipidemia. However, the increased prevalence of diabetes and hyperlipidemia may be due to selection bias, and further studies are needed to assess the significance of these findings.

Pars Plana Vitrectomy With Internal Limiting Membrane Peeling for the Treatment of Cystoid Macular Edema in a Patient With Syphilitic Uveitis and HIV Infection.

We report a case of cystoid macular edema (CME) secondary to syphilitic uveitis that was successfully treated with pars plana vitrectomy with internal limiting membrane peeling. A 37-year-old male with a history of HIV developed a CME secondary to syphilitic panuveitis. His uveitis resolved following treatment with intravenous penicillin, yet his CME persisted and was refractory to four posterior sub-tenon triamcinolone acetonide injections. A pars plana vitrectomy with internal limiting membrane peeling was performed, resulting in lasting resolution of the CME and the improvement of his visual acuity at the two-month follow-up visit. Pars plana vitrectomy with internal limiting membrane peeling may be a viable alternative for the treatment of CME in patients with syphilitic uveitis. In particular, it may serve as a viable alternative for the treatment of CME in patients with a history of infectious uveitis or other comorbidities, such as HIV infection.

Variables in the ACBD5 Gene Leading to Distinct Phenotypes: A Case Report.

We report the cases of a father and his daughter, the former diagnosed with retinitis pigmentosa (RP) and the latter with early foveal atrophy; while both shared a novel variant of uncertain significance (VUS) in the ACBD5 gene (variant c.431G>A), they exhibited different clinical profiles and disease manifestations. The father was a 48-year-old man who presented with nyctalopia that had persisted since age seven. He had mild disk pallor, vessel attenuation, retinal pigment epithelium (RPE) changes nasal to the fovea, and few mid-peripheral bone spicules. Sequencing analysis showed that he carried seven VUS in five genes: ACBD5 c.431G>A (p.Gly144Asp), CYP4V2 c.296T>C (p.Met99Thr), EYS c.1852G>A (p.Gly618Ser), HMCN1 c.280G>A (p.Val94Met), HMCN1 c.8939A>C (p.Asn2980Thr), RP1L1 c.575C>A (p.Pro192His), and RP1L1 c.1375A>C (p.Thr459Pro). He shared only the ACBD5 gene with his 18-year-old daughter. The daughter had 20/20 visual acuity, but further testing showed foveal atrophy and hyperautofluorescence. Intrafamilial phenotypic heterogeneity was detected in our patients. Studies on the role of hormonal factors leading to phenotypic variability are warranted.

Contralateral Recurrences of Post-vaccination Multiple Evanescent White Dot Syndrome.

We report on a case of multiple evanescent white dot syndrome (MEWDS) following the simultaneous administration of the human papillomavirus and meningococcal (conjugate) vaccines and two recurrences of MEWDS following the administration of the second dose of the human papillomavirus (HPV) vaccine and the COVID-19 vaccine and COVID-19 viral infection. A 17-year-old Hispanic female presented with a one-week history of photopsia and blurred vision in her left eye following the simultaneous administration of the human papillomavirus and meningococcal (conjugate) vaccines. Upon a comprehensive examination, her best-corrected visual acuity was 20/20 in the right eye and 20/100 in the left eye. A left fundus examination revealed multiple white dots in the macula and nasal periphery, consistent with a diagnosis of MEWDS. Ancillary testing, including fundus autofluorescence, fluorescein angiography, indocyanine green angiography, and optical coherence tomography, supported the diagnosis. One month following her initial diagnosis, the patient's symptoms had resolved without any therapy, and a fundus examination revealed multiple relatively ill-defined brown-colored subretinal lesions in the nasal midperiphery, corresponding to the location of the previous MEWDS lesions. Subsequently, she received the second dose of the HPV vaccine and then developed a mild COVID-19 infection. Four months after the initial presentation, she received the first dose of the BNT162b2 COVID-19 vaccine, followed by the second dose a month later. Eight months following her initial presentation, she presented with photopsia in the right eye. Her visual acuity remained 20/20 in the right eye and improved to 20/20 in the left eye, and white dots were identified nasal to the disk and surrounding the peripapillary region; the contralateral MEWDS diagnosis was confirmed by the previously mentioned ancillary tests. At her one-month follow-up, she presented new onset photopsia of the right eye. Her visual acuity remained 20/20 in both eyes, and a fundus examination revealed white lesions suggestive of active MEWDS temporal to the macula and brown-colored spots nasal to the disk, suggestive of recovering MEWDS, nasally. The aforementioned testing confirmed the coexistence of new and resolving lesions; nonetheless, the patient's symptoms resolved without any therapy, and she received the third dose of the BNT162b2 COVID-19 vaccine 11 months after her initial presentation. Our case suggests that vaccines may serve as immunological triggers of MEWDS. Recurrent MEWDS may occur when an individual is exposed to a powerful immune challenge, such as receiving a wide array of vaccinations in a short period of time. We believe this case constitutes a previously undescribed finding of multiple relatively ill-defined brown-colored subretinal lesions present in late MEWDS.