Idiopathic Acute Exudative Polymorphous Vitelliform Maculopathy: Clinical Spectrum and Multimodal Imaging Characteristics.

PURPOSE: To describe clinical findings in patients with acute exudative polymorphous vitelliform maculopathy (AEPVM). DESIGN: Retrospective, observational, multicenter case series review. PARTICIPANTS: Consecutive patients diagnosed with idiopathic AEPVM. METHODS: Review of clinical charts, multimodal imaging, electrophysiologic findings, and genetic findings in previously unpublished patients and review of the literature. MAIN OUTCOME MEASURES: Clinical features of idiopathic AEPVM and differential diagnosis. RESULTS: Eighteen patients (age range, 21-74 years) with typical features of AEPVM, including initial localized, serous detachments followed by the development of characteristic yellow-white deposits in the vitelliform space. Over time, this hyperautofluorescent material gravitated within the larger lesions, resulting in typical curvilinear deposits characteristic of later stages. Symptoms and clinical findings lasted from weeks to several years. Some patients showed previously undescribed features such as fluorescein-negative intraretinal cystic changes, choroidal neovascularization, serous retinal elevations mimicking retinal folds, increased choroidal thickness, lack of rapid visual recovery, and recurrence years after complete resolution of initial manifestations. CONCLUSIONS: Acute exudative polymorphous vitelliform maculopathy can present with a more variable natural course than previously described. Paraneoplastic retinopathy and autosomal recessive bestrophinopathy closely resemble AEPVM, necessitating medical and hereditary evaluation to exclude these clinical possibilities. This series of patients with AEPVM expands the clinical spectrum of the disorder, including demographics, clinical manifestations, imaging features, natural course, and visual prognosis.

Scleral buckling surgery and eye pain: assessment of chronic eye pain during the postoperative period of scleral buckling surgery.

PURPOSE: To assess the progression of eye pain after scleral buckling surgery to treat rhegmatogenous retinal detachment and to investigate the occurrence and characteristics of chronic eye pain. METHODS: This was a longitudinal, prospective, and observational study. Eye pain was measured according to a numerical analog scale (range, 0-10) for 6 months after scleral buckling surgery. The sample was divided into two groups, with or without chronic eye pain, to perform statistical analyses. For this study, chronic eye pain was defined as postoperative pain beyond 30 days. The following variables were assessed to investigate the etiology of chronic eye pain: age, gender, ethnicity, degree of myopia, visual acuity, intraocular pressure, and degree of scleral indentation. RESULTS: A total of 100 patients were assessed in this study. One particular sequence of levels on the pain analog scale, specifically 3-2-1-0-0 (intensity of eye pain on postoperative Days 1, 14, 30, 60, and 180, respectively), was identified more frequently during the progression of eye pain in the 180-day follow-up period. The pain resolved for 72% of patients within 30 days. Chronic eye pain occurred in 18% of the patients. Scleral indentation was the only statistically significant variable investigated relative to the etiology of chronic eye pain (P < 0.05). CONCLUSION: Chronic eye pain correlated significantly with large scleral indentation. Patients with more intense pain at the onset of the postoperative period tended to develop chronic eye pain.

CHOROIDAL NEOVASCULARIZATION AS A LATE COMPLICATION OF SYPHILITIC CHORIORETINITIS MANAGED WITH INTRAVITREAL AFLIBERCEPT.

PURPOSE: To report a rare case of choroidal neovascular membrane (CNV) developed three years after acquired syphilitic chorioretinitis successfully treated with intravitreal aflibercept. METHODS: Case report. RESULTS: A 44-year-old woman with bilateral decreased vision and a history of syphilitic chorioretinitis 3 years prior to presentation. Her best-corrected visual acuity was <20/400 in the right eye and 20/70 in the left eye. There was no evidence of anterior chamber or vitreous inflammation. Fundoscopy revealed a fibrovascular macular lesion associated with hemorrhages in both eyes. Optical coherence tomography (OCT) and OCT angiography confirmed the diagnosis of bilateral CNV. After ruling out other systemic diseases, the diagnosis of CNV as a late complication of syphilitic chorioretinitis was established. Although treatment was not recommended in the right eye, the left eye was treated with 3 monthly intravitreal injections of aflibercept as a solo therapy. Three weeks after the last injection, the visual acuity improved to 20/25 and remained stable at the 6-month follow-up with no evidence of CNV reactivation. CONCLUSION: Choroidal neovascular membranes can occur as a late complication of syphilitic chorioretinitis. Solo treatment with intravitreal injections of the anti-vascular endothelial growth factor aflibercept effectively controlled CNV activity and improved visual acuity.

Intravitreal bevacizumab for type 2 idiopathic macular telangiectasia.

BACKGROUND/AIMS: The aim of this paper is to report the treatment of type 2 nonproliferative idiopathic macular telangiectasia (IMT) with intravitreal bevacizumab (IVB). METHODS: Retrospective case series of 10 eyes of 5 patients with type 2 IMT. All patients received 3 monthly IVB injections. Visual acuity (VA), fluorescein angiography (FA) and optical coherence tomography (OCT) were performed at baseline and 4 weeks after each injection. RESULTS: Four weeks after the third IVB injection, VA remained stable for all patients. All eyes showed some decrease in fluorescein leakage, and there was a mild decrease in central macular thickness. One year later, VA, OCT and FA findings returned to the baseline levels. CONCLUSION: IVB did not improve VA in cases of type 2 IMT.

CLINICAL VARIANTS OF PRESUMED ACUTE FOVEALITIS.

PURPOSE: To describe four eyes of three patients with presumed acute fovealitis to expand the clinical variants of this recently described disorder. METHOD: The patients underwent a comprehensive ophthalmic examination, including multimodal imaging and electrophysiological tests. RESULTS: Three female patients aged from 18 to 24 years presented sudden central visual disturbances in one or both eyes. The visual acuity ranged from 20/25 to 20/70 in the affected eyes. All of them showed a subtle yellowish lesion in the foveola. Fundus autofluorescence and fluorescein angiography were unremarkable. Optical coherence tomography disclosed focal disarrangement of the outer retinal layers restricted to the fovea and hyperreflective lesions above the external limiting membrane. Multifocal electroretinography responses were attenuated. The electrooculogram response was normal. All patients recovered normal visual acuity and the outer retinal layers. CONCLUSION: Attenuated multifocal electroretinography foveal response and normal electrooculogram are newly described clinical findings in patients with acute fovealitis.

Birdshot retinochoroiditis in Brazil: a multicenter review of 40 patients.

BACKGROUND: Birdshot retinochoroiditis (BRC) is a rare and chronic bilateral uveitis mostly found in Caucasians. As few data are available about the clinical course of BRC in Hispanic patients, we aimed to report the clinical findings and the evolution of BRC in Brazilian patients. METHODS: This retrospective cohort multicenter nationwide study was performed by analyzing the records of patients with BRC diagnoses from Brazilian ophthalmological centers from April 1995 to May 2020. RESULTS: Forty patients (80 eyes) with a diagnosis of BRC were evaluated. The mean age was 53 years, and there was no sex predominance. All tested patients (34/40) were positive for HLA-A29. The diagnosis of BRC was made following the Levinson et al. criteria, and all ancillary tests were performed to exclude differential diagnoses. Clinical signs and symptoms, such as complications and treatment, were described. CONCLUSIONS: BRC evolution in Brazilian patients seems to have some peculiarities that diverge from the published literature available about Caucasians, as AS inflammation is higher in this population.

Unilateral macular serpiginous-like choroiditis as the initial manifestation of presumed ocular tuberculosis.

BACKGROUND: Classic serpiginous choroiditis (SC) usually begins in the peripapillary area and spreads centrifugally, however, in some patients, the lesion can arise in the macular region. An association between lesions resembling classic SC and tuberculosis was recognized as a possibly distinct clinical entity and named as tuberculous serpiginous-like choroiditis. The differentiation of this tuberculous entity from SC is critical because the treatment of the former with immunosuppressive drugs leads to several potential adverse effects, and such treatment can have devastating consequences because of the worsening of a concomitant tuberculous infection. CASE PRESENTANTION: A 31-year-old woman presented with unilateral decreased vision and a fundus examination consistent with macular serpiginous choroiditis. A non-reactor tuberculin skin test and normal thoracic CT scan ruled out tuberculosis. However, after 2 months of treatment with steroids and immunosuppressive drugs, the contralateral eye developed similar lesions, further raising the suspicions of ocular tuberculosis. We conducted QuantiFERON® TB Gold, which was positive; hence, antituberculous therapy was started on the patient. The lesions started healing within a few weeks. After 1 year of finishing the therapy, the lesions remained healed without any recurrence. CONCLUSIONS: Macular serpiginous-like choroiditis may be the initial presentation of presumed ocular tuberculosis. Nevertheless, the correct diagnosis of this entity can be challenging and delayed by the imprecise results from the currently available methods.

Intravitreal and posterior subtenon triamcinolone acetonide for severe acute posterior multifocal placoid pigment epitheliopathy.

A 21-year-old man presented with visual acuity of 20/200 in both eyes. The fundus picture, fluorescein angiography, and optical coherence tomography revealed severe bilateral acute posterior multifocal placoid pigment epitheliopathy and serous macular detachments. We treated the patient with triamcinolone acetonide, an intravitreal injection (4 mg/0.1 mL) in one eye and a posterior subtenon injection (40 mg/1 mL) in the other eye. Within 2 weeks the visual acuity was 20/80 in both eyes. At the 8-week follow-up visit his vision was 20/63 bilaterally. One year later the vision remained 20/63 in both eyes. In this patient, the triamcinolone acetonide injections, whether administered intravitreally or via the posterior subtenon route, achieved similar anatomic and functional recovery results.

[Cataract extraction using the phacoemulsification technique in patients with uveitis]. / Extração da catarata pela técnica de facoemulsificação em pacientes com uveíte.

PURPOSE: To report outcomes of cataract extraction by phacoemulsification in patients with uveitis. METHODS: We retrospectively reviewed the charts of 189 patients (242 eyes) with uveitis who underwent cataract extraction by phacoemulsification at the Uveitis and Ocular Immunology Unit of McGill University Health Centre, Montreal, Quebec, Canada. RESULTS: Average follow-up was 46 +/- 31.2 months. Average preoperative visual acuity was 20/100 and average postoperative visual acuity was 20/40. Hundred and forty-six eyes (59.9%) attained visual acuity better than 20/40. Visual loss occurred in 26 eyes. The most common peroperative complication was posterior capsule rupture with vitreous loss seen in 7 eyes (3%). Recurrence of uveitis was the most common postoperative complication seen in 73 eyes (30.16%). Other postoperative complications included iris atrophy (28.51%), ocular hypertension (28.09%), epiretinal membrane (26.44%), posterior capsule opacification (19%), cystoid macular edema (13.63%), ocular hypotony (12.80%), optic disc atrophy (8.67%) and posterior synechiae (6.61%). CONCLUSIONS: Cataract extraction by phacoemulsification is safe in patients with uveitis. Successful visual results are observed in long-term follow-up despite the prevalence of recurrence of uveitis, posterior capsule opacification and macular abnormalities. To the best of our knowledge this is the largest series presented to date.

Subretinal neovascularization following ruptured retinal arterial macroaneurysm: case report.

A 51-year-old black woman was referred to our Retina Clinic for evaluation of loss of vision in her left eye. She was previously diagnosed with a ruptured retinal arterial macroaneurism (RAM) in the same eye. The ophthalmoscopic examination of the affected eye disclosed a subretinal lesion with some hemorrhage and exudation in the same area previously diagnosed with ruptured RAM. Fluorescein angiography revealed a gradually hyperfluorescent net of vessels located in the subfoveal region, consistent with the diagnosis of subretinal neovascular membrane. In this paper we stress the possibility of subretinal neovascularization following a ruptured RAM, even as a rare event.

Flecked retina in systemic large-cell non-Hodgkin's lymphoma: case report.

We report on a patient with a past medical history of successfully treated systemic large-cell non-Hodgkin's lymphoma (SNHL), who presented with multifocal yellowish retinal infiltrates (flecked retina) involving the post-equatorial retina of one eye. Fluorescein angiography revealed that the retinal infiltrates were hypofluorescent throughout the examination. The correct diagnosis of this ocular picture was important because the retinal lesions indicated central nervous system recurrence of systemic large-cell non-Hodgkin's lymphoma.

The expression of cyclooxygenase 2 in retinoblastoma: primary enucleated eyes and enucleation after conservative treatment.

PURPOSE: To provide insight into the relationship between cyclooxygenase-2 (COX-2) expression and histopathologic features of retinoblastoma specimens treated either by primary or secondary enucleation. DESIGN: Laboratory investigation. METHODS: Twenty-five retinoblastoma specimens received between 1994 and 2003 were retrieved for this study from the Ocular Pathology Registry, Federal University of São Paulo, Brazil and the Henry C. Witelson Ocular Pathology Laboratory and Registry, McGill University, Montreal, Canada. The specimens retrieved were divided into two groups: Group I, enucleation was performed as a form of primary treatment (n = 15) and Group II, enucleation after failure of conservative treatment (n = 10). Patient information and formalin-fixed, paraffin-embedded specimens were obtained. New sections of these blocks were used for hematoxylin and eosin stain and for immunoassay using a monoclonal mouse anti-COX-2 antibody. Two independent ophthalmic pathologists reviewed all of the microslides. RESULTS: Twenty-three specimens (92%) presented a high expression of COX-2 (15 in Group I; eight in Group II) and there was no statistical difference in COX-2 expression between the two groups (P = .07). However, all specimens expressed COX-2 to a different degree. The areas of tumor invasion were positive for COX-2 in 87.5% of the two groups. CONCLUSION: Most retinoblastoma specimens revealed a high COX-2 expression. Future studies will be necessary to correlate the high expression of COX-2 in retinoblastoma and a possible applicability of anti-COX-2 medications in the treatment of these tumors.

Combination of azathioprine and corticosteroids in the treatment of serpiginous choroiditis.

BACKGROUND: Although therapy with immunosuppressive agents is currently accepted as the best option for treating active serpiginous choroiditis (SC), there is no consensus on the most effective immunosuppressive drug to use. In this paper, we describe the clinical course of patients with active SC treated with azathioprine (AZA) in combination with corticosteroids. METHODS: This retrospective study included 4 patients (5 eyes) with active, vision-threatening SC who received systemic immunosuppression with AZA at 1.5 to 2.0 mg/kg per day. In combination with oral AZA, patients also received 1 mg/kg oral prednisone per day. Information collected included Snellen visual acuity (VA), clinical disease activity, duration of follow-up, rate of inflammation recurrence, and side effects of AZA. RESULTS: Within 3 weeks of treatment, all patients experienced decreased ocular inflammation and improved VA. One patient, however, had a recurrence in both eyes while oral prednisone was being tapered. In this case, once the dosage of oral prednisone was increased and methotrexate was added to the therapeutic scheme, inflammation was controlled within 1 month. The other 3 patients presented no further visual loss while on AZA and were able to taper and then discontinue oral prednisone. Nevertheless, SC recurred in 1 of these patients 40 months after the initial treatment. AZA was reintroduced but the patient complained of gastrointestinal problems, and it was then successfully replaced by mycophenolate mofetil. None of the 4 patients presented serious systemic side effects secondary to AZA. INTERPRETATION: This study suggests that when AZA is used in combination with corticosteroids it is a safe and acceptable option for treating patients with active SC. Side effects and recurrences while on AZA therapy can occur, requiring either replacement of the drug or addition of another immunosuppressive agent.

Visual outcome of juvenile rheumatoid arthritis-associated uveitis in adults.

PURPOSE: Juvenile rheumatoid arthritis (JRA) is the systemic disease most frequently associated in childhood uveitis. The disease may cause several ocular complications, visual impairment, and blindness. Recent studies revealed a more favorable ocular prognosis. Our purpose was to analyze the long-term visual outcome of JRA-associated uveitis. METHODS: Ocular complications and visual outcome in adult patients with JRA-associated uveitis were evaluated. Among 18 patients included in the study, uveitis was bilateral in 12 (66.7%) and unilateral in six (33.3%), for a total of 30 eyes with ocular involvement. RESULTS: The mean durations of JRA and its associated uveitis were 24.9 and 20.5 years, respectively. All eyes (100%) had at least one ocular complication. The most frequently observed ocular complications were cataract (83.3%), band keratopathy (60%), posterior synechia (46.7%), glaucoma (33.3%), hypotony (16.7%), and macular pathology (13.3%). Final visual acuity was impaired in 40% of the eyes, poor in 20%, and totally lost in 10%. Therefore, 70% of the eyes were either visually handicapped or totally blind. Most eyes underwent at least one surgical procedure. Inflammation was active at last examination in 63.3% of eyes. All patients were still treated topically and with systemic NSAID. Sixty-one percent of the patients were using an immunosuppressive agent. CONCLUSION: JRA-associated uveitis still has a severe course and blinding potential. Patients suffer from uveitis and its complications even during the adulthood period. However, because our series represents a more severe subset of the disease, the outcome may be poorer than that of some other outcome studies.

Chemoattraction of inflammatory cells by various intraocular lens materials.

PURPOSE: To compare the chemotactic activity induced by polymethyl methacrylate (PMMA), acrylic, and silicone intraocular lens (IOL) materials, regardless of the surgical and host factors. METHODS: The chemotactic effect of five different IOLs was studied using a modified multi-well Boyden chamber. Two different non-coated PMMA IOLs, a heparin-coated PMMA IOL, an acrylic IOL, and a silicone IOL were assessed. Each mean migration distance of the IOLs was compared with the others using Student's t test. RESULTS: All IOLs induced some amount of leukocyte chemotaxis. There was no statistically significant difference between the chemotactic activity of PMMA IOLs. However, chemotactic activity was significantly higher in acrylic (p<0.05) and silicone (p<0.05) IOLs compared to one of the PMMA lenses. CONCLUSIONS: This limited study has proven that chemotaxis assay is a useful tool to assess the biocompatibility of IOLs. The IOL material itself might attract inflammatory cells to its surface in the absence of surgical and host factors. As PMMA IOLs do not cause an increase in inflammatory reaction, they may be considered safe to use. Further improvement in chemical composition and surface characteristics of IOLs should reduce the inflammatory reaction and increase the biocompatibility and safety of IOLs.

Intravitreal and posterior subtenon triamcinolone acetonide for severe acute posterior multifocal placoid pigment epitheliopathy / Triancinolona intravítrea e sub-tenoniana para epiteliopatia pigmentar placóide multifocal posterior aguda grave

ABSTRACT A 21-year-old man presented with visual acuity of 20/200 in both eyes. The fundus picture, fluorescein angiography, and optical coherence tomography revealed severe bilateral acute posterior multifocal placoid pigment epitheliopathy and serous macular detachments. We treated the patient with triamcinolone acetonide, an intravitreal injection (4 mg/0.1 mL) in one eye and a posterior subtenon injection (40 mg/1 mL) in the other eye. Within 2 weeks the visual acuity was 20/80 in both eyes. At the 8-week follow-up visit his vision was 20/63 bilaterally. One year later the vision remained 20/63 in both eyes. In this patient, the triamcinolone acetonide injections, whether administered intravitreally or via the posterior subtenon route, achieved similar anatomic and functional recovery results.

RESUMO Um homem de 21 anos apresentou acuidade visual de 20/200 em ambos os olhos. O quadro de fundo de olho, angiofluoresceinografia e a tomografia de coerência óptica revelaram epiteliopatia pigmentar placóide multifocal posterior aguda e descolamento macular seroso. Tratamos o paciente com triancinolona acetonida, uma injeção intravítrea (4 mg/0,1 ml) em um olho e uma injeção subtenoniana posterior (40 mg/1 ml) no outro olho. Após 2 semanas, a acuidade visual foi de 20/80 em ambos os olhos. Na visita de acompanhamento de 8 semanas, sua visão foi de 20/63 bilateralmente. Um ano depois, a visão permaneceu 20/63 em ambos os olhos. Neste paciente, as injeções de triancinolona, administradas por via intravítrea ou por via subtenoniana posterior, obtiveram resultados semelhantes na recuperação anatômica e funcional.