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To describe an atypical case of chronic central serous chorioretinopathy (CSCR) with acute exacerbation consisting of severe exudation at the site of a retinal pigment epithelium (RPE) aperture.Case report.A 39-year-old man presented with a recurrence of CSCR in the right eye. Initial evaluation was notable for a RPE aperture overlying a chronic avascular pigment epithelial detachment (PED). He was initially treated with topical dorzolamide and indomethacine. During follow-up, application of topical dermal steroid for a case of athlete's foot led to severe fibrinous exudation originating from the site of the RPE aperture. Half-fluence verteporfin photodynamic therapy (PDT) induced rapid and complete resolution of the retinal findings.PDT allowed for excellent resolution of an atypical exudative and fibrinous form of CSCR associated with an RPE aperture.

Purpose To evaluate the effect of tumor necrosis factor (TNF) inhibitor therapy on ocular relapses in patients with Susac syndrome. Methods Multicenter retrospective cohort study of patients diagnosed with Susac syndrome according to classical clinical criteria. We evaluated the disease activity before and after introduction of anti-TNF therapy and its value as a steroid-sparing agent. Results Five patients were included. All were initially treated with a combination of corticosteroids and classical immunosuppressive drugs. Infliximab was started in three patients, and adalimumab was started in two patients. Patients had on average 5 ocular relapses during a mean follow-up time of 2.59 years before introducing a TNF inhibitor, corresponding with on average 1.93 relapses per year. After the introduction of an anti-TNF agent, this number was reduced by factor 5.51 to an average of 0.35 relapses per year for a mean follow-up of 2.86 years (P = 0.10). Before anti-TNF introduction ocular relapses occurred at a mean daily dose of 34 mg of prednisone, whereas with anti-TNF treatment, corticosteroid administration could be completely stopped in four patients with one patient still needing 5 mg daily (P = 0.10). Infliximab and adalimumab generally were well tolerated, and no serious adverse events were reported. Conclusion Although not statistically significant, our results suggest that anti-TNF therapy can be a valuable option for the treatment of ocular Susac syndrome and may especially be considered in those patients unresponsive to more conventional immunosuppressive treatment.

Purpose To illustrate with multimodal imaging a case of HELLP syndrome (Hemolysis, Elevated Liver enzymes, Low Platelets) complicated by bilateral multifocal serous retinal detachments, subretinal exudation, and papilledema. Methods Case report. Fundus photography, spectral domain optical coherence tomography (SD-OCT), fluorescein angiography, and indocyanine green angiography were performed at presentation and the day after. We also present the SD-OCT follow-up at 8 days, 1 year, and 4 years. Results A 25-year-old 5-month-pregnant Guinean woman complained about decreased visual acuity in the right eye. Eye fundus and multimodal imaging were abnormal in both eyes. Spectral domain optical coherence tomography showed the presence of multifocal serous retinal detachments, subretinal deposits, and intraretinal cysts. Indocyanin green angiography revealed an irregular choroidal perfusion and localized choroidal ischemia. Spectral domain optical coherence tomography also provided assessment of retinal changes during the long-term follow-up, showing tissue damage in the outer retina. Conclusion Serous retinal detachments during pregnancy can be the leading sign of HELLP syndrome-a potentially life-threatening condition. Spectral domain optical coherence tomography is a noninvasive and useful tool for its diagnosis and follow-up. ICG is important to confirm the choroidal ischemia and choroidal vascular abnormalities, underlying conditions leading to main sign of HELLP syndrome in the eye.

Purpose: To describe subclinical chorioretinal lesions revealed by indocyanine green angiography (ICGA) and their evolution under systemic treatment in tubulointerstitial nephritis and uveitis (TINU) patients.Methods: Retrospective case series of three patients with TINU syndrome. Choroidal and retinal involvement were assessed by fluorescein angiography (FA) and ICGA.Results: Three patients were analyzed. FA demonstrated hot disc, associated in two cases with retinal vascular leakage, and ICGA revealed subclinical chorioretinal dots in all three cases. Given the presence of posterior uveitis and deterioration of kidney function, asystemic treatment by oral methylprednisolone was started. Persistence of retinal and choroidal inflammations under systemic corticosteroids required association with immunosuppressive agent to control the disease activityConclusion: Multimodal imaging and more precisely ICGA is useful to assess subclinical choroidal inflammation and monitor treatment response in TINU syndrome. Immunosuppression needs to be revised and adapted when uveitis and/or kidney function are unresponsive to systemic steroidsAbbreviations: TINU: tubulointerstitial nephritis and uveitis; TIN: tubulointerstitial nephritis; ACE: angiotensin-converting enzyme; RF: rheumatoid factor; Uβ2M: urinary β-2microglobulin; AMPPE: acute multifocal placoid pigment epitheliopathy; FA: fluorescein angiography; ICGA: indocyanine green angiography; CT: computed tomography.

Summary Report of a presentation of bilateral choroidal osteoma without neovascularization in a child following a school screening. Diagnosis was based on funduscopy , optical coherence tomography and B-scan ultrasonography. The child was followed regularly with cycloplegic refraction, funduscopy, macular SD-OCT and EDI-SD-OCT. Fluorescein angiography and OCT-angiography will be performed if necessary. Purpose To report a peculiar presentation of bilateral choroidal osteoma in a very young child. Methods Case report. Results During a school screening, a 4-year-old girl was detected with anisometropia as the first sign of a bilateral choroidal osteoma. The diagnosis was based on funduscopy, optical coherence tomography and B-scan ultrasonography. Conclusion Choroidal osteomas are rare benign tumors that can lead to severe visual loss due to progression or complications. They are found mostly in young adults, but in our case, the refractive screening allowed an early diagnosis despite the lack of visual symptoms.

To investigate the effect of a single intravitreal dexamethasone implant (IVT-DI; Ozurdex; Allergan, Inc.) on visual acuity, macular thickness, and intraocular pressure (IOP) in active noninfectious uveitis.Medical records of patients with noninfectious active uveitis treated by IVT-DIs were retrospectively reviewed. Uveitis etiologies, treatment indications, best corrected visual acuity (BCVA), central retinal thickness measured by ocular coherence tomography, IOP, and systemic, local, and topical treatments were collected. Parameters were analyzed before the injection of the implant, after 1.5 ± 0.8 months and 4.4 ± 0.9 months for the BCVA, after 2 ± 1.3 months and 4.6 ± 1.3 months for the ocular coherence tomography, and after 1.3 ± 0.7 months and 4.4 ± 1 months for the IOP.We included 14 patients (20 eyes, 20 implant injections) with cystoid macular edema (78%), vasculitis (7%), choroiditis (7%), and vasculitis associated with choroiditis (7%). Before the injection, mean visual acuity was 0.4 ± 0.5 logMAR (logarithm of the minimum angle of resolution) that improved to 0.3 ± 0.5 logMAR (P = 0.0002) after 1.5 ± 0.8 months and to 0.3 ± 0.5 logMAR (P = 0.005) after 4.4 ± 0.9 months. A statistically significant decrease of macular thickness was observed both at 2 ± 1.3 months and at 4.6 ± 1.3 months after IVT-DI. Mean IOP was 16 ± 5 mmHg before injections, 18 ± 6 mmHg (P = 0.13) at 1.3 ± 0.7 months, and 15 ± 4 mmHg (P = 0.65) at 4.4 ± 1 months. By Kaplan-Meier analysis, we found that after 3.3 months, 17% of the eyes still present a BCVA amelioration ≥0.3 logMAR.In our patients with active noninfectious uveitis, injection of a first single dexamethasone implant was found to improve visual acuity and decrease macular thickness without significant increase of IOP, although the effect seems limited in time.

Purpose: The aim of this study is to report a rare spectral-domain optical coherence tomography finding in the macula. Methods: This is a descriptive consecutive case series. Patients diagnosed with hyperreflective central perpendicular line in the macular spectral-domain optical coherence tomography were included. Best-corrected visual acuity assessment, standard Amsler grid test, biomicroscopic examination and macular spectral-domain optical coherence tomography were performed. Results: We examined three men and one woman, aged 56 to 91 years (average age: 75.25 years). Spectral-domain optical coherence tomography showed a hyperreflective central perpendicular line in five eyes accompanied by vitreofoveal adhesion in all of them. In two eyes, we observed a lifting of the ellipsoid zone, and in one eye the external limiting membrane was also pulled. In one eye, a subtle lifting of the interdigitation zone was revealed. In another eye, we also found a triangular foveolar detachment of the interdigitation zone. Snellen’s best-corrected visual acuity ranged from 0.2 to −0.1 logMAR (average of 0.006 logMAR). Amsler grid test was unremarkable in four eyes and metamorphopsia was detected in one eye. One eye developed a full-thickness macular hole several weeks after the phacoemulsification cataract surgery. Conclusion: The presence of a central perpendicular line can be revealed by the macular spectral-domain optical coherence tomography. We hypothesize that this finding could be considered as a sign of vitreomacular traction. In our patients, best-corrected visual acuity was only mildly reduced, and Amsler grid test was affected in only one eye.

Objective Age-related macular degeneration (ARMD) is a leading cause of visual impairment. Intravitreal injections of anti-vascular endothelial growth factor (VEGF) are the standard treatment for wet ARMD. There is however, variability in patient responses, suggesting patient-specific factors influencing drug efficacy. We tested whether single nucleotide polymorphisms (SNPs) in genes encoding VEGF pathway members contribute to therapy response. Methods and analysis A retrospective cohort of 281 European wet ARMD patients treated with anti-VEGF was genotyped for 138 tagging SNPs in the VEGF pathway. Per patient, we collected best corrected visual acuity at baseline, after three loading injections and at 12 months. We also registered the injection number and changes in retinal morphology after three loading injections (central foveal thickness (CFT), intraretinal cysts and serous neuroepithelium detachment). Changes in CFT after 3 months were our primary outcome measure. Association of SNPs to response was assessed by binomial logistic regression. Replication was attempted by associating visual acuity changes to genotypes in an independent Japanese cohort. Results Association with treatment response was detected for seven SNPs, including in FLT4 (rs55667289: OR=0.746, 95% CI 0.63 to 0.88, p=0.0005) and KDR (rs7691507: OR=1.056, 95% CI 1.02 to 1.10, p=0.005; and rs2305945: OR=0.963, 95% CI 0.93 to 1.00, p=0.0472). Only association with rs55667289 in FLT4 survived multiple testing correction. This SNP was unavailable for testing in the replication cohort. Of six SNPs tested for replication, one was significant although not after multiple testing correction. Conclusion Identifying genetic variants that define treatment response can help to develop individualised therapeutic approaches for wet ARMD patients and may point towards new targets in non-responders., SCOPUS: ar.j, info:eu-repo/semantics/published

Summary Purpose To evaluate long-term results of low-fluence photodynamic therapy (PDT) with verteporfin in the treatment of chronic central serous chorioretinopathy (CCSC). Methods Retrospective medical record review of 38 eyes (34 patients) who received low-fluence PDT for the treatment of CCSC. Visual acuity (VA), fundus biomicroscopy, fluorescein angiography (FA), indocyanine green angiography (ICG) and optical coherence tomography (OCT) were analyzed. Results Thirty-eight eyes (34 patients) with CCSC received low-fluence PDT. Mean follow-up after PDT was 43.97 months. Mean logMar best corrected VA (BCVA) improved significantly from 0.33 to 0.11 at the last follow-up which corresponds to a gain of 2.2 lines. At 3 months, complete resolution of central subretinal fluid was achieved on OCT after 1 PDT in 37 eyes and after 2 PDTs in 1 eye (retreated at 3 months after first PDT). One patient developed choroidal neovascularization (CNV) 4 years after his low-fluence PDT and received anti-vascular endothelial growth factor (VEGF) injections. Conclusion Low-fluence PDT with verteporfin for CCSC seems efficacious and safe in the long-term.

Purpose To present the case of a young Turkish pregnant patient with bilateral, sight-threatening retinal ischemic vasculopathy and systemic signs suggestive of Behcet disease (oral ulceration, arthritis, and pseudofolliculitis). Methods Case report. Results Multiple areas of superficial, retinal white lesions and few hemorrhages related to occlusions of small retinal vessels were observed at presentation in the macular zone of both eyes. There was gradual improvement of retinal lesions after the administration of corticosteroids and immunosuppressive treatment. Conclusion We report the case of a young pregnant patient with bilateral, sight-threatening retinal ischemic vasculopathy and systemic signs of Behcet disease. Although the systemic signs were highly suggestive of Behcet disease, the ocular presentation was unusual for this multisystemic inflammatory disorder. The differential diagnosis included a number of causes of ischemic retinal vasculopathy, such as systemic vasculitis, antiphospholipid syndrome, Susac syndrome, Purtscher-like retinopathy, and a new variant of acute macular neuroretinopathy (paracentral, acute middle maculopathy), which are further discussed.

PURPOSE To present a case of frosted branch periphlebitis in a young Armenian patient with familial Mediterranean fever. METHODS Case report. RESULTS A 37-year-old man presented with a unilateral decreased visual acuity and floaters for 4 days on the left eye (LE). Visual acuity was 20/20 in the right eye (RE) and 20/28 in the LE. Anterior segment and fundus examinations of the RE were normal. Slit-lamp examination of LE revealed a mild nongranulomatous anterior uveitis and vitritis. Intraocular pressure was 19 mmHg in the RE and 12 mmHg in the LE. Fundoscopy of the LE showed typical appearance of frosted branch periphlebitis with perivascular sheathing of the retinal veins and scattered retinal hemorrhages. Fluorescein angiography of the RE was normal. The LE showed optic disk and segmented vascular staining without macular leakage. Optical coherence tomography of the RE was normal; LE demonstrated a localized macular thickening and few intraretinal cysts. The detailed ophthalmologic history was negative. The general history and workup were significant for familial Mediterranean fever and a positive lupus anticoagulant. One week later, the fundus findings worsened with a severe decrease of visual acuity of the LE to 20/200. A single intravitreal (IVT) injection of bevacizumab was performed. Three weeks after injection, fundus findings progressively improved with a decrease of the macular thickening and an improvement of the visual acuity to 20/25. Clinical improvement continued up to the last visit (19 weeks after the injection) with a visual acuity that reached back 20/20 with no signs of active inflammation. CONCLUSION This case demonstrates a possible association between unilateral frosted branch periphlebitis and familial Mediterranean fever.

Purpose To analyze the effect of tumor necrosis factor inhibitor therapy on ocular relapses in patients with Susac syndrome. Methods Multicenter retrospective case series of patients with ocular Susac syndrome treated with a TNF inhibitor (either infliximab or adalimumab). Diagnosis was based on neurologic, ophthalmic, otologic, biologic and imaging typical findings. Results Five patients were included. All were initially treated with a combination of corticosteroids and classical immunosuppressive drugs. In 4 of the 5 patients a TNF-inhibitor (3 infliximab and 2 adalimumab) was started due to treatment failure. In these patients cortisone could not be decreased below a daily dose of 10 mg without the occurrence of relapses (with an average of 4.25 relapses over a mean follow-up of 330 days) despite the use of a mean of 2.75 different immunosuppressive drugs. After introduction of an anti-TNF agent the daily cortisone dose could successfully be tapered below 10 mg in all patients with a complete stop in 3 patients, with a mean number of 1.25 relapses during a mean follow-up of 1,199 days. In the fifth patient, disease activity was well controlled with only steroid sparing immunosuppressive agents and only 1 relapse during approximately 5 years of follow up until these had to be stopped for a desired pregnancy. No relapse was documented during 60 weeks of follow-up while on infliximab treatment afterwards. Conclusions Anti-TNF antibodies can be a valuable option for the treatment of ocular Susac syndrome and may especially be considered in those patients unresponsive to conventional immunosuppressive treatment.

Purpose Although photodynamic therapy (PDT) is an established treatment for choroidal neovascularization (CNV), the mechanisms are still not completely elucidated. Damage to the retinal pigment epithelium (RPE) was observed following uncomplicated PDT in young patients. Design Observational case series. Methods Four female patients between the age of 26 and 39 years presented with visual loss because of classic CNV. In two 39 years old females the CNV originated secondary to a small chorioretinal scar, in a 26 and a 36-year-old woman the CNV was of idiopathic cause. All patients received standard PDT according to the Treatment of Age-Related Macular Degeneration with Photodynamic Therapy (TAP) Study protocol. Results One to three months after an uncomplicated PDT with verteporfin, severe pigment epithelial alterations in the treatment area were observed. The neovascular membranes responded favorably to the treatment and demonstrated fibrosis and resolution of leakage. Ophthalmoscopically and angiographically, atrophy of the retinal pigment epithelium was seen precisely delineating the size of the treatment spot used. Vision declined in two patients from 0.3 to 0.1 and 0.15 to 0.1. The two other patients demonstrated an increase of visual acuity from 0.7 to 0.9 and from 0.4 to 0.9. The retinal pigment epithelium alterations did not resolve during follow-up, but remained unchanged in area and intensity. Conclusions Characteristic retinal pigment epithelium alterations were observed in young female patients with small classic CNV following PDT. Unusual retinal pigment epithelium damage in young female patients without any associated disease might be related to a possible inherent defect in the RPE or to the hormonal status of this specific patient population.

A patient with AIDS and cytomegalovirus (CMV) retinitis developed a massive bilateral peripheral occlusive vasculopathy with a bilateral neovascularization of the optic disc five weeks after the introduction of highly active antiretroviral therapy (HAART). No associate cause of occlusive vasculopathy was found. Occlusive vasculopathy and optic disc neovascularization may be an immune recovery-related ocular disorder.

The use of highly active antiretroviral therapy (HAART) has dramatically modified the natural history of cytomegalovirus retinitis (CMVR) in AIDS patients. In this context, we discontinued maintenance therapy in eight AIDS patients with previous CMVR and a good and persistent immune and virological response to HAART (CD(4)/= 75/mm(3) in all patients, viral load/=30,000 copies of RNA/ml in seven patients and negative CMV viremia in six patients whose viremia was evaluated). The median recurrence-free interval before maintenance discontinuation was 613 days (326-787). No recurrence was observed after a median follow-up of 248 days (183-368) without maintenance therapy. In conclusion, discontinuation of maintenance therapy for CMVR in AIDS patients with good immune response to HAART can be considered and appears to be safe with a careful follow-up.

Background: Preretinal hemorrhages usually occur at the interface between the posterior hyaloid and inner limiting membrane (ILM). Less frequently, they are located between the ILM and the retinal nerve fiber layer. Sub-ILM hemorrhages have been described in a variety of clinical settings and often lead to severe visual impairment due to their predilection for the macular region. Methods: A consecutive series of five cases in which sub-ILM hemorrhages were clinically suspected and confirmed during early vitrectomy with ILM-peeling. Results: Sub-ILM hemorrhages were clinically suspected in five patients (median age: 32 years) based on the fundoscopic appearance and clinical setting of Terson syndrome (n=1), Valsalva retinopathy (n=2), blood dyscrasia (n=1) and blunt facial trauma (n=1). Vision was severely impaired in all patients (to hand movements in 4 of 5) due to a premacular location of the hemorrhage. All patients were treated with early pars plana vitrectomy because of insufficient spontaneous visual recovery after a median of 6 weeks. The sub-ILM location of the hemorrhage could be confirmed intra-operatively in all patients by bio-staining of the membrane overlying the hemorrhage. ILM-peeling and aspiration of the hemorrhage resulted in excellent visual recovery in all patients. There were no procedure-related complications. Conclusions: Sub-ILM hemorrhages often occur in a specific clinical context and can lead to severe visual impairment in young patients. Given the excellent results and low complication rates, timely surgical intervention is justified when spontaneous resorption is insufficient.

Purpose : To evaluate the safety and efficacy of photodynamic therapy with verteporfin (PDT) for subfoveal classic choroidal neovascularization (CNV) related to punctate inner choroidopathy (PIC) or presumed ocular histoplasmosis-like syndrome (POHS-like). Methods : Retrospective review of 16 eyes from 14 patients with subfoveal classic CNV associated with PIC or POHS-like and treated with PDT. Results : The mean visual acuity increased from 4.5/10 (range: 1/10-9/10) to 7/10 (range: 2/10-10/10) after a mean follow-up of 21 months (range: 8-32 months) and a mean number of 2 PDT (range: 1-6). Visual acuity remained stable or improved in 13 of the 16 eyes (81%) and decreased in three. Conclusion : This nearly two-year follow-up study suggests that PDT could be helpful for patients with subfoveal classic CNV related to PIC or POHS-like.

PURPOSE: To evaluate the effect of photodynamic therapy on subfoveal neovascular membrane related to type 2A idiopathic juxtafoveolar retinal telangiectasia. DESIGN: Interventional case series. METHODS: Retrospective review of four eyes of four patients who underwent photodynamic therapy for subfoveal neovascular membrane secondary to idiopathic juxtafoveolar retinal telangiectasia. Ocular photodynamic therapy with verteporfin was performed in all cases using standard protocols. Results are given in terms of final visual acuity and neovascular membrane activity based on clinical examination, fluorescein and indocyanin green angiography, and, in two cases, optical coherence tomography. RESULTS: Baseline visual acuity of 20/30 and 20/40 (x2) was maintained in three patients after one, two, and three sessions of photodynamic therapy respectively, and a follow-up of 23, 21, and 9 months. Leakage specific to the subfoveal neovascular membrane ceased on the fluorescein angiography. In the other patient, the final vision decreased from 20/50 to 20/200 after four sessions of photodynamic therapy and a follow-up of 14 months. Although there was still mild persistent leakage on the fluorescein angiography, neovascular membrane size was unchanged, and no subretinal fluid was demonstrated on optical coherence tomography. CONCLUSIONS: Data from this case series suggest that photodynamic therapy may be effective in managing subfoveal neovascular membrane associated with idiopathic juxtafoveolar retinal telangiectasia, which usually carries a poor visual prognosis. Prospective study is required to confirm the beneficial effect of this treatment.

We read with interest the article by Aisenbrey et al 1 who have described the results of surgical treatment of peripapillary choroidal neovascularisation in eight patients. As reported, peripapillary choroidal neovascularisation is a relatively uncommon entity that can be a variant of macular choroidal neovascularisation in elderly patients. According to the MPSG,2 early small peripapillary choroidal neovascularisation should be first treated with …