Cross-Sectional Study on Vitamin D, Zinc Oxide and Fatty Acid Status in a Population with a Moderate to High Risk of AMD Identified by the STARS® Questionnaire.

INTRODUCTION: A prospective study was carried out in Belgium to determine the proportion of subjects with a moderate to high risk of developing age-related macular degeneration (AMD), identified using the STARS® (Simplified Théa AMD Risk-Assessment Scale) questionnaire, who were in need of nutritional supplementation, by assessing the vitamin D, zinc oxide and fatty acid profile status. METHODS: This multicentre cross-sectional pilot study involved 50 Belgian subjects with no or early AMD, aged > 55 years who were at moderate to high risk for AMD. Subjects were assessed using the STARS® questionnaire, visual acuity assessment, an optical coherence tomography scan of the macula and fundus photography. Blood samples were collected, and serum analyses were performed to determine the the omega-6:omega-3 (Ω6:Ω3) ratio and the levels of eicosapentaenoic acid (EPA), docosahexaenoic acid (DHA), zinc and cupric oxides and vitamin D. RESULTS: Abnormal serum levels for at least one of the micronutrients was detected in 94% of the subjects. Lower than optimal vitamin D levels were found in 76% of the participants, and 68% of the subjects demonstrated at least one abnormal fatty acid profile. The Ω6:Ω3 ratio was above the reference range for normal values in 54% of the subjects; DHA and EPA levels were below the reference range in 60 and 46% of the subjects, respectively; and zinc oxide concentration was below the reference range in 50% of the subjects. Only 12% of the subjects exhibited cupric oxide deficiency. CONCLUSION: In this study, the STARS® questionnaire was used for early identification of patients at moderate to high risk of AMD in real life. These patients presented a suboptimal nutritional status. Further research is needed to determine if specific diet modification or micronutrient supplement intake delays the onset or slows down the progression of AMD in these subjects. TRIAL REGISTRATION: Trial registration: ClinicalTrials.Gov, identifier: NCT04482465.

Long-Term Ranibizumab Treatment in Neovascular Age-Related Macular Degeneration: A Belgian Subanalysis from the Global Real-World LUMINOUSTM Study.

PURPOSE: To evaluate long-term, real-world treatment patterns and outcomes of ranibizumab 0.5 mg for neovascular age-related macular degeneration (nAMD) in a Belgian cohort. PATIENTS AND METHODS: This Belgian (BE) cohort of the 5-year global observational LUMINOUS study included 229 patients with nAMD. Outcomes included visual acuity (VA), central retinal thickness (CRT) and safety. RESULTS: The mean age was 79.5±7.7 years. The majority of patients (67.7%) were female and all patients were Caucasian. Most patients previously received ranibizumab with only 17.5% of patients being treatment-naïve. The injection frequency declined over time irrespective of prior treatment status (p<0.0001), with treatment-naïve eyes receiving a mean of 4.2±2.9 yearly injections and prior-ranibizumab eyes 3.6±2.7. Regression analysis confirmed first-year VA increases for treatment-naïve eyes (p=0.002) followed by a slight decrease of -1.8 letters per year. For prior-ranibizumab eyes, the visual changes over 1 year were statistically non-significant (p=0.90) but declined slightly after year one (p<0.0001). Anatomically, the CRT of treatment-naïve eyes decreased over time from baseline (p<0.0001), whereas the CRT of prior-ranibizumab eyes remained stable (p=0.43). No new safety findings were identified. CONCLUSION: LUMINOUS-BE reconfirms the well-characterized benefit-risk profile of ranibizumab for nAMD treatment. The observed low injection frequency reflects a need for more rigorous treatment in real-world settings. CLINICAL TRIAL REGISTRATION: NCT01318941.

SEVERE CASE OF RENAL COLOBOMA SYNDROME IN LONG-TERM FOLLOW-UP.

PURPOSE: To characterize the ocular features of a severe case of renal coloboma syndrome in a long-term follow-up. METHODS: Observational case report over a period of 45 years. Examination under anesthesia at the age of 3 months, repeated ophthalmologic examination (age 7, 14, 25, 45 years), fluorescein and indocyanine green angiography, electroretinography, ocular ultrasound, optical coherence tomography, computed tomography scan orbits, and magnetic resonance imaging of the brain. RESULTS: Presentation with severe bilateral posterior eye defects, optic nerve aplasia and a retrobulbar cyst in the left eye, renal abnormalities, and mental retardation. Over time, a progressive axial myopia in the right eye, band keratopathy in the left eye, and progressive bilateral posterior lens opacities were noted. There was only a minor decrease in visual acuity and visual field of the only functional right eye. The mother of this patient had a mild optic disk hypoplasia, progressive lens opacities, and late-onset renal disease. Both had a confirmed mutation in exon 2 of the PAX2 gene. CONCLUSION: This first published long-term follow-up of renal coloboma syndrome shows progressive posterior lens opacities, axial myopia, and band keratopathy with only a small decline in visual function over time.

Vitamin D and Age-Related Macular Degeneration.

In recent years, the relationship between vitamin D and health has received growing attention from the scientific and medical communities. Vitamin D deficiencies have been repeatedly associated with various acute and chronic diseases, including age-related macular degeneration (AMD). Its active metabolite, 1α,25-dihydoxy vitamin D, acts as a modulator of cell proliferation, differentiation and apoptosis, and cumulative data from experimental and observational studies suggest that relatively a lower vitamin D status could be a potential risk factor for the development of early and/or late AMD. Herein, we made a narrative review of the mechanisms linking a potential role of vitamin D with the current concepts of AMD pathophysiology.

Six-year outcomes in neovascular age-related macular degeneration with ranibizumab.

AIM: To evaluate the outcomes of ≥6y ranibizumab therapy in neovascular age-related macular degeneration (AMD). METHODS: HELIX was a retrospective, observational effectiveness study using medical records of patients treated in three clinics in Belgium. Patients had neovascular AMD and were initially treated with intravitreal ranibizumab (0.5 mg) between November 1, 2007 and October 31, 2008, had ≥6y of data available, and were treated on an ongoing, as-needed basis. Outcomes included best-corrected visual acuity (BCVA) and central retinal thickness (CRT). RESULTS: The sample consisted of 88 eyes from 69 patients. Mean age was 76.4±6.5y, most patients were female (62.3%). Most eyes (62.5%) were treatment-naive, 33 previously treated eyes had received predominantly other anti-vascular endothelial growth factor agents and verteporfin. Mean baseline BCVA was 57.4±12.7 ETDRS letters and CRT was 291.5±86.1 µm. On average, patients received 20.6±11.9 ranibizumab injections over the ≥6y. Intervals between injections were on average 12.7±16.1wk. Mean change in BCVA from baseline to last observation for the sample was less than one letter (-0.9±17.3 letters), with an average loss of -3.2±15.6 letters in previously treated eyes versus a gain of 0.6±18.4 letters in treatment-naïve eyes. When considering a loss of <15 letters over 6y as stabilization of disease, 75.9% of all eyes showed a positive (improvement or stabilization) outcome. Mean change in CRT from baseline to last observation for the sample was -26.9±148.4 µm with the greatest reduction observed in treatment-naive eyes. CONCLUSION: This retrospective study of 69 neovascular AMD patients treated for ≥6y with ranibizumab demonstrates long-term visual stabilization. In light of the natural evolution of the disease, these data confirm that ranibizumab is effective long-term under real-world conditions of heterogeneity of patients, clinicians, and centers.

Omega-3 Fatty Acids and Age-Related Macular Degeneration.

Against a background of considerable epidemiological and other evidence implicating omega-3 fatty acids in the prevention of age-related macular degeneration (AMD), the negative results of the Age-Related Disease Study 2 (AREDS2) were unexpected. The possibility that the design, setting, intake or subjects of AREDS2 may not have permitted the prophylactic potential of omega-3 to be adequately demonstrated is considered. Epidemiological studies had indicated potential preventative effects of omega-3, and an earlier randomised prospective study (NAT2) showed that patients who achieved high red blood cell membrane EPA/DHA (eicosapentaenoic acid/docosahexaenoic acid) levels were significantly protected against AMD compared with those with permanently low EPA/DHA levels. Various methodological differences between these studies are considered. NAT2 included a true placebo group, whereas control subjects in AREDS2 received a nutritional formula already found to be effective in AREDS1, but no placebo for DHA/EPA supplementation. Differences in the handling of non-compliant subjects and the formulation of the test formulations are considered. Given these considerations, and other lines of evidence from laboratory and clinical studies, closing the chapter on omega-3 in AMD prevention may be premature.

Early diagnosis and successful treatment of paraneoplastic melanocytic proliferation.

BACKGROUND: Paraneoplastic melanocytic proliferation (bilateral diffuse uveal melanocytic proliferation, BDUMP) is a rare but devastating disease that causes progressive visual loss in patients who usually have an occult malignancy. Visual loss occurs as a result of paraneoplastic changes in the uveal tissue. METHODS: In a masked fashion, the serum of two patients with BDUMP was evaluated for the presence of cultured melanocyte elongation and proliferation (CMEP) factor using cultured human melanocytes. We evaluated the efficacy of plasmapheresis as a treatment modality early in the disease in conjunction with radiation and chemotherapy. RESULTS: The serum of the first case patient was investigated after plasmapheresis and did not demonstrate proliferation of cultured human melanocytes. The serum of the second case was evaluated prior to treatment with plasmapheresis and did induce this proliferation. These findings are in accordance with the diminution of CMEP factor after plasmapheresis. Treatment with plasmapheresis managed to stabilise the ocular disease progression in both patients. CONCLUSIONS: In the past, visual loss due to paraneoplastic melanocytic proliferation was considered progressive and irreversible. We treated two patients successfully with plasmapheresis and demonstrated a relation between CMEP factor in the serum of these patients and proliferation of cultured melanocytes.

Ocular features in Alport syndrome: pathogenesis and clinical significance.

Alport syndrome is an inherited disease characterized by progressive renal failure, hearing loss, and ocular abnormalities. Mutations in the COL4A5 (X-linked), or COL4A3 and COL4A4 (autosomal recessive) genes result in absence of the collagen IV α3α4α5 network from the basement membranes of the cornea, lens capsule, and retina and are associated with corneal opacities, anterior lenticonus, fleck retinopathy, and temporal retinal thinning. Typically, these features do not affect vision or, in the case of lenticonus, are correctable. In contrast, the rarer ophthalmic complications of posterior polymorphous corneal dystrophy, giant macular hole, and maculopathy all produce visual loss. Many of the ocular features of Alport syndrome are common, easily recognizable, and thus, helpful diagnostically, and in identifying the likelihood of early-onset renal failure. Lenticonus and central fleck retinopathy strongly suggest the diagnosis of Alport syndrome and are associated with renal failure before the age of 30 years, in males with X-linked disease. Sometimes, ophthalmic features suggest the mode of inheritance. A peripheral retinopathy in the mother of a male with hematuria suggests X-linked inheritance, and central retinopathy or lenticonus in a female means that recessive disease is likely. Ocular examination, retinal photography, and optical coherence tomography are widely available, safe, fast, inexpensive, and acceptable to patients. Ocular examination is particularly helpful in the diagnosis of Alport syndrome when genetic testing is not readily available or the results are inconclusive. It also detects complications, such as macular hole, for which new treatments are emerging.

Bilateral diffuse uveal melanocytic proliferation with good clinical response to plasmapheresis and treatment of the primary tumor.

PURPOSE: To report successful treatment of bilateral diffuse uveal melanocytic proliferation. METHODS: Case report of a patient with clinical diagnosis of bilateral diffuse uveal melanocytic proliferation, imaged with fluorescein angiography and spectral domain optical coherence tomography, and with follow-up data after treatment with plasmapheresis, radiation, and chemotherapy. RESULTS: A 67-year-old white man presented with a history of bilateral rapidly declining vision. The ophthalmoscopic examination, fluorescein angiography, and optical coherence tomography showed in both eyes an exudative retinal detachment and subretinal lesions highly suggestive for bilateral diffuse uveal melanocytic proliferation. Further investigation demonstrated a large cell lung carcinoma. Subsequently, the patient was treated with plasmapheresis, radiation, and chemotherapy. In a few weeks, the exudative retinal detachments resolved with gain of vision, and the retina remained flat during the 5-month follow-up. CONCLUSION: Plasmapheresis and treatment of the primary tumor are valuable treatment options for visually impaired patients with bilateral diffuse uveal melanocytic proliferation.

European survey on the opinion and use of micronutrition in age-related macular degeneration: 10 years on from the Age-Related Eye Disease Study.

PURPOSE: To evaluate ophthalmologists' opinion of, and use of, micronutritional dietary supplements 10 years after publication of the first Age-Related Eye Disease Study (AREDS) study. METHODS: Participation was solicited from 4,000 European ophthalmologists. Responding physicians were screened, and those treating at least 40 patients with age-related macular degeneration (AMD) per month and prescribing nutrition supplements at least 4 times per month were admitted and completed a 40-item questionnaire. RESULTS: The surveyed sample included 112 general ophthalmologists and 104 retinal specialists. Most nutritional supplements (46%) were initiated when early/intermediate AMD was confirmed, although 18% were initiated on confirmation of neovascular AMD. Clinical studies were well known: 90% were aware of AREDS, with 88% aware of AREDS1 and 36% aware of the, as-yet-unpublished, AREDS2 studies. Respondents considered lutein, zeaxanthin, zinc, omega-3, and vitamins to be the most important components of nutritional supplements, with the results of AREDS2 already having been taken into consideration by many. Ophthalmologists anticipate more scientific studies as well as improved product quality but identify cost as a barrier to wider uptake. CONCLUSION: Micronutrition is now part of the routine management of AMD for many ophthalmologists. Ophthalmologists choosing to use nutritional supplements are well-informed regarding current scientific studies.

Macular telangiectasia type 2 (MacTel) in a 34-year-old patient.

We report macular telangiectasia type 2 (MacTel) in a 34-year-old man, the youngest patient so far published with MacTel type 2. The patient presented with metamorphopsia and impaired reading ability. Diagnosis was based on bilateral abnormal macular autofluorescence, perifoveal telangiectasia with fluorescein angiographic hyperfluorescence without cystoid oedema, a small foveal avascular zone, asymmetric configuration of the foveal pit, disruptions in the inner segment/outer segment layer and hyper-reflective haze and spots in the outer nuclear layer. Although MacTel usually manifests with a slowly progressive decrease in visual acuity in the fifth to seventh decades of life, younger patients may occasionally be diagnosed with the disease. Awareness of subtle signs of the condition is essential for early diagnosis.

Effect of pegaptanib sodium 0.3†mg intravitreal injections (Macugen) in intraocular pressure: posthoc analysis from V.I.S.I.O.N. study.

OBJECTIVE: To assess the rate of pegaptanib-associated sustained intraocular pressure (IOP) elevation. METHODS: A posthoc analysis was conducted on all IOP measurements, except the immediate 30-min postinjection, from all subjects randomised to pegaptanib 0.3 mg or sham injections continuously in the first 2 years of the Vascular endothelial growth factor Inhibition Study in Ocular Neovascularisation (V.I.S.I.O.N.) study. Measurements were taken with Goldmann applanation tonometer or Tonopen, except at baseline and in cases of an IOP reading >30 mm Hg when a Goldmann applanation tonometer was mandatory. RESULTS: Of 221 subjects, IOP measurements ≥22 mm Hg were seen in 28/114 and 23/107 subjects of the pegaptanib and sham subgroups, respectively (p=0.6338) and measurements ≥24 mm Hg were observed in eight and eight subjects in the pegaptanib and sham groups, respectively. More than two measurements ≥22 mm Hg occurred in six and 10 subjects (p=0.3025), and more than two measurements ≥24 mm Hg were observed in one and four subjects in the pegaptanib and sham groups, respectively. One patient with sustained IOP elevation in the pegaptanib study group, and four in the sham group, had IOP lowering medication added during the course of the study. No subject required glaucoma surgery. CONCLUSIONS: In V.I.S.I.O.N., after 2 years, there was no evidence of sustained IOP elevation associated with pegaptanib 0.3 mg use. TRIAL REGISTRATION NUMBER: NCT00321997.

Immunogammopathies and acquired vitelliform detachments: a report of four cases.

PURPOSE: To describe the nature and evolution of acquired macular detachments in patients with immunogammopathies and to propose a mechanism for their development. DESIGN: Retrospective observational case series. METHODS: Three patients with multiple myeloma and 1 with light chain deposition disease were diagnosed with vitelliform macular detachments based on clinical examination, fundus autofluorescence, fluorescein angiography, and optical coherence tomography. These patients were followed over time and their clinical examinations and imaging studies were compared and contrasted. RESULTS: Three patients (5 eyes) with multiple myeloma and 1 patient (2 eyes) with light chain deposition disease presented with acquired macular yellowish subretinal deposits on funduscopic examination that corresponded to hyperautofluorescent lesions on fundus autofluorescence imaging and subretinal hyperreflective material on spectral-domain optical coherence tomography. One patient (2 eyes) had diffuse serous retinal detachments involving not only the macular region but also the midperiphery of the retina. These acquired macular vitelliform detachments were not associated with signs of hyperviscosity retinopathy in 5 eyes and resolved after successful treatment of the multiple myeloma in 6 eyes. CONCLUSION: Patients with an immunogammopathy such as multiple myeloma or light chain deposition disease may develop serous elevations of the macula that we classify as acquired vitelliform detachments using multimodal imaging. Appropriate evaluation including serum protein electrophoresis and hematology consultation should be considered in the management of patients with acquired vitelliform detachments of uncertain etiology.

Intravitreal bevacizumab for nonsubfoveal choroidal neovascularization associated with angioid streaks.

PURPOSE: To evaluate the effects of intravitreal bevacizumab injections in the treatment of nonsubfoveal choroidal neovascularization (CNV) associated with angioid streaks. DESIGN: Nonrandomized, interventional, prospective case series. METHODS: Fifteen patients (15 eyes) affected by juxtafoveal or extrafoveal CNV secondary to angioid streaks were enrolled in the study. All patients underwent a complete ophthalmologic examination, including best-corrected visual acuity (BCVA) measurement on Early Treatment Diabetic Retinopathy Study (ETDRS) chart, optical coherence tomography (OCT), and fluorescein angiography (FA). The protocol treatment included a first injection, followed by repeated injections over a 12-month follow-up period on the basis of the detection of new hemorrhage on biomicroscopic examination, any type of fluid on OCT, or presence of leakage on FA. PRIMARY OUTCOME MEASURES: Mean changes in BCVA and proportion of eyes gaining at least 10 letters (2 ETDRS lines) at the end of the follow-up. SECONDARY OUTCOMES: Mean changes of central macular thickness (CMT) and extension to the fovea. RESULTS: Mean BCVA did not change throughout the follow-up period, being 0.2 ± 0.2 logMAR at baseline and 0.2 ± 0.3 logMAR at the 12-month examination. A functional improvement of at least 2 ETDRS lines was achieved by 5 eyes (33%), with 3 eyes (20%) gaining 3 lines. Mean CMT at baseline was 215 ± 13 µm and 225 ± 85 µm at the 12-month examination. Two eyes (13.3%) showed CNV extension to the fovea. CONCLUSIONS: Intravitreal bevacizumab injection can be a beneficial approach for the management of nonsubfoveal CNV secondary to angioid streaks over a 1-year follow-up.

Long-term fundus changes in acquired partial lipodystrophy.

We describe long-term fundus changes in a patient with partial lipodystrophy (PL). Retinal pigment alterations, drusen and subretinal neovascularisation were seen without evidence for membranoproliferative glomerulonephritis. Fundus alterations similar to those seen in age-related macular degeneration can occur at an earlier age in patients with PL, even without renal disease. Dysregulation of an alternative complement pathway with low serum levels of C3 has been implicated as a pathogenetic mechanism.

Real-world variability in ranibizumab treatment and associated clinical, quality of life, and safety outcomes over 24 months in patients with neovascular age-related macular degeneration: the HELIOS study.

INTRODUCTION: The aim of this study was to examine ranibizumab treatment patterns in "real-world" practice and clinical settings, as well as to assess quality of life outcomes over a 24-month period. MATERIALS AND METHODS: This was a prospective, observational, multicenter, open-label study of 0.5 mg of ranibizumab administered intravitreally. Patients were followed over 24 ± 3 months with intermediate data points at 6 ± 2 months and 12 ± 2 months, and a limited data point at 2.5 ± 1 month that coincided with the end of the loading phase. Outcomes included visual acuity (Early Treatment Diabetic Retinopathy Study), visual function (National Eye Institute Visual Function Questionnaire-25 [NEI VFQ-25]), quality of life (Health Utilities Index Mark III [HUI3]), and safety. RESULTS: A total of 267 patients with wet age-related macular degeneration (mean ± standard deviation [SD] age = 78.5 ± 7.3 years; 62.4% were female; 34.5% with dual eye involvement; 74.9% were treatment-naïve) were treated (309 eyes were treated). The mean ± SD Early Treatment Diabetic Retinopathy Study score at baseline was 56.3 ± 14.3 letters. The mean ± SD number of injections over 24 months was 7.6 ± 4.1, including 2.5 ± 0.7 and 5.9 ± 3.6 during the loading and maintenance phases, respectively, with corresponding treatment intervals of 4.8 ± 1.4 weeks and 11.5 ± 9.5 weeks, respectively. Improvements in visual acuity over baseline were reached at 2.5 months and maintained at 6 months (both P < 0.0001). The mean visual acuity increase over baseline at 12 months was not significant (P = 0.08); the decline over baseline at 24 months statistically significant (P = 0.02). Overall, 94.3% of patients showed stable or improved disease at 6 months and 81.5% of patients showed stable or improved disease at 24 months. At 6 months, improvements over baseline were significant for VFQ-25 (P = 0.03) and HUI3 (P = 0.02), but not at 12 months and 24 months. Improvements in VFQ-25 and HUI3 were maintained at 24 months in 38% and 34% of patients, respectively. In total 78 serious adverse events were reported in 40 patients and 77 nonserious adverse events in 34 patients. Nine serious adverse events and nine nonserious adverse events in 14 patients were suspected to be related to ranibizumab treatment. CONCLUSION: The "real-world" clinical effectiveness of ranibizumab was evidenced by the initial improvements over baseline in visual acuity and quality of life, as well as the maintenance of these outcomes at baseline levels at 24 months, and this was observed under variable treatment conditions. The findings underscore the need for individualized treatment with regular monitoring to achieve optimal vision and quality of life outcomes.

Traumatic retinopathy presenting as acute macular neuroretinopathy.

AIM: Traumatic retinopathy presenting as acute macular neuroretinopathy (AMNR) is an uncommon disease causing paracentral scotomas after indirect trauma. METHODS: We report on five patients (six eyes) with AMNR with a temporary reduction of visual acuity and persistent paracentral scotomas after indirect trauma. The findings were documented using multimodal imaging and the follow-up was up to 32 months. RESULTS: Initially, fundoscopy was unremarkable in all patients while visual acuity (Snellen equivalents) varied between 0.03 and 1.0, and a paracentral scotoma was present in all patients. During follow-up, visual acuity recovered to 1.0 in all patients while the paracentral scotomas persisted. Spectral-domain optical coherence tomography revealed a disruption of the inner/outer segment junction within the macular lesion and changes in the outer nuclear layer, which slowly recovered partly during the follow-up. CONCLUSIONS: These findings suggest that indirect trauma can cause changes in the outer retina resembling those seen in AMNR, resulting in persisting paracentral scotomas.

Retinal pigment epithelial detachments and tears, and progressive retinal degeneration in light chain deposition disease.

BACKGROUND/PURPOSE: Light-chain deposition disease (LCDD) is a rare condition characterised by deposition of monoclonal immunoglobulin light chains (LCs) in tissues, resulting in varying degrees of organ dysfunction. This study reports the characteristic clinical ocular findings seen in advanced LCDD upon development of ocular fundus changes. This is the first report to describe this entity in vivo in a series of patients. METHODS: A case series of ocular fundus changes in three patients with kidney biopsy-proven LCDD. All patients underwent best corrected visual acuity (BCVA) exam, perimetry, colour fundus photography and fluorescein angiography; two patients underwent indocyanine green angiography, optical coherence tomography, ultrasound and electroretinography; and one patient underwent fundus autofluorescence. RESULTS: Three patients, 53-60 years old at initial presentation, were studied. All three presented with night blindness, poor dark adaptation, metamorphopsia and visual loss. Examination revealed serous and serohaemorrhagic detachments, multiple retinal pigment epithelial (RPE) tears, diffuse RPE degeneration and progressive fibrotic changes. Neither choroidal neovascularisation nor other vascular abnormalities were present. Final best corrected visual acuity (BCVA) ranged from 20/40 to 20/300. CONCLUSIONS: Progressive LC deposition in the fundus seems to damage RPE pump function with flow disturbance between choroid and retina. This pathogenesis can explain the evolution to RPE detachments and subsequent rips and progressive retinal malfunction.

Micronutrients in age-related macular degeneration.

Several lines of evidence from in vitro and in vivo studies suggest that specific micronutrients may have beneficial effects in age-related macular degeneration (AMD). Such effects appear to be complex and may include filtering short wavelength light and attenuating oxidative and inflammatory damage as well as other structural and physiological factors. There is clinical evidence for potential benefits from vitamin C, ß-carotene, vitamin E and zinc, as well as emerging epidemiological and clinical data for the carotenoids lutein and zeaxanthin and for omega-3 fatty acids. A survey of the literature suggests that some specific micronutrients may be of value in treating or preventing AMD, but further prospective studies are needed to further identify and characterize their effects and place in therapy.

Flecked retina, choroidopathy, and retinal pigment epithelial tear in familial pulmonary arterial hypertension.

BACKGROUND: Thanks to advancement in treatment modalities, the medial survival rate of patients with familial pulmonary arterial hypertension (FPAH) has been improved. Unfortunately, ocular complications because of the chronically elevated systemic venous pressure become more frequent. METHODS: The authors report new FPAH-associated fundus changes in a 50-year-old male patient treated with sildenafil. The anomalies were studied with autofluorescence, fluorescein and indocyanine green angiography, and spectral optical coherence tomography including enhanced depth imaging. RESULTS: Loss of vision and cystoid macular edema were associated with a flecked retina, a thick choroid, central serous choroidopathy-like changes in both eyes and with retinal pigment epithelium detachments and a retinal pigment epithelium tear in the right eye. CONCLUSION: Treatment of ocular findings associated with FPAH mainly involves optimal control of pulmonary arterial hypertension and ophthalmic supportive treatment toward preventing acute ocular complications. The ocular complications occur as a result of enhanced pressure in the superior vena cava and in the ophthalmic veins, resulting in dilation of the ocular veins and congestion of the choroid. Sildenafil treatment in FPAH may enhance the congestion of the choroid and can induce central serous choroidopathy-like changes. A flecked retina, central serous choroidopathy-like changes, and retinal pigment epithelium tear are rare complications of FPAH.