The ophthalmic diagnosis and management of four siblings with Werner syndrome.

INTRODUCTION: Werner syndrome is a rare autosomal recessive disorder caused by mutations in the Werner syndrome WRN gene, on chromosome 8. Those affected manifest early the features of ageing. DISCUSSION: Cataract surgery is prone to post-operative complications in those with Werner syndrome. The development of cystoid macular oedema (CMO) is likely multifactorial. Patients with WS have diabetes mellitus type 2 which can contribute to macular oedema. There is a deposition of abnormal WRN proteins in the macula which also predisposes to macular oedema. The trauma of cataract surgery appears to be the main stimulus for the development of CMO. CMO may, as a result, be difficult to manage in Werner syndrome patients. CONCLUSION: Further study is needed to elucidate the precise role of retinal WRN protein expression in the development of CMO in those with Werner syndrome. A tailored and more successful approach to the treatment of CMO in such patients may result.

Macular Edema in Childhood Uveitis.

BACKGROUND: Pediatric uveitis is associated with a high incidence of severe and frequently permanent visual loss. This article summarizes the current understanding of the disease and the therapeutic options that are available to improve treatment outcomes. METHODS: A Medline search spanning the last 10 years was undertaken using the key terms "pediatric uveitis" or "childhood uveitis" and "macular edema". Articles which appertained to case reports or small case series were excluded from consideration, whereas those in which the opinions of experts were expressed, as well as reviews, were not. The information contained in these latter two forms of publication was particularly valuable, owing to the scarcity of prospective clinical data appertaining to the treatment of pediatric uveitis-associated macular edema. RESULTS: Ten years ago, 34% of children who presented with uveitis manifested secondary complications at the time of diagnosis. During the ensuing 3 years of treatment, this rose to 86%. Heightening awareness of the disease with earlier referrals to expert centers, as well as the advent of therapeutic strategies involving anti-TNF agents and intravitreal corticosteroids, have led to a decrease in the incidence of legal blindness in the affected eyes from 18 - 69% to below 8% during a five-year course of treatment. CONCLUSION: Early diagnosis and strict control of inflammatory activity have led to a dramatic reduction in the incidence of vision-threatening secondary complications. In the majority of cases, it has also been possible to resolve cystoid macular edema, which, if insufficiently controlled by systemic therapy, usually responds well to intravitreal dexamethasone implants.

Combined Laser Photocoagulation and anti-VEGF Injection Treatment in Radiation Retinopathy.

Background Radiation retinopathy can lead to a considerable reduction in visual acuity. We report 2 cases of radiation retinopathy in which a therapy with combined intravitreal anti-VEGF injection and laser photocoagulation (LPC) was used. Patient History Two 55-year-old women were referred to our clinic with radiation retinopathy, in the first case unilaterally after radiation due to endocrine orbitopathy (P1), in the second case in both eyes after palliative cerebral radiation for metastatic breast cancer (P2). Both cases were treated with combined intravitreal anti-VEGF injection and focal LPC. Therapy After the initiation therapy macular edema decreased considerably. One year after the beginning of therapy visual acuity increased in the first case from 0,05 to 0,16 p, in the second case from 0,5/0,4 to 0,6/0,5. Despite the positive response there is still continuing need for therapy in all three eyes. Conclusions Combined anti-VEGF injection and LT can reduce macular edema due to radiation retinopathy. The follow-up observation shows the necessity of long-term care.

Fulminant proliferative diabetic retinopathy in the non-photocoagulated eye following acute renal failure.

Management of diabetic retinopathy should follow more strict and aggressive rules in patients at risk for severe acute renal impairment. Such patients should be identified and possibly prophylactically laser treated to avoid the severe consequences demonstrated in this case report. A 34-year-old type 2 diabetes patient with a stabilized diabetic retinopathy developed acute and severe retinal decompensation within weeks after acute renal failure complicated his chronic stable renal impairment. Fluorescein angiographic and optical coherence tomographic illustrations of the rapid evolution of the retinal condition are presented. The patient had previously been treated with panretinal photocoagulation in his left eye. After 8 years of regular 6-monthly checked stability, he developed rapid-onset proliferative diabetic retinopathy and macular edema in his right eye within 3 months of his last ocular check-up. Fluorescein angiography showed neovessels and major ischemic areas. Emergency panretinal photocoagulation and a sub-Tenon's injection were necessary to achieve control of the situation with regression of neovessels and complete regression of macular edema. This case shows that it is imperative for nephrologists to be well informed about a patient's ocular situation in order to give timely information to the ophthalmologist who can intervene to protect the retina in case of renal failure. On the other hand, the ophthalmologist should be familiar with the renal function of his patient with renal impairment so that he can decide to perform prophylactic retinal panphotocoagulation that should be imperatively considered even without strict indications in patients with renal impairment at risk for further deterioration of renal function, in order to prevent such explosive ischemic and proliferative retinopathy putting vision at risk.

Current treatment modalities in diabetic macular edema.

Diabetic macular edema is the major cause of vision impairment in the diabetic population. Its treatment requires a multidisciplinary approach and the use of both systemic and local therapies. Current management includes the vascular endothelial growth factor inhibitors and steroids administered as intravitreal injections, as well as laser therapy and surgical treatment. Treatment modalities in diabetic macular edema have changed in recent years, after the introduction of vascular endothelial growth factor inhibitors, which are gradually replacing laser photocoagulation. All anti-VEGF agents tested so far showed efficacy in the treatment of diabetic macular edema. Due to structural differences, the therapeutic efficacy of different anti-VEGF products varies. According to recent results, 2,0 mg of aflibercept is more effective than 0,3 mg of ranibizumab in patients with lower visual acuity at baseline. Additionally, its superiority was shown in reducing the number of required macular laser photocoagulation procedures. This article provides a comprehensive overview of available therapeutic modalities based on laser photocoagulation, anti-VEGF agents, steroid injections or surgical interventions, and discusses their efficacy in patients with diabetic macular edema.

Intravitreal Ranibizumab for diabetic macular edema with prompt versus deferred laser treatment: 5-year randomized trial results.

OBJECTIVE: To report 5-year results from a previously reported trial evaluating intravitreal 0.5 mg ranibizumab with prompt versus deferred (for ≥24 weeks) focal/grid laser treatment for diabetic macular edema (DME). DESIGN: Multicenter, randomized clinical trial. PARTICIPANTS: Among participants from the trial with 3 years of follow-up who subsequently consented to a 2-year extension and survived through 5 years, 124 (97%) and 111 (92%) completed the 5-year visit in the prompt and deferred groups, respectively. METHODS: Random assignment to ranibizumab every 4 weeks until no longer improving (with resumption if worsening) and prompt or deferred (≥24 weeks) focal/grid laser treatment. MAIN OUTCOME MEASURES: Best-corrected visual acuity at the 5-year visit. RESULTS: The mean change in visual acuity letter score from baseline to the 5-year visit was +7.2 letters in the prompt laser group compared with +9.8 letters in the deferred laser group (mean difference, -2.6 letters; 95% confidence interval, -5.5 to +0.4 letters; P = 0.09). At the 5-year visit in the prompt versus deferred laser groups, there was vision loss of ≥10 letters in 9% versus 8%, an improvement of ≥10 letters in 46% versus 58%, and an improvement of ≥15 letters in 27% versus 38% of participants, respectively. From baseline to 5 years, 56% of participants in the deferred group did not receive laser. The median number of injections was 13 versus 17 in the prompt and deferral groups, including 54% and 45% receiving no injections during year 4 and 62% and 52% receiving no injections during year 5, respectively. CONCLUSIONS: Five-year results suggest focal/grid laser treatment at the initiation of intravitreal ranibizumab is no better than deferring laser treatment for ≥24 weeks in eyes with DME involving the central macula with vision impairment. Although more than half of eyes in which laser treatment is deferred may avoid laser for at least 5 years, such eyes may require more injections to achieve these results when following this protocol. Most eyes treated with ranibizumab and either prompt or deferred laser maintain vision gains obtained by the first year through 5 years with little additional treatment after 3 years.

The Narrative Medicine Approach in the Treatment of Diabetic Macular Edema: An Italian Experience.

The study retraces the healthcare pathway of patients affected by diabetic macular edema (DME) through the direct voice of patients and caregivers by using a "patient journey" and narrative method approach. The mapping of the patient's journey was developed by a multidisciplinary board of health professionals and involved four Italian retina centers. DME patients on intravitreal injection therapy and caregivers were interviewed according to the narrative medicine approach. Narratives were analyzed through a quali-quantitative tool, as set by the narrative medicine method. The study involved four specialized retina centers in Italy and collected a total of 106 narratives, 82 from DME patients and 24 from caregivers. The narratives reported their difficulty in identifying the correct pathway of care because of a limited awareness of diabetes and its complications. Patients experienced reduced autonomy due to ocular complications. In the treatment of diabetes and its complications, a multidisciplinary approach currently appears to be missing. DME reduces the quality of life of affected patients. The narrative medicine approach offers qualitative and emotional patient-guided information. The patient journey provides all of those involved in the management of DME with flowcharts to refer to, identifying the critical points in the healthcare journey of DME patients to improve the management of the disease.

EFFECT OF LASER PHOTOCOAGULATION ON MACULAR EDEMA ASSOCIATED WITH MACULAR HOLES.

PURPOSE: To report the outcomes of laser therapy to barricade eccentric full-thickness macular hole with associated cystoid macular edema. METHODS: We report two patients who developed an eccentric full-thickness macular hole with persistent cystoid macular edema after pars plan vitrectomy with and without internal limiting membrane peel for epiretinal membrane and the results of argon laser therapy. RESULTS: Barricade argon laser therapy was applied concentric to the full-thickness macular hole. Associated cystoid macular edema was noted to resolve within 1 to 3 months of therapy in both cases. CONCLUSION: Barricade laser therapy surrounding a macular hole can lead to resolution of associated cystoid macular edema. Pathogenic mechanisms to explain this favorable outcome are discussed.

Diabetic macular edema.

Diabetic retinopathy (DR) is a leading cause of preventable blindness world-wide. Diabetic macular edema (DME) is the most common cause of moderate vision loss in patients with diabetes. Although treatments for DME have improved significantly over the past decades, the burden of this disease remains high for patients and the healthcare system alike. The role of the primary care provider is critical in the prevention and prompt referral for management of DME.

[Focal laser photocoagulation in patients with diabetic macular edema after pretreatment with triamcinolone: 6-month results]. / Fokale Laserkoagulation des diabetischen Makulaödems nach vorbereitender Triamcinolon-Injektion: Ergebnisse nach 6 Monaten.

PURPOSE: The aim of this study was to analyse the 6-months results of focal laser photocoagulation (LC) applied after previous intravitreal injection of triamcinolone in eyes with non-proliferative diabetic retinopathy and clinically significant diabetic macular edema (DME). PATIENTS AND METHODS: 32 eyes (32 patients) with DME were included in this study. The number of laser treatments was: 1 in 14 eyes, 2 in 11, 3 in 4 eyes, 4 in 2 and 5 in 1 eye. The first LC was performed at a median of 17 days (mean: 20.3 +/- 6.5, range: 14 - 42 days) after triamcinolone injection. The second, if necessary, at a median of 32.5 (mean: 45.6 +/- 36.6, range: 22 - 144) days after injection. RESULTS: Mean logMAR visual acuity improved from baseline 0.56 (median: 0.48; 95 %-confidence interval [CI]: 0.41 - 0.71) to 0.47 (median: 0.40, CI: 0.36 - 0.58) after 1 month, and 0.49 (median: 0.40, CI: 0.38 - 0.61) at 3 months, and 0.47 (median: 0.40, CI: 0.36 - 0.58) at 6 months (p < 0.0065). Mean central retinal thickness (OCT-CRT) of optical coherence tomography measurements improved from 417 microm (median: 402.5, CI: 375 - 460 microm) at baseline to 301 microm (median: 280, CI: 271 - 331) at 1 month, 318 microm (median: 305, CI: 276 - 360) at 3 months and 348 microm (median: 329.5, CI: 299 - 398 microm) at 6 months (p = 0.0256). A second combination therapy was performed in 1 eye 3 months after the first injection. In 7 / 32 eyes (22 %) further injections and LC were indicated after 6 months. 8 / 32 eyes received newly prescribed antiglaucomatous medication and 4 / 32 eyes cataract surgery during follow-up. CONCLUSION: Focal LC after previous injection of triamcinolone results in an early and in most cases persistent functional improvement in eyes with DME.

[Diagnosis, treatment and monitoring of diabetic eye disease (Update 2019)]. / Diagnose, Therapie und Verlaufskontrolle der diabetischen Augenerkrankung (Update 2019).

Diabetes mellitus can cause diabetic retinopathy, diabetic macular edema, optic neuropathy, cataract or dysfunction of the eye muscles. The incidence of these defects correlates with disease duration and quality of metabolic control. Recommendations of the Austrian Diabetes Association for diagnosis, therapeutic procedures and requirements for adequate follow-up depending on stage of diabetic eye disease are summarized.

How do we evaluate the role of focal/grid photocoagulation in the treatment of diabetic macular edema?

Vascular endothelial growth factor inhibitors (anti-VEGF) have consistently demonstrated efficacy and safety and changed both the aim and perspectives of diabetic macular edema (DME) treatment. Hence, the present and future role of focal/grid laser photocoagulation in DME treatment has been subjected to some debate. However, extensive insight into technical advances in novel laser systems, treatment protocols of anti-VEGF trials and the functional impact of modern focal/grid photocoagulation is needed to evaluate the present and future role of photocoagulation in DME treatment. Across a wide range of clinical trials laser therapy was required as adjunctive/rescue treatment in approximately 20-50% of patients receiving anti-VEGF monotherapy for centre involving DME. Further, a lower retreatment rate and a more stable reduction in retinal thickness have been demonstrated in more studies. However, lacking information on the laser systems used, their technical specifications and protocols of application often complicates direct comparison of results in anti-VEGF trials. Hence, this paper aimed to provide an overview of the currently available data relevant to the potential role of focal/grid laser photocoagulation in DME treatment including a thorough overview of the current most commonly used laser systems. Results with subthreshold diode micropulse laser photocoagulation are intriguing and may offer a valuable option as adjunctive therapy to anti-VEGF treatment. However, more well-designed studies on combination therapy are warranted to determine the full potential of modern retinal photocoagulation systems. In conclusion, current data suggest that focal/grid laser therapy should still be an option for consideration as adjunctive therapy in many patients.

Update on treatments for diabetic macular edema.

PURPOSE OF REVIEW: Due to modest outcomes with macular laser, other treatment modalities for diabetic macular edema have been evaluated. Intravitreal triamcinolone acetonide, pars plana vitrectomy, oral protein kinase C inhibitors and, from more recently, anti-vascular endothelial growth factor therapy are reviewed. RECENT FINDINGS: Intravitreal triamcinolone acetonide may be more effective than laser. Intravitreal triamcinolone acetonide followed by laser may be more effective than intravitreal triamcinolone acetonide alone. Ruboxistaurin, a selective protein kinase C betainhibitor, reduced retinal vascular leakage in patients with diabetic macular edema and reduced the rate of sustained moderate visual loss in those with moderately severe to very severe non proliferative diabetic retinopathy. Several anti-vascular endothelial growth factor agents are under evaluation. Intravitreal pegaptanib sodium (0.3 mg) improved vision and reduced central retinal thickness compared to sham. Data on the other anti-vascular endothelial growth factor agents is limited, but there are promising results, with ranibizumab (0.5 mg) and bevacizumab reducing foveal thickness and improving visual acuity in some patients with diabetic macular edema. SUMMARY: There remains no proven intervention that consistently prevents or reverses visual loss from diabetic macular edema in all patients. A variety of promising new medical and surgical therapies are under investigation, but further research is required to determine their role alone or in combination.

Argon Laser Photocoagulation For Macular Edema In Branch Vein Occlusion.

The Branch Vein Occlusion Study is a multi-center, randomized, controlled clinical trial designed to answer several questions regarding the management of complications of branch vein occlusion. This report discusses the question, "Is argon laser photocoagulation useful in improving visual acuity in eyes with branch vein occlusion and macular edema reducing vision to 20/40 or worse?" One hundred thirty-nine eligible eyes were assigned randomly to either a treated or an untreated control group. Comparing treated patients to control patients (mean follow-up 3.1 years for all study eyes), the gain of at least two lines of visual acuity from baseline maintained for two consecutive visits was significantly greater in treated eyes (P = .00049, logrank test). Because of this improvement in visual acuity with argon laser photocoagulation of macular edema from branch vein occlusion, we recommend laser photocoagulation for patients with macular edema associated with branch vein occlusion who meet the eligibility criteria of this study.

Management of diabetic retinopathy: a systematic review.

CONTEXT: Diabetic retinopathy (DR) is the leading cause of blindness in the working-aged population in the United States. There are many new interventions for DR, but evidence to support their use is uncertain. OBJECTIVE: To review the best evidence for primary and secondary intervention in the management of DR, including diabetic macular edema. EVIDENCE ACQUISITION: Systematic review of all English-language articles, retrieved using a keyword search of MEDLINE (1966 through May 2007), EMBASE, Cochrane Collaboration, the Association for Research in Vision and Ophthalmology database, and the National Institutes of Health Clinical Trials Database, and followed by manual searches of reference lists of selected major review articles. All English-language randomized controlled trials (RCTs) with more than 12 months of follow-up and meta-analyses were included. Delphi consensus criteria were used to identify well-conducted studies. EVIDENCE SYNTHESIS: Forty-four studies (including 3 meta-analyses) met the inclusion criteria. Tight glycemic and blood pressure control reduces the incidence and progression of DR. Pan-retinal laser photocoagulation reduces the risk of moderate and severe visual loss by 50% in patients with severe nonproliferative and proliferative retinopathy. Focal laser photocoagulation reduces the risk of moderate visual loss by 50% to 70% in eyes with macular edema. Early vitrectomy improves visual recovery in patients with proliferative retinopathy and severe vitreous hemorrhage. Intravitreal injections of steroids may be considered in eyes with persistent loss of vision when conventional treatment has failed. There is insufficient evidence for the efficacy or safety of lipid-lowering therapy, medical interventions, or antivascular endothelial growth factors on the incidence or progression of DR. CONCLUSIONS: Tight glycemic and blood pressure control remains the cornerstone in the primary prevention of DR. Pan-retinal and focal retinal laser photocoagulation reduces the risk of visual loss in patients with severe DR and macular edema, respectively. There is currently insufficient evidence to recommend routine use of other treatments.

Considerations for management of patients with diabetic macular edema: Optimizing treatment outcomes and minimizing safety concerns through interdisciplinary collaboration.

Diabetes is a growing worldwide epidemic and a leading cause of blindness in working-age people around the world. Diabetic retinopathy (DR) and diabetic macular edema (DME) are common causes of visual impairment in people with diabetes and often indicate the presence of diabetes-associated preclinical micro- and macrovascular complications. As such, patients with DR and DME often display complex, highly comorbid profiles. Several treatments are currently available for the treatment of DME, including anti-vascular endothelial growth factor (VEGF) agents, which are administered via intravitreal injection. While the safety profiles of approved ocular anti-VEGF therapies have been reassuring, the high-risk nature of the DME patient population means that treatment must be carefully considered and a holistic approach to disease management should be taken. This requires multidisciplinary, collaborative care involving all relevant specialties to ensure that patients not only receive prompt treatment for DME but also appropriate consideration is taken of any systemic comorbidities to evaluate and minimize potentially serious safety issues.

A multimodal approach to diabetic macular edema.

Diabetic retinopathy is a common complication of uncontrolled diabetes. A complication is diabetic macular edema, which is the leading cause of blindness in patients with diabetic retinopathy. Historically, management of these conditions was laser photocoagulation with regulation of blood pressure, blood sugar, and cholesterol. The initial studies demonstrated that this treatment regimen prevented further visual deterioration but did not improve visual acuity. Novel studies identifying the presence of vascular endothelial growth factor (VEGF) in the eye with accompanying elucidation of diabetic pathophysiology allowed for the development of alternative therapies, namely antibodies against VEGF and corticosteroids. These two therapies revolutionized the management of diabetic macular edema by not only preventing vision loss, but also improving overall vision. In this review, we outline the major breakthroughs and underlying thought processes of the paradigm shifts that have occurred in management of these conditions. Further, we present how the evolving role of anti-inflammatory and anti-VEGF therapies, in a combinatorial approach, may provide further permutations to optimize treatment.

[Diagnosis, treatment and monitoring of diabetic eye control]. / Diagnose, Therapie und Verlaufskontrolle der diabetischen Augenerkrankung.

Diabetes mellitus can cause diabetic retinopathy, diabetic macular edema, optic neuropathy, cataract or dysfunction of the eye muscles. The incidence of these defects correlates with disease duration and quality of metabolic control. Recommendations of the Austrian Diabetes Association for diagnosis, therapeutic procedures and requirements for adequate follow up depending on stage of diabetic eye disease are summarized.

[Management of macular edema secondary to retinal vein occlusion]. / Prise en charge de l'œdème maculaire secondaire à une occlusion veineuse rétinienne.

BACKGROUND: In recent years, intravitreal injections have added to the treatment modalities available for macular edema (ME) secondary to retinal vein occlusion (RVO). This article aims to provide an update regarding the management of ME secondary to RVO. METHODS: A work group met in order to analyze the literature available on Embase/PubMed, regarding treatments for venous occlusion that have received market approval and are reimbursed in France. In total, 33 articles were selected. Consensus within the group for recommendations was based on this data from the literature review and clinical experience and was reported in this article. RESULTS: The management of ME secondary to branch retinal vein occlusion (BRVO) or central vein occlusion of the retina (CRVO) differs on a number of points. Methods of best practice were discussed separately for BRVO and CRVO, taking into account various ocular and associated parameters. DISCUSSION: Ranibizumab and dexamethasone implant are the first-line treatments for visual impairment due to ME secondary to RVO. The choice of either of these drugs may take into account various ocular and extraocular parameters. A change of treatment to one or the other or to laser may also be considered during follow-up.

Prophylaxis and therapy of aphakic cystoid macular edema.

Aphakic cystoid macular edema (ACME) can be a visually significant complication of modern cataract surgery. Prophylaxis of ACME is preferable to therapy of established ACME. Selection of the appropriate cataract operation, control of systemic factors, avoidance of topical catecholamines, control of intraocular inflammation and use of topical or systemic pharmacologic agents may play a role in the prevention of ACME. If it is proven that light toxicity influences the development of ACME, filters in operating microscopes or in intraocular lenses, cataract glasses, or contact lenses may be helpful. Once ACME is present, antiinflammatory therapy has been the main intervention, although its longterm value remains uncertain. Surgical approaches of unproven value have included photocoagulation, vitrectomy, and removal of an intraocular lens.