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Leukocoria is an aberration of the eyeball that interferes with normal reflection. It shows up as a white or gray pupillary reflex rather than the bright red or orange pupil of the other eye. Leukocoria can be brought on by a variety of ocular pathologies, with retinoblastoma being the most common. We present the case of a 17-month-old guy who had unilateral leukocoria and whose orbital MRI was ordered on the basis of retinoblastoma suspicion. The results, however, were more suggestive of Coats disease than retinoblastoma. Telangiectasia and exudate, which frequently afflict males’ unilateral eyes, are the hallmarks of Coats’ illness. Depending on the stage of the disease, there are differences in its severity, course, and outlook. It is crucial to get therapy and a diagnosis for retinal problems as soon as possible. Although uncommon, there are still many people who are not familiar with Coats’ disease. This paper aims to describe imaging findings in Coats' disease.

Abstract Background Coats’ disease was originally defined as a unilateral idiopathic exudative retinopathy in young males, characterized by abnormal retinal vascular telangiectasia with intraretinal and subretinal lipid exudation. The retinal detachment is usually exudative. Herein, we describe a case of rhegmatogenous retinal detachment with detectable retinal break in a patient with Coats’ disease. Case presentation A 15-year-old Indo-Aryan male patient presented with sudden painless diminution of vision in his right eye of 4 days duration. Upon examination, the anterior segment in both eyes and left fundus was within normal limits. Dilated fundus evaluation of the right eye revealed telangiectasia of the retinal vessels, with subretinal exudation in superotemporal and superonasal quadrants and presence of subretinal fluid in the superotemporal area extending into fovea. There was also presence of single flap horseshoe tear in the superotemporal quadrant at around the 10 o’clock position in the equatorial region, with no secondary changes. The retina was reattached with encircling band buckle combined with vitrectomy and silicon oil tamponade. Seven months post vitrectomy, lenticular opacification developed, for which he underwent silicon oil removal, along with lens aspiration and implantation of foldable intraocular lens. Over the period of 1 year, his best corrected visual acuity improved from 6/60 to 6/18 in the affected eye at the last follow-up visit. The recovery was uneventful following the subsequent surgery. Conclusion Coats’ disease has a remarkable diversity in clinical presentation and morphology. The disease can also present with an underlying break, which may not be attributed to any iatrogenic modality. The treatment modalities in coats’ disease should be tailored individually due to the low incidence of the disease and the great variation in severity upon presentation. Prompt management restores the best possible anatomical outcome and maintains good vision.

An 8-year-old child with Stage 3A Coats' disease and severe submacular lipid exudation was initially treated with intravitreal injections of bevacizumab followed by triamcinolone. The exudative retinal detachment was then treated by scleral buckling, cryotherapy of persistent telangiectasia, and subretinal fluid drainage. The residual telangiectasia on the reattached retina was finally ablated by photocoagulation. The patient had a near-total resolution of submacular hard exudates without macular fibrosis. The peripheral telangiectasia and exudative detachment also regressed, with the sustained recovery of excellent visual acuity.

ObjectiveTo investigate the associations of macular microvascular abnormalities with the characteristics and progression of macular edema or exudation in Coats' disease, toward an updated understanding of possible risk factors for macular edema or exudation.MethodsTwenty-six eyes (26 patients) with Coats' disease and macular edema or exudation underwent multimodal imaging and were followed for 18 months. The eyes were classified according to their outcomes (refractory or improved). Macular capillary affections were assessed by optical coherence tomography angiography (OCTA) and fluorescein angiography (FA). Histopathological analysis of the macular region of an additional enucleated eye was performed.ResultsOCTA revealed telangiectasia in the deep capillary plexus (DCP) in 76.9% and the superficial capillary plexus (SCP) in 34.6% of 26 eyes with macular edema or exudation of Coats' disease, exceeding the rate detected by FA (21.4%). Eyes with intraretinal cystoid spaces/exudates of the macula presented higher presence of telangiectasia in the SCP (57.1% with vs. 8.3% without, X2 = 6.801, P = 0.009) and DCP (92.9 with vs. 58.3% without, X2 = 4.338, P = 0.037). The parafoveal vessel densities (VDs) and fractal dimension in the SCP and DCP were lower in affected eyes than in contralateral eyes (all P < 0.001). The VD in SCP (P = 0.009) and DCP (P = 0.010) were lower in refractory group than in improved group. Dilated capillaries with incomplete vessel walls and adjacent inflammatory cells were detected in the neuroretina of the macula in histopathological specimen.ConclusionsMacular capillary abnormalities, including telangiectasia and VD loss, were positively detected in eyes with macular edema or exudation of Coats' disease. Intraretinal cystoid spaces/exudates of the macula, rather than subretinal exudates, may be related to macular telangiectasia. VD losses in the SCP and DCP may be risk factors for refractory macular edema or exudation.

Mohamed Nowara,1,2 Yousef A Fouad,1,3 Ihab Abdel Aziz,1,4 Ahmed M Habib,1,3 Mariam Al-feky,1,3 Hisham Hassan1 1Al Mashreq Eye Center, Cairo, Egypt; 2Department of Ophthalmology, Electricity Hospital, Cairo, Egypt; 3Department of Ophthalmology, Ain Shams University Hospitals, Cairo, Egypt; 4Memorial Institute for Ophthalmic Research, Giza, EgyptCorrespondence: Yousef A FouadDepartment of Ophthalmology, Ain Shams University Hospitals, Ramses Street, 11517 Abbasiya, Cairo, EgyptTel +201063781237Email yousef.a.fouad@gmail.comBackground: Coats’ disease is a rare entity with retinal vascular telangiectasia that can progress to exudative retinal detachment, neovascular glaucoma, and a blind painful eye requiring enucleation. Despite recent therapeutic advances decreasing the need for enucleation, no consensus exists about the optimum management of exudative Coats’ disease. The use of intravitreal anti-vascular endothelial growth factor agents as an adjunct to ablation therapy has been shown to achieve favorable outcomes, but some reports suggest an increased incidence of vitreoretinal (VR) fibrosis and tractional retinal detachment (TRD).Methods: We retrospectively reviewed records of patients presenting with exudative Coats’ disease (stages 2 and 3) from April 2016 till November 2020. Extracted data included clinical and radiological assessment, stage (Shields’ classification), interventions, and follow-up.Results: Sixteen eyes were included in the final analysis, of which 4 (25%) were stage 2 and 12 (75%) were stage 3. All eyes underwent intravitreal ranibizumab injection combined with ablation therapy, 14 (87.5%) underwent cryotherapy, 4 (25%) underwent laser ablation, 3 (18.75%) underwent external subretinal fluid drainage, and 3 (18.75%) underwent buckle or vitrectomy surgery. After a median follow-up of 16 months, 11 eyes (68.75%) had complete resolution, 4 (25%) had incomplete resolution, and only one (6.25%) progressed but did not require enucleation. Three eyes (18.75%) developed VR fibrosis, but none progressed to TRD.Conclusion: Combining intravitreal ranibizumab injection with ablation therapy is effective in managing exudative Coats’ disease. External drainage should be preserved for when ablation therapy is not feasible. Future prospective trials with pre-defined outcomes are required.Keywords: Coats’ disease, Leber’s miliary aneurysms, exudative retinal detachment, vascular endothelial growth factor, cryotherapy

Background: Coats' disease, described by George Coats in the early 1900s, is an idiopathic unilateral retinal vascular abnormality with exudation occurring in young males. It is characterized by retinal telangiectasia with intraretinal or subretinal exudation. Coats' disease is mostly diagnosed in the first to the second decade of life, with a common presentation of leukocoria. Younger patients have a more severe presentation and are associated with poor visual prognosis. Management of Coats' disease varies from observation, cryotherapy with anti-VEGFs (Vascular Endothelial Growth Factor), and surgery to enucleation. The mode of treatment depends on the age of presentation, the severity of the disease, and the stage of the disease. Through this video, we describe the clinical features, pathology, and surgical management of a 2-year-old child with grade 3B of Coats' disease. Purpose: To demonstrate successful surgical management of grade 3B of Coats' disease in a 2-year-old boy. Synopsis: Coats' disease mostly presents with a diagnostic dilemma due to its varied presentation. Early detection and treatment are the keys to salvaging the eye as well as the vision, hence, avoiding dreadful complications such as neovascular glaucoma or phthisis bulbi. We demonstrate successful surgical management of a child who presented with grade 3B of Coats' disease. Highlights: Through this video, we aim to describe the clinical features, pathology, and surgical management of a 2-year-old child with grade 3B of Coats' disease. Combination of external drainage with vitrectomy, challenges faced, and the importance of visual rehabilitation postoperatively. Video Link: https://youtu.be/0obpVTOkKKs

Purpose: Coats' disease is associated with poor outcomes, and there are limited studies on long-term outcomes of Coats' disease. The purpose of our study is to identify various predictive factors to help in prognosticating the treatment outcomes in advanced Coats' disease in children.Methods: This is a retrospective case series from a single tertiary eye care center of children (<18 years) diagnosed with coat's disease. Sixty-seven patients with Coat's disease were identified from the medical records from 2009 to 2020. Patients' demographic data, clinical presentation, stage, extent of involvement, detailed treatment history, clinical sequelae post-treatment (including complications and anatomical and functional outcomes) were noted. Binary logistic regression was performed to correlate the predictive factors for anatomical and functional improvement.Results: Of the 67 patients, 51 eyes of 51 patients were included in the study. The male to female proportion was 2.2. Mean age at presentation was 4.98 ± 3.55 years (range: 2 months-15 years). Mean duration of follow-up was 31.53 ± 26.38 months. Overall, our globe salvage rate was 92.2%. We found that vitreoretinal fibrosis (P < 0.001), subretinal gliosis (P < 0.001), vitreous hemorrhage (P = 0.02), tractional or combined retinal detachment (P < 0.001), foveal scar (P < 0.006), and cataract (P < 0.001) to be important factors to affect the outcome.Conclusion: Advanced stage of presentation (stage 3B and above), diffuse involvement, cataract, vitreoretinal fibrosis (preretinal and subretinal), vitreous hemorrhage, tractional or combined retinal detachment, and anterior hyaloid proliferation are poor prognostic factors for globe salvage in advanced disease. Subretinal gliotic nodule or scar and lack of visual rehabilitation suggest poor functional outcomes. [ABSTRACT FROM AUTHOR]

Background: Coats' disease was originally defined as a unilateral idiopathic exudative retinopathy in young males, characterized by abnormal retinal vascular telangiectasia with intraretinal and subretinal lipid exudation. The retinal detachment is usually exudative. Herein, we describe a case of rhegmatogenous retinal detachment with detectable retinal break in a patient with Coats' disease.Case Presentation: A 15-year-old Indo-Aryan male patient presented with sudden painless diminution of vision in his right eye of 4 days duration. Upon examination, the anterior segment in both eyes and left fundus was within normal limits. Dilated fundus evaluation of the right eye revealed telangiectasia of the retinal vessels, with subretinal exudation in superotemporal and superonasal quadrants and presence of subretinal fluid in the superotemporal area extending into fovea. There was also presence of single flap horseshoe tear in the superotemporal quadrant at around the 10 o'clock position in the equatorial region, with no secondary changes. The retina was reattached with encircling band buckle combined with vitrectomy and silicon oil tamponade. Seven months post vitrectomy, lenticular opacification developed, for which he underwent silicon oil removal, along with lens aspiration and implantation of foldable intraocular lens. Over the period of 1 year, his best corrected visual acuity improved from 6/60 to 6/18 in the affected eye at the last follow-up visit. The recovery was uneventful following the subsequent surgery.Conclusion: Coats' disease has a remarkable diversity in clinical presentation and morphology. The disease can also present with an underlying break, which may not be attributed to any iatrogenic modality. The treatment modalities in coats' disease should be tailored individually due to the low incidence of the disease and the great variation in severity upon presentation. Prompt management restores the best possible anatomical outcome and maintains good vision. [ABSTRACT FROM AUTHOR]

Background: The long-term treatment outcomes in Coat's disease – particularly in the era of newer pharmacotherapies such as anti-vascular endothelial growth factor (VEGF) agents and depot steroids – are poorly understood. Aim: To describe the clinical features and treatment outcomes of 148 eyes with Coats' disease assessed in a referral centre over 30 years. Materials and methods: We conducted a retrospective chart review of patients diagnosed with Coats' disease between 1 June 1987 and 31 July 2017. The demographic, clinical and treatment data were collected and long-term functional and anatomical outcomes were analysed based on the treatment either with conventional therapy (cryo/laser) or along with adjuvants like intravitreal steroids or anti-VEGFs. Results: The mean age at presentation was 15.22 years (median 11). Familial exudative vitreoretinopathy was the most common referral diagnosis, 76/148 (51.5%), followed by Coats' disease, 37/148 (25%), and retinoblastoma, 35/148 (23.6%). Stage 3B was most common at presentation (31.8%), followed by 2B (22.3%) and 2A (16.9%). A total of 107 patients were treated either with conventional therapy or in combination with adjuvants. The mean follow-up period was 24.95 months. The visual acuity improved from baseline logMAR 2.17 (Snellen-20/2958) to logMAR 1.88 (Snellen-20/1517) at final follow-up (p = 0.004). The improvement in visual acuity was better when the presenting BCVA was <1 logMAR (Snellen 20/200), p = 0.004. No statistically significant change in BCVA was noted between conventional and adjuvant groups, p = 0.5. However, the final anatomical outcome was good in 78/99 (78.8%) in the conventional group and 45/49 (91.8%) in the adjuvant group, respectively (p = 0.046). Conclusion: In this series of patients with Coats' disease over three decades, the use of intravitreal steroids or anti-VEGFs as adjuvants resulted in better anatomical outcomes. A better baseline visual acuity, lower stage of the disease, and older age at presentation were found to be the factors leading to favourable visual outcomes. Summary: In the current series of 148 eyes with Coats' disease, adjuvant treatment with intravitreal steroids or anti-VEGFs resulted in better outcomes as compared with conventional cryotherapy or laser photocoagulation alone. Patients with Coats' disease who had presented with better visual acuity at baseline, lower stage of the disease and older age had better final visual outcomes. [ABSTRACT FROM AUTHOR]

Purpose: To assess the demographic and treatment features of pediatric patients of Coats' disease with retinal cyst using wide-angle FA.Design: A retrospective, hospital based, cross-sectional study.Participants: Pediatric patients of Coats' disease underwent wide-angle FA.Methods: A retrospective review of pediatric patients of Coats' disease who underwent wide-angle FA at a single center from January 2015 to July 2020. Demographic and treatment features were compared between patients with or without retinal cyst.Main Outcome Measures: Demographic and treatment outcomes.Results: There were 123 pediatric Coats' patients in our study, and 18.70% (23/123) of the patients developed complications with retinal cyst, 73.9% (17/23) of the retinal cysts were located in the inferior-temporal quadrant and 82.6% (19/23) of the retinal cysts were located in the peripheral retina anterior to the vortex veins. Compared with patients without retinal cyst, patients with retinal cyst had more clock-hours of telangiectasia on FA (7.32 vs. 5.41, p = 0.031), and may need more total treatments (7.47 vs. 3.53, p = 0.023) including laser photocoagulation (4.08 vs. 2.31, p = 0.019) or intravitreal anti-VEGF (3.13 vs. 2.23, p = 0.039), and also required a longer time for telangiectasia resolution (22.33 vs. 18.53 months, p = 0.043).Conclusion: Pediatric patients with Coats' disease complicated by retinal cyst presented with more clock-hours of telangiectasia on FA and needed more total treatments and longer time for telangiectasia resolution.

An 8-year-old child with Stage 3A Coats' disease and severe submacular lipid exudation was initially treated with intravitreal injections of bevacizumab followed by triamcinolone. The exudative retinal detachment was then treated by scleral buckling, cryotherapy of persistent telangiectasia, and subretinal fluid drainage. The residual telangiectasia on the reattached retina was finally ablated by photocoagulation. The patient had a near-total resolution of submacular hard exudates without macular fibrosis. The peripheral telangiectasia and exudative detachment also regressed, with the sustained recovery of excellent visual acuity. [ABSTRACT FROM AUTHOR]

Purpose: To illustrate the spectrum of clinical and imaging features in patients with unilateral Coats’ disease at baseline and in response to treatment with laser, intravitreal bevacizumab, and regional steroids. Observations: Telangiectasias, macular exudates, and vascular leakage were present in all 3 patients included in this series. After treatment with laser and bevacizumab, OCT angiography findings included an anomalous foveal vascular loop and chorioretinal anastomoses. Choroidal flow voids appeared to improve after intravitreal bevaziumab and laser treatment in 2 patients with OCT angiography obtained at follow up. A-scan axial lengths in affected eyes were 1.5–1.8 mm smaller than fellow eyes. Conclusions and importance: OCT angiography is a non-invasive tool that can be a useful adjunct to multimodal imaging studies in the management of Coats’ disease. Improved vascular density following anti-VEGF injection suggests a possible role of the choroidal vasculature in this retinal vascular pathology.

AIM: To elucidate the association of treatment modality to vitreoretinal fibrosis and traction retinal detachment (TRD) in Coats’ disease. METHODS: A PubMed search for Coats’ disease with included studies describing eyes with clinical features and treatment course of Coats’ disease. Binary logistic regression with fibrosis at presentation and treatment type as independent variables was performed to determine predictors of TRD historically (since 1921) and in the anti-vascular endothelial growth factor (VEGF) era (since 2007). Odds ratios (OR) with 95% confidence intervals (CI) reported. RESULTS: Of 175 articles described 1183 eyes. Vitreoretinal fibrosis increased from presentation (5.4%) to follow-up (15.5%) and TRD increased from 0.44% to 3.9% at follow up. Laser was protective against vitreoretinal fibrosis (OR 0.6, 95%CI 0.4-0.9) but TRD was borderline (OR 0.6, 95%CI 0.3-1.1). Cryotherapy showed a higher association with TRD (OR 1.9, 95%CI 1.0-3.7) than with vitreoretinal fibrosis (OR 0.8, 95%CI 0.5-1.2). Similarly, intravitreal anti-VEGF alone was not associated with fibrosis (OR 1.1, 95%CI 0.6-1.8) nor TRD (OR 1.1, 95%CI 0.5-2.6) but the combination of laser and anti-VEGF therapy was protective [Fibrosis: 0.1 (0.03, 0.35); TRD: 0.05 (0.01, 0.23)] compared to anti-VEGF plus cryotherapy (P

To report the use of intravitreal dexamethasone implant and its short-term efficacy in a pediatric patient with Coats' disease. A 7-year-old patient was referred to the clinic because of decreased visual acuity in the right eye of 10-month duration. Best-corrected visual acuity was 20/400. Funduscopic examination showed evidence of Stage 2B Coats' disease. The patient underwent treatment with laser photocoagulation, intravitreal injection of bevacizumab, and intravitreal injection of dexamethasone implant. During the 10-month follow-up, there was no evidence of side effects. Anatomical resolution was evident on funduscopic examination and macular optical coherence tomography, with best-corrected visual acuity being 20/60. Intravitreal dexamethasone implant was effective during the 10-month follow-up; providing improvement in visual acuity without any side effects.

To report the use of intravitreal dexamethasone implant and its short-term efficacy in a pediatric patient with Coats' disease. A 7-year-old patient was referred to the clinic because of decreased visual acuity in the right eye of 10-month duration. Best-corrected visual acuity was 20/400. Funduscopic examination showed evidence of Stage 2B Coats' disease. The patient underwent treatment with laser photocoagulation, intravitreal injection of bevacizumab, and intravitreal injection of dexamethasone implant. During the 10-month follow-up, there was no evidence of side effects. Anatomical resolution was evident on funduscopic examination and macular optical coherence tomography, with best-corrected visual acuity being 20/60. Intravitreal dexamethasone implant was effective during the 10-month follow-up; providing improvement in visual acuity without any side effects. [ABSTRACT FROM AUTHOR]

The paper presents a case of a 58-year-old woman with hypertension, who suffered unilateral visual loss due to vitreous hemorrhage in the right eye. Clinical examination, fluorescein and indocyanine green angiography and ultrasonography were performed. Following vitreous hemorrhage resorption, fundoscopy showed atypical retinal vessels located temporally to the macula. Fluorescein and indocyanine green angiograms confirmed atypical vessels in the peripheral retina. Diagnosis of adult onset Coats' disease was made for the atypical lesions and treatment with retinal laser photocoagulation was administered to prevent further hemorrhage. Coats' disease, most commonly seen in male children, is rarely diagnosed in the adult female population. In adults, the course of the disease tends to be less severe than in children. When investigating the cause of vitreous hemorrhage, we have to take pathological neovascularization typical for Coat's disease into consideration as a differential diagnosis. Fundoscopy of the peripheral retina is an examination that can aid in diagnosis after absorption of vitreous hemorrhage. Wide-field angiography confirms the diagnosis, enables precise localization of pathological vessels and their activity, and helps determine treatment effectiveness. [ABSTRACT FROM AUTHOR]

Purpose: To report the first use the intravitreal anti-VEGF brolucizumab for the treatment of macular exudates and edema in a patient with Coats’ disease. Observations: A 9-year-old boy was referred with a decrease in vision in the right eye. Visual acuity was 20/400 OD on presentation, and examination was remarkable for peripheral telangiectasias, exudates, microaneurysms, macular edema, and an inferior exudative retinal detachment. A diagnosis of Stage 3A2 Coats’ disease was made, and the patient was treated with intravitreal bevacizumab as well as peripheral diode laser. However, on follow up, there was persistence of subretinal fluid accompanied by a decrease in visual acuity. The patient was then treated with intravitreal brolucizumab. Post injection visual and anatomical improvements were significant with complete resolution of macular edema within two weeks. Throughout the 5 months of follow up, there has been no re-accumulation of fluid and no further required therapy. Conclusions and Importance: Intravitreal brolucizumab was effective for the treatment of retinal edema and exudates in Coats’ disease.

Retinal detachment is rare in children. It is different from adults in terms of its presentation, etiologies, and management. It remains a challenge to manage these cases due to delayed presentation and mostly, bilateral involvement. The various etiologies, their management and the surgical tips that is specific to each type of retinal detachment is discussed here.

Paediatric retinal detachment (PRD) is an uncommon and challenging disease; it differs from adult detachments in etiology, anatomical characteristics, management and prognosis. PRDs can be particularly challenging, even for the most expert paediatric surgeons due to the higher prevalence of total retinal detachments, late diagnosis and bilateral involvement with respect to those which occur in adulthood. Moreover, the anatomical success, when achieved, is frequently not related to a functional recover. Postsurgical adverse events, refractive errors and amblyopia may additionally undermine the final outcome. Up to date there are few reviews regarding the approach of retinal detachment in children, mainly dealing with rhegmatogenous retinal detachment. In this review, rhegmatogenous, retinopathy of prematurity-related and Coats’-related PRDs were considered. The available literature from the last decades were reviewed and summarized. Epidemiology, etiology and clinical presentation, together with therapeutic approaches and outcomes have been reviewed and discussed.

Background: Coats' disease is characterized by vascular hyperpermeability, oedema and accumulation of exudates related to impairment of retinal vascular function. The background for the development of the disease is unknown, but it is likely that the study of diameter changes of retinal vessels may contribute to understanding the pathophysiology of the disease. Methods: In seven patients with unilateral Coats' disease (mean age = 34.7 years, range: 11–69 years), the baseline diameter and reactivity of retinal vessels during an increase in the arterial blood pressure by isometric exercise and in the metabolism by flicker stimulation were measured on video recordings of the retina obtained with the Dynamic Vessel Analyzer. Results: The baseline diameter of retinal vessels was larger in the affected than in the unaffected eyes which was significant for the arterioles (p = 0.02), but not for the venules (p = 0.15). During an increase in the arterial blood pressure induced by isometric exercise, the normal contraction of arterioles was absent in both eyes (p > 0.7), whereas there was a significant dilatation of the venules in the unaffected eyes (p = 0.04). Stimulation with flickering light induced normal dilatation of retinal vessels in both affected and unaffected eyes. Conclusion: Unilateral Coats' disease is accompanied by vasodilatation in the affected eye but impaired pressure autoregulation in both eyes. A further investigation of the disease should include an elucidation of the background for dilatation of retinal vessels in affected eyes and whether impaired pressure autoregulation can be found in vessels elsewhere in the body. [ABSTRACT FROM AUTHOR]

Retinal vein occlusion (RVO) is a differential diagnosis for Coats’ disease due to retinal arterial Leber’s aneurysms. Occasionally, RVO shows a Coats-like appearance. The differential diagnosis between Coats’ disease and RVO is essential for clinical therapy, especially for those obsolete RVOs with collateral vessels and without retinal hemorrhage. In this case report, we describe and discuss the imaging characteristics of bilateral RVO-simulated Coats’ disease with tortuous retinal arterioles and its prognosis after anti-vascular endothelial growth factor therapy, which will be beneficial for its definite diagnosis and aid further investigation.

Purpose: To report use of widefield fluorescein angiography (FA) for targeted therapy of Coats’ disease and familial exudative vitreoretinopathy (FEVR). Study design: Retrospective, noncomparative, consecutive case series. Material and Methods: Patients diagnosed with Coats’ disease or FEVR and evaluated with widefield FA, treated with indirect laser from January 1, 2003 to December 31, 2012. Visual acuity (VA) and anatomic status of eyes was evaluated. Results: 17 eyes were treated for Coats’ disease, and 25 eyes treated for FEVR. Mean VA in the Coats’ group was 20/384 at baseline, and mean VA at the last followup was 20/258. In the FEVR group, mean VA was 20/100 at baseline, and 20/358 at last follow-up. None of the Coats’ eyes required enucleation or additional surgical intervention. Two of the eyes with FEVR required surgery due to advanced disease. Conclusion: Widefield angiographyguided laser therapy in Coats’ disease and FEVR led to visual and anatomic stability in these diseases.

Objectives: The aim of this study was to evaluate the clinical features, follow-up and treatment results of patients diagnosed with Coats’ disease. Materials and Methods: Medical records of 27 patients diagnosed with Coats’ disease in our clinic were reviewed retrospectively. All patients underwent complete ophthalmological examination and fundus photography was taken. Disease stage and treatment methods used were recorded. Results: Twenty-seven eyes of 27 patients were included in the study. Mean age was 9.03 years; 21 patients were male and 6 were female. Three patients were older than 18 years old. Based on the Shields classification, 1 (3.7%) eye was stage 2A, 4 (14.8%) eyes were stage 2B, 6 (22.2%) were stage 3A1, 3 (11.1%) were stage 3A2, 1 (3.7%) was stage 3B, 4 (14.8%) were stage 4 and 8 (29.6%) were stage 5. Fourteen patients underwent treatment, 12 of whom had combined therapy. The most common treatment modalities were laser photocoagulation and cryotherapy. Encircling band was done in one patient and pars plana vitrectomy in 3 patients. Enucleation was done in 5 patients. Conclusion: Coats’ disease is a chronic disease and main goal of treatment is to eliminate the vascular anomalies and their complications using repetitive combination therapies. Treatment in the early stages can lead to functional success, and in advanced stages can result in a salvageable eye.

Coats' Disease is an idiopathic condition of the eye affecting young children although it can be seen in adults. Most patients present early in life with unilateral decreased vision, strabismus or leukocoria. The most important differential diagnosis is unilateral retinoblastoma. In this study we report a case of coat's disease in an young girl, and evaluate histopathological and clinical findings.

Surgical management of advanced coats' disease has always been a challenge to a surgeon. Various different techniques have been tried. With the advancement in surgical instruments and machines, surgeons are now modifying older surgical techniques. We have tried a novel minimally invasive surgical technique for the management of advanced coats' disease patient having high bullous exudative retinal detachment. Intravitreal injection of Bevacizumab at the end of the surgery has a role in such cases.

A 30-year-old female presented with macular edema and discoid exudation at the posterior pole. Diffuse vascular sheathing was observed at the peripheral retina. Fluorescein angiography revealed multiple microaneurysms at the posterior pole and leakage from the peripheral vessels. Two monthly intravitreal bevacizumab led to minimal improvement, and resolution of macular edema was achieved by an additional intravitreal triamcinolone. The findings at the posterior pole resembled those of Leber's miliary aneurysm. However, this case also demonstrated a peculiar vascular sheathing at the periphery and showed response to triamcinolone, which are evidences for an inflammatory condition.

Purpose: To investigate the associations of cytokine concentrations in aqueous humour with the severity of retinal vascular abnormalities, exudation and fibrosis in patients with Coats' disease. Methods: Aqueous humour samples were collected in 23 paediatric patients (23 eyes) with Coats' disease and six age‐matched control patients (six eyes) with congenital cataract in this cross‐sectional, case–control study. Through Cytometric Bead Array technology, six angiogenic, inflammatory and fibrotic cytokines were measured for their concentrations in aqueous humour. Ophthalmologic characteristics including retinal vessel abnormalities, exudation and fibrosis of Coats' disease were also clinically evaluated for analysis. Results: The aqueous levels of vascular endothelial growth factor (VEGF) (p = 0.006) and monocyte chemoattractant protein‐1 (MCP‐1) (p < 0.001) were significantly higher in the Coats' disease group than in the control group. The concentrations of angiogenin were peaked in eyes with first‐grade vessels tortuosity (p < 0.001), and also positively correlated with the severity of retinal capillary abnormalities (r = 0.910, p < 0.001). The concentrations of MCP‐1 (r = 0.966, p < 0.001) and VEGF (r = 0. 765, p = 0.002) were significantly correlated with the extent of retinal exudation. The aqueous humour transforming growth factor‐β (TGFβ) concentrations were higher in eyes with retinal fibrosis than in non‐fibrotic eyes with Coats' disease (p = 0.004). Conclusion: In Coats' disease, angiogenin may act as a potential biomarker for retinal vascular abnormalities. The concentrations of VEGF and MCP‐1 may positively correlate with the severity of retinal exudation. [ABSTRACT FROM AUTHOR]

Purpose: To review and summarize the recent progress in the diagnosis and treatment of Coats' disease. Methods: Literature was collected from Web of Science, Medline and Pubmed, through searching of these keywords: "Coats' disease", "diagnosis" and "treatment". Results: Coats' disease is characterized by idiopathic leaky retinal vascular telangiectasia and microvascular abnormalities often accompanied by intraretinal or subretinal exudation and retinal detachment. Neovascular glaucoma and phthisis bulbi often occur in advanced cases. Coats' disease has significant diversity in terms of its clinical presentation and morphology. Anti-VEGF therapy combined with laser photocoagulation for early Coats' disease and anti-VEGF therapy combined with minimally invasive vitrectomy for advanced Coats' disease can achieve good efficacy. Conclusion: Early diagnosis and timely treatment based on clinical stage are critical to retaining the patient's visual function. Patients should be aware that close long-term follow-up is necessary. [ABSTRACT FROM AUTHOR]

Retinoblastoma mimickers, or pseudoretinoblastoma, are conditions that show similarities with the pediatric cancer retinoblastoma. However, false-positive retinoblastoma diagnosis can cause mistreatment, while false-negative diagnosis can cause life-threatening treatment delay. The purpose of this study is to identify the MR imaging features that best differentiate between retinoblastoma and the most common pseudoretinoblastoma diagnoses: Coats’ disease and persistent fetal vasculature (PFV). Here, six expert radiologists performed retrospective assessments (blinded for diagnosis) of MR images of patients with a final diagnosis based on histopathology or clinical follow-up. Associations between 20 predefined imaging features and diagnosis were assessed with exact tests corrected for multiple hypothesis testing. Sixty-six patients were included, of which 33 (50%) were retinoblastoma and 33 (50%) pseudoretinoblastoma patients. A larger eye size, vitreous seeding, and sharp-V-shaped retinal detachment were almost exclusively found in retinoblastoma (p < 0.001–0.022, specificity 93–97%). Features that were almost exclusively found in pseudoretinoblastoma included smaller eye size, ciliary/lens deformations, optic nerve atrophy, a central stalk between optic disc and lens, Y-shaped retinal detachment, and absence of calcifications (p < 0.001–0.022, specificity 91–100%). Additionally, three newly identified imaging features were exclusively present in pseudoretinoblastoma: intraretinal macrocysts (p < 0.001, 38% [9/24] in Coats’ disease and 20% [2/10] in PFV), contrast enhancement outside the solid lesion (p < 0.001, 30% [7/23] in Coats’ disease and 57% [4/7] in PFV), and enhancing subfoveal nodules (38% [9/24] in Coats’ disease). An assessment strategy was proposed for MR imaging differentiation between retinoblastoma and pseudoretinoblastoma, including three newly identified differentiating MR imaging features.