Eales Disease Research Articles

Steroid Sparing Immunosuppression as a Novel Approach in the Treatment of Eales Disease with Neurological Complications: A Series of Two Cases

Steroid Sparing Immunosuppression as a Novel Approach in the Treatment of Eales Disease with Neurological Complications: A Series of Two Cases

Evaluation of Aetiological Factors of Vasculitis Retinae in a Tertiary Specialized Eye Hospital in Bangladesh

Vasculitis retinae (VR) is a potentially sight threatening inflammatory condition of eye which may occur as isolated intraocular disorder or in association with various systemic diseases. It may occur as an idiopathic condition, as a manifestation of infections or non-infectious auto immune disorders. Impractical or unthoughtful approach increases the burden of treatment cost because of long investigation list. This prospective, observational study was carried out at the National Institute of Ophthalmology & Hospital (NIOH), a tertiary specialized eye hospital, Dhaka, Bangladesh, between March 2020 and February 2021, which aims to determine the common aetiology of VR patients. A total of 45 patients of VR were prospectively examined and investigated thoroughly. The mean age of mean age of onset of disease was 29.5 years where 82.2% were male. Among the 72 eyes of 45 patients of vasculitis retinae, the most common aetiology was Eales disease (52.8%), followed by tubercular VR (33.3%). Other causes of VR found in the study were toxoplasmosis (8.3%), systemic lupus erythematosus (SLE) (2.8%) and sarcoidosis (2.8%). The most common cause of VR was found Eales disease followed by Tubercular VR. Although the study has the limitations of being cross sectional and hospital based, the information from the study can be used by the ophthalmologist while dealing with the VR patients. CBMJ 2024 July: vol. 13 no. 02 P: 192-197

THE EFFECTIVENESS OF INTRAVITREAL ANTI-VEGF INJECTION IN EALES' DISEASE: A CASE REPORT

Introduction : Eales’ Disease (ED) is an idiopathic inflammatory retinal vasculitis, affecting retinal veins of small and large caliber that results in extraretinal neovascularization with vitreous hemorrhage. The etiopathogenesis of ED is poorly understood. However, many studies have attributed inflammation as a response to mycobacterium tuberculosis. Treatment of this disease can be done with corticosteroids, anti-VEGF therapy, laser photocoagulation, and vitreoretinal surgery.
 Case Illustration : We reported a patient, a 31-year-old woman with complaints of blurred vision and floaters in both eyes. An initial examination showed the visual acuity of the RE was 0,1F1 and the LE was 0,1. Examination of the posterior segment found perivascular exudate and peripheral retinal neovascularization. We performed an OCT examination on both eyes and found that there was extensive macular edema, especially in the RE. Patient was diagnosed with ED and received steroidsas an initial treatment. But there was no improvement in both eyes. In addition, patient received two anti-VEGF injections in the RE and one injection in the LE and showed good improvement. After that injection, the posterior segment showed very minimal lesions on the macula and repeated OCT results showed normal central macular thickness.
 Discussion : Anti-VEGF may also play a role in the treatment and management of ED. This is because, besides inflammation, there is an increased expression of VEGF in vitreous patients with ED. Several case reported there is significant improvement in anti-VEGF injection in the management of patients with ED.
 Conclusion : Intravitreal anti-VEGF injection shows good results in ED that did not improve with steroid treatment.

MANAGEMENT EALES’ DISEASE GRADE III B: A RARE CASE

Introduction : Eales' disease is an idiopathic occlusive retinal vasculopathy that mostly affects the peripheral retina bilaterally in young, predominately male patients. A hallmark of eales disease is recurrent vitreous hemorrage can all cause some patients to experience severe vision loss
 Case Illustration : A 39-year-old male with vitreous hemorrhage caused by Eales Disease grade III B with initial visual acuity 1/300. Ultrasound imaging showed the membrane like lesion with 1/2 retinal reflectivity, after moderate movement and vitreous hemorrhage on both eyes. The photo-fundus showed there is decreased fovea reflex and perimacular exudate with visible contours of sclerotic blood vessels, visible cotton wall spot, and macroaneurysm. There is also haze grade 3 on media, visible folds in the inferior part, visible shadows of cotton wall spots. The patient was managed with pars plana vitrectomy with endolaser
 Discussion : In this case, patient was diagnosed as vitreous hemorrhage caused by Eales Disease grade III B on both eyes but it getting worse on left eye further. We chose to perform PPV with endolaser on right eye. The outcome was good on right eye based on better visual acuity and decreased hemorrhage, but in the left eye, which not treated, worsen outcome occurred
 Conclusion : The management of this patient’s case showed good results in both anatomic and functional outcomes for right eye, but unfortunately left eye is on serious problem

A CASE OF EALES DISEASE OBSERVED BY WIDEFIELD OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY

Purpose: The purpose of this study was to report a 30-year-old woman with Eales disease, showing bilateral proliferative tissue. The retinal vessels were evaluated using widefield optical coherence tomography angiography (widefield OCTA), which has a wider angle of view compared with conventional instruments. Methods: This is a case report. Results: Widefield OCTA showed an extensive nonperfusion area and A-V shunt in both eyes and a vascular structure in the left eye that appeared to be retinal neovascularization. Ultra-widefield fluorescein angiography revealed no filling delay in the early phase, a vast nonperfusion area from the midperiphery to the ora serrata in bilateral eyes, and retinal neovascularization in the left eye. Based on the results of ultra-widefield angiography, OCTA examination, and systemic examination, a diagnosis of Eales disease was finally made after all the differential diseases had been excluded. Conclusion: In a patient with Eales disease, the peripheral nonperfusion area and retinal neovascularization that were consistent with the ultra-widefield angiography findings were noninvasively confirmed by widefield OCTA. Widefield OCTA is useful in the detection of peripheral changes of the fundus.

The outcome of cataract surgery in paediatric uveitis

Aims/Purpose: Cataract is a frequent complication of chronic uveitis. It can be a consequence of intraocular inflammation or the use of corticosteroids. In children, uveitic cataract is a source of amblyopia, hence the interest in surgery. Our work aims to study the therapeutic particularities, the peri and post‐operative complications and the visual and functional results of these cataracts.Methods: We performed a retrospective study of 14 patients (18 eyes) with uveitis; involving children under 15 years of age; who underwent cataract surgery between January 2011 and November 2022.Results: The average age of the operated children was 9.5 years (4–14 years) with a female predominance of 70%. Uveitis was bilateral in 8 children. The aetiology of the uveitis was found in 9 patients. It was juvenile idiopathic arthritis (JIA) in 6 cases, ocular toxoplasmosis in 1 case, tuberculosis in 1 case and Eales disease in 1 case. The aetiology of the uveitis was not found in 5 patients. The initial visual acuity varied between light perception and 2/10. Total white cataract was found in 44% of cases, posterior subcapsular in 39% of cases, anterior subcapsular in 5.5% of cases and corticonuclear and posterior subcapsular in 11% of cases. Phacoemulsification was performed with implantation in the lens bag in 17 eyes, only 1 patient in our series was not implanted. Post‐operative visual acuity was greater than 7/10 in 68% of cases, between 7/10 and 3/10 in 16% of cases and less than 3/10 in 16% of patients. Post‐operative complications were dominated by the inflammatory reaction of the anterior segment, which was controlled by corticosteroid treatment in 40% of cases, macular oedema occurred in 2 patients in our series post‐operatively and Elsching's beads in 6 eyes (34%). Immunosuppressive treatment was maintained in 75% of cases.Conclusions: Cataract surgery in uveitic children requires careful management to ensure successful outcomes. The control of peri‐operative inflammation in the surgery of paediatric uveitic cataracts is the key to obtaining good visual outcomes and preventing amblyopia.ReferencesAlAmeer AM, Al Shamrani M. Outcome of cataract surgery in pediatric uveitis (experience at King Khalid Eye Hospital). Oman J Ophthalmol. 2022 Mar 2;15(1):43–48.

An abnormal immediate increase in the intraocular pressure post-intravitreal injection in patients with Eales disease

Introduction In 1880, British ophthalmologist Henry Eales described Eales' disease for the first time(1). Recurrent vitreous hemorrhaging is a hallmark of Eales' disease(2). The systemic anti- tuberculosis and the steroids usually control the disease and subsequently resolve the vitreous hemorrhage(3), but when the vitreous hemorrhage persists, anti-VEGF is an option(4).

Less Frequent Vascular Disorders of the Retina - Part 1

This review provides an overview of retinal vascular disorders that are less frequent in Germany and Europe compared to diabetic retinopathy and retinal venous or arterial occlusive disorders. The knowledge of these disorders is important for the differential diagnosis of retinal vascular disorders as well as potentially associated systemic disorders. In the current part one epidemiology, pathophysiology, clinical presentation, and therapy are discussed for hypertensive retinochoroidopathy, ocular ischemic syndrome, retinal alterations in sickle cell disease, Eales disease, radiation retinopathy, peripheral exudative hemorrhagic chorioretinopathy, and retinal disorders associated with pregnancy.

Eales Disease

Eales disease, first described by Henry Eales in 1880, constitutes an idiopathic occlusive retinal vasculopathy apparently multifactorial, and with a likely immunologic origin, which mainly affects the peripheral retina bilaterally in young patients predominantly males. Most cases are asymptomatic in the early stages of the disease however, in the course of the disease floaters and blurry vision arise and hemorrhages lead to significant visual loss. The clinical appearance depends on the peripheral nonperfusion and neovascularization that follow retinal perivasculitis. Visual loss is commonly due to vitreous hemorrhage and tractional retinal detachment. Routine biochemical tests, fundus fluorescein angiography, widefield angiography, optical coherence tomography, optical coherence tomography angiography, and ultrasound are used in the diagnosis of the disease. Corticosteroids, immunosuppressive agents, laser photocoagulation, intravitreal triamcinolone, anti-VEGF injections, and vitrectomy are treatment modalities.

EFFICACY OF JIVANTYADI GHRITA NASYA AND NETRA TARPANA IN THE MANAGEMENT OF EALES DISEASE (TIMIRA) – A CASE STUDY

Introduction – Eales disease is an idiopathic occlusive vasculitis involving the peripheral retina. Clinical find-ings in Eales disease are characterized by avascular areas in the peripheral retina followed by microaneurysms, and neovascularization and are hallmarked by recurrent vitreous hemorrhages and vision loss. The symptoms of Eales disease can be correlated to Dwitiya Patalagata Doshdushtijanya Timira in Ayurveda. When the vitiat-ed Doshas are situated in the Dwitiya Patala, it causes confused visual perception and appearance of bees, flies, and circles in front of the eyes, which can be correlated with Timira described by Acharya Sushruta. According to Acharya Vagbhata, Nasya and Netra Tarpana are indicated in Timira’s samanya chikitsa. Here, we present a case of 38 years old male patient, previously diagnosed and treated with Eales disease that underwent Ayurve-dic treatment at our institution and showed remarkable improvement. Case presentation – A 38-year-old male patient, presented to our institution's OPD with complaints of diminution of vision in both eyes, floaters, specks, and cobwebs in the vision of both eyes in the last eight months. He was previously diagnosed with Eales disease in both eyes with the help of FFA and fundus photography. He was treated with oral steroids, AKT (Anti- Koch's Therapy), Intravitreal injection in both eyes, and Pan-retinal photocoagulation therapy in the left eye. But due to poor response and recurrent retinal haemorrhages and neovascularization, he was ad-vised of left eye Vitrectomy. Hence the patient opted for Ayurvedic management. He underwent Jivantyadi Ghrita Nasya, and Netra Tarpana along with oral medications like Saptamrut Lauha, Panchatiktaghrita Guggul, and Avipattikar Churna for one month. Result- His visual acuity improved significantly from 6/18 to 6/9 in the right eye and 6/36 to 6/12 in the left eye. In fundoscopic findings, vitreous haze decreased, and active haemor-rhages were arrested after one month of treatment. Conclusion- Thus it can be concluded that Jivantyadi Ghri-ta Nasya and Netra Tarpana were found to be effective in treating signs and symptoms of Eales disease.

The reply to the comment on "Clinical characteristics of Eales disease and the efficacy of different interventions for neovascular complications".

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Correspondence.

1Retina-Vitreous Service, Ratan Jyoti Netralaya, Gwalior, INDIA aCORRESPONDENCE: Dhananjay Shukla, Ratan Jyoti Netralaya, 18 Vikas Nagar, Gwalior 474002 INDIA Ph.: 91-751-2423350-51; Fax: 91-751-4070921; E-mail: [email protected]

Cocaine Abuse as an Immunological Trigger in a Case Diagnosed with Eales Disease

Background: Eales disease is a clinical syndrome affecting the mid-peripheral retina with an idiopathic occlusive vasculitis and possible subsequent retinal neovascularization. The disease can develop into visually threatening complications. Case Presentation: We report the case of a 40-year-old Caucasian male with a history of cocaine abuse who presented with blurred vision in the left eye (LE). Fundus examination showed vitreous hemorrhages, peripheral sheathing of venous blood vessels, areas of retinal neovascularization in the LE, and peripheral occlusive phlebitis in the right eye. The full serologic panel was negative except for the heterozygous mutation of factor V Leiden. Clinical and biochemical parameters suggested a diagnosis of Eales disease. Therapy with dexamethasone, 1 mg per kg per day, tapered down slowly over 4 months, and peripheral laser photocoagulation allowed a regression of clinical signs and symptoms. Conclusion: This case shows an uncommon presentation of Eales disease associated with cocaine abuse. Both cocaine abuse and a thrombophilic pattern, as cofactors, might have sensitized the retinal microcirculation on the pathogenetic route to this retinal pathology. Furthermore, in view of this hypothesis, a thorough ocular and general medical history investigating drug abuse and coagulation disorders is recommended for ophthalmologists in such cases.

CLINICAL CHARACTERISTICS OF EALES DISEASE AND THE EFFICACY OF DIFFERENT INTERVENTIONS FOR NEOVASCULAR COMPLICATIONS.

To evaluate clinical features and manifestations of Eales disease and the efficacy of different interventions. The databases PubMed, EMBASE, and Ovid from inception until February 2021 were searched. Pooled analyses included 1) presenting features, 2) symptoms and signs, and 3) postoperative vision outcomes and complications. Statistical analyses were conducted with R software version 3.6.3. Forty-seven studies involving 3,557 patients and 4,959 eyes were included. The pooling results showed that Eales disease was male dominated (94%), bilateral involvement (64%), and mainly occurred in adolescents. Approximately 83% of patients were positive in Mantoux test, and 31% of patients have vitreous tap polymerase chain reaction positive for tuberculosis genome. More than half of the patients had decreased vision at the first clinic visit. The common signs of Eales disease included sclerotic vessels (83%), neovascularization of retina elsewhere (64%) and periphlebitis (51%). Macular changes could be detected in 24% of patients. The most common complication was vitreous hemorrhage, followed by cataracts and retinal detachment. Photocoagulation was directed at nonperfusion or neovascularization areas, with a decrease in the incidence of later pars plana vitrectomy (19%). Anti-vascular endothelial growth factor treatment was suggested for vitreous hemorrhage, which could achieve high visual acuity improvement rate (82%) while increasing the possibility of retinal detachment (18%). Pars plana vitrectomy was indicated in cases with persistent vitreous hemorrhage or retinal detachment, with 56% of patients obtaining visual improvement. Still, 17% of patients needed a second pars plana vitrectomy, mainly because of recurrent vitreous hemorrhage or retinal detachment. Eales disease most commonly affects young men. It has diverse clinical features, and management should be based on the progression of the disease.

A retrospective study on demographic profile and etiology of vitreous hemorrhage

Vitreous haemorrhage(VH) is due to blood leak from ruptured vessels into the vitreous cavity. It results in painless loss of vision. Visual acuity depends on degree and location of haemorrhage.To study the demographic profile and etiology of all patients with vitreous hemorrhage in tertiary care eye hospital. Medical records of patients who presented with VH between Jan 2017 to July 2018 were retrospectively reviewed. A detailed analysis of records will be noted. Out of 47 patients, 35 were males and 12 were females. Common age group affected were more than 60 years with 22 cases followed by that among 40 to 60 years involving 15 eyes. 46 cases were unilateral and 1 was bilateral. Majority of cases was noted from the rural areas with low socioeconomic status. Among the etiological causes proliferative diabetic retinopathy was the major cause (41.6%), followed by retinal vein occlusion (14.5%), rhegmatogenous retinal detachment(RRD) (12.5%), blunt trauma (10.41%) which was major cause among children presenting with vitreous haemorrhage, hypertension (4.16%), Eales disease (4.16%), AMD (4.16%), open globe injury (4.16%), retinal artery microaneurysm (2.08%), and PVD with tear (2.08%).Most common age groups affected were greater than 60 years. Among the 47 cases majority were males. 46 were unilateral and 1 was bilateral. More cases were from the rural areas having lower socioeconomic status. Among the etiological causes proliferative diabetic retinopathy formed the major cause.

Eales Disease Debut in Childhood

Introduction: Eales disease is considered a peripheral retinal vascular disease characterized by inflammation (vasculitis), ischemia, vascular occlusion, and neovascularization. Its etiology is not yet defined, it generally affects young men in the second decade of life. Case: Male patient, 10 years old of age with a red eye with blurred right eye vision of 1 month of evolution that worsened 4 days before coming to the service, accompanied by photophobia and eye pain. Discussion: Eales disease affect to more often young males, whose main characteristic peripheral phlebitis which can cause retinal ischemia and neovascularization. Although its etiopathogenesis is unknown. Some cases in the literature that relate it to a type IV hypersensitivity reaction to M. tuberculosis’s antigens The treatment of choice was systemic and topical corticosteroids, with a very good clinical response and periodic follow-up of the patient, with warning signs.

Comparison of fundus fluorescein angiography, optical coherence tomography and optical coherence tomography angiography features of macular changes in Eales disease: a case series

PurposeTo study the macular features in Eales disease patients observed with fundus fluorescein angiography (FA), optical coherence tomography (OCT) and optical coherence tomography angiography (OCTA).MethodsA cross-sectional study was done on treatment naïve 31 eyes (23 patients) with Eales disease. Baseline parameters such as Best-corrected visual acuity (BCVA), slit-lamp bio microscopy (SLB), indirect ophthalmoscopy, FA, spectral-domain OCT {quantitative (central macular thickness [CMT]) and qualitative analysis on SD-OCT} and OCTA were performed. Any media opacity precluding the above investigations was excluded.ResultsMacular findings comprised of- epiretinal membrane, macular exudation, full thickness macular hole, sub internal limiting membrane bleed, cystoid macular oedema, neurosensory detachment and retinal thickening. Sixteen (51.6%) of our patients had macular changes as seen on all modalities together. SLB and indirect ophthalmoscopy missed macular findings in 50% patients and FA in 18.8% patients. OCT and OCTA diagnosed all macular findings. On comparison of mean BCVA in patients with macular involvement on FA, OCT and OCTA, compared to those without macular involvement, patients with macular involvement had lower BCVA (p 0.000, 0.01 and 0.001 respectively). Thus, FA missed many patients who had significant macular involvement and hence less vision.ConclusionEales disease though described in literature as classically being peripheral retina disease process, also has macular involvement. OCT and OCTA are useful guides to evaluation of macular involvement in these patients. The latter seems to be superior to FA in detecting macular abnormalities in this ailment. OCTA is non-invasive and shows deep capillary plexus changes which are not shown by any other modality.

Image characteristics and quantitative analysis of multicolor imaging in eyes with cystoid macular edema

Objective To observe the imaging features of cystoid macular edema (CME) in multicolor imaging (MC), and to evaluate the value of MC in the diagnosis of CME. Methods Descriptive case series study. From August 2017 to June 2018, 42 eyes of 37 patients with CME diagnosed in the people's Hospital of Wuhan University were included in the study. Among them, there were 24 males and 13 females, with an average age of 48.51±10.29 years. There were 14 eyes with diabetic retinopathy, 14 eyes with central retinal vein occlusion, 8 eyes with branch retinal vein occlusion, 4 eyes with uveitis, and 2 eyes with Eales disease. The macular color fundus photography (CFP) was performed with Visucam 200 non-mydriatic fundus camera of Zeiss company in Germany. MC, frequnce domainoptical OCT (SD-OCT) and FFA were examined by Spectralis HRA2 + OCT of Heidelberg company in Germany. According to the MC standard method, five images, including 488 nm blue reflection (BR), 515 nm green reflection (GR), 820 nm infrared reflection (IR) imaging and standard MC and blue-green enhancement (BG), were obtained at the same time. Compared with SD-OCT, CFP and MC images were scored. Friedman M test and Wilcoxon signed rank test were used for statistical analysis. Results The standard MC and BG images showed blue-green uplift area or petal-shaped appearance, surrounded by green reflection areas with clear boundaries. BR image can be seen in the low reflexes area. On the GR image, there were patches or cystic low reflection areas, surrounded by a slightly high reflection. On the IR image, patches or cystoid high reflexes can be seen, surrounded by low reflection dark areas with clear boundaries. The average scores of CFP, standard MC, GB, IR, GR and BR were 1.20±0.94, 3.05±0.99, 2.90±1.04, 2.55±1.27, 2.00±0.94, 0.51±0.85 respectively, and the differences were statistically significant (χ2= 151.61, P=0.000). The score of CFP were significantly lower than that of standard MC (Z=-5.421), BG (Z=-5.354), IR (Z=-4.714), GR (Z=-4.438) and higher than that of BR (Z=-3.435). The differences were statistically significant (P=0.000, 0.000, 0.000, 0.000, 0.001). Conclusions The quality of MC imaging is better than that of CFP. Combined with SD-OCT, it can be used as an assistant method to diagnose CME. Key words: Macular edema/diagnosis; Tomographyoptical coherence; Fluorescein angiography; Multicolor imaging

Diagnosis of Eales disease from a macular epiretinal membrane

Macular involvement is a common finding in patients with Eales disease . The purpose of this communication is to describe the diagnosis of Eales disease from the finding of a macular epiretinal membrane in a young patient. The case is presented of a 38-year-old man referred to this medical service unit with blurred vision developed over the past 3 months, and was associated with vitreoretinal traction and a macular epiretinal membrane. After an ophthalmological examination including the retinal periphery, optical coherence tomography , tuberculin test , interferon gamma release assay , and a systemic study, the patient was diagnosed with Eales disease. Macular oedema or epiretinal membranes due to Eales disease are relatively common. Sd-OCT is recommended in all patients with Eales Disease. On the other hand, the presence of epiretinal membranes in young patients usually suggests a non-idiopathic aetiology. Presentamos el caso de un varón de 38 años, remitido a nuestro servicio por tracción vítreo-retiniana y membrana epirretiniana asociada a pérdida de visión de 3 meses de evolución. Tras una exploración oftalmológica que incluyó el examen de la periferia retiniana, tomografía de coherencia óptica, prueba de tuberculina, IGRA y estudio sistémico se llegó al diagnóstico de enfermedad de Eales. La afectación macular en pacientes con enfermedad de Eales es un hallazgo común, ya sea en forma de edema macular o membrana epirretiniana. Por ello, es aconsejable realizar un estudio macular mediante OCT. Por otra parte, el hallazgo de una membrana epirretiniana en un paciente joven debe hacernos pensar en una posible etiología no idiopática.

Diagnóstico de enfermedad de Eales a partir de una membrana epirretiniana

Macular involvement is a common finding in patients with Eales disease. The purpose of this communication is to describe the diagnosis of Eales disease from the finding of a macular epiretinal membrane in a young patient.The case is presented of a 38-year-old man referred to this medical service unit with blurred vision developed over the past 3 months, and was associated with vitreoretinal traction and a macular epiretinal membrane. After an ophthalmological examination including the retinal periphery, optical coherence tomography, tuberculin test, interferon gamma release assay (IGRA), and a systemic study, the patient was diagnosed with Eales disease.Macular oedema or epiretinal membranes due to Eales disease are relatively common. Sd-OCT is recommended in all patients with Eales disease. On the other hand, the presence of epiretinal membranes in young patients usually suggests a non-idiopathic aetiology.