Eales’ disease: epidemiology, diagnostic and therapeutic concepts

Background To describe the epidemiological traits, clinical characteristics, diagnostic procedures, therapeutic interventions and evolution in a large series of patients with diagnosis of Eales’ disease. Methods A clinical retrospective review of patients with Eales’ disease, evaluated and treated between April 2009 and April 2018, with a 1-year minimum follow-up. Thirty patients (59 eyes), were included. Age, sex, laboratory results (CBC, glycemia, protein electrophoresis, ACE levels) immunological profile and a Quantiferon-TB Gold Plus test were recorded. The patients were divided into groups according to their evolution, medical or surgical treatment, and visual outcomes. Results Seventeen male patients and 13 female patients were included, and their ages ranged from 14 to 35 years. The Quantiferon-TB Gold Plus test was positive in 25 patients. Twenty-eight patients had unilateral vitreous hemorrhage, 10 of whom presented with vasculitis and non-perfusion areas in the contralateral eye, 9 presented contralateral peripheral neovascularization and 9 had contralateral fibrovascular proliferation. The remaining 2 patients presented with a rhegmatogenous retinal detachment. In 6 patients, conservative treatment with intravitreal anti-VEGF injections and photocoagulation was performed after the hemorrhage cleared. Twenty-two patients, required vitrectomy, with good visual outcomes. Macular edema was found in 16 eyes, which responded to periocular and/or systemic corticosteroid therapy, except for 9 eyes that required intravitreal bevacizumab, with complete resolution in 7 eyes and partial resolution in 2 eyes. Conclusions Eales’ disease is a pathology of significant prevalence in our country. The distribution according to sex, tends to be equivalent. The etiology, even when it is not specifically determined, according to laboratory tests, confirms the probable immunologic response in the presence of Mycobacterium tuberculosis antigens. This is still a diagnosis of exclusion, and therefore, it is advisable to perform a complete laboratory work-up in each case. Timely application of laser and other medical treatments, help to avoid progression to more advanced stages and their complications. The surgical treatment of vitrectomy for vitreous hemorrhage, and/or tractional vitreous detachment yields good primary anatomical and functional outcomes. Secondary macular edema responds to periocular and intravitreal corticosteroids, and in refractory cases, the use of anti-VEGF therapy leads to an effective resolution.

Cerebral Artery Stenosis in a Young Stroke Patient with Eales’ Disease

Eales’ disease is an idiopathic disorder induced by ischemic retinal vasculitis, usually affecting the peripheral retina. We encountered a young patient diagnosed as cerebral infarction with a history of Eales’ disease. The patient’s middle cerebral artery showed multifocal stenosis with contrast enhancement, suggesting vasculitis. These findings might give clues to a rare cause of cerebral artery stenosis.

Treatment Outcomes in Eales’ Disease with Vitreous Hemorrhage at Tertiary Center in Southern Thailand

Objective: To assess the anatomical and functional outcomes of Eales’ disease with secondary vitreous hemorrhage after conservative treatment and/or pars plana vitrectomy (PPV).Material and Methods: A retrospective chart review of patients, diagnosed with Eales’ disease, at Songklanagarind Hospital from January 2003 to December 2017 was performed. Thirty-two eyes, from 28 patients, with secondary vitreous hemorrhage underwent conservative treatment and/or PPV; depending on the degree of vitreous hemorrhage, clinician consideration and patient’s decision.Results: Twenty-five eyes, with low-grade (grade 1-3) and dense (grade 4) vitreous hemorrhage, initially underwent conservative treatment; in which 64.0% achieved final best corrected visual acuity (BCVA) ≥20/70 and 56.0% achieved final BCVA ≥20/40 with complete resolution of vitreous hemorrhage. However, 8 eyes eventually required additional PPV as a result of non-resolving vitreous hemorrhage. Alternatively, 7 eyes with dense vitreous hemorrhage (grade 4) at the presentation, preferred PPV as primary treatment, which resulted in final BCVA ≥20/70 in 57.0% and final BCVA ≥20/40 in 43.0%. Post vitrectomy complications included; cataract progression (52.9%), high intraocular pressure (35.3%), epiretinal membrane (23.5%), and rhegmatogenous retinal detachment (17.6%).Conclusion: Anatomical and functional outcomes of Eales’ disease with vitreous hemorrhage were relatively good following either conservative treatment or PPV. Each treatment option offered both advantages and disadvantages. The risks and benefits of each treatment option should be discussed with each individual patient to achieve the optimal outcome. Due to being a less invasive procedure, conservative treatment should be initially performed with close monitoring. In the minority of patients, vitrectomy still plays an important role to improve visualization and enable laser photocoagulation, despite potential risk of cataract progression.

Histopathological, immunohistochemical and molecular biologic study of an enucleated specimen of a case of Eales’ disease

Eales’ disease is a retinal vasculitis characterized by retinal inflammation, ischemia, and neovascularization. Exact pathogenesis of this disease is yet to be found out. We present a 29-year-old male, diagnosed with Eales’ disease in both eyes with persistent intraocular inflammation. Enucleation of the pthisical right eye was subjected for histopathological examination immunohistochemistry and molecular biologic study for mycobacterial tuberculosis DNA. Our study showed that Eales disease is probably a T cell mediated disease which is triggered by mycobacterial TB DNA. Further studies are needed to confirm our findings.

Optical Coherence Tomography Angiography Features of Macular Epiretinal Neovascularization In Eales’ Disease: A Case Rreport

Background: Eales disease primarily affects the peripheral retina. However, posterior involvement can be seen. Macular epiretinal neovascularization is not commonly seen in Eales disease. This report highlights the morphology and origin of macular epiretinal neovascularization (ERN) using multimodal retinal imaging, including optical coherence tomography angiography (OCTA). Results: A 35-year-old man with no history of systemic disorders presented with gradual decrease of vision in his left eye. Fundus examination of his right eye showed peripheral sclerosed blood vessels, neovascularization of the optic disc and elsewhere, and macular ERN. The view of the left fundus was limited by vitreous haemorrhage. Fluorescein angiography (FA), of the right eye showed widespread peripheral capillary nonperfusion and leakage of dye from the retinal neovascularization and macular ERN. Macular Spectral domain optical coherence tomography (SD-OCT) of the right eye showed an epiretinal membrane and the presence of epiretinal neovascular lesions extending above the internal limiting membrane towards the vitreous. Optical coherence tomography angiography (OCTA) showed multiple tiny blood vessels at the macula that arose from the superficial retinal capillary plexuses and extended toward the vitreous. The corresponding B-scan showed flow signal through these vessels and the signal extend above the internal limiting membrane. Systemic work-up was negative except for strongly positive tuberculin skin testing giving the classic diagnosis of Eales disease. Patient was started on empirical anti-tubercular therapy and oral corticosteroids. Scatter laser photocoagulation was applied to nonperfused retinal zones. Despite adequate scatter laser ablation, the ERN failed to regress fully. Conclusions: Macular ERN can be seen in cases of classic Eales disease. The origin of macular ERN in our case was shown to be from the superficial retinal capillary plexuses. We also noted the slower regression rate of macular ERN as compared to the major neovascularizations of the optic disc and peripheral retina. Further research is needed to establish the pathogenesis of ERN and its optimal management.

Eales’ Disease: When the Rare Sounds Frequent

Eales’ disease is a peripheral occlusive retinal phlebitis, with an unclear pathogenesis. The classic association with hypersensitivity to Mycobacterium tuberculosis protein infers that immunologic disturbance may be involved. Here, we described three cases of Eales’ disease. All patients are Caucasian men aged 27-58 years and presented with vitreous hemorrhage and/or peripheral venous vasculitis. Tuberculin skin sensitive test (Mantoux screening test) and interferon-gamma release assay (IGRA) were positive in all patients. Therapeutic approach included antituberculosis therapy and systemic steroids, associated or not to immunosuppressive therapy, and retinal scatter photocoagulation in all cases. Antivascular endothelial grow factor (VEGF) intravitreal injections were also required in two cases. Since various retinal diseases can resemble this presentation, Eales’ disease is considered a diagnosis of exclusion. Early diagnosis and appropriate therapeutic approach are both essential to accomplish disease control and reduce ophthalmologic complications.

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.