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International Journal of Ophthalmology & Eye Science (IJOES) Epidemiology of Eales Disease in the Central Western India
Shah MA*, Shah SM, Kalyani PJ, Shah AH, Shah PD, Pandya JS Drashti Netralaya, Dahod, Gujarat, India.
Objective: To investigate the clinical features and propose a new staging system based on the clinicopathological correla-
tion and formulate new guidelines for management of Eales' disease in healthy young males.
Design: Prospective cohort study.
Setting: Vitreo Retinal Department of a tertiary eye care center in Western Central India.
Participants: Seventy-four eyes diagnosed with Eales' disease.
Materials and methods: From 2004 to 2010, patients clinically diagnosed with Eales' disease were enrolled in this study
using specific inclusion and exclusion criteria. We examined the patients' anterior and posterior segments thoroughly. We performed the necessary ocular and systemic investigations. We divided the investigations into three stages: inflammatory, ischemic and complications and the patients were treated accordingly. We treated the patients using medical management, photocoagulation or pars plana vitrectomy. The patients were monitored according to standard schedules and formats. All information was documented using a pre-tested online format and statistical analyses were performed using SPSS ver. 17. A p-value < 0.05 was considered to indicate significance.
Outcome measures: Visual acuity.
Results: The cohort comprised 74 cases with a mean age of 30 ± 8.73 years. The visual acuity of the presenting cohort
was < 3/60 in 64.9% of eyes. The final visual outcome was > 6/12 in 40 eyes (54.1%), 6/60 to 6/18 in 14 eyes (18.9%) and < 1/60 in the remaining 9 eyes at a mean follow-up of 592 days. The visual parameters differed significantly pre-and post-treatment. We evaluated the visual outcome following surgical management. Conclusion: We studied epidemiological facts about anterior and posterior segment findings.
Key Words: Eales Diseases; Grading System; Periphlebitis; Vitreous Hemorrhage; Systemic Corticosteroids.
*Corresponding Author:
Mehul A. Shah MD, Drashti Netralaya, Nr. GIDC, Chakalia Road, Dahod-389151, Gujarat, Eales' disease was first described by Henry Eales, a British oph- thalmologist, in 1880 and 1882. He identified this disease in Tel: 00-91-2673-645364 seven male patients ranging in age from 14 to 29 years with recur- Fax: 00-91-2673-221232 rent retinal hemorrhages. In addition, these patients had histories of headache, variation in peripheral circulation, chronic constipa- Received: October 05, 2014
tion, and epistaxis Accepted: December 30, 2014
Published: January 08, 2015
Eales' believed that these symptoms were caused by vasomotor neurosis with constriction of the alimentary vessels and compen- Citation: Shah MA, et al., (2015). Epidemiology Of Eales Disease In
satory dilatation of the retinal and nasal vessels. Eales' did not The Central Western India. Int J Ophthalmol Eye Res. 03(1), 1-5.
describe retinal vasculitis. However, 5 years later, Wadsworth (1887) described signs of retinal vasculitis. Eales' has subse- Copyright: Shah MA 2015. This is an open-access article distributed
quently been honored with the eponym for idiopathic retinal Pe- under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution and reproduction in any medium, provided the original author and source are credited. Eales' disease is an idiopathic inflammatory vasoproliferative dis- order of the retinal veins, which leads to the occlusion of these vessels. It affects primarily healthy young adults between the Shah MA, et al., (2015). Epidemiology Of Eales Disease In The Central Western India. Int J Ophthalmol Eye Res. 03(1), 1-5.
ages of 20 and 40 years. It affects primarily males, (80-90%), but Subjects with a history of diabetes mellitus, hypertension, colla- can affect females. Elder et al. (1971) reported the possibility of gen vascular disease, sarcoidosis, Bechet's disease, systemic lupus bilateral involvement in 90% of cases. It may rarely affect the erythematosus, Coats' disease, or syphilis were excluded from the arterioles. This vascular occlusion, induced by the inflammation of veins, can lead to proliferative vascular retinopathy and subse- quent sequelae, such as recurrent vitreous hemorrhage, and trac- Ophthalmic examinations included best-corrected visual acuity tional or combined retinal detac using Snellen's chart, intraocular pressure measurement by appla- nation tonometry, and anterior segment evaluation by slit-lamp Eales' disease was common a century ago, but its incidence has declined in western countries. However, Eales' disease is wide- spread in the Indian subcontinent and certain areas of the Mid- Evaluations of the posterior segment at the initial and each sub- dle East. It is usually seen unilaterally, but can present bilaterally. sequent visit were performed by direct and indirect ophthalmos- Eales' disease is characterized by three overlapping stages of ve- copy, slit-lamp biomicroscopy with 90-D and three-mirror lenses, nous inflammation (vasculitis), occlusion, and retinal neovascu- and fundus fluorescein angiography.
larization. Diagnosis is mostly clinical and requires exclusion of other systemic or ocular conditions that could present with simi- Investigations included complete blood count with sickle-cell lar retinal features. Recurrent vitreous hemorrhage is the hallmark screening, erythrocyte sedimentation count, liver function test, of Eales' disease. fasting plasma sugar level, blood coagulation profiles, Mantoux test using 2 TU/0.1 mL purified protein derivative (PPD), and Treatment options include systemic steroids during the inflam- chest X-ray.
matory stage, retinal photocoagulation usually in the proliferative stage to the non-perfused retina, and early vitrectomy for recur- Ultrasonography was performed to detect fractional retinal de- rent vitreous hemorrhage. Oral steroids are the primary tachment in eyes affected by vitreous hemorrhage with no fun- agents used most frequently for the management of Eales' dis- dus view. The location and extent of retinal involvement due ease, but prolonged systemic use of steroids is associated with to Eales' disease, neovascularization, and fibrovascular traction serious ocular and systemic side effects. Photocoagulation is were documented in all patients by fundus drawing and digital the mainstay of treatment in the proliferative stage of Eales' dis- fundus color photography. Optical coherence tomography (OCT) ease. Intravitreal steroids and anti-VEGF have been used in was performed using high-density OCT (Cirrus, 2000, Carl Zeiss many posterior segment ocular pathologies. Visual prog- Meditec, CA,) as required after it has became available.
nosis is good if treated early in the course of the disease Recently, anti-metabolite therapies, especially methotrexate, have The inflammatory stage was treated with medical management, been used in Eales' disease with some success. periocular steroids, systemic steroids or methotrexate. Complete blood counts and liver function tests were performed at least once The prognosis of patients with Eales' disease is variable and de- per month in patients prescribed methotrexate. Prior to prescrib- pends on the availability of medical care. Many of the patients in ing methotrexate, a thorough medical consultation was performed the region in which this study was conducted live in areas that are by a medical oncologist. The ischemic stage was treated with anti- underserved by medical care. Therefore, the treatment outcome VEGF or photocoagulation according to availability. The stage of may also vary. This study assessed the demographics, clinical pres- complications was treated with surgical management. The details entation and treatment outcome of Eales' disease in Central West were documented in a pre-tested, online Eales' disease form and India, which is inhabited by mainly tribal populations.
then exported to a Microsoft Excel™ spreadsheet.
Data were analyzed using the SPSS software (ver. 17.0; IBM SPSS Inc., Chicago, IL, USA). Univariate parametrical analyses were This study was a prospective interventional case series, designed used. A p-value of < 0.05 was considered to indicate statistical to evaluate the epidemiology of Eales' disease patients in a region of Central Western India; data were collected from January 2004 to December 2012. The study was conducted in a tertiary care teaching hospital and a tribal center for ophthalmic care, which serves the second largest tribal belt in India. It is located at the The cohort comprised 74 male patients with a mean ages of 30 junction of three states, Gujarat, Rajasthan and Madhya Pradesh.
± 8.73 years (Table 1). Demographic factors including age, diet, smoking, religion and race were evaluated (Table 2). No signifi- Approval from the Institutional Ethics Committee was obtained cant differences in the demographic factors were observed, with and all efforts were made to adhere to the guidelines of the Dec- the exception of age of presentation (p = 0.02).
laration of Helsinki.
Within the cohort, the presenting visual acuity was < 3/60 in Patients were briefed about the procedures of the study and con- 64.9% of eyes. The final visual outcome was > 6/12 in 40 eyes sent was obtained before enrolment.
(54.1%), 6/60 to 6/18 in 14 eyes (18.9%) and < 1/60 in 9 eyes at a mean follow-up of 592 days.
Patients suspected to have Eales' disease aged between 20 and 40 years underwent exhaustive ocular and medical evaluation for We found the anterior segment normal in 65 (87.8%) cases (Table confirmation of a diagnosis of Eales' disease. Our cohort com- 3). We then evaluated the findings in the posterior segment (Ta- prised patients diagnosed with Eales' disease with no other sys- ble 4). The treatments prescribed were systemic steroids (64.9%), temic or ocular disease.
anti-metabolites (35.1%), photocoagulation (13%), pars plana vit- Shah MA, et al., (2015). Epidemiology Of Eales Disease In The Central Western India. Int J Ophthalmol Eye Res. 03(1), 1-5.
rectomy (32.1%) and revision vitrectomy (9.5%) (Table 4).
been reported, but the incidences of vitritis, vasculitis and vitre- ous hemorrhage hav We evaluated visual parameters pre- and post-treatment, and found a significant difference (p = 0.000, Table 5). We next com- We observed total posterior vitreous detachment in 24.3% of pared visual outcome following surgical management, and again cases. Badrinath et al. reported multifocal vitreo retinal adhesions found a significant difference (p = 0.000, Table 6).
in 87.8% of cases There was no difference in the response to treatment in the Mantoux-positive and -negative groups Oral corticosteroids and deep sub-tenon triamcinolone, used for Our cohort comprised 74 cases with a mean age of 30 ± 8.73 quadrants two and three reported by Biswas et al., has a significant years. Other reports with a similar demographic profile, including effect on visual outcomeWe used anti-metabolites in the age and gender reported similar findings. study, form of methotrexate in 35.1% of cases As suggested by we have simplified the classification according to the clinical find- many investigators we did not use anti-tubercular therapy for the ings. In the inflammatory stage (stage 1), patients with vitritis and treatment of Eales' disease. We used laser photocoagulation in vasculitis were treated with systemic and periocular steroids. In the the ischemic stage (stage 2) or in those with neovascularization, as ischemic stage (stage 2), patients with neovascularization and is- reported by Biswas et al. chemia were treated with laser or anti-VEGF therapy. During the stage of complications (stage 3), cases of traction or mixed retinal In the current study, vitrectomy was performed in 32.1% of cases, detachment were treated with pars plana vitrectomy. Saxena et al. which is in contrast to the 6–18% reported by Badrinath et reported a classification and grading system, the proposed clas- This may be due to the greater number of patients being in the sification system may provide guidelines for the management of stage of complications (stage 3) in our study.
Eales' diseases Periphlebitis and vitreous hemorrhage have Table. 1 Age Category
AGE CATEGORY NUMBER (N)
PERCENT (%)
Table. 2 Demographic Results
Socio Economic Status Poor No Systemic Disorder Follow Up In Days Table. 3 Clinical Features in Anterior Segment
Chemosis, Rubeosis Iridis Shah MA, et al., (2015). Epidemiology Of Eales Disease In The Central Western India. Int J Ophthalmol Eye Res. 03(1), 1-5.
Table. 4 Clinical Features In Posterior Segment
Normal Anterior Segment Vitreous Haze 2 Quadrants Vitreous Haemorrhage Retinal Detachment Systemic Steroids Pars Plana Vitrectomy Revision Pars Plana Vitrectomy NVD-Neovascularization Disc; NVE-Neovascularization Elsewhere Table. 5 Comparative Study Of Vision Following Medical Mamnagment Of Eales' Disease
PRE TREATMENT VISION
POST TREATMENT VISION
<1/60 1/60 TO 3/60 6/60 TO 6/36 6/24 TO 6/18 6/12 TO 6/9
Table.6 Comparative Study Of Vision Following Surgical Management Of Eales Diseases
POST OPERATIVE VISION
P=0.000 PP VIT-PARS PLANA VITRECTOMY Shah MA, et al., (2015). Epidemiology Of Eales Disease In The Central Western India. Int J Ophthalmol Eye Res. 03(1), 1-5.
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