Eales' disease: epidemiology, diagnostic and therapeutic concepts

Abstract

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.

Keywords: Eales’ disease; Macular edema; Occlusive retinal vasculopathy; Vitreous hemorrhage.

Fig. 1

A Retinal fundus photography showing vascular tortuosity and sheathing in the right eye and vitreous hemorrhage in the left, in a 19 year-old male, with active Eales’ disease. B Retinography of a 23 year-old female with vitreous hemorrhage on the right eye and an apparent normal fundus in the left

Fig. 2

Fundus pictures showing vascular sheathing to different degrees: A, B mild, C moderate and D severe

Fig. 3

Fluorescein angiography from the same patient as in Fig. 1B. Blocked fluorescence due to vitreous hemorrage in the right eye (A) and peripheral vascular anomalies: AV shunts (red arrows) with hyperfluorescent leakage corroborating neovascularization elsewhere (yellow arrowheads) in both eyes. It also shows peripheral hypofluorescence in non-perfusion ischemic areas (red arrowheads) in the left eye (B)

Fig. 4

A Composed widefield retinography showing peripheral vascular sheathing, peripheral hemorrhages, and apparent nasal non-perfusion areas. B Peripheral nasal non-perfusion areas and vascular tortuosity and sheathing confirmed on composed widefield retinal fluorescein angiography in the same eye

Fig. 5

A Widefield fluorangiography showing nasal peripheral non-perfusion areas (left), Retinography immediately post laser application of non-perfusion areas guided by fluorangiography (right) B Late phase of fluorescein angiography shows peripheral leakage confirming neovascularization in a previously laser treated patient (left). Fundus photography with recent laser spots on the identified leakage and non-perfusion areas (right)

Fig. 6

A Postvitrectomy retinography of a female patient, with a history of vitreous hemorrhage in the right eye. Laser scars distributed on neovascularization and non-perfusion areas identified during surgery, and residual fibrosis on the superotemporal vascular arcade are shown. B Postoperative fundus photography of a 17-year-old female with a history of vitreous hemorrhage. Image shows persistent vascular sheathing in nasal and inferotemporal retina and laser scars

Fig. 7

A Two weeks postoperative male, with a history of vitreous hemorrhage. Vitrectomy, with silicon oil tamponade and photocoagulation of ischemic areas was performed, and retinography showed persistent nasal vascular sheathing and recent laser spots on non-perfusion areas, identified during surgery. B Six weeks postoperatively fundus images of the same patient showed improvement of peripheral nasal vascular sheathing and laser scars in the inferotemporal quadrants

Fig. 8

Macular optical coherence tomography of a 15-year-old patient at initial presentation (left) with cystoid macular edema, and post-treatment with periocular steroids (right)