A 23-year old female patient with Eales Disease: case report
SPMC J Health Care Serv. 2015;1(1)
Billie Jean Tang Cordero,1 Redentor Caesar Gonzales1
1Department of Ophthalmology, Southern Philippines Medical Center, JP Laurel Ave, Davao City, Philippines
Correspondence Billie Jean Tang Cordero,
billiejeancordero@yahoo.com
Received 4 June 2015
Accepted 11 September 2015
Cite as Cordero BJT, Gonzales RC. A
23year
old female patient with
Eales Disease: case report. SPMC J Health
Care Serv. 2015;1(1):42-46.
Abstract
During the 19th century, Henry Eales described the clinical features of recurrent retinal hemorrhages in young
patients. The condition was later established as a vaso-occlusive
inflammatory disease of the peripheral retina
that predominantly affects healthy young male individuals, especially in developing countries. We present the
case of a 23-year-old
female with recurrent floaters, sometimes accompanied by blurring of vision, in the left eye.
Flourescein angiography of the left eye showed vitreous hemorrhage on the inferior and temporal portions of the
retina. Hematologic and autoimmune diseases were ruled out as possible causes of the condition during the first
few years. Further systemic work-up
revealed increased erythrocyte sedimentation rate and a positive Mantoux
test. A diagnosis of Eales Disease was made on the sixth year of follow up. The eye condition has been managed
with intermittent focal laser treatment and short courses of topical prednisolone acetate. Despite the delay in the
establishment of the diagnosis and the patients refusal to undergo vitrectomy, the recurrent condition has been
tolerated by the patient who has been functional at work most of the time.
Keywords. recurrent vitreous hemorrhage, retinal vasculitis, retinal neovascularization, photocoagulation
Introduction
In 1880, Henry Eales, a British ophthalmologist, described a condition characterized by recurrent vitreous hemorrhages among young
men.
1
For the succeeding centuries, many have followed Eales' findings, and further studies emerged regarding the immunologic, molecular, and biochemical aspects of Eales Disease that implicate Mycobacterium tuberculosis genome, human leukocyte antigen, retinal autoimmunity, and radicalmediated damage in the pathogenesis of the
disease.
2
In one study in India, one out of 200-250 ophthalmic patients was reported to have Eales
Disease.
3
The condition primarily affects
males,
3
4
but in this report, we describe the clinical features, diagnostic and therapeutic management, and outcomes of an otherwise healthy young female who we eventually diagnosed as having Eales Disease.
Clinical Features
A 23-year-old female came to our clinic in 2008 with a 2-year history of floaters in her left eye. There were no accompanying systemic signs and symptoms. The patient had no history of ocular trauma or previous tuberculosis infection. She tolerated the condition until she noticed a marked decrease in visual acuity, which prompted her to seek consultation. The patient works as a nurse. She had unremarkable past medical, family, and social history. A review of systems was also unremarkable, save for her eye comA 23-year-old female came to our clinic in 2008 with a 2-year history of floaters in her left eye. There were no accompanying systemic signs and symptoms. The patient had no history of ocular trauma or previous tuberculosis infection. She tolerated the condition until she noticed a marked decrease in visual acuity, which prompted her to seek consultation. The patient works as a nurse. She had unremarkable past medical, family, and social history. A review of systems was also unremarkable, save for her eye complaint. Visual acuity of the left eye was 20/80. A fundus examination on presentation revealed hemorrhages at the inferotemporal portion of the retina of the left eye. The right eye findings were within normal limits. The patient's clinical course is summarized in Table 1.
|
Table 1 Chronology of signs and symptoms, physical examination findings, diagnostics, therapeutics and outcomes* |
| Timeline |
Signs and symptoms |
Physical examination |
Diagnostics |
Working diagnoses |
Therapeutic management |
Outcome |
| 2008 |
Floaters in inferotemporal portion of the eye |
VA: 20/80; SLIO: hemorrhages in the inferotemporal portion of the retina |
FA: vitreous hemorrhages in the inferior and temporal portions of the retina, capillary dropouts and neovascularizations in the inferior portion of the retina. |
Vitreous hemorrhages |
Focal laser treatment |
VA improved to 20/20 after 9 months |
| 2010 |
Blurring of vision and recurrence of floaters |
VA: 20/80;SLIO: new-onset vitreous hemorrhage |
FA: new retinal neovascularizations; WBC: leukocytosis, 11 x 109 per liter; ESR raised, 26mm/hr; ANA: normal; RF: normal; CRP: normal |
Vitreous hemorrhage, possibly secondary to any of the following: tuberculosis, systemic lupus erythematosus, Eales Disease, leukemia |
Focal laser treatment; ketorolac 1 drop three times a day on the affected eye for 1 month; oral prednisone 50mg/kg/day for 1 month, then tapered to 10mg/kg/day for 6 weeks |
VA improved to 20/20 after 3 months |
| 2013 |
Blurring of vision and recurrence of floaters |
VA: 20/30 |
FA: new vitreous hemorrhages and retinal neovascularizations; ESR: raised, 29mm/hr, ANA: normal; RF: normal; CRP: normal |
Strongly considering Eales Disase
| Focal laser treatment; topical prednisolone acetate 4 times a day for 1 month |
The patient had the same VA, but floaters were minimized and bleeding was controlled |
| Early 2014 |
Asymptomatic |
... |
... |
The patient was referred to another ophthalmologist who also considered Eales Disease |
... |
The need to establish past history or ongoing tuberculosis infection was reiterated to the patient |
| Late 2014 |
New-onset minimal floaters |
VA: 20/30; SLIO: minimal new-onset vitreous hemorrhage |
CXR: within normal limits; Mantoux test: positive |
Eales Disease, Stage 3b |
No intervention. The patient was monitored for new signs and symptoms |
No change in VA; persistent minimal floaters |
| 2015 |
Blurring of vision and minimal floaters |
VA: 20/100; SLIO: new-onset vitreous hemorrhage, inflammatory cells noted on the posterior segment of the retina |
... |
Eales Disease, Stage 3b |
Topical prednisolone acetate 1 drop every 4 hours for 1 month |
Visual acuity improved to 20/40 after 1 month of treatment |
* All signs and symptoms, physical examination findings, ophthalmologic investigation findings, diagnoses, management of the eye, and outcomes pertain to the left eye. The patient's right
eye has been normal throughout the disease course.
ANA — antinuclear antibody; CRP — C-reactive
protein; CXR — chest X-ray;
ESR — erythrocyte sedimentation rate; FA — fluorescein
angiography; RF — rheumatoid factor; SLIO — slit lamp indirect ophthalmoscopy; VA — visual acuity; WBC — white blood cells. |
Diagnostic and Therapeutic Approaches
A fluorescein angiogram (FA) of the left eye done in 2008 (Figure 1) revealed vitreous hemorrhages on the inferior and temporal parts of the retina. Capillary dropouts were seen on the inferior portion of the retina on the AV phase of the angiogram. Neovascularizations were also noted, mostly located at the inferotemporal area of the retina. Our working diagnosis at this point was vitreous hemorrhage in the left eye. Focal laser treatment improved the visual acuity of the affected eye.
|
|
Figure 1
(2008) Fluorescein angiogram of the left eye, showing capillary dropouts on the inferior portion of the retina on the AV phase of the angiogram (A).
Vitreous hemorrhages were seen on the inferior and temporal areas of the retina (B and C). Neovascularizations were also seen, mostly located in the
inferotemporal portion of the retina.
|
The floaters in the left eye recurred almost yearly over the next seven years, sometimes accompanied by blurring of vision in the same eye. Visual acuity in the left eye fluctuated between 20/30 and 20/100. Repeat funduscopic examinations and FAs in 2010 and 2013 revealed new-onset hemorrhages and retinal neovascularizations. During these years, we attempted to establish the underlying cause of the recurrent retinal hemorrhages. We knew that there was no past or ongoing trauma to the left eye. We were able to rule out systemic lupus erythematosus (normal antinuclear antibody, rheumatoid factor and C-reactive protein). The slightly elevated white blood cell count (11 x 109 per liter) was not convincing enough to think of a hematologic condition, such as leukemia. The raised erythrocyte sedimentation rates (26-29 mm/hour) in 2012 and 2013 were not specific enough to point us to a particular explanatory condition, either.
We requested for chest radiograph and Mantoux test to check if the patient had tuberculosis, but she was apprehensive for some time about her condition and did not comply with the added diagnostics. However, after we were able to rule out trauma, systemic lupus nephritis, and leukemia, we were already strongly considering Eales Disease in 2013. Early in 2014, another ophthalmologist who saw the patient was also considering Eales Disease. Later in 2014, we were finally able to convince the patient to have the pending diagnostics done. The chest X-ray result was within normal limits, but the Mantoux test was positive. We then decided on a diagnosis of Eales Disease, Stage 3b at that point.
Focal laser treatments were repeated in 2010 (Figure 2) and 2013 (Figure 3). Oral steroids and topical ketorolac were added to the treatment in 2010, and visual acuity of the left eye returned to normal in that year. While on focal laser treatment in 2013, the patient was also prescribed with topical prednisolone acetate. Floaters were minimized subsequent to the treatment, but visual acuity persisted to be decreased since 2013. During this time, we noted fibrous proliferation at the inferior and temporal portions of the retina (Figure 3). Since this can cause traction on the retina that could lead to retinal detachment, we advised the patient to undergo pars plana vitrectomy in the left eye, but she opted for conservative therapy. After establishing the diagnosis of Eales Disease, we prescribed intermittent short courses of topical prednisolone acetate when the hemorrhages would recur.
|
|
Figure 2
(2010) Fundus photo (A) shows new-onset
vitreous hemorrhage at the inferotemporal portion of the retina (A: red ring). Laser marks are also
visible in the area. Fluorescein angiogram (B) shows newonset
blocked hypofluorescence (B: yellow ring) that corresponds to the vitreous hemorrhage seen
in the fundus photo. Hyperfluorescence seen on the late phase of the angiogram shows new-onset
leakage due to newonset
neovascularizations elsewhere.
|
|
|
Figure 3
(2013) Fundus photo (A) shows newonset
vitreous hemorrhage on areas with laser marks (A: red ring). Fibrous proliferations (A: yellow ring)
were also noted on the same area. Fluorescein angiogram (B) shows new-onset
multiple blocked hypofluorescence (B: blue rings) that correspond to the
vitreous hemorrhages seen in the fundus photo. Hyperfluorescence seen on the angiogram shows staining on the laser marks and leakage due to new-onset
neovascularizations elsewhere.
|
Outcomes
Follow-up eye examinations have been aimed at closely monitoring any new or recurrent vitreous hemorrhages and new blood vessel formations. As of 2015, the patient reported seeing minimal floaters, however, eye examinations showed controlled inflammation and neovascularization. Currently, the patient is functional at work as a nurse and performs activities of daily living with ease.
Discussion
Our patient came due to floaters on her left eye. This is consistent with the initial presentation of patients with Eales Disease. Funduscopic examination and FA findings in our patient proved the presence of hemorrhages and neovascularizations in the affected eye. Over several years, and despite repeated laser treatments and corticosteroid courses, the floaters recurred, sometimes accompanied by blurring of vision. We requested laboratory examinations that would help us rule out autoimmune and hematologic diseases as possible causes of the vitreous hemorrhages. When most of the laboratory results came out normal, we tried to establish a possible previous exposure to tuberculosis. However, our patient was hesitant about having a tuberculosis work-up, and we had to respect her decision, so the performance of the chest X-ray and Mantoux test was rather delayed. The Mantoux test, which turned out to be positive, confirmed the patient's exposure to tuberculosis and helped us decide on a diagnosis of Eales Disease.
Eales Disease is an inflammatory, vaso-occlusive retinal disease usually affecting young adults. It is believed to be associated with exposure to tuberculosis or tuberculin
protein.
2
As in our patient's case, the onset of symptoms typically occurs during the second to third decade of
life.
5
6
7
The inflammatory process of the retinal vessels leading to vaso-occlusion is still not fully understood. However, this pathologic process leads to retinal ischemia that causes neovascularization. The newly formed vessels tend to bleed easily, and this plays a major role in decreasing the patient's
vision.
8
Eighty percent of patients with Eales Disease develop neovascularization either on the disc or
elsewhere,
9
and bilateral involvement of the eyes is
common.
5
6
7The classification system of Eales Disease is useful in assessing disease severity and in monitoring the effects of medical, laser or surgical
treatment.
10
It also provides a method of categorization based on both funduscopic and fluorescein angiographic variables that have a bearing on visual outcome prognostication. According to the classification system, our patient who experiences vitreous hemorrhages has Eales Disease,
Stage 3b.
10The treatment of Eales Disease is usually geared towards alleviating the symptoms of the disease. This is achieved by controlling retinal perivasculitis and decreasing the risks of vitreous hemorrhage from new blood vessels.
3 For almost all neovascularizations in the retina, regardless of the cause and if there is no contraindication, laser photocoagulation, which prevents proliferation of new vessels in the ischemic retina, is the mainstay of treatment.
11 Observation and corticosteroids are the ideal treatment options for inactive retinal vasculitis and active perivasculitis, respectively. Our patient was treated with corticosteroids and underwent laser photocoagulation, which improved her visual acuity. However, the recurrence of symptoms led to new tissue formation that would benefit from pars plana vitrectomy. For non-resolving vitreous hemorrhage, pars plana vitrectomy can be perfomed.
12 Vitrectomy ensures a clear optical axis that facilitates photocoagulation, relieves vitreous traction, and may promote regression of new blood vessels.
13 Having early vitrectomy - within 3 to 6 months from the onset of vitreous hemorrhage - yields improved visual acuity and favorable prognosis.
14 We explained the benefits of vitrectomy to the patient, but she was not willing to undergo the surgical procedure.
Eales Disease has a generally good prognosis and, with appropriate treatment, rarely leads to blindness.
5 7Our patient with Eales Disease has been coming to our clinic to be relieved of two rather bothersome symptoms in her left eye - floaters and blurring of vision. We were able to observe our patient long enough to document the recurrent nature of her symptoms. While we were giving her intermittent symptomatic treatments for the symptoms, we offered to do pars plana vitrectomy in the left eye to provide a more definitive treatment for her condition. She refused such surgical treatment, and she somehow came to stand having to go through laser photocoagulation and apply topical corticosteroids on her left eye every time these treatments are indicated.
In essence
Eales Disease is an inflammatory vaso-occlusive disease of the retina. It has been associated with tuberculosis or tuberculin protein exposure.
This disease among otherwise healthy young individuals is characterized by neovascularizations in the retina and subsequent vitreous hemorrhages.
Laser photocoagulation usually controls the formation of neovascularizations, and anti-inflammatory drugs decrease intra-ocular inflammation. Non-resolving hemorrhages may require vitrectomy. In general, prognosis is good among patients receiving prompt diagnosis and adequate treatment.
Acknowledgments
Our heartfelt gratitude goes to our mentors Dr Perfecto Domingo Jr, Dr Jocelyne Gonzales-Sy, and Dr David Miller who guided us in the management of our patient. We also sincerely appreciate the unwavering support of our colleagues and the staff of the Ophthalmology Outpatient Department at Southern Philippines Medical Center. We thank our patient, most of all, for her full cooperation during the preparation for this publication, for inspiring us to become efficient physicians, and for reminding us to always provide the best medical care to all our patients.
Competing interests
None declared
Access and license
This is an Open Access article licensed under the Creative Commons AttributionNonCommercial 4.0 International License, which allows others to share and adapt the work, provided that derivative works bear appropriate citation to this original work and are not used for commercial purposes. To view a copy of this license, visit https://creativecommons.org/licenses/by-nc/4.0/
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