Bilateral central serous chorioretinopathy in a patient with angioid streaks.

This report describes a case of bilateral central serous chorioretinopathy (CSCR) in a patient with angioid streaks. A 39-year-old male was referred for worsening vision. Best-corrected visual acuity (BCVA) at presentation was 5/10 in his right eye and 4/10 in his left eye. Fundus examination, fluorescein angiography, and optical coherence tomography were compatible with angioid streaks in both eyes associated with macular atrophy due to previous CSCR attack in the right eye and active nonresolving CSCR in the left eye. Macular laser photocoagulation was done in the left eye. After 3 months, subretinal fluid was absorbed and BCVA improved to 7/10. CSCR may occur rarely in patients with angioid streaks and ophthalmologists should consider CSCR in cases with angioid streaks and vision deterioration. Copyright:

Introduction

In 1889, Doyne described the angioid streaks as irregular lines that extend from optic disc margin to the peripheral retina and in 1892, Knapp initially used the term “angioid streaks” for these lesions.[12]

Typically, angioid streaks are bilateral irregular, dark reddish brown, band-shaped dehiscence in the fragile, and calcified Bruch's membrane.[3] Concurrent systemic disease is present in more than 50% of patients with angioid streaks.[4] Pseudoxanthoma elasticum is the most common systemic disease in association with angioid streaks and Ehlers–Danlos syndrome, Marfan syndrome, Paget disease, hemoglobinopathies, and hypercalcemia are other common systemic associated disorders.[5]

Frequently, angioid streaks are incidental findings and patients are asymptomatic unless the lesions involve the foveal center.[4] Optic disc drusen, retinal or subretinal hemorrhage, macular hole, retinal telangiectasia, polypoidal choroidal vasculopathy, choroidal neovascularization (CNV), and traumatic choroidal rupture may be observed over time in patients with angioid streaks.[67891011]

Central serous chorioretinopathy (CSCR) is a rare ocular disorder in association with angioid streaks, and to our knowledge, only one case of concurrent CSCR and angioid streaks has been reported.[12] Here, we report a case of bilateral CSCR in a patient with angioid streaks.

Case Report

A 39-year-old male was referred to the Retina Clinic of Baqiyatallah Hospital, Tehran, Iran, because of bilateral vision loss. Best-corrected visual acuity (BCVA) at presentation was 5/10 in his right eye and 4/10 in his left eye. The patient noted a history of past vision loss attacks that improved spontaneously in his both eyes and a history of stable vision loss in his left eye for the duration of 4 months. On slit-lamp examination, the anterior segment of both eyes was unremarkable. On fundus examination, irregular jagged bands that radiate from optic disc to retinal periphery were seen in both eyes. Furthermore, macular pigmentation and retinal pigmented epithelium (RPE) mottling in the right eye and serous retinal detachment in the left eye were noticed. OCT was done and revealed outer retinal irregularity and ellipsoid zone (EZ) disruption in the right eye and subretinal fluid (serous retinal detachment) with a small pigment epithelial detachment in the left eye [Figure 1]. In fluorescein angiography (FA), hyperfluorescence lines (window defect) radiating from optic disc were seen in both eyes along with multiple hyperfluorescence dots and 3 pin-point leaking areas in the macula in the right eye and expansile dot associated with RPE atrophic track in the left eye [Figure 2]. Overall, macular atrophy due to previous CSCR attacks in the right eye and active nonresolving CSCR in the left eye associated with bilateral angioid streaks were diagnosed. The patient had no underlying systemic disease or risk factor for CSCR.

Figure 1

Optical coherence tomography. The right eye macular optical coherence tomography (up) shows outer retina irregularity and ellipsoid zone disruption. The left eye macular optical coherence tomography (down) shows subretinal fluid (serous retinal detachment)

Figure 2

Fluorescein angiography shows hyperfluorescence lines (window defect) radiating from optic disc in both eyes along with multiple hyperfluorescence dots and 3 pin-point leaking areas in the macula in the right eye and expansile dot associated with RPE atrophic track in the left eye

Macular laser photocoagulation was done in the left eye. Laser photocoagulation was performed using an argon laser with a spot size of 100μ and duration of 0.1 s to create white burns in leaky area in the FA.

After 3 months, subretinal fluid was absorbed and BCVA improved to 7/10.

Discussion

Both CSCR and angioid streaks may be associated with multiple systemic diseases or underlying risk factors. For example, angioid streaks are more common in patients with pseudoxanthoma elasticum, Ehlers–Danlos syndrome, Marfan syndrome, Paget disease, hemoglobinopathies, and hyperkalcemia, and none of them are common risk factors for CSCR. On the other hand, several factors such as pregnancy, type A personality, Cushing's disease, alcohol consumption, organ transplant, and hypertension are considered to be predisposing factors for CSCR.

In the present report, bilateral CSCR was diagnosed in a patient with angioid streaks. In our opinion, this accompaniment rarely happens. Sarmad et al. reported a known case of angioid streaks with disciform scar due to choroidal neovascular membrane in patient's right eye and recent serous macular detachment in patient's left eye.[12] BCVA was 3/60 in patient's right eye and 6/9 in patient's left eye. A diagnosis of CSCR was made based on OCT, FA, and indocyanine green angiography in the patient's left eye. After 3 years of follow-up, visual acuity was deteriorated to 6/12 in the left eye and therefore half-fluence photodynamic therapy (PDT) with verteporfin was done but PDT had poor result and vision loss progressed further.

Usually, angioid streaks need no treatment, but its sight-threatening complications may require treatment. CNV is one of the most common complications of angioid streaks that frequently leads to considerable visual loss.[131415] Laser photocoagulation, PDT, and intravitreal anti-vascular endothelial growth factor (VEGF) injection were used with different results in the treatment of angioid streaks-associated CNV.[111617181920]

CSCR usually resolves spontaneously and does not require treatment; however, in chronic cases, therapeutic intervention, including PDT, anti-VEGF injection, and laser photocoagulation, is recommended.[12] Because extrafoveal fluorescein leakage was seen in the FA, we used macular laser photocoagulation to treat active chronic CSCR in the left eye of this patient with acceptable anatomic and visual results. However, in the right eye, due to inactivity of CSCR and lack of subretinal fluid, only follow-up of the patient was performed without medical intervention.

Conclusion

Ophthalmologist should be aware that CSCR may occur in eyes with angioid streaks and they have to differentiate it from CNV. Macular laser photocoagulation can help subretinal fluid absorption and improve vision in these patients.

Declaration of patient consent

The authors certify that they had obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initial will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.