Relentless Placoid Chorioretinitis With Branch Retinal Vein Occlusion and Secondary Peripheral Retinal Neovascularization

Abstract

Purpose:

We report a case of relentless placoid chorioretinitis (RPC) that developed branch retinal vein occlusion and peripheral retinal neovascularization in one eye.

Methods:

A case report is presented.

Results:

A 33-year-old healthy man presented with decreased visual acuity (20/150) in both eyes. Slit-lamp examination revealed anterior chamber and vitreous inflammation. Multiple yellow-white lesions were evident in the macula and scattered throughout the fundus. Following workup, a diagnosis of RPC was made. The patient was started on Pred Forte (prednisolone acetate 1%) and atropine drops. Three months later, visual acuity improved to 20/70 and 20/100 in the right and left eyes, respectively. At this time, fundus examination and fluorescein angiography revealed peripheral retinal neovascularization. Sectoral scatter laser photocoagulation was performed in the areas of nonperfusion.

Conclusions:

We describe a novel presentation of RPC associated with branch retinal vein occlusion and retinal neovascularization. Although the pathophysiology of RPC is believed to be primarily a choroidal vasculitis, retinal vascular changes may also occur, as observed in other white dot syndromes.

Introduction

Lim et al used the term “ampiginous” to describe a disease process that has features both of serpiginous chorioretinopathy (SC), and acute posterior multifocal placoid pigment epitheliopathy (APMPPE). ¹ Jones and colleagues coined the name “relentless placoid chorioretinitis” (RPC) for this condition in 2000, in light of the recurrent and progressive nature of the disease. ²

Patients with RPC typically present with sudden-onset painless blurry vision. Bilateral creamy, yellowish white placoid lesions are observed at the level of the choroid and retinal pigment epithelium. With time, these areas may develop atrophic pigmentary changes. A characteristic feature of RPC is the eventual presence of more than 50 lesions scattered bilaterally throughout the fundus, typically at different activity stages. ³ The lesions are smaller than those of SC or APMPPE. A prolonged course is not unusual for RPC, with recurrences months to years after onset. The similarities between SC, APMPPE, and RPC suggest a common etiology; however, the exact pathogenesis is still unknown.

Atypical features of APMPPE and SC include papillitis, periphlebitis, optic disc neovascularization, serous retinal detachment, and preretinal hemorrhage. ^1,4 Retinal vein occlusion (RVO) may occur uncommonly in both APMPPE ⁵ and SC ⁶ but to our knowledge has not been reported in RPC. This case report describes a unique case of RPC complicated by peripheral neovascularization secondary to branch vein occlusion.

Methods

Case Report

A 33-year-old healthy man was referred to the uveitis service with a 3-day history of decreased vision in each eye. His medical and ocular history was unremarkable. Findings from a review of systems was unremarkable. The patient did not experience a viral prodrome or headache, and he denied any recent travel, high-risk sexual activity, intravenous drug use, or exposure to tuberculosis.

Examination revealed a best-corrected visual acuity of 20/150 OU. Confrontational visual fields were full. Intraocular pressure was 9 mm Hg in each eye. Pupils were equal in size and reactive to light and accommodation. Slit-lamp examination demonstrated 1+ cell and 1+ flare in the anterior chamber in each eye and 1+ vitreous cell in each eye. Multiple creamy, yellow-white lesions were present at the level of the retinal pigment epithelium in the macula of both eyes (Figure 1A). Scattered throughout the rest of each fundus, similar but more atrophic lesions were observed, suggesting various stages of disease activity (Figure 1B.) Fluorescein angiography demonstrated early hypofluorescence and late hyperfluorescence of the active lesions in the macula (Figure 1C).

Figure 1.

(A) Color fundus photographs of the right and left eye demonstrate multifocal creamy, yellowish placoid lesions of the posterior pole at presentation. (B) Color fundus montage photographs of the right and left eye illustrate a multitude of peripheral lesions scattered throughout the fundus, many of which appear more atrophic than those in the macula. (C) Fluorescein angiography in the right macula illustrates early hypofluorescence with late hyperfluorescence and staining.

Serologic tests were performed and found to be negative for Lyme and syphilis, as was the result from a purified protein derivative Mantoux tuberculosis skin test. The angiotensin-converting enzyme level was normal. A chest x-ray revealed no evidence of hilar adenopathy. Magnetic resonance imaging of the brain produced normal results, with no evidence of cerebral vasculitis.

Based on the negative workup findings and clinical picture with widespread distribution of the lesions that appeared to be at different activity stages, a diagnosis of RPC was made. Oral prednisone 80 mg daily was prescribed; however, the patient discontinued the therapy after a few days. He agreed to take topical Pred Forte (prednisolone) 1 drop 4 times a day and atropine 1 drop twice a day.

Results

The patient’s best-corrected visual acuity improved to 20/70 OD and 20/100 OS by 3 months. Slit-lamp examination revealed a deep and quiet anterior chamber in each eye. The dilated fundus examination of both eyes revealed pigmentary changes of the previously observed lesions. Additionally, peripheral retinal neovascularization and intraretinal hemorrhages were appreciated inferotemporally in the left eye. (Figure 2, A and B). Fluorescein angiography illustrated capillary nonperfusion and dye leakage in the areas of neovascularization (Figure 2, C and D). Atrophic changes were appreciated on optical coherence tomography of the macula of both eyes, with disruption of the external limiting membrane and ellipsoid zone (Figure 2, E and F). Sectoral scatter laser photocoagulation was performed in the area of nonperfusion.

Figure 2.

Ultrawide-field pseudocolor fundus photographs of (A) the right and (B) left eye illustrate pigmented chorioretinal atrophy of most of the lesions 7 months after presentation. Inferotemporally in (B) the left eye, intraretinal hemorrhages and neovascularization (arrows) are observed. Ultrawide-field fluorescein angiography of the left eye demonstrates (C) early hyperfluorescence inferotemporally with leakage (D) in later phases in the area of neovascularization. Spectral domain–optical coherence tomography of the macula shows focal disruptions of the external limiting membrane and ellipsoid zone in (E) the fovea of the right eye and (F) perifoveally in the left eye.

Conclusions

RPC is a rare disease of unknown etiology that exhibits features of both APMPPE and SC. ¹ Optical coherence tomography angiography has shown inner choroidal ischemia to be the main focus of pathology in these diseases. Not surprisingly, choroidal neovascularization may form. ^1,7 However, abnormalities of the retinal circulation may also occur in APMPPE and SC, including retinal vasculitis and occlusion. Central RVO has been reported in APMPPE, and both central and branch RVO ^6,8,9 have been observed in SC. Additionally, neovascularization of the disc without obvious vein occlusion may occur in APMPPE and SC. ^8,10 Owing to the similarities of RPC with APMPPE and SC, one might expect these presentations to occur in RPC.

In cases of retinal vasculitis with or without inflammatory papillitis, RVO may occur via local inflammation, leading to leukostasis as well as injury to the vascular endothelium. These changes ultimately lead to thrombosis and insufficient oxygen supply to retinal tissues.

However, Blumenkranz and colleagues have reported a case of SC without obvious vasculitis that developed a peripheral BRVO with secondary retinal neovascularization. ⁶ Our case is similar and, to our knowledge, the first to show BRVO and retinal neovascularization in RPC. One possible explanation for BRVO formation in these cases is that the vasculitis was subclinical yet significant enough to cause thrombus formation. Another explanation may be that our imaging studies did not capture an “active” stage of occlusive vasculitis that subsequently led to neovascularization.

The optimal management strategy for RPC has not yet been established. Owing to the disease’s potentially recurrent and prolonged nature, steroid-sparing agents such as azathioprine, cyclosporine, mycophenolate mofetil, and adalimumab have been explored. Intravitreal triamcinolone has shown promise and has the benefit of reducing systemic medication adverse effects. If RVO occurs, patients should be followed for retinal neovascularization, in which case, scatter laser photocoagulation may be of benefit.

In this report, we described a rare and atypical presentation of RPC with neovascularization secondary to BRVO. Although the pathophysiology of RPC is believed to be primarily a choroidal vasculitis, retinal vascular changes may also occur, as observed in SC and APMPPE.