Venous Overload Choroidopathy With Coexisting Chronic Central Serous Chorioretinopathy and Uveal Effusion Syndrome

Abstract

Purpose: To report a rare case of bilateral pachychoroid spectrum disease presenting asymmetrically as chronic central serous chorioretinopathy (CSCR) in 1 eye and type 3 uveal effusion syndrome in the contralateral eye. Methods: A single case was reviewed. Results: A 47-year-old man presented with a 1-month history of decreased vision in the left eye while being on oral corticosteroids. Best-corrected visual acuity was 20/20 OD and 2/80 OS. Multimodal imaging revealed features of CSCR with an ink-blot leak in the right eye and idiopathic type 3 uveal effusion syndrome with exudative retinal and choroidal detachment in the left eye. The patient was treated with a tapering course of oral corticosteroids, resulting in complete resolution of both conditions. During a 2-year follow-up period, a recurrence of subretinal fluid was observed in the right eye. Conclusions: This unusual case highlights the spectrum of venous overload choroidopathy and emphasizes the overlapping yet distinct clinical features of CSCR and uveal effusion syndrome. Multimodal imaging plays a pivotal role in the diagnosis and management of such unique presentations.

Introduction

Central serous chorioretinopathy (CSCR), considered part of the pachychoroid spectrum disorders, is characterized by serous neurosensory detachment with or without pigment epithelial detachment (PED) and mostly affects middle-aged men. ¹ Depending on disease duration and the extent of retinal pigment epithelium (RPE) involvement, CSCR may present in acute or chronic forms. The acute form presents with serous retinal detachment (RD) with or without PED and minimal RPE alterations. It typically follows a self-limiting course, with spontaneous resolution within 3 to 4 months with a good visual prognosis. ¹ In contrast, the chronic form is characterized by widespread RPE atrophy secondary to diffuse retinal pigment epitheliopathy. Symptoms tend to be persistent and are often associated with a more severe vision loss. ² The bullous variant of CSCR is usually associated with exudative RD with multiple leakage points, PEDs, fibrinous exudation, RPE tears, and, rarely, serous choroidal detachments. ³ Numerous treatment modalities have been described for CSCR, with focal and micropulse laser photocoagulation, oral eplerenone, and low fluence photodynamic therapy being the most common. ⁴ Systemic corticosteroids are known to exacerbate CSCR and are therefore generally contraindicated. ⁵

Uveal effusion syndrome is a rare condition characterized by idiopathic exudative RD involving the choroid, ciliary body, and retina, thought to arise from impaired posterior segment venous drainage, usually associated with scleral thickening. ⁶ Uveal effusion syndrome has been classified into 2 types, namely nanophthalmic (type 1) and idiopathic, which is further subdivided into type 2 uveal effusion syndrome, in which the size of the eyeball is normal with associated scleral abnormalities, and type 3 uveal effusion syndrome, in which the size of the eyeball is normal, with no clinically evident scleral abnormalities. ⁷ The disease course is typically relapsing and remitting, with some patients responding to medical management with systemic corticosteroids, while others require surgical intervention in the form of deep sclerectomy. ⁷

Recent studies indicate that both uveal effusion syndrome and CSCR result from choroidal venous overload, placing them along a spectrum of venous overload choroidopathy and highlighting a significant overlap between the 2 conditions with regard to clinical features, imaging findings, and disease pathogenesis. ⁴ We present the long-term follow-up of a distinctive case exhibiting multimodal imaging features of chronic CSCR in 1 eye and uveal effusion syndrome in the contralateral eye that improved with systemic steroid therapy, underscoring the simultaneous occurrence of these 2 conditions.

Case Report

A 47-year-old man with no history of systemic illness presented with complaints of decreased vision in the left eye for the past 1 month. He had been previously diagnosed elsewhere with a case of Harada’s disease and was advised a tapering course of oral corticosteroids, which he did not take. He had no known systemic illnesses. There was no history of hearing loss, fever, headache, or neck stiffness. General examination did not reveal vitiligo, poliosis, or alopecia. His best-corrected visual acuity (BCVA) was 20/20 OD and 20/80 OS. Intraocular pressure was within normal limits in both eyes. Anterior segment examination of both eyes was unremarkable. Fundus examination of the right eye revealed RPE degenerative changes at the posterior pole, while that of the left eye revealed RPE degeneration at the posterior pole with subretinal fluid (SRF) at the macula, associated retinal folds, inferior exudative RD, and choroidal detachment (Figure 1).

Figure 1.

Color fundus photograph and fundus autofluorescence (FAF) of both eyes at presentation. (A) Color fundus photograph of the right eye showing retinal pigment epithelial (RPE) degenerative changes at the posterior pole, with an oval area of RPE atrophy just below the inferior arcade. (B) Color fundus photograph of the left eye showing RPE degeneration at the posterior pole along with subretinal fluid at the macula, retinal folds, and inferior exudative retinal detachment with choroidal detachment (white arrows). (C) FAF of the right eye revealing an oval area of descending hypoautofluorescence (black arrow). (D) FAF of the left eye demonstrating speckled hypoautofluorescence at the posterior pole.

Spectral-domain optical coherence tomography (SD-OCT) of the right eye revealed a normal foveal contour with a dry macula. SD-OCT of the left eye revealed serous macular detachment with subretinal hyperreflective material suggestive of fibrinous exudation, along with hyperreflective dots. Enhanced depth imaging OCT revealed thickened choroids in both eyes, with a subfoveal choroidal thickness of 432 µm in the right eye and 560 µm in the left eye (Figure 2).

Figure 2.

Spectral-domain optical coherence tomography of both eyes at presentation. (A) Diagonal radial line scan through the fovea of the right eye showing a normal foveal contour with photoreceptor atrophy corresponding to the area of the oval-shaped defect. A thickened choroid is also visualized. (B) Horizontal raster line scan of the left eye revealing serous macular detachment with subretinal hyperreflective material and posterior shadowing, suggestive of subretinal fibrin (white asterisk). (C) Vertical raster line scan of the left eye demonstrating subretinal hyperreflective material suggestive of subretinal fibrin.

Fundus autofluorescence (FAF) of the right eye revealed an oval area of descending hypoautofluorescence at the inferotemporal arcade with surrounding hyperautofluorescence, suggestive of a teardrop-shaped RPE atrophic tract. FAF of the left eye revealed speckled hypoautofluorescence at the posterior pole (Figure 1). Fundus fluorescein angiography (FA) of the right eye showed diffuse retinal pigment epitheliopathy with an ink-blot leak temporal to the fovea, along with a descending oval-shaped window defect at the inferotemporal arcade, consistent with an RPE atrophic tract suggestive of chronic CSCR. Fundus FA of the left eye showed multiple ink-blot leaks at the posterior pole, suggestive of bullous CSCR, along with mid-peripheral diffuse granular hyperfluorescence. Additionally, disc hyperfluorescence was observed in the mid phase, increasing in intensity toward the late phase.

Ultra-widefield fundus FA demonstrated inferior peripheral choroidal detachment with associated RD (Figure 3). An A-scan revealed axial lengths of 22.81 mm in the right eye and 22.67 mm in the left eye. Anterior chamber depth of the right eye was 2.27 mm and that of the left eye was 2.28 mm. B-scan ultrasound of the left eye confirmed the presence of exudative RD and serous choroidal detachments, primarily localized inferiorly and temporally. The posterior retinochoroidoscleral thickness was normal (1.78 mm), with no features suggestive of posterior scleritis (Figure 4).

Figure 3.

Fundus fluorescein angiography (FA) of both eyes. (A, B) Early and late phase fundus FAs of the right eye revealing an ink-blot leak temporal to the fovea, along with a descending oval-shaped window defect at the inferotemporal arcade corresponding to an RPE atrophic tract, suggestive of chronic central serous chorioretinopathy. (C, D) Fundus FA of the left eye showing multiple ink-blot leaks at the posterior pole, along with midperipheral granular hyperfluorescence. Additionally, disc hyperfluorescence with late leakage is observed. (E, F) Early and late phase ultra-widefield fundus FAs of the left eye revealing inferior peripheral retinal detachment and associated choroidal detachment.

Figure 4.

B-scan ultrasound of both eyes. (A, B) Horizontal axial and transverse inferior B-scans of the right eye, respectively, revealing an optically empty vitreous cavity with normal retinochoroidoscleral thickness. (C, D) Transverse temporal and inferior B-scans of the left eye, respectively, revealing increasing retinochoroidoscleral thickness, along with a hypoechoic dome-shaped detachment suggestive of choroidal detachment and a hyperechoic line with poor aftermovement, suggestive of inferior retinal detachment.

Based on the clinical examination and imaging findings, the patient was diagnosed with chronic CSCR in the right eye and type 3 uveal effusion syndrome in the left eye. He was started on a tapering course of oral prednisolone (60 mg). Three months after treatment, his BCVA was 20/20 OD and improved to 20/25 OS. Multimodal imaging, including color fundus photography and FAF, showed diffuse RPE degeneration and pigmentary changes with descending atrophic tracts in both eyes, along with resolution of the RD and choroidal detachments in the left eye (Figure 5). SD-OCT revealed a normal foveal contour in both eyes with residual RPE changes, along with minimal reduction in subfoveal choroidal thickness; the left eye also showed an organized fibrin clump along the nasal macula (Figure 6). A repeat B-scan ultrasound confirmed the absence of retinal and choroidal detachments and showed a reduction in retinochoroidoscleral thickness in the left eye to 1.41 mm (Figure 7).

Figure 5.

Color fundus photographs and fundus autofluorescence (FAF) of both eyes after treatment and at final follow-up. (A) Color fundus photograph of the right eye showing retinal pigment epithelial (RPE) degenerative changes at the posterior pole, with an oval area of RPE atrophy just below the inferior arcade. (B) Color fundus photograph of the left eye showing RPE degeneration at the posterior pole. (C) FAF of the right eye revealing an oval area of descending hypoautofluorescence (black arrow). (D) FAF of the left eye demonstrating speckled hypoautofluorescence at the posterior pole.

Figure 6.

Spectral-domain optical coherence tomography of both eyes after treatment and at final follow-up. (A) Diagonal radial line scan through the fovea of the right eye showing a normal foveal contour with shallow subretinal fluid in the inferotemporal macula, suggestive of recurrence of chronic central serous chorioretinopathy. A thickened choroid is also visualized. (B) Diagonal radial line scan of the left eye revealing a normal foveal contour, along with a dense subretinal hyperreflective lesion with posterior shadowing suggestive of organized fibrin (white asterisk), in the presence of a thickened choroid.

Figure 7.

B-scan ultrasound of both eyes after treatment and at final follow-up. (A, B) Horizontal axial and transverse inferior B-scans of the right eye, respectively, revealing an optically empty vitreous cavity with a normal retinochoroidoscleral thickness. (C, D) Transverse temporal and inferior B-scans of the left eye, respectively, demonstrating an optically empty vitreous cavity with normal retinochoroidoscleral thickness.

The patient was observed for the next 2 years with no evidence of disease recurrence. However, at the most recent follow-up visit, he showed shallow temporal macular SRF with a dry foveal center in the right eye, suggestive of a recurrence of CSCR, whereas the left eye remained stable over the long term (Figure 6).

Conclusions

We describe the multimodal imaging features of a unique case presenting with chronic CSCR in 1 eye, with a late recurrence, and type 3 uveal effusion syndrome in the other eye that responded well to systemic corticosteroids. CSCR is a chorioretinal disease characterized by serous detachment of the neurosensory retina and/or the RPE, resulting in the accumulation of SRF. ¹ When SRF persists beyond 3 months, CSCR is considered chronic, which may lead to permanent visual impairment, with patients often continuing to suffer from the disease even in advanced age. ⁸

Characteristic findings include RPE atrophic patches and gravitating RPE tracts on fundus examination; photoreceptor outer segment irregularities, PEDs, and intraretinal cystoid degeneration on SD-OCT; widespread stippled hyperfluorescence with progressive late oozing suggestive of diffuse retinal pigment epitheliopathy seen on fundus FA; and multifocal choroidal vascular hyperpermeability and congestion on indocyanine green angiography (ICGA). ¹ An atypical bullous variant of chronic CSCR associated with exudative RD and choroidal detachment has also been reported in the literature. ³ Low-fluence PDT has been shown to induce disease resolution in chronic CSCR with this bullous variant. ³

The right eye of our patient maintained good vision at presentation, and multimodal imaging revealed a dry macula on SD-OCT. However, after 2 years, the eye developed serous macular detachment temporally, along with features of diffuse retinal pigment epitheliopathy on fundus FA, along with an ink-blot leak temporal to the fovea, suggestive of chronic CSCR with late recurrence.

Uveal effusion syndrome is a relatively uncommon condition characterized by a series of fundus changes, including exudative RD and choroidal detachments, with type 3 uveal effusion syndrome demonstrating a normal axial length and clinically normal sclera, and hence termed idiopathic. Uveal effusion syndrome is a chronic disorder that typically occurs in otherwise healthy middle-aged individuals, with a male preponderance. Proposed hypotheses on its pathogenesis include vortex vein occlusion, increased choroidal permeability, intrinsic choroidal changes, and decreased scleral permeability. ⁹

Thickened sclera, with disordered collagen fibers and increased mucopolysaccharide content, reduces permeability to albumin, disrupting osmotic gradients and leading to fluid retention in the suprachoroidal and supraciliary spaces. ⁹ Multimodal imaging of the left eye in our patient revealed posterior-pole pigmentary changes with exudative RD and choroidal detachments, along with midperipheral diffuse granular hyperfluorescence on fundus FA, normal axial length, and normal scleral thickness, findings suggestive of type 3 uveal effusion syndrome.

A differential diagnosis of atypical chronic CSCR with exudative RD and choroidal detachment was also considered for the left eye of our patient; however, the extensive exudative retinal and choroidal detachments, midperipheral granular hyperfluorescence with optic disc staining on fundus FA, absence of typical focal RPE leaks seen in CSCR, and complete resolution with oral systemic corticosteroids were not consistent with CSCR. We acknowledge that CSCR typically worsens with corticosteroid therapy; however, in our patient, no such deterioration was observed in the right eye during high-dose treatment. This lack of worsening may reflect individual variability in steroid responsiveness, ¹⁰ the chronic and relatively quiescent nature of the disease in the right eye at presentation, or the possibility that both eyes were manifestations of a broader venous overload process.

The terms pachychoroid and pachychoroid disease describe a phenotype characterized by focal or diffuse choroidal thickening resulting from dilatation of Haller’s layer vessels (pachyvessels), accompanied by thinning of Sattler’s layer, with or without overlying RPE abnormalities. ¹¹ Similar to the pachychoroid spectrum, which includes CSCR, type 3 uveal effusion syndrome is also characterized by a thickened choroid with dilated choroidal vessels. In addition, increased peripheral choroidal thickness has been reported in type 3 uveal effusion syndrome.

Recent ultra-widefield ICG studies have highlighted choroidal venous outflow stasis, causing an overload of the choroidal venous circulation, as a potential mechanism underlying pachyvessel and pachychoroid formation in CSCR. Choroidal venous overload and vortex vein congestion may result from regional factors such as obstruction of vortex veins, increased anterior scleral thickness, and asymmetry of the vortex veins. ⁴

CSCR is a pachychoroid spectrum disorder characterized by the accumulation of fluid in the macular region, causing serous macular detachment. Among recent insights into CSCR pathophysiology, choroidal venous congestion has been highlighted as a potential mechanism underlying pachyvessel and pachychoroid formation. ⁴ Obstruction of the dominant vortex veins draining the macula may cause enlargement of Haller’s layer and subsequent development of SRF. Vortex vein dilatation has been demonstrated in 83.35% of eyes with CSCR imaged using ultra-widefield ICGA, thereby contributing to atypical forms of CSCR that overlap with choroidal effusion and even choroidal detachments, resembling uveal effusion syndrome. ⁴ Venous outflow abnormalities caused by vortex vein obstruction produce a constellation of findings, including venous dilatation and choroidal hyperpermeability, collectively termed venous overload choroidopathy. Extraocular factors, such as vortex vein occlusion due to scleral thickening, can contribute to choroidal congestion and lead to uveal effusion syndrome, while intrinsic abnormalities in the local control of venous outflow may also lead to choroidal congestion, as observed in CSCR.^4,12

Type 3 uveal effusion syndrome was diagnosed in the left eye of our patient based on multimodal imaging findings, as well as the complete resolution of the disease following treatment with systemic corticosteroids, with no recurrence observed over long-term follow- up. Onoe et al ¹² reported a similar case of bilateral pachychoroid disease, with type 3 uveal effusion syndrome manifesting as exudative RD and choroidal detachment in the right eye and CSCR in the left eye. In their case, PDT was performed in both eyes; however, the right eye did not respond, following which deep sclerectomies with mitomycin C were performed, leading to disease resolution in the right eye. They also reported peripheral pachychoroid in the right eye, along with the absence of scleral thickening and glycosaminoglycan deposition, thereby confirming the diagnosis of type 3 uveal effusion syndrome. ¹²

In summary, we describe a rare case of chronic CSCR in 1 eye coexisting with type 3 uveal effusion syndrome in the contralateral eye, both being overlapping entities within the spectrum of venous overload choroidopathy. Multimodal imaging plays a crucial role in distinguishing between these 2 conditions, thereby enabling the provision of appropriate treatment for each.