Case report: Bilateral central serous chorioretinopathy-like abnormalities in a man with pulmonary arterial hypertension

Abstract

Background: Pulmonary arterial hypertension (PAH) leads to progressive increases in pulmonary vascular resistance, right heart failure, and death if left untreated. Ocular complications secondary to PAH were less reported. In this study, we reported a case of bilateral visual loss and metamorphopsia in a patient with PAH, who developed central serous chorioretinopathy (CSCR)-like abnormalities and optic disc atrophy.

Case summary: A 45-year-old man presented with decreasing central vision and metamorphopsia in both eyes. He had a history of PAH and 6-year history of low-dose oral sildenafil treatment. Slit-lamp examination revealed prominent dilated and tortuous episcleral and conjunctival vessels. Ultrawide-field color picture showed retinal pigment epithelial mottling and atrophy in ring-like configurations. Ultrawide-field autofluorescence showed multiple irregular hyper-autofluorescence with a constellation-like pattern surrounding the optic nerve head and macular region. Optical coherence tomography angiography (OCTA) b-scan demonstrated CSCR-like changes. Swept-source optical coherence tomography (SS-OCT) analysis showed optic nerve atrophy with enlarged cup/disc ratio in right eye, which was confirmed with perimetry. Fluorescein angiography (FA) showed marked leakage of macula and optic nerve head with time, cystoid macular edema, early blocking with late staining of the flecks as shown in the backgrounds of infrared and autofluorescence, and mild leakage in peripheral retina. Indocyanine green angiography (ICGA) showed dilation, tortuosity and congestion of all vortex veins without obvious leakage.

Conclusion: Undertreated PAH may cause the congestion of the choroid and induce CSCR-like abnormalities.

Keywords: case report; central serous chorioretinopathy; fundus abnormalities; hypertension - complications; macular edema; pulmonary arterial hypertension; vascular disease.

FIGURE 1

Prominent dilated episcleral and conjunctival vessels were noted in both eyes without signs of rubeosis of iris or iridocorneal angle closure. OD, right eye; OS, left eye.

FIGURE 2

Ultrawide-field color pictures and autofluorescence in both eyes. (A,B) Ultrawide-field color pictures showed mild retinal venous dilation without hemorrhages and numerous flecks with subtle yellow subretinal flecks surrounding the papillomacular region. (C,D) Fundus autofluorescence showed mild hypo-autofluorescence in the foveal center and marked hyper-autofluorescence of these flecks surrounding the optic nerve head and macular region in both eyes. In left eye, gravitational track was observed on fundus autofluorescence.

FIGURE 3

Marked cystoid macular edema with intraretinal fluid, subfoveal neurosensory retinal detachment and pachychoroid on OCTA b-scan in both eyes. OD, right eye; OS, left eye; SCP, superficial capillary plexus; DCP, deep capillary plexus.

FIGURE 4

Fluorescein angiography (FA) and indocyanine green angiography (ICGA) showed bilateral changes of retinal and choroidal vessels. Infrared (IR) image showed patched hypo-reflection in macula and hyper-reflective flecks surrounding the macula and optic discs in both eyes. Background autofluorescence (BAF) image showed patched hypo-autofluorescence in macula and hyper-autofluorescence flecks surrounding the macula and optic discs in both eyes. Both FA and ICGA showed delayed and slow perfusion of the retinal and choroidal vessels. The flecks were early blocking with late staining. FA showed macular leakage with pronounced cystoid macular edema, deep masking in the area of the choroidal detachment. ICGA showed dilation and congestion of the choroidal vessels, especially vortex veins, in both eyes. SD-OCT with the Heidelberg Spectralis showed cystoid macular edema with intraretinal fluid and subretinal fluid accumulation in both eyes. FA, fluorescein angiography; ICGA, indocyanine green angiography; IR, infrared; BAF, background autofluorescence; OD, right eye; OS, left eye.