Central serous chorioretinopathy and angioid streaks: coincidental?

Abstract

Background To report an unusual case of central serous chorioretinopathy in a patient with angioid streaks. Case presentation The authors describe a case report of a 26-year old male patient presenting acute scotoma and metamorphopsia in OD. He had been diagnosed with angioid streaks complicated with choroidal neovascularization and referred to us for treatment. The patient presented an ETDRS score of 85 letters (20/20) in OD and in OS. The anterior segment examination was unremarkable. Fundoscopy revealed bilateral angioid streaks (AS) and peau d’orange, as well as a small neurosensory retinal detachment in the macula of OD. A multimodal retinal analysis, including fundus photography, infra-red and fundus autofluorescence imaging, spectral-domain optical coherence tomography, optical coherence tomography angiography, fluorescein and indocyanine green angiography was performed. The diagnosis of central serous chorioretinopathy was made in the absence of any identifiable choroidal neovascularization. He was submitted to half-dose photodynamic therapy with verteporfin. One month later, he reported no visual complaints, his vision was 85 letters (20/20) in OD and a complete resolution of the sub-retinal fluid was registered. No signs of choroidal neovascularization were detected on the optical coherence tomography angiography (OCTA). A complete medical workup evaluation was made to exclude systemic diseases usually associated with AS. Conclusions To the authors’ knowledge, this is the second reported case of CSC associated with angioid streaks. The focal abnormalities in the Bruch’s membrane and the irregular vascular choriocapillary network associated with AS might predispose to CSC.

Angioid Streaks Remain a Challenge in Diagnosis, Management, and Treatment

“…Another interesting point is that OCT may be able to detect sub-retinal fluid in the absence of an active CNV in some PXE patients [ 5 , 76 ]. This OCT finding may resemble central serous retinopathy (CSR) [ 5 , 76 , 77 ] ( Figure 3 ). It is proposed that the fluid in these cases may have appeared due to RPE pump dysfunction, increased hydrophobicity of the Bruch’s membrane, or both [ 5 , 77 ].…”

“…This OCT finding may resemble central serous retinopathy (CSR) [ 5 , 76 , 77 ] ( Figure 3 ). It is proposed that the fluid in these cases may have appeared due to RPE pump dysfunction, increased hydrophobicity of the Bruch’s membrane, or both [ 5 , 77 ]. Limited data suggest that this type of sub-retinal fluid does not respond to anti-VEGF agents or carbonic anhydrase inhibitors [ 5 , 77 ].…”

“…It is proposed that the fluid in these cases may have appeared due to RPE pump dysfunction, increased hydrophobicity of the Bruch’s membrane, or both [ 5 , 77 ]. Limited data suggest that this type of sub-retinal fluid does not respond to anti-VEGF agents or carbonic anhydrase inhibitors [ 5 , 77 ]. In chronic cases, the persistence of this non-CNV-associated sub-retinal fluid may result in the development of a vitelliform lesion that will appear markedly hyper-reflective on an OCT scan and will demonstrate increased hyper-autofluorescence on FAF imaging [ 5 ].…”