Purpose: To investigate, using multimodal imaging, the anatomy of neovascularization in eyes with enhanced S-cone syndrome.
Methods: Three eyes with neovascularization, from two patients with enhanced S-cone syndrome, were analyzed using fluorescein angiography, indocyanine-green and optical coherence tomography angiography imaging.
Results: The eyes reported had a demonstrable Type 3 neovascularization with evidence of retinal-retinal anastomoses on fluorescein angiography, indocyanine-green and optical coherence tomography angiography imaging. One eye that was initially without neovascularization, but with chronic macular edema developed a macular hemorrhage. This eye was treated with 8 injections of intravitreal bevacizumab over 29-months resulting in a final fibrovascular lesion. The characteristics of this final lesion share similarities to the two other eyes described. In all eyes and all exams, retinal vessels are observed to communicate with the subretinal fibrovascular lesion.
Conclusion: We provide evidence of retinal arteriovenous anastomosis of the superficial retinal plexus to a subretinal neovascular complex in patients with enhanced S-cone syndrome and point to similarities with Type 3 neovascularization in macular telengiectasia Type 2 (MacTel2) and age-related macular degeneration. These findings provide insights into the anatomy of neovascularization in these pathologies and may lead to hypotheses of their etiologies.