Table 2.

Overview of imaging and pathology findings of RVCL-S.

System	Imaging findings	Pathology findings
Eyes
	Fundoscopy/fluorescein angiography:- Telangiectasia- Micro-aneurysms- Cotton wool spots- Perifoveal capillary obliterations- Neovascularization's- Fluorescein leaks- Ischemia Optical coherence tomography:- Peripapillary retinal nerve fiber layer thinning- Reduced total macular volume	Scattered microinfarcts Thickened hyalinized retinal arterial walls Focal areas of disruption of the ganglion cell layer and inner nuclear layer
Brain
	MRI:- White Matter Hyperintensities- T2 hyperintense lesions with long-lasting contrast enhancement- Tumefactive lesions surrounded by edema CT:- Calcifications	Multiple (often confluent) foci of ischemic necrosis of white matter Vessel wall thickening, luminal stenosis and telangiectasias A modest chronic inflammatory cell infiltrate Focal calcifications and reactive astrocytosis Myelin loss
Kidney
	–	Renal arteriolosclerosis Focal or diffuse glomerulosclerosis
Liver
	Ultrasound:- Liver cirrhosis	Nodular regenerative hyperplasia Micro and macro vesicular steatosis Periportal inflammation Bridging and portal fibrosis
Gastrointestinal
	Gastroscopy:- Telangiectasia- Angiodysplasia Colonoscopy:- Blotchy, erythematous mucosa- Ulcerations- Telangiectasia- Angiodysplasia	Telangiectasias Thin-walled vasculopathy Focal or diffuse (chronic) inflammation

CT = computed tomography; MRI = magnetic resonance imaging.