Purpose To further elucidate retinal findings and retinal vessel changes in
Cerebral autosomal dominant arteriopathy with subcortical infarcts and
leukoencephalopathy (CADASIL) patients by means of high resolution retinal
imaging. Methods 28 eyes of fourteen CADASIL patients and an equal number of
control subjects underwent confocal scanning laser ophthalmoscopy (cSLO),
spectral-domain optical coherence tomography (SD-OCT), retinal nerve fibre
layer (RNFL) measurements, fluorescein and indocyanine angiography. Three
vessel measurement techniques were applied: RNFL thickness, a semiautomatic
software tool based on cSLO images and manual vessel outlining based on SD-
OCT. Results Mean age of patients was 56.2±11.6 years. Arteriovenous nicking
was present in 22 (78.6%) eyes and venous dilation in 24 (85.7%) eyes. Retinal
volume and choroidal volume were 8.77±0.46 mm3 and 8.83±2.24 mm3. RNFL
measurements showed a global increase of 105.2 µm (Control group: 98.4 µm; p =
0.015). Based on semi-automatic cSLO measurements, maximum diameters of
arteries and veins were 102.5 µm (106.0 µm; p = 0.21) and 128.6 µm (124.4 µm;
p = 0.27) respectively. Manual SD-OCT measurements revealed significantly
increased mean arterial 138.7 µm (125.4 µm; p<0.001) and venous 160.0 µm
(146.9; p = 0.003) outer diameters as well as mean arterial 27.4 µm (19.2 µm;
p<0.001) and venous 18.3 µm (15.7 µm; p<0.001) wall thicknesses in CADASIL
patients. Conclusions The findings reflect current knowledge on
pathophysiologic changes in vessel morphology in CADASIL patients. SD-OCT may
serve as a complementary tool to diagnose and follow-up patients suffering
from cerebral small-vessel diseases
