Multimodal retinal vessel analysis in CADASIL patients


  • F. Alten
  • J. Motte
  • C. Ewering
  • N. Osada
  • C.R. Clemens
  • E.M. Kadas
  • N. Eter
  • F. Paul
  • M. Marziniak


  • PLoS ONE


  • PLoS ONE 9 (11): e112311


  • 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 microm (Control group: 98.4 microm; p = 0.015). Based on semi-automatic cSLO measurements, maximum diameters of arteries and veins were 102.5 microm (106.0 microm; p = 0.21) and 128.6 microm (124.4 microm; p = 0.27) respectively. Manual SD-OCT measurements revealed significantly increased mean arterial 138.7 microm (125.4 microm; p<0.001) and venous 160.0 microm (146.9; p = 0.003) outer diameters as well as mean arterial 27.4 microm (19.2 microm; p<0.001) and venous 18.3 microm (15.7 microm; 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.