Multimodal imaging including spectral domain OCT and confocal near infrared reflectance for characterisation of outer retinal pathology in pseudoxanthoma elasticum

¹ Department of Ophthalmology, University of Bonn, Ernst-Abbe-Str. 2, Bonn, 53127, Germany
² Department of Ophthalmology, University of Bonn, Bonn, Germany

^* To whom correspondence should be addressed. E-mail: peter.issa{at}ukb.uni-bonn.de.

	Abstract

Purpose: To investigate the value of multimodal confocal scanning laser ophthalmoscopy (cSLO) for phenotyping fundus lesions in patients with pseudoxanthoma elasticum (PXE) and to correlate these findings with morphological alterations detected by spectral domain optical coherence tomography (SD-OCT). Methods: Imaging was performed with a combined and simultaneous SD-OCT-cSLO (SpectralisHRA-OCT, Heidelberg Engineering, Heidelberg, Germany). OCT scans were placed at locations of interest on near-infrared (NIR) reflectance, fundus autofluorescence (FAF) and fluorescein angiography (FA) images. The instrument allowed for exact topographic correlation of findings on OCT and cSLO images. Results: NIR reflectance imaging showed highest sensitivity to detect angioid streaks and peau d orange compared to FAF or FA. On OCT scans, angioid streaks reliably showed breaks in Bruch membrane. Peau d orange was associated with alternating reflectivity within Bruch membrane. Characteristic mid-peripheral chorioretinal atrophies showed hyporeflective spaces involving the outer neurosensory retina. In eyes with pattern dystrophy like alterations, sub-neurosensory accumulation of material was observed within areas of increased FAF. Conclusions: SD-OCT in combination with cSLO imaging using NIR light locates the primary pathology of angioid streaks and peau d orange to Bruch membrane. NIR reflectance imaging may be superior to detect PXE-related fundus lesions at the level of Bruch membrane because blue laser light as used for FAF and FA is markedly absorbed by the pigment epithelium and therefore may only detect alterations if this cell layer is also affected. The findings indicate that multimodal cSLO and SD-OCT imaging of the outer retina allows for screening of PXE related retinal alterations.

Key Words: scanning laser ophthalmoscopy, optical coherence tomography, retinal pigment epithelium

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