Abstract

P 187

Centrifugal spread of fundus abnormalities in pseudoxanthoma elasticum

Peter Charbel Issa1, Robert P. Finger1, Frank G. Holz2, Hendrik P. Scholl1
1Universitäts-Augenklinik Bonn, 2GRADE Reading Center, Universitäts-Augenklinik Bonn

Objective
Patients with pseudoxanthoma elasticum (PXE) exhibit characteristic findings at the ocular fundus such as angioid streaks, peau d’orange or salmon spots. To further investigate these lesions in-vivo, the Bruch’s membrane-retinal pigment epithelium (RPE) complex in patients with PXE was recorded with multi-modal imaging techniques capable of visualizing deep retinal structures with high contrast.
Methods
16 patients with confirmed PXE were investigated by indocyanine green (ICG) and fluorescein angiography, confocal near-infrared reflectance (NIR) and fundus autofluorescence imaging using a confocal scanning laser ophthalmoscope (Spectralis HRA-OCT, Heidelberg Engineering, Heidelberg, Germany). Composite fundus photography was performed using a fundus camera.
Results
A central area of decreased fluorescence on late phase ICG angiography was a characteristic finding in all patients. A second area of increased fundus reflectivity on NIR reflectance imaging extended further peripherally. The most eccentric area did neither show decreased ICG fluorescence nor increased fundus reflectivity. The three areas were separated by two transition zones of which the more peripheral one was the equivalent of peau d’orange. Angioid streaks did not extend into the third area.
Conclusions
The abnormalities detected by this multimodal imaging approach suggest a centrifugal spread of retinal pathology of the Bruch’s membrane-RPE complex in PXE. Decreased late phase ICG fluorescence appears to be a consistent sign of retinal pathology in PXE. Bruch’s membrane calcification may be the underlying cause for such increased reflectivity.

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