A histopathological correlation of Bruch’s membrane calcification in pseudoxanthoma elasticum with clinical retinal imaging

摘要: Purpose: To investigate the histology of Bruch’s membrane calcification in pseudoxanthoma elasticum (PXE) and correlate this to clinical retinal imaging.Methods: Six post-mortem eyes from four PXE patients and one age-matched control were investigated. Eyes were stained with Osteosense 680RD, a fluorescent dye specific for apatite. Labelling was visualized with a confocal microscope with an excitation wavelength of 620nm and an emission wavelength of 680nm. Scanning electron microscopy coupled with energy-dispersive x-ray spectroscopy (SEM-EDX) and time of flight-secondary ion mass spectrometry (TOF-SIMs) were used to analyze the elemental and ionic composition at specific anatomical locations. The findings of one PXE patient were compared with prior clinical retinal imaging.Results: All whole-mounted PXE eyes revealed extensive Osteosense labelling in the central retina, changing to a …