PURPOSE: To describe optical coherence tomography (OCT) findings of ocular lesions in pseudoxanthoma elasticum (PXE). METHODS: Sixteen eyes of 8 patients with PXE and 20 eyes of 10 age-matched healthy volunteers were included in the study. All patients in the study and control group underwent a complete ophthalmologic examination and OCT. Fluorescein angiography was performed on the patients with PXE. RESULTS: In the areas of peau de orange mottling, OCT demonstrated increased reflectivity on the level of retinal pigment epithelium (RPE), Bruch membrane, and choriocapillaris complex. OCT scans of crystalline body showed a hyperreflective shell and an isoreflective or hyporeflective core. OCT scans of the angioid streaks demonstrated thinning in RPE-Bruch membrane-choriocapillaris complex. CONCLUSIONS: Disturbances on the level of RPE, choroid, and Bruch membrane may be responsible for the ocular lesions in PXE. OCT may give clues to the pathophysiology of the retinal lesions. Spectral domain OCT could provide more details and information. Further studies using this new technology should be performed.
PURPOSE: To describe optical coherence tomography (OCT) findings of ocular lesions in pseudoxanthoma elasticum (PXE). METHODS: Sixteen eyes of 8 patients with PXE and 20 eyes of 10 age-matched healthy volunteers were included in the study. All patients in the study and control group underwent a complete ophthalmologic examination and OCT. Fluorescein angiography was performed on the patients with PXE. RESULTS: In the areas of peau de orange mottling, OCT demonstrated increased reflectivity on the level of retinal pigment epithelium (RPE), Bruch membrane, and choriocapillaris complex. OCT scans of crystalline body showed a hyperreflective shell and an isoreflective or hyporeflective core. OCT scans of the angioid streaks demonstrated thinning in RPE-Bruch membrane-choriocapillaris complex. CONCLUSIONS: Disturbances on the level of RPE, choroid, and Bruch membrane may be responsible for the ocular lesions in PXE. OCT may give clues to the pathophysiology of the retinal lesions. Spectral domain OCT could provide more details and information. Further studies using this new technology should be performed.
Authors: Sara Risseeuw; Edwin Bennink; Maarten G Poirot; Pim A de Jong; Wilko Spiering; Saskia M Imhof; Redmer van Leeuwen; Jeannette Ossewaarde-van Norel Journal: Transl Vis Sci Technol Date: 2020-07-23 Impact factor: 3.283