Optical Coherence Tomography Findings in Pseudoxanthoma Elasticum

• Published in: European journal of ophthalmology Vol. 20; no. 2; pp. 397 - 401
• Main Authors: Yaylali, Sevil Ari, Akcakaya, Aylin Ardagil, Erbil, Hasan Hasbi, Salar, Senem, Karakurt, Yücel
• Format: Journal Article
• Language: English
• Published: London, England SAGE Publications 01-03-2010
• Subjects: Adult; Choroid - pathology; Eye Diseases - diagnosis; Eye Diseases - etiology; Fluorescein Angiography; Fundus Oculi; Humans; Lens, Crystalline - pathology; Pseudoxanthoma Elasticum - complications; Pseudoxanthoma Elasticum - diagnosis; Reproducibility of Results; Retinal Pigment Epithelium - pathology; Tomography, Optical Coherence - methods; Optical coherence tomography; Pseudoxanthoma elasticum
• ISSN: 1120-6721; 1724-6016
• DOI: 10.1177/112067211002000221

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.