Optical coherence tomography findings in pseudoxanthoma elasticum.
OBJECTIVE
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.
Ari Yaylali
Sevil
S
Department of Ophthalmology, Istanbul Goztepe Educational Hospital, Istanbul, Turkey. sevil.yaylali@gmail.com
Akcakaya
Aylin Ardagil
AA
Erbil
Hasan Hasbi
HH
Salar
Senem
S
Karakurt
Yücel
Y
D000328:Adult; D002829:Choroid / Q000473:pathology*; D005128:Eye Diseases / Q000175:diagnosis* / Q000209:etiology; D005451:Fluorescein Angiography; D005654:Fundus Oculi; D006801:Humans; D007908:Lens, Crystalline / Q000473:pathology*; D011561:Pseudoxanthoma Elasticum / Q000150:complications / Q000175:diagnosis*; D015203:Reproducibility of Results; D055213:Retinal Pigment Epithelium / Q000473:pathology*; D041623:Tomography, Optical Coherence / Q000379:methods*