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Adaptive Optics Imaging in Patients Affected by Pseudoxanthoma Elasticum
Vittoria Murro1, Dario Pasquale Mucciolo1, Dario Giorgio1
1Department of Neuroscience, Psychology, Drug Research and Child Health, University of Florence, Florence, Italy.
American Journal of Ophthalmology
|December 14, 2020
Summary
Adaptive optics ophthalmoscopy revealed distinct photoreceptor changes and three types of angioid streaks in pseudoxanthoma elasticum (PXE) retinopathy. This advanced imaging identified small crystalline bodies previously unseen.
Area of Science:
- Ophthalmology
- Genetics
- Medical Imaging
Background:
- Pseudoxanthoma elasticum (PXE) is a rare genetic disorder affecting elastic tissue, often leading to significant vision impairment due to characteristic retinal changes.
- Retinal manifestations in PXE, including angioid streaks and peau d'orange, can be challenging to fully characterize with conventional imaging techniques.
Purpose of the Study:
- To detail the retinal findings in patients with pseudoxanthoma elasticum (PXE) using a comprehensive multimodal imaging approach.
- To specifically evaluate the utility of flood-illumination adaptive optics ophthalmoscopy (AO) in visualizing fine retinal details in PXE.
Main Methods:
- A retrospective case series involving 18 patients (21 eyes) diagnosed with PXE.
- Multimodal imaging included color, infrared, and autofluorescence fundus photography, optical coherence tomography (OCT), and adaptive optics (AO) ophthalmoscopy.
- Photoreceptor counts and angioid streak morphology were assessed using AO imaging.
Main Results:
- Adaptive optics (AO) imaging allowed for the classification of angioid streaks into three distinct types: "crack," "band," and "hypopigmented."
- Comet lesions were identified as hyper-reflective round structures on AO imaging.
- A significant reduction in cone photoreceptor density was observed within the angioid streaks compared to adjacent retinal areas.
Conclusions:
- Adaptive optics (AO) ophthalmoscopy provides unprecedented visualization of photoreceptors within angioid streaks in PXE.
- AO imaging facilitates differentiation of angioid streak subtypes and identification of subtle crystalline deposits.
- This advanced imaging modality enhances the understanding of PXE-related retinopathy and its impact on photoreceptor integrity.
