Abstract
Angioid streaks (AS) are the most common ocular manifestation in patients with pseudoxanthoma elasticum (PXE). The major cause of severe visual loss in patients with AS is choroidal neovascularization (CNV). We report the optical coherence tomography angiography (OCTA) findings of CNV in a patient with PXE and angioid streaks. A 51-year-old man with PXE presented with visual disturbance in his right eye. Best corrected visual acuity was 20/30 OD and 20/30 OS. Funduscopic examination revealed angioid streaks and type 1 NV in his right eye. Multimodal imaging including OCTA demonstrated CNV nasal to the fovea. The morphology and configuration of CNV followed the path of the AS. OCTA combined with other multimodal imaging modalities may be a useful tool for diagnosing CNV secondary to angioid streaks in patients with pseudoxanthoma elasticum. The configuration of CNV in these may follow the path of angioid streaks implicating Bruch’s membrane disruption as an important anatomical change in the pathogenesis of CNV.
Highlights
Pseudoxanthoma elasticum (PXE) is a rare inherited multisystem disorder
There are many ocular manifestations associated with PXE of which angioid streaks (AS) are the most common
AS represent breaks within calcified and thickened elastic fibers of Bruch’s membrane. They often do not affect visual function, but they may be complicated by the development of choroidal neovascularization (CNV), which is a major cause of visual loss in these patients
Summary
Pseudoxanthoma elasticum (PXE) is a rare inherited multisystem disorder. There are many ocular manifestations associated with PXE of which angioid streaks (AS) are the most common. They often do not affect visual function, but they may be complicated by the development of choroidal neovascularization (CNV), which is a major cause of visual loss in these patients. Optical coherence tomography angiography (OCTA) is relatively new non-invasive imaging modality that demonstrates flow-characteristics of the vascular network. *Correspondence: kbfnyf@aol.com 2 Vitreous-Retina-Macula Consultants of New York, 460 Park Avenue, 5th Floor, New York, NY 10022, USA Full list of author information is available at the end of the article and spatial relationships of CNV and angioid streaks using OCTA.
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