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Published on: August 11, 2023
Prevalence, Features, and Outcomes of Type 1 Neovascularization in Eyes with Angioid Streaks
Maria Vittoria Cicinelli1, Prithvi Ramtohul2, Lorenzo Bianco1
1School of Medicine, Vita-Salute San Raffaele University, Milan, Italy; Department of Ophthalmology, IRCCS San Raffaele Scientific Institute, Milan, Italy.
Purpose:
This study aimed to delineate the characteristics, prevalence, and outcomes of neovascularization (NV), particularly aneurysmal type 1 NV, in patients with angioid streaks (AS) secondary to pseudoxanthoma elasticum (PXE), and to introduce a clinical classification based on multimodal imaging.
Design:
Retrospective longitudinal cohort study.
Participants:
Eighty-five patients (168 eyes) with AS secondary to PXE at 2 tertiary referral centers.
Methods:
Data collection included demographic, medical, and ocular histories. Diagnostic methods comprised fundus photography, autofluorescence, indocyanine green angiography, OCT, and OCT angiography.
Main Outcome Measures:
Prevalence of type 1 NV, visual acuity (VA), risk of exudation.
Results:
Type 1 NV was identified in 127 eyes (76%), with 85 of these (67%) showing exclusively type 1 NV. These lesions often originated around the disc, at sites of Bruch membrane dehiscences, and followed the path of AS, extending to the macula in 101 eyes (80%). Despite 65% of type 1 NV remaining nonexudative, 35% evolved into exudative over 5 years, and 11 eyes experienced midperipheral subretinal hemorrhages. Aneurysmal dilations, observed in 57% of eyes, substantially increased exudation risk (hazard ratio = 3.86, P = 0.02). Despite treatment, VA significantly deteriorated in exudative type 1 NV (P = 0.02). Type 2 NV, detected in 42 eyes (33%), often coexisted with type 1 NV and was associated with poorer visual outcomes and higher rates of macular atrophy. A classification of AS was developed, ranging from empty AS (stage 0, no NV) to advanced NV (stage 3, both type 1 and type 2 NV).
Conclusions:
Type 1 NV predominates in AS. Although predominantly nonexudative, its progression correlates with substantial visual impairment, similar to the deficits observed with type 2 NV. Aneurysmal type 1 NV poses a significant exudation risk, underscoring the need for vigilant monitoring.
Financial Disclosure(S):
Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.
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