Maria Vittoria Cicinelli MD , Prithvi Ramtohul MD , Lorenzo Bianco MD , Ugo Introini MD , Francesco Bandello MD , K. Bailey Freund MD , Maurizio Battaglia Parodi MD
{"title":"Prevalence, Features, and Outcomes of Type 1 Neovascularization in Eyes with Angioid Streaks","authors":"Maria Vittoria Cicinelli MD , Prithvi Ramtohul MD , Lorenzo Bianco MD , Ugo Introini MD , Francesco Bandello MD , K. Bailey Freund MD , Maurizio Battaglia Parodi MD","doi":"10.1016/j.oret.2024.08.002","DOIUrl":null,"url":null,"abstract":"<div><h3>Purpose</h3><div>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.</div></div><div><h3>Design</h3><div>Retrospective longitudinal cohort study.</div></div><div><h3>Participants</h3><div>Eighty-five patients (168 eyes) with AS secondary to PXE at 2 tertiary referral centers.</div></div><div><h3>Methods</h3><div>Data collection included demographic, medical, and ocular histories. Diagnostic methods comprised fundus photography, autofluorescence, indocyanine green angiography, OCT, and OCT angiography.</div></div><div><h3>Main Outcome Measures</h3><div>Prevalence of type 1 NV, visual acuity (VA), risk of exudation.</div></div><div><h3>Results</h3><div>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, <em>P</em> = 0.02). Despite treatment, VA significantly deteriorated in exudative type 1 NV (<em>P</em> = 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).</div></div><div><h3>Conclusions</h3><div>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.</div></div><div><h3>Financial Disclosure(s)</h3><div>Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.</div></div>","PeriodicalId":19501,"journal":{"name":"Ophthalmology. Retina","volume":"9 2","pages":"Pages 166-179"},"PeriodicalIF":4.4000,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Ophthalmology. Retina","FirstCategoryId":"1085","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S2468653024003646","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"OPHTHALMOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
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.