Retinal imaging including optical coherence tomography angiography for detecting active choroidal neovascularization in pseudoxanthoma elasticum

Birtel, Johannes; Lindner, Moritz; Mishra, Divyansh; Müller, Philipp L.; Hendig, Doris; Herrmann, Philipp; Holz, Frank G.; Fleckenstein, Monika; Gliem, Martin; Issa, Peter Charbel

doi:10.1111/ceo.13385

Cited by 11 publications

(5 citation statements)

References 44 publications

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“…The ocular phenotype is characterized by so-called peau d'orange which marks the transition zone between the centrally calcified and peripheral uncalcified Bruch's membrane, peripheral chorioretinal atrophic spots (comet lesions), and angioid streaks which represent breaks within the thickened and brittle Bruch's membrane [5][6][7]. At later stages, macular atrophy may develop, or the disease course may be complicated by choroidal neovascularizations (CNV) that typically originate from angioid streaks [7][8][9].…”

Section: Introductionmentioning

confidence: 99%

Aflibercept for choroidal neovascularizations secondary to pseudoxanthoma elasticum: a prospective study

Gliem

Birtel

Herrmann

et al. 2019

Graefes Arch Clin Exp Ophthalmol

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Purpose To evaluate the use of 2 mg intravitreal aflibercept for treatment of choroidal neovascularization (CNV) secondary to angioid streaks in patients with pseudoxanthoma elasticum (PXE). Methods In this 12-month prospective, open-label, uncontrolled, non-randomized interventional clinical trial, 15 PXE patients with CNV (mean age: 53 years, range 22–65) received one initial intravitreal injection of 2 mg aflibercept. Further injections were based on CNV activity at monthly examinations. The primary endpoint was change of best corrected visual acuity (BCVA) after 12 months. Secondary outcomes were change of central retinal thickness (CRT), leakage from CNV, retinal sensitivity, and vision-related quality of life. Results BCVA improved from 75.0 ± 10.8 (± SD, Snellen equivalent 20/32) to 79.3 ± 7.3 ETDRS letters (20/32) at final visit (p = 0.083). CRT decreased from 317 ± 81 to 279 ± 51 μm (p = 0.004). Retinal sensitivity on microperimetry changed from 17.8 ± 4.5 to 18.5 ± 4.3 dB (p = 0.103) and vision-related quality of life from a VQF-25 score of 80.7 ± 10.4 to 83.5 ± 14.5 (p = 0.554). The mean number of injections was 6.7 ± 2.6, and 5 participants had persistent or reactivated CNV activity at final visit. The observed adverse events were comparable with studies on aflibercept for other indications. Conclusion The results of this study indicate that intravitreal aflibercept is a treatment option for CNV secondary to PXE.

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Section: Introductionmentioning

confidence: 99%

Aflibercept for choroidal neovascularizations secondary to pseudoxanthoma elasticum: a prospective study

Gliem

Birtel

Herrmann

et al. 2019

Graefes Arch Clin Exp Ophthalmol

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“…Birtel et al found that OCT shows a higher diagnostic accuracy of CNV in PXE than OCT angiography and FA, especially with longitudinal follow-up images, and FA and OCT angiography might contribute to the diagnostic accuracy in more complex cases. The systematic use of OCT, OCT angiography, and FA may facilitate the diagnosis and monitoring of AS-related CNV in PXE [31]. Furthermore, in PXE, the transition from classic retinal dystrophies to systemic disease with a retinal phenotype could be indistinct and requires careful observations, as noted previously [1,12].…”

Section: Discussionmentioning

confidence: 95%

Clinical Characterization of Korean Patients with Pseudoxanthoma Elasticum and Angioid Streaks

Jin

Joo

Woo

2021

Genes

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This study aimed to characterize Korean patients with pseudoxanthoma elasticum (PXE) presenting with angioid streaks. Retinal phenotypes were longitudinally evaluated by multimodal ophthalmic imaging, and targeted gene panel sequencing for inherited retinal diseases was conducted. Seven subjects from unrelated families (median age, 51.2 years) were enrolled and followed for a median of 3.2 years. Four asymptomatic patients were significantly younger than three symptomatic patients with decreased visual acuity at presentation (mean age; 38.1 vs. 61.5 years, p = 0.020). The asymptomatic patients maintained good vision (20/32 or better) and had no choroidal neovascularization (CNV) over the observation period. The symptomatic patients showed additional reduction in visual acuity and bilateral CNV occurrence during the longitudinal follow-up. Pathogenic ABCC6 variants were identified in all patients, leading to a diagnosis of PXE. Heterozygous monoallelic variants were identified in four patients and compound heterozygous variants were detected in three patients. Nine ABCC6 variants were identified, including one novel variant, c.2035G>T [p.Glu679Ter]. This is the first genetic study of Korean patients with PXE.

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“…Unfortunately, fluorescence angiography was not available to detect leakage of CNV. We believe that this should not be a problem, because angiography does not add information regarding CNV activity in PXE patients in most cases [15]. CNV activity was diagnosed if at least one of the following criteria was present: 1) hemorrhage in posterior pole on color fundus photography and/or 2) one or more intraretinal cysts of >50 μm diameter and/or subretinal fluid of >200 μm width, with signs of a CNV nearby, and/or 3) obvious growth of a subretinal neovascular complex.…”

Section: Assessment Of Outcomementioning

confidence: 99%

The effect of etidronate on choroidal neovascular activity in patients with pseudoxanthoma elasticum

et al. 2020

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To assess the effect of the bisphosphonate etidronate on choroidal neovascular (CNV) activity in patients with pseudoxanthoma elasticum (PXE). Methods This is an ancillary study in a single center, randomized, double-blind placebo-controlled trial (RCT) in which 74 patients with PXE were assigned to either one-year etidronate or placebo treatment. Spectral domain optical coherence tomography (SD-OCT) imaging and color fundus photography were performed every three months for one year and were systematically assessed on signs of CNV activity. Results In the etidronate group, 11 (30%) of the patients had CNV activity at baseline, compared to 25 (67%) of the patients in the placebo group (P = 0.005). The proportion of eyes with CNV activity during the study ranged from 18-33% in the etidronate group and 42-56% in the placebo group and no significant difference in improvement or worsening of CNV activity was found (P = 0.168). Using a generalized mixed model for repeated measures, there was a protective effect of etidronate in crude analysis (RR 0.86, 95% CI 0.75-0.98) that disappeared when adjusting for baseline CNV activity (RR 0.97, 95% CI 0.84-1.13). Conclusion In this post-hoc RCT analysis we did not observe a protecting or deteriorating effect of etidronate on CNV activity in patients with PXE after adjustment for baseline CNV.

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Retinal imaging including optical coherence tomography angiography for detecting active choroidal neovascularization in pseudoxanthoma elasticum

Cited by 11 publications

References 44 publications

Aflibercept for choroidal neovascularizations secondary to pseudoxanthoma elasticum: a prospective study

Aflibercept for choroidal neovascularizations secondary to pseudoxanthoma elasticum: a prospective study

Clinical Characterization of Korean Patients with Pseudoxanthoma Elasticum and Angioid Streaks

The effect of etidronate on choroidal neovascular activity in patients with pseudoxanthoma elasticum

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