CHARACTERISTICS OF THE FOVEAL MICROVASCULATURE IN CHILDREN WITH MARFAN SYNDROME: An Optical Coherence Tomography Angiography Study

Hui Chen, Kit Yee Ng, Songshan Li, Guangming Jin, Qianzhong Cao, Zhangkai Lian, Xiaoling Luo, Xiaoyan Ding, Danying Zheng, Hui Chen, Kit Yee Ng, Songshan Li, Guangming Jin, Qianzhong Cao, Zhangkai Lian, Xiaoling Luo, Xiaoyan Ding, Danying Zheng

Abstract

Purpose: To investigate the characteristics of foveal microvasculature in children with Marfan syndrome (MFS).

Methods: Ninety eyes from 45 MFS patients and 76 eyes from 38 healthy individuals of age-matched, sex-matched, and axial length-matched were enrolled. Characteristics of the superficial capillary plexus including the vessel density, perfusion density, and foveal avascular zone were analyzed by optical coherence tomography angiography.

Results: The vessel density and the circularity index of the foveal avascular zone were significantly decreased in the MFS group compared with the controls (P = 0.017 and P = 0.004 respectively). In MFS group, the central vessel density (P = 0.003) and perfusion density (P = 0.001) were negatively correlated with the best-corrected visual acuity. The foveal avascular zone area was correlated with the aortic diameters (P = 0.001) and the paratemporal perfusion density was correlated with the ejection fraction (P = 0.003). Moreover, the paratemporal perfusion density and the circularity index of foveal avascular zone were found to be correlated with the aortic Z-score (P < 0.001 and P = 0.003 respectively).

Conclusion: Retinal microvascular decrease and its correlation with best-corrected visual acuity and cardiac functions were observed in the MFS group. The optical coherence tomography angiography may help to characterize the underlying pathophysiology features of MFS and enable early detection and prevention of vascular changes in MFS.

Conflict of interest statement

None of the authors has any conflicting interests to disclose.

Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the Opthalmic Communications Society, Inc.

Figures

Fig. 1.
Fig. 1.
A representative OCTA image (A–C) of a 5-year-old boy with Marfan syndrome and a 11-year-old healthy boy (D–F). The vessel densities for the total area of the two participants were 22 mm−1 (A) and 23.3 mm−1 (D), and the perfusion densities were 0.338 (B) and 0.406 (E), respectively. The automatically detected foveal avascular zone areas were 0.26 mm2 (C) and 0.11 mm2 (F), respectively.
Fig. 2.
Fig. 2.
Receiver operating characteristic curves of the OCTA-based parameters in the task of distinguishing the control eyes from the eyes with Marfan syndrome. A. Vascular density parameters. B. Perfusion density parameters.

References

    1. Judge DP, Dietz HC. Marfan's syndrome. Lancet 2005;366:1965–1976.
    1. Izquierdo NJ, Traboulsi EI, Enger C, Maumenee IH. Glaucoma in the Marfan syndrome. Trans Am Ophthalmol Soc 1992;90:111–117.
    1. Lee B, Godfrey M, Vitale E, et al. . Linkage of Marfan syndrome and a phenotypically related disorder to two different fibrillin genes. Nature 1991;352:330–334.
    1. Patton N, Aslam T, Macgillivray T, et al. . Retinal vascular image analysis as a potential screening tool for cerebrovascular disease: a rationale based on homology between cerebral and retinal microvasculatures. J Anat 2005;206:319–348.
    1. Wong TY, Klein R, Klein BE, et al. . Retinal microvascular abnormalities and their relationship with hypertension, cardiovascular disease, and mortality. Surv Ophthalmol 2001;46:59–80.
    1. Hoffman RS, Snyder ME, Devgan U, et al. . Management of the subluxated crystalline lens. J Cataract Refract Surg 2013;39:1904–1915.
    1. Al-Sheikh M, Falavarjani KG, Akil H, Sadda SR. Impact of image quality on OCT angiography based quantitative measurements. Int J Retina Vitreous 2017;3:13.
    1. Baumgartner H, Bonhoeffer P, Groot NM, et al. . ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J 2010;31:2915–2957.
    1. Colan SD, McElhinney DB, Crawford EC, et al. . Validation and re-evaluation of a discriminant model predicting anatomic suitability for biventricular repair in neonates with aortic stenosis. J Am Coll Cardiol 2006;47:1858–1865.
    1. van Kimmenade RR, Kempers M, de Boer MJ, et al. . A clinical appraisal of different Z-score equations for aortic root assessment in the diagnostic evaluation of Marfan syndrome. Genet Med 2013;15:528–532.
    1. DeLong ER, DeLong DM, Clarke-Pearson DL. Comparing the areas under two or more correlated receiver operating characteristic curves: a nonparametric approach. Biometrics 1988;44:837–845.
    1. Awais M, Williams DM, Deeb GM, Shea MJ. Aneurysms of medium-sized arteries in Marfan syndrome. Ann Vasc Surg 2013;27:1188.e5–1188.e7.
    1. Eberth JF, Taucer AI, Wilson E, Humphrey JD. Mechanics of carotid arteries in a mouse model of Marfan Syndrome. Ann Biomed Eng 2009;37:1093–1104.
    1. Cao Q, Xiao B, Jin GM, et al. . Expression of transforming growth factor beta and matrix metalloproteinases in the aqueous humor of patients with congenital ectopia lentis. Mol Med Rep 2019;20:559–566.
    1. Takata M, Amiya E, Watanabe M, et al. . Impairment of flow-mediated dilation correlates with aortic dilation in patients with Marfan syndrome. Heart Vessels 2014;29:478–485.
    1. Grillo A, Salvi P, Marelli S, et al. . Impaired central pulsatile hemodynamics in children and adolescents with Marfan syndrome. J Am Heart Assoc 2017;6:e006815.
    1. Sandor GG, Hishitani T, Petty RE, et al. . A novel Doppler echocardiographic method of measuring the biophysical properties of the aorta in pediatric patients. J Am Soc Echocardiogr 2003;16:745–750.
    1. Masugata H, Senda S, Murao K, et al. . Aortic root dilatation as a marker of subclinical left ventricular diastolic dysfunction in patients with cardiovascular risk factors. J Int Med Res 2011;39:64–70.
    1. De Backer JF, Devos D, Segers P, et al. . Primary impairment of left ventricular function in Marfan syndrome. Int J Cardiol 2006;112:353–358.
    1. Morbach C, Gelbrich G, Breunig M, et al. . Impact of acquisition and interpretation on total inter-observer variability in echocardiography: results from the quality assurance program of the STAAB cohort study. Int J Cardiovasc Imaging 2018;34:1057–1065.
    1. Coscas F, Sellam A, Bernard AG, et al. . Normative data for vascular density in superficial and deep capillary plexuses of healthy adults assessed by optical coherence tomography angiography. Invest Ophthalmol Vis Sci 2016;57:OCT211–OCT223.
    1. Li C, Zhong P, Yuan H, et al. . Retinal microvasculature impairment in patients with congenital heart disease investigated by optical coherence tomography angiography. Clin Exp Ophthalmol 2020;48:1219–1228.
    1. Romano PE, Kerr NC, Hope GM. Bilateral ametropic functional amblyopia in genetic ectopia lentis: its relation to the amount of subluxation, an indicator for early surgical management. Binocul Vis Strabismus Q 2002;17:235–241.
    1. Wong ES, Zhang XJ, Yuan N, et al. . Association of optical coherence tomography angiography metrics with detection of impaired macular microvasculature and decreased vision in amblyopic eyes: the Hong Kong children eye study. JAMA Ophthalmol 2020;138:858–865.
    1. Lee MW, Lee WH, Ryu CK, et al. . Effects of prolonged type 2 diabetes on the inner retinal layer and macular microvasculature: an optical coherence tomography angiography study. J Clin Med 2020;9:1849.
    1. Cheung CY, Li J, Yuan N, et al. . Quantitative retinal microvasculature in children using swept-source optical coherence tomography: the Hong Kong Children Eye Study. Br J Ophthalmol 2018. Epub ahead of print.
    1. Trinh M, Kalloniatis M, Nivison-Smith L. Vascular changes in intermediate age-related macular degeneration quantified using optical coherence tomography angiography. Transl Vis Sci Technol 2019;8:20.
    1. Tiryaki Demir S, Kiray Bas E, Karapapak M, et al. . Effect of prematurity on foveal development in early school-age children. Am J Ophthalmol 2020;219:177–185.
    1. You QS, Chan JC, Ng AL, et al. . Macular vessel density measured with optical coherence tomography angiography and its associations in a large population-based study. Invest Ophthalmol Vis Sci 2019;60:4830–4837.
    1. Drobnjak D, Munch IC, Glümer C, et al. . Retinal vessel diameters and their relationship with cardiovascular risk and all-cause mortality in the Inter99 eye study: a 15-year follow-up. J Ophthalmol 2016;2016:6138659.

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