Abstract
Objective
To explore the structural differences between X-linked retinoschisis (XLR) and stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR) using swept-source optical coherence tomography angiography (SS-OCTA).
Methods
A case series of two patients, a 9-year-old male with XLR and a 58-year-old woman with SNIFR were imaged with swept-source optical coherence tomography angiography (SS-OCTA; PLEX Elite 900, Carl Zeiss Meditec, Inc, Dublin, CA). Automated segmentation was manually adjusted to include the areas of retinoschisis within en face flow and structural slabs. The flow data were binarized using ImageJ 1.51s (Wayne Rasband, National Institutes of Health, USA, http://imagej.nih.gov.ij) and superimposed onto the structural slab.
Results
In the eye with XLR, OCTA flow data superimposed on the structural slab demonstrated flow signal within numerous bridging structures connecting the inner and outer plexiform layers containing the intermediate (ICP) and deep (DCP) capillary plexuses. In contrast, the same technique applied to the eye with SNIFR demonstrated an absence of flow signal in the cystic retinal spaces within Henle’s fiber layer.
Conclusions
The vascular pattern of bridging vessels between the ICP and DCP is closely related to the structural “retinoschisis” pattern of XLR and appears to be structurally different from that seen in SNIFR. Moreover, the connecting vessels appear to be highly represented and regularly distributed, thereby supporting a serial arrangement of the retinal capillary plexuses within the perifoveal macula.
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References
Orès R, Mohand-Said S, Dhaenens C-M, Antonio A, Zeitz C, Augstburger E, et al. Phenotypic characteristics of a French cohort of patients with X-linked retinoschisis. Ophthalmology. 2018. https://doi.org/10.1016/j.ophtha.2018.03.057.
Ober MD, Freund KB, Shah M, Ahmed S, Mahmoud TH, Aaberg TM, et al. Stellate nonhereditary idiopathic foveomacular retinoschisis. Ophthalmology. 2014;121:1406–13.
Snodderly DM, Weinhaus RS, Choi JC. Neural−vascular relationships in central retina of macaque monkeys (Macaca fascicularis). J Neurosci. 1992;12:1169-93.
Campbell JP, Zhang M, Hwang TS, Bailey ST, Wilson DJ, Jia Y, et al. Detailed vascular anatomy of the human retina by projection-resolved optical coherence tomography angiography. Sci. Rep. 2017;7:42201.
Lee EJ, Kim T-W, Kim M, Choi YJ. Peripapillary retinoschisis in glaucomatous eyes. PLoS ONE. 2014;9:e90129.
Condon GP, Brownstein S, Wang NS, Kearns JAF, Ewing CC. Congenital hereditary (juvenile X-linked) retinoschisis: histopathologic and ultrastructural findings in three eyes. Arch Ophthalmol. 1986;104:576–83.
Makiyama Y, Oishi A, Otani A, Ogino K, Nakagawa S, Kurimoto M, et al. Prevalence and spatial distribution of cystoid spaces in retinitis pigmentosa. Retina. 2014;34:981–8. http://content.wkhealth.com/linkback/openurl?sid=WKPTLP:landingpage&an=00006982-201405000-00020
Hasegawa T, Akagi T, Yoshikawa M, Suda K, Yamada H, Kimura Y, et al. Microcystic inner nuclear layer changes and retinal nerve fiber layer defects in eyes with glaucoma. PLoS ONE. 2015;10:e0130175.
Fortune B, Ma KN, Gardiner SK, Demirel S, Mansberger SL. Peripapillary retinoschisis in glaucoma: association with progression and OCT signs of Müller cell involvement. Investig Ophthalmol Vis Sci. 2018;59:2818–27.
Gelfand JM, Nolan R, Schwartz DM, Graves J, Green AJ. Microcystic macular oedema in multiple sclerosis is associated with disease severity. Brain. 2012;135:1786-93.
Abegg M, Dysli M, Wolf S, Kowal J, Dufour P, Zinkernagel M. Microcystic macular edema. Ophthalmology. 2014;121:142–9.
Brazerol J, Iliev ME, Höhn R, Fränkl S, Grabe H, Abegg M. Retrograde maculopathy in patients with glaucoma. J Glaucoma. 2017; 26:423–9.
Mrejen S, Gallego-Pinazo R, Freund KB, Paques M. Recognition of henle’s fiber layer on OCT images. Ophthalmology. 2013;120:e32-3.e1.
Freund K, Sarraf D, Leong BCS, Garrity S, Vupparaboina KK, Dansingani KK. Association of optical coherence tomography angiography of collaterals in retinal vein occlusion with major venous outflow through the deep vascular complex. JAMA Ophthalmol. 2018. https://doi.org/10.1001/jamaophthalmol.2018.3586.
Garrity ST, Paques M, Gaudric A, Freund KB, Sarraf D. Considerations in the understanding of venous outflow in the retinal capillary plexus. Retina. 2017;37:1809–12.
Garrity ST, Iafe NA, Phasukkijwatana N, Chen X, Sarraf D. Quantitative analysis of three distinct retinal capillary plexuses in healthy eyes using optical coherence tomography angiography. Invest Ophthalmol Vis Sci. 2017;58:5548–55.
Fouquet S, Vacca O, Sennlaub F, Paques M. The 3D retinal capillary circulation in pigs reveals a predominant serial organization. Investig Ophthalmol Vis Sci. 2017;58:5754–63
Nesper PL, Fawzi AA. Human parafoveal capillary vascular anatomy and connectivity revealed by optical coherence tomography angiography. Invest Ophthalmol Vis Sci. 2018;59:3858–67.
Ghasemi Falavarjani K, Phasukkijwatana N, Freund KB, Cunningham ET, Kalevar A, McDonald HR, et al. En face optical coherence tomography analysis to assess the spectrum of perivenular ischemia and paracentral acute middle maculopathy in retinal vein occlusion. Am J Ophthalmol. 2017;177:131–8.
Garrity ST, Tseng VL, Sarraf D. Paracentral acute middle maculopathy in a perivenular fern-like distribution with en-face optical coherence tomography. Retin Cases Brief Rep. 2017;12 Suppl 1:S25-S28.
Acknowledgements
Funding
This work was supported by The Macula Foundation, Inc., New York, NY. SF was supported by the H2CU—Honors Center of Italian Universities.
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KBF is a consultant to Genentech, Allergan, Optos, Optovue, Zeiss, Heidelberg Engineering, and Novartis. He receives research funding from Genentech/Roche. JS is a consultant for Topcon, Diopsys, and INNOVA. The remaining authors declare that they have no conflict of interest.
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Fragiotta, S., Leong, B.C.S., Kaden, T.R. et al. A proposed mechanism influencing structural patterns in X-linked retinoschisis and stellate nonhereditary idiopathic foveomacular retinoschisis. Eye 33, 724–728 (2019). https://doi.org/10.1038/s41433-018-0296-8
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DOI: https://doi.org/10.1038/s41433-018-0296-8
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