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Case Reports
. 2019 May;33(5):724-728.
doi: 10.1038/s41433-018-0296-8. Epub 2018 Dec 5.

A proposed mechanism influencing structural patterns in X-linked retinoschisis and stellate nonhereditary idiopathic foveomacular retinoschisis

Serena Fragiotta  1   2   3   4 , Belinda C S Leong  1   2   3 , Talia R Kaden  1   2   3   5 , Sherry J Bass  6 , Jerome Sherman  6 , Lawrence A Yannuzzi  1   2   3   5 , K Bailey Freund  7   8   9   10
Affiliations
Case Reports

A proposed mechanism influencing structural patterns in X-linked retinoschisis and stellate nonhereditary idiopathic foveomacular retinoschisis

Serena Fragiotta et al. Eye (Lond). 2019 May.

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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Conflict of interest statement

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.

Figures

Fig. 1
Fig. 1
Optical coherence tomography angiography (OCTA) in X-linked retinoschisis. a Binarized en face OCTA with segmentation of the schitic area and projection removal. b En face structural slab. c OCTA flow signal (red) superimposed on the structural slab shows that flow signal representing bridging vessels between the intermediate and deep capillary plexuses is present in the large majority of connecting tissue within the inner nuclear layer. Black spaces correspond to intraretinal cysts. d OCT B-scan with flow overlay shows segmentation lines (dashed magenta lines) used for the en face projections (a and b) and confirms correspondence of flow signal with the tissue bridging the schisis
Fig. 2
Fig. 2
Optical coherence tomography angiography (OCTA) in stellate nonhereditary idiopathic foveomacular retinoschisis. a Binarized en face OCTA of the schitic area with projection removal. b En face structural slab. c OCTA flow signal (red) superimposed on the structural slab demonstrates the absence of flow signal within retinal tissue separating cystic spaces within Henle’s fiber layer. d OCT B-scan with flow overlay shows segmentation lines (dashed magenta lines) used for the en face projections (a and b) and confirms an absence of flow signal within the tissue bridging the schisis
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