Wide-Field Swept-Source OCT and Angiography in X-Linked Retinoschisis

Ian C Han; S Scott Whitmore; D Brice Critser; Sun Young Lee; Adam P DeLuca; Heather T Daggett; Louisa M Affatigato; Robert F Mullins; Budd A Tucker; Arlene V Drack; Edwin M Stone

doi:10.1016/j.oret.2018.09.006

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Wide-Field Swept-Source OCT and Angiography in X-Linked Retinoschisis

Journal article

Peer reviewed

Wide-Field Swept-Source OCT and Angiography in X-Linked Retinoschisis

Ian C Han, S Scott Whitmore, D Brice Critser, Sun Young Lee, Adam P DeLuca, Heather T Daggett, Louisa M Affatigato, Robert F Mullins, Budd A Tucker, Arlene V Drack, …

Ophthalmology retina, Vol.3(2), pp.178-185

02/2019

DOI: 10.1016/j.oret.2018.09.006

PMID: 31014769

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Abstract

Retinal vascular and structural changes, particularly outside of the central macula, are not well characterized in X-linked retinoschisis (XLRS). We aim to describe wide-field swept-source OCT (SS-OCT) and swept-source OCT angiography (SS-OCTA) findings in XLRS. Retrospective, cross-sectional study at a tertiary referral center. Nine consecutive male patients with molecularly confirmed XLRS. All patients underwent complete ophthalmic examination with multimodal imaging, including SS-OCT with SS-OCTA (PLEX Elite 9000; Carl-Zeiss Meditec Inc., Dublin, CA). Images were then reviewed by 2 retinal specialists as independent graders to determine the frequency and distribution of retinal structural and vascular abnormalities. Structural and vascular abnormalities seen on SS-OCT and SS-OCTA in patients with XLRS, with attention to the retinal layers involved, the regional distribution of schitic spaces in the posterior pole, and vascular abnormalities within the superficial and deep capillary plexuses. Eighteen eyes from 9 male patients (mean age, 20 years; range 9-40) with molecularly confirmed XLRS were included. Median best-corrected visual acuity measured 20/63 (range, 20/25-10/300). A total of 17 of 18 eyes (94.4%) were noted to have schitic spaces on SS-OCT, and these were observed to be predominantly within the inner nuclear layer in all 17 eyes. A regional variation in the distribution of cysts was noted, with schitic spaces within the ganglion cell layer (13/17 eyes; 76.5%) observed to be perifoveal and those within the outer nuclear layer (8/17 eyes, 47.1%) observed to be mostly extramacular. All eyes had vascular abnormalities on SS-OCTA, including an irregular foveal avascular zone and flow loss within the deep capillary plexus corresponding to the distribution of the schisis. Wide-field SS-OCT and SS-OCTA provide detailed visualization of structural and vascular changes in XLRS and may be helpful for monitoring disease progression or treatment response in clinical trials for the disease.

Cross-Sectional Studies

Fluorescein Angiography - methods

Humans

Male

Tomography, Optical Coherence - methods

Visual Acuity

Retinoschisis - diagnosis

Retinal Ganglion Cells - pathology

Retinal Vessels - pathology

Young Adult

Multimodal Imaging

Adolescent

Adult

Female

Retrospective Studies

Child

Diagnostic Techniques, Ophthalmological

Macula Lutea - pathology

Details

Title: Subtitle: Wide-Field Swept-Source OCT and Angiography in X-Linked Retinoschisis
Creators: Ian C Han - University of Iowa Institute for Vision Research, Department of Ophthalmology and Visual Sciences, Carver College of Medicine, Iowa City, Iowa. Electronic address: ian-han@uiowa.edu
S Scott Whitmore - University of Iowa Institute for Vision Research, Department of Ophthalmology and Visual Sciences, Carver College of Medicine, Iowa City, Iowa
D Brice Critser - University of Iowa Institute for Vision Research, Department of Ophthalmology and Visual Sciences, Carver College of Medicine, Iowa City, Iowa
Sun Young Lee - University of Iowa Institute for Vision Research, Department of Ophthalmology and Visual Sciences, Carver College of Medicine, Iowa City, Iowa
Adam P DeLuca - University of Iowa Institute for Vision Research, Department of Ophthalmology and Visual Sciences, Carver College of Medicine, Iowa City, Iowa
Heather T Daggett - University of Iowa Institute for Vision Research, Department of Ophthalmology and Visual Sciences, Carver College of Medicine, Iowa City, Iowa
Louisa M Affatigato - University of Iowa Institute for Vision Research, Department of Ophthalmology and Visual Sciences, Carver College of Medicine, Iowa City, Iowa
Robert F Mullins - University of Iowa Institute for Vision Research, Department of Ophthalmology and Visual Sciences, Carver College of Medicine, Iowa City, Iowa
Budd A Tucker - University of Iowa Institute for Vision Research, Department of Ophthalmology and Visual Sciences, Carver College of Medicine, Iowa City, Iowa
Arlene V Drack - University of Iowa Institute for Vision Research, Department of Ophthalmology and Visual Sciences, Carver College of Medicine, Iowa City, Iowa
Edwin M Stone - University of Iowa Institute for Vision Research, Department of Ophthalmology and Visual Sciences, Carver College of Medicine, Iowa City, Iowa
Resource Type: Journal article
Publication Details: Ophthalmology retina, Vol.3(2), pp.178-185
Publisher: United States
DOI: 10.1016/j.oret.2018.09.006
PMID: 31014769
ISSN: 2468-6530
eISSN: 2468-6530
Grant note: P30 EY025580 / NEI NIH HHS
Language: English
Date published: 02/2019
Academic Unit: Stead Family Department of Pediatrics; The University of Iowa Institute for Vision Research; Iowa Neuroscience Institute; John and Marcia Carver Nonprofit Genetic Testing Laboratory; Center for Bioinformatics and Computational Biology; Ophthalmology and Visual Sciences
Record Identifier: 9984070407102771

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