Dataset Information

Segmented swept source optical coherence tomography angiography assessment of the perifoveal vasculature in patients with X-linked juvenile retinoschisis: a serial case report.

ABSTRACT: PURPOSE:To describe perifoveal microvascular changes occurring in X-linked juvenile retinoschisis (XLRS) using swept source optical coherence tomography angiography (SS OCTA). PATIENTS AND METHODS:This is a serial case report of three patients. Retrospective data of patients affected by XLRS were collected. Structural optical coherence tomography (OCT) and color fundus photography (CFPh) were carried out with Topcon® OCT 2000 3D OCT as part of the standard care. Two patients were imaged on Topcon Atlantis® SS OCTA and one on Topcon Triton® SS OCTA. SS OCTA images were acquired using the 3 × 3 mm fovea-centered cubes scanning protocol. Analysis of both perifoveal superficial vascular plexus (pSVP) and perifoveal deep vascular plexus (pDVP) was performed by two observers after automated segmentation. RESULTS:Four eyes of three males (mean age 14 ± 3.8 years) were analyzed. All eyes showed foveoschisis on CFPh images. OCT B-scans of three eyes showed schistic cysts in the ganglion cell layer, inner nuclear layer (INL) and outer nuclear layer (ONL); in one eye, cysts were depicted in INL and ONL only. In two eyes, SS OCTA showed abnormal foveal avascular zone (FAZ) shape in the pSVP, and in the other two, FAZ shape was abnormal in both plexuses. In all eyes, retinal vascular abnormalities (ie, microvascular protrusions) were present in pDVP. CONCLUSION:SS OCTA can depict perifoveal microvascular changes in young patients affected by XLRS. In this study, the structural and vascular changes seem to be more evident in the pDVP and may represent a useful biomarker of prognosis.

SUBMITTER: Stringa F

PROVIDER: S-EPMC5652910 | biostudies-literature | 2017

REPOSITORIES: biostudies-literature

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Segmented swept source optical coherence tomography angiography assessment of the perifoveal vasculature in patients with X-linked juvenile retinoschisis: a serial case report.

Stringa Francesco F Tsamis Emmanouli E Papayannis Alessandro A Chwiejczak Katarzyna K Jalil Assad A Biswas Susmito S Ahmad Hassan H Stanga Paulo Eduardo PE

International medical case reports journal 20171011

<h4>Purpose</h4>To describe perifoveal microvascular changes occurring in X-linked juvenile retinoschisis (XLRS) using swept source optical coherence tomography angiography (SS OCTA).<h4>Patients and methods</h4>This is a serial case report of three patients. Retrospective data of patients affected by XLRS were collected. Structural optical coherence tomography (OCT) and color fundus photography (CFPh) were carried out with Topcon<sup>®</sup> OCT 2000 3D OCT as part of the standard care. Two pat ...[more]

PMID: 29081674

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Project description:ObjectiveTo report the morphologic characteristics of tumor-related vasculatures and their association with secondary choroidal neovascularization (CNV), subretinal fluid (SRF), choroidal thickness, retinal pigment epithelium (RPE) alterations, subretinal hemorrhage, and tumor decalcification in eyes with choroidal osteoma (CO), using swept-source optical coherence tomographic angiography (SS-OCTA).DesignCross-sectional observational study.ParticipantsWe included 26 patients recruited from Beijing Tongren Hospital with a diagnosis of CO, based on the presence of yellow-orange mass deep to the RPE under indirect ophthalmoscopy and occupying the choroid with well-defined margins and bone density on ultrasonography or computed tomography and focal hyperfluorescent spots with no homogeneous pattern on fluorescein angiography/indocyanine green angiography (FA/ICGA). Data were collected from April 1, 2020, to April 1, 2021, and analyzed from April 30 through May 30, 2021.MethodsApplying SS-OCTA systems operating at 1,050-nm wavelengths, eyes with CO were imaged.Main outcome and measuresTumor-related vasculature in eyes with CO was characterized using multimodal imaging that included fundus photography, FA/ICGA, SS-OCT, and SS-OCTA, and the images were anatomically aligned. CO thickness was manually measured as the distance between the upper boundary of the tumor and the underlying sclerochoroidal interface on the SS-OCT images. Subfoveal choroidal thickness was manually measured as the distance between the Bruch membrane and the sclerochoroidal interface on the SS-OCT images.ResultsOf the 26 Asian patients, 16 (62%) were women and 10 (38%) were men. The mean age was 26.8 years (median, 23; range, 8-45 years), and the mean best corrected visual acuity (BCVA) was 20/40. Thirty-three eyes underwent imaging and were diagnosed with CO. Indocyanine green angiography identified inhomogeneous hyperfluorescence due to tumor-related vasculature, and all corresponded to the structures that appeared as sea-fan vascular networks (SFVNs) combined with clusters of tangled vessels on SS-OCTA images. SFVNs were detected on SS-OCTA imaging in all eyes (100%), terminal tangled vascular structures in 32 of 33 eyes (97%), but not identified on ICGA. Of the 33 tangled vascular structures, 32 (97%) were located at the edge of or inside the tumor, and only 1 (3%) was associated with type 2 neovascularization. In addition, SS-OCT revealed SRF in 33 eyes (100%), 33 (100%) were located at the edge of CO, and only 1 was underlying macular. SRF with retinal edema was seen in 30 of 32 eyes (94%).ConclusionsIn eyes with CO undergoing SS-OCTA imaging, tumor-related vasculature appears as SFVNs combined with tangled vascular structures or few type 2 neovascularization. The identification of actual tumor vasculature in patients with CO as SFVNs with inner or terminal vascular tangles rather than previously described CNV may help facilitate understanding of their pathogenesis, tumor control, and response to treatment.

Dataset Information

Segmented swept source optical coherence tomography angiography assessment of the perifoveal vasculature in patients with X-linked juvenile retinoschisis: a serial case report.

Publications

Segmented swept source optical coherence tomography angiography assessment of the perifoveal vasculature in patients with X-linked juvenile retinoschisis: a serial case report.

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