PMC9289179_01
Tractional retinal disease evaluated via OCT. (A) Tractional retinal fold secondary to FEVR in a 3-year-old patient. (B) Tractional fold with peripheral detachment secondary to IP in a 1-year-old patient. For (A,B), top left is the FA, top right is the mean-intensity en face projection taken with the 55  FOV configuration, and bottom is the B-scan corresponding to the dotted line. (C) Stage 4a ROP in a 4-month-old born at 24 weeks gestation. Top left image is a montage of fundus photographs. Top right is a 105  FOV OCT en face with dotted lines indicating the locations of the color-coded B-scans in the bottom row. Vitreoretinal traction is indicated by white arrows, and areas of schisis are indicated by black arrows.
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Figure 2 reveals en face and selected B-scans from several children with tractional retinal detachment (TRD). TRDs are most commonly related to peripheral epiretinal neovascularization with fibrosis, with the resulting vitreoretinal traction leading to macular dragging (as seen in Figure 2A), distortion of the normal retinal architecture, and if there is sufficient anterior-posterior traction, separation of the retina from the retinal pigment epithelium (RPE). OCT is more sensitive for detection of this spectrum of changes, as seen in Figure 2C which reveals an early stage 4a detachment in ROP with the selected B-scan demonstrating tractional schisis but no subretinal fluid.
Rhegmatogenous retinal detachment (RRD) in a 5-year-old girl, evaluated using widefield OCT. (Top left) Image is an ultra-widefield fundus photograph, with white arrows indicating a large temporal break. (Top right) Image is the 105  FOV OCT en face, with dotted lines indicating the locations of the color-coded B-scans shown below.
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FOV, as previously seen with our 55  FOV prototype. Figure 3 demonstrates a rhegmatogenous retinal detachment (RRD) with a large temporal retinal break in a 5-year-old girl.
Tractional and exudative retinal detachments. (A) ERD in a 15-year-old girl in the setting of a vasoproliferative lesion. (B) Chronic exudative retinopathy in a 16-year-old girl. For (A,B), top left image is fluorescein angiography (FA), top right is the mean-intensity en face projection [105  FOV for panel (A) and 40  FOV for panel (B)], with dotted lines indicating the locations of the color-coded B-scans shown below. (C) ERD in a 5-month-old patient with ROP. From left: fundus photograph, 105  FOV OCT en face, and three-dimensional rendering of the same OCT volume.
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Figure 4 provides several examples ERDs and combined tractional and exudative RDs in children.
Macular exudation in a 2-year-old with Coats disease. (Left) Image shows 55  FOV OCT en face projection, with dotted lines indicating the locations of the color-coded B-scans shown on the (right). White arrows point to the area of subretinal exudation.
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Figure 5 demonstrates several examples of the visualization of subretinal exudative in Coats' disease, including the potential benefit of en face visualization with topographic volume rendering.
Retinoblastoma evaluated with OCT in three patients with bilateral disease. (A) 3-year-old with multifocal RB in the left eye who has completed therapy. Left image is the fundus photograph, middle image is the 105  FOV OCT en face projection showing multiple regressed tumors, with dashed purple line corresponding to the location of the B-scan on the bottom right. White arrows point toward a vitreous band, while black arrows point to an area of retinal atrophy. Top right image shows three-dimensional rendering of the same volume shown in the middle panel. (B) Large partially calcified retinoblastoma in a 5-month-old patient undergoing systemic chemotherapy. Fundus photograph is shown on the left, and three-dimensional rendering of 40  FOV, high-resolution OCT volume is shown on the right. (C) 7-month-old undergoing systemic chemotherapy with active RB including diffuse vitreous seeding and multifocal tumors in the left eye. Top left image shows the fundus photograph, top right shows the 105  FOV OCT en face projection, with dashed purple line corresponding to the B-scan below.
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Figure 6 demonstrates several examples of RB documented with our device during routine RB EUAs. WF-OCT system was successful in capturing three dimensional images of elevated tumors, including those in the far periphery, and provided better images than traditional fundus photography in the setting of diffuse vitreous seeding (Figure 6C). WF-OCT was also able to identify very small subclinical tumors, indicating that it may prove useful in surveillance for new tumors in children with known RB or in those being screened due to family history of the disease.
Peripheral retinal and vitreoretinal interface abnormalities. (A) OCT evaluation in an 8-year-old patient with XLRS. Top left shows the 55  FOV en face projection, with corresponding volume rendering shown below. White arrows denote a blood vessel extending into the area of vitreoschisis (vitreous veils) and black arrows denote the area of foveoschisis. Top right images show high-resolution scans of an area of retinoschisis taken with 40  FOV. The inset in the bottom-right corner of the purple B-scan shows the locations of the cross-sections in dotted lines. (B) OCT evaluation of 7-month-old patient with history of non-accidental trauma, displaying disorganization of the retinal architecture, as well as vitreoretinal traction in region of prior breakthrough vitreous hemorrhage. Left image shows a 40  FOV en face projection, with dashed yellow and purple lines denoting the location of the corresponding B-scans on the right. White arrows denote vitreoretinal traction. (C) Vitreoretinal traction in a 2-month-old at site of regressed stage 3 extraretinal neovascularization in ROP. Left image shows 105  FOV en face view with dashed line indicating the location of the cross-sectional B-scan pictured on the right. White arrows denote vitreoretinal traction. (D) 1-month-old with ectopic PFV. Top left image shows the 105  FOV en face projection with complex oval-shaped vitreoschisis. Dotted lines correspond to B-scan locations demonstrating retinal fold through the macula.
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Figure 7A shows an example of X-linked retinoschisis (XLRS), in which retinoschisis may manifest both in the macula and periphery. Figure 7B, and regressed neovascularization in ROP, as in Figure 7C. Finally, in persistent fetal vasculature (PFV), there is a cellular connection through the vitreous cavity that connects the retina to the anterior segment, which can be associated with traction at the nerve or in an extramacular location, as seen in Figure 7D.
Incontinentia pigmenti (IP). (A) OCT volume of a 1-year-old with IP. Top left image is the OCT 55  FOV en face projection. Corresponding color-coded B-scans at top right show irregularities in the nerve fiber layer and retinal surface. Bottom image in teal shows the retina after laser treatment, with en face view on the left, and corresponding B-scan on the right. (B) OCTA evaluation of a 2-year-old patient with IP. Top left image shows the OCTA en face projection with 55  FOV, while top right image shows the same volume with extraretinal neovascularization highlighted in bright yellow. Bottom image is the B-scan corresponding to the dashed yellow line above, showing automated segmentation of capillary plexus layers.
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Nontheless, particularly under anesthesia, it is possible to explore the potential role OCTA may play in the diagnosis of pediatric retinal diseases when used in conjunction with structural OCT (Figure 8A). Figure 8B demonstrates an OCTA taken during an EUA for a child with incontinentia pigmenti (IP), revealing both non-perfusion and neovascularization without the need for fluorescein dye.
Retinal hemangioblastomas in the setting of Von Hippel Lindau (VHL) syndrome. (A) Volume rendering of retinal hemangioblastomas in a 15-year-old. Left image shows an en face of a three-dimensional volume rendering of the tumors taken with a 40  FOV, while image on the right shows an angled view of the same volume. (B) Three-dimensional visualization of vessels generated from OCTA of the same volume as in panel (A). The image on the left shows a three-dimensional rendering of the OCTA volume, showing flow signal within the tumors. Image on the right shows the same volume with higher contrast between vessels and surrounding tissue.
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Figure 9 demonstrates several VHL tumors visualized with en face OCT and OCTA.
Anterior segment (AS)-OCT. (A) AS-OCT in a 6-month-old with PFV. Leftmost en face OCT demonstrates good pupillary dilation and central cataract. Middle image shows en face of AS-OCT, with corresponding color-coded B-scans on the right. The scan outlined in yellow was taken at the limbus, providing visualization of the ciliary body. The scan outlined in purple shows the iris, with a reflection artifact affecting the cornea. (B) Silicone oil in the anterior chamber on AS-OCT in a 14-year-old. Leftmost image shows three-dimensional rendering of the iris and anterior chamber, while rightmost image shows a close up of retained oil above the lens. Both (A,B) were captured with a 105  FOV.
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Figure 10 reveals en face and cross-sectional AS-OCT in several patients with both preoperative and post-operative abnormalities of the anterior segment.