Clinical Implications of Alternating Hypointense Bands on OCT-Angiography in Retinal Vascular Occlusive Disease.

PURPOSE: To demonstrate the relationship between alternating hypointense signal bands on optical coherence tomography-angiography (OCTA), real-time fluorescein angiography (FA), and structural OCT findings in patients with retinal vascular occlusive disease (RVOD). DESIGN: Retrospective, consecutive case series. SUBJECTS: Consecutive patients with a clinical diagnosis of acute RVOD and alternating bands of hypointense OCTA flow signal on en face projections. METHODS: Complete ophthalmic examination and multimodal imaging, including color fundus photography (CFP), real-time FA, SD-OCT, and OCTA performed with different instruments having different scan speeds and acquisition protocols. MAIN OUTCOME MEASUREMENTS: The primary outcomes were: Hypointense OCTA band characteristics (number, width, orientation, and location), OCTA acquisition characteristics (speed and scan direction), and FA findings including delayed arteriovenous (AV) transit and pulsatile flow. Secondary outcomes were: Structural OCT changes including retinal fluid, paracentral acute maculopathy lesion (PAMM), and a prominent middle limiting membrane (p-MLM) sign. RESULTS: OCTA-hypointense bands were detected in the superficial and deep vascular plexuses in 9 eyes of 9 patients with either partial CRAO or nonischemic RVO. When obtained on the same device, hypointense bands were thinner and more numerous at lower (100 kHz) scan speeds compared with higher (200 kHz) scan speeds. Band orientation was parallel to the OCTA scan direction, and their extent correlated with the area of delayed AV transit on FA. Structural OCT showed multiple PAMM lesions in 78% of cases and a p-MLM sign centered in the fovea in 44% of cases. CONCLUSIONS: OCTA-hypointense bands are a novel biomarker in RVOD indicating delayed AV transit and pulsatile filling without the need for dye angiography. Structural OCT often shows PAMM in these eyes, and less commonly, a p-MLM sign.

When the second comes first- rhabdomyosarcoma preceding heritable retinoblastoma- a case report.

BACKGROUND: Retinoblastoma (rb) is the most frequent intraocular tumor, accounting for 3% of all childhood cancers. Heritable rb survivors are germline carriers for an RB1 mutation and have a lifelong risk to develop non-ocular second primary tumors (SPTs) involving multiple other organs like the bones, soft tissues, or skin. These SPTs usually become manifest several years succeeding the diagnosis of rb. In our instance, however, a non-ocular SPT presented prior to the diagnosis of heritable rb. CASE PRESENTATION: We report a rare case of a monozygotic twin who presented with primary rhabdomyosarcoma (RMS) preceding the manifestation of heritable rb. The rb was diagnosed when the child developed strabismus while already on therapy for the RMS. The child underwent therapy for both as per defined treatment protocols. The rb regressed well on treatment, but the RMS relapsed and the child developed multiple refractory metastatic foci and succumbed to his disease. CONCLUSIONS: Non-ocular SPTs like sarcomas are usually known to manifest in heritable rb survivors with a lag of two to three decades (earlier if exposure to radiation is present) from the presentation of the rb. However, in our case, this seemed to be reversed with the RMS being manifest at an unusual early age and the rb being diagnosed at a later point in time.

FROM DRUSEN TO TYPE 3 MACULAR NEOVASCULARIZATION.

PURPOSE: To investigate the imaging features preceding the occurrence of type 3 (T3) macular neovascularization (MNV) using tracked spectral-domain optical coherence tomography. METHOD: From a cohort of eyes with T3 MNV and ≥ 12 months of previously tracked spectral-domain optical coherence tomography, T3 lesions that developed above soft drusen were selected for optical coherence tomography analysis. Retinal imaging findings at the location where type T3 MNV occurred were analyzed at each follow-up until the onset of T3 MNV. The following optical coherence tomography parameters were assessed: drusen size (height and width), outer nuclear layer/Henle fiber layer thickness at the drusen apex, and the presence of intraretinal hyperreflective foci, retinal pigment epithelium disruption, incomplete retinal pigment epithelium and outer retina atrophy, and complete retinal pigment epithelium and outer retina atrophy. RESULTS: From a cohort of 31 eyes with T3 MNV, T3 lesions developed above soft drusen in 20 eyes (64.5%). Drusen showed progressive growth ( P < 0.001) associated with outer nuclear layer/Henle fiber ( P < 0.001) thinning before T3 MNV. The following optical coherence tomography features were identified preceding the occurrence of T3 MNV, typically at the apex of the drusenoid lesion: disruption of the external limiting membrane/ellipsoid zone and/or the retinal pigment epithelium, hyperreflective foci, and incomplete retinal pigment epithelium and outer retina atrophy/complete retinal pigment epithelium and outer retina atrophy. CONCLUSION: The results demonstrate specific anatomic alterations preceding the occurrence of T3 MNV that most commonly originates above soft drusen. Drusen growth, reduced outer nuclear layer/Henle fiber thickness, and retinal pigment epithelium atrophy at the drusen apex precede the development of T3 MNV. Identifying these optical coherence tomography features should warrant close monitoring for identification of T3 MNV, which can benefit from prompt intravitreal anti-vascular endothelial growth factor therapy.

Distinctive Optical Coherence Tomography Angiography and Indocyanine Green Angiography Imaging Patterns in Topiramate-Induced Choroidal Effusion.

PURPOSE: To describe novel findings seen on optical coherence tomography angiography (OCTA) and indocyanine green angiography (ICGA) in a young male patient presenting with bilateral topiramate-induced choroidal effusion. METHODS: Retrospective case report. A comprehensive ophthalmic examination was conducted and multimodal imaging techniques, including B-scan ultrasound, OCT, OCTA, and ICGA were analyzed. RESULTS: A male in his 30s presented with a myopic shift due to bilateral choroidal effusion induced by a medication containing topiramate prescribed for weight loss. ICGA showed multiple hypofluorescent spots within the choroid corresponding to areas of reduced OCTA flow signal in both the inner and deeper en face choroidal slabs. Symptoms and abnormal imaging findings resolved within five days of discontinuing the medication. CONCLUSION: Findings observed with OCTA and ICGA together suggest multifocal reversible areas of reduced choroidal vascular flow occurring in a topiramate-induced choroidal effusion. We propose that this transient hypoperfusion is due to compression from deeper choroidal vessels with a congested choroid.

Transdifferentiation and Intrachoroidal Migration of Melanotic Retinal Pigment Epithelium in Punctate Inner Choroiditis.

PURPOSE: To report the multimodal imaging features of hyperpigmented chorioretinal lesions originating from the retinal pigment epithelium (RPE) within punched-out lesions of punctate inner choroidopathy (PIC). METHODS: Retrospective case report. Multimodal imaging findings including fundus photography, optical coherence tomography (OCT), and OCT-angiography (OCTA) were analyzed. RESULTS: A 49-year-old female with myopic degeneration developed progressive lesions of PIC requiring immunosuppressive therapy with adalimumab. Within areas of punched-out chorioretinal atrophic lesions, the occurrence of hyperpigmented lesions were observed which enlarged and extended into the choroid over a multiyear follow-up. CONCLUSION: This case illustrates the development of pigmented choroidal lesions appearing to originate from the RPE through transdifferentiation following previous chorioretinal inflammatory lesions. The introduction of adalimumab treatment may have activated the cellular migration of the RPE. To the best of our knowledge, this is the first report of intrachoroidal RPE migration in PIC.

Retinal Findings and Cardiovascular Risk: Prognostic Conditions, Novel Biomarkers, and Emerging Image Analysis Techniques.

Many retinal diseases and imaging findings have pathophysiologic underpinnings in the function of the cardiovascular system. Myriad retinal conditions, new imaging biomarkers, and novel image analysis techniques have been investigated for their association with future cardiovascular risk or utility in cardiovascular risk prognostication. An intensive literature search was performed to identify relevant articles indexed in PubMed, Scopus, and Google Scholar for a targeted narrative review. This review investigates the literature on specific retinal disease states, such as retinal arterial and venous occlusions and cotton wool spots, that portend significantly increased risk of future cardiovascular events, such as stroke or myocardial infarction, and the implications for personalized patient counseling. Furthermore, conditions diagnosed primarily through retinal bioimaging, such as paracentral acute middle maculopathy and the newly discovered entity known as a retinal ischemic perivascular lesion, may be associated with future incident cardiovascular morbidity and are also discussed. As ever-more-sophisticated imaging biomarkers and analysis techniques are developed, the review concludes with a focused analysis of optical coherence tomography and optical coherence tomography angiography biomarkers under investigation for potential value in prognostication and personalized therapy in cardiovascular disease.

Multiple Small Floaters Associated With Silicone Oil Droplets Following Intravitreal Pegcetacoplan Injection.

This case series analyzes the presence of floaters associated with silicone oil droplets after intravitreal pegcetacoplan injection.

Cavitary Choroidal Nevus Showing Thickness Fluctuations in Response to Anti-VEGF Therapy for Diabetic Macular Edema: A Case Report.

PURPOSE: To report the multimodal imaging features of a cavitary choroidal nevus showing thickness fluctuations that mirrored the response of diabetic macular edema (DME) to intravitreal anti-vascular endothelial growth factor (VEGF) therapy. METHODS: Retrospective case report. Multimodal imaging findings including fundus photography, optical coherence tomography (OCT), fluorescein (FA) and indocyanine green angiography (ICGA), OCT-angiography (OCTA), and B-scan ultrasonography were analyzed. RESULTS: A female in her 80s with a cavitary choroidal nevus and DME was treated with intravitreal anti-VEGF therapy using a pro re nata regimen over 5 years. The choroidal nevus showed thickness fluctuations paralleling the response of DME to anti-VEGF therapy. Worsening of the DME was associated with marked increased choroidal lesion thickness on OCT. Conversely, resolution of DME after intravitreal anti-VEGF injections was followed by choroidal lesion flattening on OCT. Variations of the choroidal lesion thickness were mainly dependent on changes of intralesional hyporeflective caverns on OCT. CONCLUSION: Our report shows thickness variations of a cavitary choroidal nevus that paralleled the clinical course of DME treated with intravitreal anti-VEGF therapy. To the best of our knowledge, this is the first report on volume variations of a cavitary choroidal nevus following anti-VEGF therapy.

Development and validation of a pixel wise deep learning model to detect cataract on swept-source optical coherence tomography images.

PURPOSE: The diagnosis of cataract is mostly clinical and there is a lack of objective and specific tool to detect and grade it automatically. The goal of this study was to develop and validate a deep learning model to detect and localize cataract on Swept Source Optical Coherance Tomography (SS-OCT) images. METHODS: We trained a convolutional network to detect cataract at the pixel level from 504 SS-OCT images of clear lens and cataract patients. The model was then validated on 1326 different images of 114 patients. The output of the model is a map repreenting the probability of cataract for each pixel of the image. We calculated the Cataract Fraction (CF), defined as the number of pixel classified as "cataract" divided by the number of pixel representing the lens for each image. Receiver Operating Characteristic Curves were plotted. Area Under the Curve (ROC AUC) sensitivity and specitivity to detect cataract were calculated. RESULTS: In the validsation set, mean CF was 0.024 ± 0.077 and 0.479 ± 0.230 (p < 0.001). ROC AUC was 0.98 with an optimal CF threshold of 0.14. Using that threshold, sensitivity and specificity to detect cataract were 94.4% and 94.7%, respectively. CONCLUSION: We developed an automatic detection tool for cataract on SS-OCT images. Probability maps of cataract on the images provide an additional tool to help the physician in its diagnosis and surgical planning.

Valsalva-Induced Spontaneous Suprachoroidal Hemorrhage: A Case Report and Review of the Literature.

We report a rare case of spontaneous suprachoroidal hemorrhage and present a systematic review of the literature using PubMed/Medline databases. Patients that developed a spontaneous suprachoroidal hemorrhage with a history of previous intraocular surgery were excluded. An 82-year-old male patient with no known ocular pathologies or surgical history was referred with acute ocular pain and decreased vision in the right eye (BCVA: 0.6 with hyperopic correction) following a Valsalva maneuver. General history included chronic heart failure and coronary artery disease, treated with anticoagulant and antihypertensive drugs. Dilated fundus examination revealed a posterior red-brown choroidal mass, with a thickness of 1.5 mm on B-scan ultrasonography. The lesion was not visible on fluorescein or indocyanine green angiography and was located under the choroid on B-scan optical coherence tomography. The diagnosis of a spontaneous suprachoroidal hemorrhage was evoked, and the patient was observed. Five months later, BCVA was 1.0 uncorrected, with a normal-appearing fundus. In a literature review, eight cases of spontaneous suprachoroidal hemorrhage following an episode of increased intrathoracic pressure were identified, including our patient. M/F ratio was 1 : 1, with a median age of 66.5 years. All cases presented systemic pathologies. All cases presented with a unilateral suprachoroidal hemorrhage. Only 2/8 patients had ocular comorbidities. Complications requiring treatment were noted in 4/8 cases, including 2 cases that resulted in the loss of the vision following an acute angle-closure glaucoma. Spontaneous resolution of the hemorrhage was observed in the other 4 patients. In 6/8 cases, vision recovered over a mean period of 10 weeks. In conclusion, spontaneous suprachoroidal hemorrhage following a Valsalva maneuver in eyes with no history of ocular surgery or trauma is rare, and has been associated with advanced age, cardiovascular disease and asthma. In severe cases (2/8) the eye was lost, while most cases (6/8) recovered, presenting a good visual outcome.

Combined Astigmatic Arcuate Keratotomy with Descemet Automated Endothelial Keratoplasty.

We report the results of simultaneous astigmatic arcuate keratotomy (AK) and Descemet automated endothelial keratoplasty (DSAEK). A 55-year-old patient with a history of high myopia was referred for the management of bullous keratopathy secondary to an anterior chamber phakic intraocular lens (pIOL). IOL explantation through a 5.5-mm corneal incision, cataract extraction, and posterior chamber IOL implantation, combined with DSAEK, were performed. Postoperatively, increased astigmatism up to 2.0 diopters (Dpt) was observed, attributed to the large corneal incision, and remained stable, despite suture removal at 3 months. One year postoperatively, the graft showed signs of progressive endothelial dysfunction. A combined procedure of astigmatic AK and DSAEK was thus performed. After 6 months, topographic astigmatism was significantly reduced to 0.5 Dpt and best-corrected visual acuity increased. In conclusion, simultaneous astigmatic AK and DSAEK could be an effective combination for treating patients with well-documented pre-existing astigmatism and endothelial decompensation.