A Case of Refractory Posterior Scleritis with Marked Retinochoroidal Detachment Associated with Panuveitis.

An 84-year-old man presented with decreased right-eye visual acuity. Upon initial examination, the rightand left-eye visual acuities were 0.03 and 1.2, respectively; moreover, the right- and left-eye intraocular pressure was 12 mmHg and 13 mmHg, respectively. Examination revealed a shallow anterior chamber of the right eye, anterior chamber inflammation, vitreous opacity, and marked retinochoroidal detachment. Optical coherence tomography (OCT) revealed retinal detachment (RD) and choroidal folds; moreover, B-scan ultrasonography (B-scan) showed RD as well as thickened sclera with fluid in Tenon's space. Fluorescent fundus angiography revealed hyperfluorescence in the optic disc and vascular hyperpermeability in the right eye. The left eye lacked extra-ocular symptoms or abnormalities. The right ocular axis measured 23.4 mm with no apparent subretinal fluid migration due to positional changes. Accordingly, the patient was diagnosed with panuveitis associated with posterior scleritis and immediately started on 40 mg prednisolone, which improved his symptoms. However, at 3 post-treatment months, choroidal folds were observed and was restarted on 20 mg prednisolone. The choroidal folds subsequently disappeared, with a current visual acuity of 0.3 in the right eye and no recurrence. Our findings indicated the utility of accurate diagnosis of posterior scleritis by B-scan and prompt systemic steroid administration.

Visual functions and multimodal imaging of patients with idiopathic focal choroidal excavation.

This study aimed to evaluate visual function and perform multimodal imaging on patients with focal choroidal excavation without any chorioretinal disease (idiopathic focal choroidal excavation [iFCE]). Seventeen eyes of 15 patients with iFCE (8 men, 7 women; mean ± standard deviation age, 56.0 ± 10.8 years) were assessed for visual function including visual acuity, metamorphopsia, aniseikonia, and retinal sensitivity. Multimodal imaging included optical coherence tomography (OCT), fundus autofluorescence (FAF), and OCT angiography. This study found that the maximum width and depth of the excavation were 597 ± 330 (238-1809) µm and 123 ± 45 (66-231) µm, respectively, and that FAF showed normal or hypoautofluorescence corresponding to iFCE. The fundus examination findings were stable during the follow-up period (96 ± 48 months). None of the eyes showed any abnormalities in central retinal sensitivity or aniseikonia. Metamorphopsia was detected using Amsler grid testing and M-CHARTS in two eyes. Therefore, this study is the first to quantitatively and qualitatively study metamorphopsia of patients with iFCE. Our results showed that most patients with iFCE did not have visual impairments, despite the presence of morphological changes in the outer retina and choroid.

Choroidal manifestations of non-ocular sarcoidosis: an enhanced depth imaging OCT study.

BACKGROUND: Although choroidal thickening was reported as a sign of active inflammation in ocular sarcoidosis, there has been no research on the choroidal changes in non-ocular sarcoidosis (defined as systemic sarcoidosis without overt clinical signs of ocular involvement). Therefore, this study aimed to investigate choroidal structural changes in patients with non-ocular sarcoidosis. METHODS: This retrospective case-control study was conducted at Asan Medical Center, a tertiary referral center. We evaluated 30 eyes with non-ocular sarcoidosis and their age- and spherical equivalent-matched healthy control eyes. The subfoveal choroidal thickness, area ratio (Sattler layer-choriocapillaris complex [SLCC] area to Haller layer [HL] area), and choroidal vascularity index (CVI, luminal area to choroidal area) were analyzed using enhanced depth imaging in optical coherence tomography. Systemic and ocular factors associated with the choroidal thickness were investigated. RESULTS: Compared with the healthy control group, the non-ocular sarcoidosis group had significantly thicker subfoveal choroid (total and all sublayers [SLCC and HL]) and lower area ratio. There were no significant differences in the CVIs at all sublayers between groups. In the non-ocular sarcoidosis group, eyes under oral steroid treatment had thinner choroid than eyes under observation. In the control group, eyes with older age and more myopic spherical equivalent had thinner choroidal thickness. CONCLUSION: Total and all sublayers of the subfoveal choroid were significantly thicker without significant vascularity changes in non-ocular sarcoidosis eyes than in healthy control eyes. The degree of choroidal thickening was disproportionally greater at HL than at SLCC. These characteristic choroidal changes may be the subclinical manifestations in non-ocular sarcoidosis.

Long-term follow-up demonstrates change in conformation shape of the focal choroidal excavation lesions.

PURPOSE: This study aims to present long-term observation of 5 eyes with focal choroidal excavation (FCE), focusing on morphological changes in conformity of the lesion. METHODS: A retrospective case series was conducted, including 5 eyes of 5 patients with FCE. The study utilized multimodal imaging including color fundus photography, optical coherence tomography (OCT), enhanced depth imaging OCT (EDI-OCT), fundus fluorescein angiography (FFA), fundus autofluorescence (FAF), red free imaging, and OCT angiography. RESULTS: The mean age at diagnosis was 51 ± 10.65 years, with a mean follow-up period 37 ± 13.59 months. All cases were unilateral, with 1 presenting FCE as an isolated lesion, and one patient exhibiting 2 FCEs in one eye. The mean choroidal thickness measured by EDI-OCT was 268.2 ± 63.39 µm in the affected eye. One patient displayed choroidal thickening and pachyvessels. Of the 5 eyes, one had conforming and 4 non-conforming FCE. We observed a conversion in conformity in all patients, with 4 cases transitioning from non-conforming FCE to conforming type (3 spontaneously, 1 treatment-induced). In conforming FCE, a hyporeflective space appeared twice between neuroretina and retinal pigment epithelium with spontaneous regression. CONCLUSION: We observed change in shape from the conforming to non-conforming FCE and vice versa in all patients. We consider this small change in the hyporeflective space as non-pathologic and clinically insignificant.

Multimodal and retro-mode imaging in sclerochoroidal calcification: A case report.

We present a case report on sclerochoroidal calcification (SCC), a rare condition involving calcium pyrophosphate deposits in the posterior pole of the eye in a 70-year-old patient. We provide an account of the clinical presentation and its appearance in multimodal images, using color fundus photography, swept-source optical coherence tomography (SS-OCT), ocular ultrasound, and the novel retro-mode imaging (RMI) technique. Visual acuity was 20/25 in the right eye (OD) and 20/20 in the left eye (OS). Color fundus photography revealed yellowish deposits located in the upper temporal arcade of both eyes. SS-OCT demonstrated masses of scleral origin. Ocular ultrasounds confirmed the calcification of these masses. RMI detected hyper-reflective images with marked superficial elevation. Systemic laboratory results did not detect any abnormalities, leading to the diagnosis of bilateral idiopathic SCC.

Choroid cavitation associated with macular coloboma. Multimodal study. Image en face.

Intrachoroidal cavitation is a finding identified with OCT initially described in myopic patients, it also appears in non-myopic patients. It can occur in both the peripapillary area and the posterior pole. Macular coloboma is a defect of embryonic development of the posterior pole, in structural OCT the absence of the retinal pigment epithelium and choroidal vessels is essential. In this case, intrachoroidal cavitation circumscribes the macular coloboma, in the absence of an intercalary membrane. The en face image allows us to assess the relationship between the two structures as well as their magnitude.

SCLERAL EXCAVATION AT OBLIQUE MUSCLE INSERTIONS: IMAGING CHARACTERISTICS AND CORRELATION WITH SCLERAL PLAQUES AND SCLEROCHOROIDAL CALCIFICATION.

PURPOSE: To compare the imaging features of lesions showing hyporeflective posterior scleral excavation found near the insertions of the oblique extraocular muscles to the features and the natural course of Cogan scleral plaques. METHODS: Multimodal imaging with color fundus photography, spectral-domain optical coherence tomography (OCT), swept-source optical coherence tomography, and B-scan ultrasonography. RESULTS: A 71-year-old man and an 89-year-old man presented with ring-shaped hypopigmented lesions measuring between 200 µm and 300 µm transversally, and located along the superior vascular arcade and temporal to the fovea. All lesions were identified near the insertion of oblique muscles, with one observed in the temporal macula, and two found along the superotemporal arcades. Enhanced depth imaging-optical coherence tomography showed hyporeflective boat-shaped areas of scleral excavation with reduced choroidal thickness along their margins. B-scan ultrasonography showed the lesions to be intensely reflective with varying degrees of posterior shadowing. CONCLUSION: To our knowledge, this is the first report of excavated hyporeflective scleral lesions found near the oblique muscle insertions. Imaging and clinical data support the diagnosis of a posterior form of Cogan scleral plaque and are consistent with the natural course of this entity.

Deep learning based diagnostic quality assessment of choroidal OCT features with expert-evaluated explainability.

Various vision-threatening eye diseases including age-related macular degeneration (AMD) and central serous chorioretinopathy (CSCR) are caused due to the dysfunctions manifested in the highly vascular choroid layer of the posterior segment of the eye. In the current clinical practice, screening choroidal structural changes is widely based on optical coherence tomography (OCT) images. Accordingly, to assist clinicians, several automated choroidal biomarker detection methods using OCT images are developed. However, the performance of these algorithms is largely constrained by the quality of the OCT scan. Consequently, determining the quality of choroidal features in OCT scans is significant in building standardized quantification tools and hence constitutes our main objective. This study includes a dataset of 1593 good and 2581 bad quality Spectralis OCT images graded by an expert. Noting the efficacy of deep-learning (DL) in medical image analysis, we propose to train three state-of-the-art DL models: ResNet18, EfficientNet-B0 and EfficientNet-B3 to detect the quality of OCT images. The choice of these models was inspired by their ability to preserve the salient features across all the layers without information loss. To evaluate the attention of DL models on the choroid, we introduced color transparency maps (CTMs) based on GradCAM explanations. Further, we proposed two subjective grading scores: overall choroid coverage (OCC) and choroid coverage in the visible region(CCVR) based on CTMs to objectively correlate visual explanations vis-à-vis DL model attentions. We observed that the average accuracy and F-scores for the three DL models are greater than 96%. Further, the OCC and CCVR scores achieved for the three DL models under consideration substantiate that they mostly focus on the choroid layer in making the decision. In particular, of the three DL models, EfficientNet-B3 is in close agreement with the clinician's inference. The proposed DL-based framework demonstrated high detection accuracy as well as attention on the choroid layer, where EfficientNet-B3 reported superior performance. Our work assumes significance in bench-marking the automated choroid biomarker detection tools and facilitating high-throughput screening. Further, the methods proposed in this work can be adopted for evaluating the attention of DL-based approaches developed for other region-specific quality assessment tasks.

Vasculopatía coroidea oclusiva tras quimioterapia intraarterial: hallazgos en la RMN / Choroidal occlusive vasculopathy after intraarterial chemotherapy: MRI findings

Objetivo: Evaluar los hallazgos en las imágenes de resonancia magnética nuclear (RMN) en pacientes con vasculopatía coroidea oclusiva (VCO) tras quimioterapia intraarterial (QIA) por retinoblastoma. Métodos: Se realizó un estudio retrospectivo de 37 ojos de 34 pacientes que recibieron QIA entre 2016 y 2021 como tratamiento de primera o segunda línea del retinoblastoma intraocular. De estos pacientes, 22 recibieron quimioterapia sistémica y el resto QIA como primera línea, con melfalán (3-4mg), carboplatino (40mg) y topotecan (20mg). Los pacientes fueron examinados cada mes para observar la regresión tumoral y posibles complicaciones de los tratamientos. A los pacientes que presentaron VCO se les realizaron estudios con RMN para evaluar el grosor coroideo y la longitud del globo ocular. Resultados: Se observó VCO en cinco de los 37 ojos (13,51%), todos ellos con una coroidopatía sectorial completa con afectación foveal (grado 2). En cuatro de los cinco pacientes el grosor coroideo se vio disminuido, mientras que en tres casos el tamaño del globo afectado era claramente inferior. El control tumoral fue posible en todos los casos. Conclusiones:En esta muestra, la VCO se asocia con adelgazamiento coroideo y diminución del tamaño ocular en la RMN. Puede ser necesaria una nueva clasificación para correlacionar mejor la severidad de la coroidopatía que afecta a la fóvea. Los resultados iniciales son favorables respecto al uso de la QIA; aunque es necesaria la realización de estudios a largo plazo y una documentación exhaustiva para valorar tanto el papel de la QIA, como los efectos derivados de ella. (AU)

Purpose: To evaluate magnetic resonance imaging (MRI) findings in patients suffering choroidal occlusive vasculopathy (COV) after intra-arterial chemotherapy (IAC) for retinoblastoma. Methods: A retrospective study of 37 eyes of 34 patients receiving IAQ between 2016 to 2021 as primary or secondary treatment for retinoblastoma was conducted. Twenty-two patients received systemic chemotherapy with carboplatin, vincristine and etoposide. The rest received IAC as primary treatment. The drugs administered were melphalan (3-4mg), carboplatin (40mg) plus topotecan (20mg). The patients were examined under general anaesthesia every month to observe tumor regression and possible complications of the treatment. For the patients with COV an MRI was obtained to analyse the choroidal thickness and axial ocular length. Results: A COV was observed in 5 of the 37 eyes receiving IAC (13,51%), all of them with a complete sectorial choroidopathy not sparing the fovea (grade 2). In 4 of the 5 patients the choroidal thickness was decreased and in three cases the size of the eye which presented COV was clearly smaller than the contralateral eye. Tumor control was archived in all 5 patients. Conclusion: In our cases COV was associated with reduction of thinning of choroid and eye length in the MRI. A new classification maybe needed to correlate better with the severity of the complication affecting the fovea. Although early results generally are favorable to the use of IAC, longer follow up and scrupulous documentation of side effects will be necessary to know the true role of IAC for retinoblastoma. (AU)

Choroidal Caverns in Stargardt Disease.

Purpose: To report choroidal caverns in patients affected by recessive Stargardt disease (STGD1) and to investigate its clinical features. Methods: Retrospective analysis of STGD1 patients recruited at the Regional Reference Center for Hereditary Retinal Degenerations at the Eye Clinic in Florence from 2012 to 2017. Patients included in the study underwent a complete ophthalmic examination including best-corrected visual acuity, color fundus photography, fundus autofluorescence, optical coherence tomography (OCT) and OCT angiography. Results: Eighty-six patients (172 eyes) were included in the study. Twenty-three eyes (13.3%) of 21 patients presented choroidal caverns. The total number of detected choroidal caverns was 63. Choroidal caverns were only present in patients with stage III and IV STGD. Interestingly, patients with choroidal caverns presented larger macular atrophy (20.53 ± 16.9 mm2 vs. 18.11 ± 20.39 mm2), worse visual acuity (1.03 ± 0.29 vs. 0.83 ± 0.26), and a thinner choroidal thickness (245.9 ± 88.7 vs. 266.0 ± 110.5 µm). Conclusions: Choroidal caverns are present only in the advanced stage of STGD1, and a possible degenerative origin of the finding has been hypothesized.

Diagnosis of Choroidal Disease With Deep Learning-Based Image Enhancement and Volumetric Quantification of Optical Coherence Tomography.

Purpose: The purpose of this study was to quantify choroidal vessels (CVs) in pathological eyes in three dimensions (3D) using optical coherence tomography (OCT) and a deep-learning analysis. Methods: A single-center retrospective study including 34 eyes of 34 patients (7 women and 27 men) with treatment-naïve central serous chorioretinopathy (CSC) and 33 eyes of 17 patients (7 women and 10 men) with Vogt-Koyanagi-Harada disease (VKH) or sympathetic ophthalmitis (SO) were imaged consecutively between October 2012 and May 2019 with a swept source OCT. Seventy-seven eyes of 39 age-matched volunteers (26 women and 13 men) with no sign of ocular pathology were imaged for comparison. Deep-learning-based image enhancement pipeline enabled CV segmentation and visualization in 3D, after which quantitative vessel volume maps were acquired to compare normal and diseased eyes and to track the clinical course of eyes in the disease group. Region-based vessel volumes and vessel indices were utilized for disease diagnosis. Results: OCT-based CV volume maps disclose regional CV changes in patients with CSC, VKH, or SO. Three metrics, (i) choroidal volume, (ii) CV volume, and (iii) CV index, exhibit high sensitivity and specificity in discriminating pathological choroids from healthy ones. Conclusions: The deep-learning analysis of OCT images described here provides a 3D visualization of the choroid, and allows quantification of features in the datasets to identify choroidal disease and distinguish between different diseases. Translational Relevance: This novel analysis can be applied retrospectively to existing OCT datasets, and it represents a significant advance toward the automated diagnosis of choroidal pathologies based on observations and quantifications of the vasculature.

LONGITUDINAL ANALYSIS OF DIABETIC CHOROIDOPATHY IN PROLIFERATIVE DIABETIC RETINOPATHY TREATED WITH PANRETINAL PHOTOCOAGULATION USING WIDEFIELD SWEPT-SOURCE OPTICAL COHERENCE TOMOGRAPHY.

PURPOSE: Widefield swept-source optical coherence tomography (OCT) imaging was used to characterize choroidal thickness and vascularity at baseline in proliferative diabetic retinopathy (PDR) and longitudinally after panretinal photocoagulation (PRP). METHODS: Patients with treatment-naive PDR were imaged at baseline and at 1 week, 1 month, and 3 months after PRP. Previously validated algorithms were used to calculate the mean choroidal thickness (MCT) and choroidal vascularity index (CVI) in 5 regions of 12 mm × 12 mm scans. RESULTS: Fourteen PDR eyes were included. Baseline MCT in PDR eyes did not differ significantly from normal eyes, but CVI measurements in PDR eyes were lower in all regions (P < 0.001-0.008). After PRP, MCT measurements in PDR eyes were significantly lower at 1 month and 3 months in all regions (P < 0.001-0.005) except the fovea (P = 0.074). However, CVI measurements did not change over time in any region after PRP. CONCLUSION: The choroid in PDR eyes has a smaller CVI than that in normal eyes. After PRP, the choroidal thickness decreases outside the fovea, but the CVI remains constant, which suggests that a relative decrease in choroidal vascularity persists. These widefield swept-source OCT results are consistent with choroidal alterations found in histopathological reports of diabetic choroidopathy.

CHORIORETINAL FOLDS IN PATIENTS WITH CENTRAL SEROUS CHORIORETINOPATHY.

BACKGROUND AND PURPOSE: To the best of our knowledge, there is no study of patients with central serous chorioretinopathy associated with chorioretinal folds, since a short mention in Gass' stereoscopic atlas. We report here six cases with this association. METHODS: Six patients with both conditions were examined in our institution and underwent fluorescein angiography and optical coherence tomography. RESULTS: Patients were 3 men and 3 women, aged 44 years to 82 years. All patients were hyperopic and two received corticosteroids. Fluorescein angiography showed pigmentary changes, diffuse leakage areas typical of chronic central serous chorioretinopathy, and chorioretinal folds mainly located in the upper temporal part of the fundus. Enhanced depth imaging optical coherence tomography was performed in 5 cases and revealed a thick choroid in all cases (mean subfoveal choroidal thickness: 381 µm, range: 280-510 µm). CONCLUSION: Although possibly coincidental, the presence of chorioretinal folds in hyperopic central serous chorioretinopathy eyes could be due to the excessive thickness of the choroid in eyes with hyperopia related to short axial length.

MULTIMODAL IMAGING IN HELLP-RELATED CHORIORETINOPATHY.

PURPOSE: To illustrate with multimodal imaging a case of HELLP syndrome (Hemolysis, Elevated Liver enzymes, Low Platelets) complicated by bilateral multifocal serous retinal detachments, subretinal exudation, and papilledema. METHODS: Case report. Fundus photography, spectral domain optical coherence tomography (SD-OCT), fluorescein angiography, and indocyanine green angiography were performed at presentation and the day after. We also present the SD-OCT follow-up at 8 days, 1 year, and 4 years. RESULTS: A 25-year-old 5-month-pregnant Guinean woman complained about decreased visual acuity in the right eye. Eye fundus and multimodal imaging were abnormal in both eyes. Spectral domain optical coherence tomography showed the presence of multifocal serous retinal detachments, subretinal deposits, and intraretinal cysts. Indocyanin green angiography revealed an irregular choroidal perfusion and localized choroidal ischemia. Spectral domain optical coherence tomography also provided assessment of retinal changes during the long-term follow-up, showing tissue damage in the outer retina. CONCLUSION: Serous retinal detachments during pregnancy can be the leading sign of HELLP syndrome-a potentially life-threatening condition. Spectral domain optical coherence tomography is a noninvasive and useful tool for its diagnosis and follow-up. ICG is important to confirm the choroidal ischemia and choroidal vascular abnormalities, underlying conditions leading to main sign of HELLP syndrome in the eye.