A case of choroidal melanocytoma treated by transscleral resection: A clinicopathological study.

Purpose: Choroidal melanocytoma is a rare benign melanocytic tumor. We report a case of choroidal melanocytoma that was definitively diagnosed by histopathological findings after local resection. Observation: A 71-year-old female complained of blurred vision in her left eye. Her best-corrected visual acuity (BCVA) was 1.0. A dark-brown elevated lesion, measuring 5 papilla-diameter was found in the periphery of the fundus in her left eye. The mass showed hyperfluorescence on fluorescein angiography, early hypofluorescence and late hyperfluorescence on indocyanine green angiography. B-mode echography indicated the mass was originated from the choroid. Orbital magnetic resonance imaging showed isointense signal intensity on T1-weighted images (WI) and hypointense signal intensity on T2-WI, and poor Gadolinium enhancement on T1WI. The tumor was suspected to be melanocytoma, but it was difficult to differentiate from malignant melanoma. Transscleral tumor resection combined with 25-gauge vitrectomy was performed. Histopathological examinations led to the diagnosis of choroidal melanocytoma. Two years after local resection, her BCVA was 1.0 with no tumor recurrence. Conclusions/importance: Local resection was useful as a diagnostic treatment for choroidal tumors confined to the periphery of the fundus that were difficult to clinically differentiate from malignant melanoma.

Choroidal Circulatory and Vascular Morphological Changes in Acute Macular Neuroretinopathy After Infection With Severe Acute Respiratory Syndrome Coronavirus 2: A Case Report With Literature Review.

BACKGROUND/AIM: The aim of this study was to analyze choroidal circulatory and structural changes using laser speckle flowgraphy (LSFG) and optical coherence tomography (OCT) in acute macular neuroretinopathy (AMN) after infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), respectively. CASE REPORT: A 24-year-old woman complained of distorted vision after SARS-CoV-2 infection oculi uterque (OU) and referred to our hospital because of ellipsoid zones (EZ) disruption on OCT. Her best-corrected visual acuity (BCVA) was 1.2 OU. Color fundus photographs revealed dark red lesions in the macula, and scanning laser ophthalmoscopy infrared images showed hypointensity consistent with dark red lesions OU. We diagnosed the patient with AMN after SARS-CoV-2 infection, and posterior sub-Tenon injections of triamcinolone acetonide were performed OU. Five months after the initial visit, her BCVA was 1.2 OU, and EZ disruption improved. The rate of change in macular blood flow assessed by the mean blur rate on LSFG was 20.4% and 29.6% increase oculus dexter (OD) and oculus sinister (OS) 5 months after the initial visit, respectively. The central choroidal thickness showed 13.5% increase OD and 16.1% increase OS. The binarization technique demonstrated that the ratio of luminal areas in choroidal area increased by 12.6% OD and 14.2% OS, and stromal areas increased by 7.3% OD and 16.9% OS. CONCLUSION: Before and after treatment for AMN, the luminal component may have increased with improvement of acute choroidal circulatory disturbance caused by SARS-CoV-2, and increased stromal components may be due to chronic inflammation and tissue remodeling of the stroma.

Alterations in choroidal circulatory dynamics and choroidal thickness before and after treatment in posterior scleritis.

BACKGROUND: Posterior scleritis is an inflammatory reaction of the sclera that occurs posterior to the ora serrata. The aim of this study was to present a case of posterior scleritis and to analyze choroidal circulatory and structural changes using laser speckle flowgraphy (LSFG) and optical coherence tomography (OCT), respectively. CASE PRESENTATION: A 64-year-old man presented to our department because of hyperemia of the left eye for one week, diplopia, ocular pain, and distorted vision when looking leftward. At an initial examination, his best-corrected visual acuity was 1.0 Oculi uterque (OU), with mild conjunctival hyperemia oculus dexter (OD) and marked ciliary hyperemia oculus sinister (OS). Color fundus photographs revealed a cluster of choroidal folds extending from the macula to the inferior retinal region OS. Swept-Source OCT showed choroidal thickening OD, and bacillary layer detachment and paracentral middle maculopathy on the paracentral side of the optic nerve papilla, suggesting severe inflammation. Fluorescein angiography showed hyperfluorescence in the optic disc and window defects around the macula OU. Indocyanine green angiography showed mottled choroidal vascular hyperpermeability findings in the late stage. B-mode echography displayed thickening of the posterior wall of the left eye. Orbital magnetic resonance imaging showed the thickened posterior eyeball. The patient was diagnosed with posterior scleritis, and 30 mg of oral prednisolone was then given and tapered off over the next 4 months. The hyperemia and intraocular inflammation resolved after the treatment. The rate of change in macular blood flow assessed by the mean blur rate on LSFG was 20.5% and 20.2% decrease OD and OS, respectively, before and after treatment. The central choroidal thickness showed 8.8% and 37.8% decrease OD and OS, respectively. CONCLUSION: Posterior scleritis complicated with choroiditis was suggested to show different choroidal circulatory dynamics from those in other choroidal inflammations.

Alterations of choroidal circulation and choroidal thickness before and after chemoradiotherapy in a case of metastatic choroidal tumor.

BACKGROUND: Metastatic choroidal tumors are hematogenous intraocular metastases of malignant tumors in systemic organs; however, the details of choroidal circulation and morphological changes in the choroid are unknown. The aim of this study is to present a case of metastatic choroidal tumor and examine laser speckle flowgraphy (LSFG)-based choroidal circulation and central choroidal thickness (CCT) before and after chemoradiotherapy. CASE PRESENTATION: A 66-year-old woman with a medical history of breast cancer 16 years ago was referred to our department struggling with blurred vision in her right eye. At the time of initial examination, her best-corrected visual acuity (BCVA) was 0.4 oculus dexter (OD) and 0.9 oculus sinister. Fundus revealed a yellowish-white choroidal elevated lesion measuring 8 papillary diameters with serous retinal detachment (SRD) in the posterior pole. Fluorescein angiography showed diffuse hyperfluorescence and fluorescent leakage due to SRD, and indocyanine green angiography demonstrated no abnormalities in the macula but hypofluorescence in the center of the tumor. Based on these clinical findings, she was diagnosed with metastatic choroidal tumor. After chemoradiotherapy, the metastatic choroidal tumor became scarred, and SRD disappeared. The rate of changes in macular blood flows assessed by mean blur rate on LSFG and CCT of her right eye were 33.8 and 32.8% decrease at 5 months after the initial visit, respectively. BCVA was 0.5 OD 27 months after the initial examination. CONCLUSION: Chemoradiotherapy resulted in regression of the metastatic choroidal tumor and disappearance of SRD, with a decrease in central choroidal blood flow and CCT. The choroidal blood flow on LSFG could reflect an increased oxygen demand by cancer cells invading the choroid and substantial blood supply.

A case of choroidal melanocytosis observed by multimodal imaging with laser speckle flowgraphy.

BACKGROUND: Choroidal melanocytosis is characterized by congenital diffuse melanin pigmentation with extensive parenchymal infiltration of spindle cells in the choroid; however, little is known about the choroidal circulation and morphological changes. We herein report a case of choroidal melanocytosis observed by multimodal imaging with laser speckle flowgraphy (LSFG). CASE PRESENTATION: A 56-year-old woman was referred to our hospital because of serous retinal detachment (SRD) in her left eye. At the initial examination, her best-corrected visual acuity (BCVA) was 1.5 oculus dexter (OD) and 0.8 oculus sinister (OS). An irregular, flat, brownish lesion was noted around the macula OS. Optical coherence tomography showed a choroidal structure with marked hyporeflectivity and SRD where the retinal thickness was preserved. Indocyanine green angiography demonstrated fluorescence blockade throughout. Fundus autofluorescence revealed enlarged macular hypofluorescence, suggesting chronic retinal pigment epithelium damage associated with prolonged SRD. B-mode echography showed no choroidal elevation. Based on the clinical findings, the left eye was diagnosed with choroidal melanocytosis. Four years and 10 months after the initial visit, her BCVA was 0.5 and SRD remained. During the entire period of observation, the mean blur rate (MBR) (mean ± standard deviation) of choroidal blood flow velocity on LSFG was 10.15 ± 0.72 arbitrary units (AU) OD and 1.31 ± 0.06 AU OS. CONCLUSION: Choroidal melanocytosis presented with chronic minor circulatory disturbances due to melanocyte proliferation in the choroid, but the markedly low MBR values by LSFG were dissociated from her retinal thickness and visual function. The proliferation of melanocytes may be a cause of overestimating the cold-color signal of LSFG due to their pigmentation.

Peripapillary circulatory dysfunction precedes structural loss in treatment-naive diabetic retinopathy.

PURPOSE: The aim of this study was to compare the timing of peripapillary vascular damage between functional and structural parameters and examine their involvement with neurovascular coupling at different stages of diabetic retinopathy (DR). METHODS: One hundred ninety eyes of 143 patients with type 2 diabetes mellitus (DM) and 88 healthy control eyes were enrolled. Eyes of DM patients were divided into 3 stages with no diabetic retinopathy (NDR), non-proliferative diabetic retinopathy (NPDR), and proliferative diabetic retinopathy (PDR). NPDR and PDR eyes were treatment-naive. OCT angiography was used to calculate radial peripapillary capillary (RPC)-flux index (FI) and RPC-perfusion density (PD). Spectral domain OCT was used to measure retinal nerve fiber layer (RNFL) thickness within the corresponding RPC areas. RESULTS: RPC-FI significantly decreased in NDR eyes compared to control eyes and thereafter remained unchanged among DM (NDR, NPDR, and PDR) eyes. In contrast, RPC-PD stayed unaltered between control and NDR eyes and significantly decreased in NPDR followed by PDR eyes at similar levels. From control to NPDR eyes, RNFL thickness showed positive correlations with both RPC-FI and RPC-PD, indicative of functional and structural neurovascular coupling. These vascular parameters were also correlated with each other in control and NPDR eyes but not NDR eyes, consistent with the difference in the timing of vascular damage between functional and structural parameters. CONCLUSIONS: Circulatory dysfunction preceded structural loss while maintaining peripapillary neurovascular coupling during progression of DR stages. RPC-FI would likely be more sensitive than RPC-PD in detecting early vascular damage in DR.

RETINAL HYPOPERFUSION DETECTED BY WIDE-FIELD OPTICAL COHERENCE TOMOGRAPHIC ANGIOGRAPHY IN A CASE OF RETINAL RACEMOSE HEMANGIOMA.

PURPOSE: Retinal racemose hemangioma is a rare, unilateral, nonhereditary, arteriovenous malformation characterized by the appearance of dilated and tortuous retinal vessels. Retinal racemose hemangioma can develop complications associated with retinal ischemia, such as vitreous hemorrhage, retinal vein occlusion, and neovascular glaucoma. Here, a case of retinal racemose hemangioma with retinal hypoperfusion detected by wide-field swept-source optical coherence tomographic angiography was reported, which was not unambiguously illustrated by fluorescein angiography. METHODS: Case report. RESULTS: A 57-year-old woman was referred to our hospital for the evaluation of severe retinal vascular tortuosity, dilation, and retinal hemorrhages in the left eye. Fundus examination revealed arteriovenous communications temporal to the fovea and multiple microaneurysms surrounded by retinal hemorrhages at the midperipheral temporal fundus. In fluorescein angiography, multiple hyperfluorescent lesions with leakage corresponding to microaneurysms were observed in the temporal and lower midperipheral areas; however, nonperfused areas were apparently absent. By contrast, wide-field optical coherence tomographic angiography clearly showed low-density retinal capillaries in the superotemporal quadrant in comparison with those in the inferotemporal quadrant. CONCLUSION: Wide-field optical coherence tomographic angiography detected sparse retinal capillaries, which were not well illustrated by fluorescein angiography, in a patient with retinal racemose hemangioma. This indicates the presence of low-grade retinal hypoperfusion caused by altered retinal hemodynamics, potentially leading to ischemia-related retinal disorders during a prolonged course, in patients with clinically quiescent retinal racemose hemangioma.

Diffuse large b-cell lymphoma of the ocular adnexa with flow cytometry analysis and review of the literature.

PURPOSE: The aim of this study was to evaluate the clinicopathological features and flow cytometry (FCM) of tumor tissues in ocular adnexal diffuse large B-cell lymphoma (DLBCL). METHODS: This retrospective, multicenter case study was designed to evaluate the clinical and immunohistochemical features of tumors. DLBCL was diagnosed based on histopathology, immunoglobulin (Ig) heavy chain gene rearrangement, and FCM in all surgically removed periocular tumor tissues. This study involved assessing percentages (%) of B-cell/T-cell markers, a natural killer cell marker, and cell-surface Ig kappa/lambda (κ/λ) expression measured by FCM analysis in tumor tissues. RESULTS: Eleven DLBCL patients (4 men and 7 women) with 11 tumors were enrolled in this study. The median age at the time of initial presentation was 73 years. The tumor cells were immunohistochemically positive for cluster of differentiation (CD) 20, while CD5 was negative in all 8 cases tested. At the time of ophthalmic diagnosis, two cases already showed systemic dissemination of DLBCL throughout the body. FCM of tumor tissues detected a high percentage of B-cell markers including CD19 and CD20 in all 11 tumors. One case with high CD10 levels in FCM was histologic transformation from follicular lymphoma. One case with a relatively low CD20 population involved a history of systemic treatments including intravenous rituximab. CONCLUSION: Although caution should be exercised when interpreting the data, FCM is useful for not only supportive diagnosis complementary to immunohistochemistry, but also facilitates a better understanding of immunopathology including histologic transformation of follicular lymphoma to DLBCL in the ocular adnexa.

Alterations of choroidal circulation and vascular morphology in a patient with chronic myeloid leukemia before and after chemotherapy.

BACKGROUND: Chronic myeloid leukemia (CML) is known to cause leukemic retinopathy due to leukemia cell invasion into the choroid; however, details of the circulatory dynamics and morphological changes in the choroid are unknown. The aim of this study was to present a case of leukemic retinopathy and examine choroidal circulatory and structural analyses using laser speckle flowgraphy (LSFG) and optical coherence tomography with a binarization method, respectively. CASE PRESENTATION: A 15-year-old male diagnosed with CML complained of blurred vision in his right eye. He was ophthalmologically diagnosed with leukemic retinopathy due to retinal hemorrhage in both eyes. Tyrosine kinase inhibitors achieved complete cytogenetic remission and resolution of retinal hemorrhages at 6 months after treatment. After the treatment, the best-corrected visual acuity had recovered from 0.1 to 1.2 oculus dexter (OD) and remained at 1.5 oculus sinister (OS). The rate of change in macular blood flow assessed by the mean blur rate on LSFG was 18.3% increase OD and 25.2% decrease OS 19 months after treatment. The central choroidal thickness showed 0.4 and 3.1% reductions OD and OS, respectively. The binarization technique demonstrated that the rate of luminal areas in choroidal areas exhibited 3.2% increase OD but 4.8% decrease OS. CONCLUSION: Choroidal blood flow improved OD after treatment for CML, while it deteriorated OS, together with choroidal thinning due to reduction of luminal areas. The degrees of leukemia cell invasion into the choroidal tissue and tissue destruction might be different between the eyes in this case.

Follicular lymphoma of the ocular adnexa: Clinicopathological findings with flow cytometry analysis of eight cases.

PURPOSE: Flow cytometry (FCM) is used to evaluate cell surface markers of various leukocyte populations quantitatively. However, little is known about the usefulness of FCM in follicular lymphoma (FL) of the ocular adnexa. The aim of this study was to evaluate the clinicopathological features and FCM results in ocular adnexal FL. MATERIALS: This is a retrospective multicenter case study on clinical and immunohistochemical features. All tumors, surgically excised, were diagnosed based on histopathology, immunoglobulin heavy chain gene rearrangement, and FCM. The percentages (%) of B-cell markers, T-cell markers, a natural killer cell marker, and cell surface kappa/lambda measured by FCM analysis in tumor tissues were searched based on medical records. RESULTS: This study enrolled nine tumors in eight FL patients (three men and five women). The median age at the time of initial presentation was 74 years. All the tumors surgically excised histologically exhibited cluster of differentiation (CD)10, CD20, and BCL2-positive cells. At the time of ophthalmic diagnosis, lymphomas were already disseminated throughout the body in five cases. FCM demonstrated high percentage of B-cell markers including CD10, CD19, CD20, and CD23 in all nine tumors. CD10 population was 73.5% ± 11.9% in seven out of nine tumors, while that in the other two tumors was particularly low being 11.7% ± 1.13%, which showed the relatively high T-cell lineages compared to the other seven tumors. CONCLUSION: For ophthalmologists involving managements of ocular adnexal tumors, FCM can provide useful information for complementing the diagnosis and understanding pathophysiology of FL.

A case of pterygium-like proliferation containing postoperative limbal dermoid remnants: a clinicopathological study.

BACKGROUND: This study reports a case of pterygium-like proliferation containing postoperative limbal dermoid remnants and its clinicopathological features. CASE PRESENTATION: A 79-year-old Japanese woman, with a history of congenital limbal dermoid resection at age 12, presented with a pterygium-like tissue growth in the left eye. Its temporal location and marked thickness with abundant fatty droplets were atypical of primary pterygium. We performed pterygium surgery and ocular surface reconstruction. Pathological findings included squamous metaplasia, neovessels, and elastic degeneration, as well as prominent subepithelial and stromal accumulation of collagen fibers, adipose tissue formation, and presence of a peripheral nerve corresponded with the frequent findings of limbal dermoid. Ki67, a marker for cell proliferation, was immunopositive in pterygial epithelial cells and neovascular endothelial cells, but not in dermoid components. CONCLUSIONS: Although the pathological finding of degenerative elastic fibers indicated the common feature of ultraviolet-induced pterygium, clinical appearances were atypical possibly due to modification with dermoid remnants.

Solitary Fibrous Tumor of the Orbit: A Clinicopathologic Study of Two Cases With Review of the Literature.

BACKGROUND/AIM: Orbital solitary fibrous tumor (SFT) is a rare lesion among orbital tumors, which can be misdiagnosed as another mesenchymal tumor. In this study we report two cases of orbital SFT, focusing on the imaging and pathological findings of the vascular structure inside the tumor. CASE REPORT: A 26-year-old woman and 43-year-old man presented with orbital SFT. The pathological findings revealed a patternless growth pattern of the tumor cells and hemangiopericytoma-like vascularity as well as thickened, dilated blood vessels within the tumor tissue. Tumor cells revealed a diffuse strong positivity for cluster of differentiation 34 (CD34) and signal transducer and activator of transcription 6 (STAT6) in both cases, while B-cell lymphoma 2 (bcl-2) and CD99 were positive in one case. Characteristic findings within the tumor were the arterial components, where a variety of STAT6, CD99 and bcl-2-positive smooth muscle cells were intermingled. CONCLUSION: Histologically, the tumor tissues might be characterized by not only conventional hemangiopericytoma-like vasculature but also dilated arterial vessels, which were shown to be part of the tumor components.

Laser speckle flowgraphy in juxtapapillary retinal capillary hemangioblastoma: a case report on natural course and therapeutic effect.

Juxtapapillary retinal capillary hemangioblastoma (JRCH), a benign intraocular vascular tumor, is usually progressive and may lead to severe vision loss due to various complications. We herein present a case of JRCH observed with laser speckle flowgraphy (LSFG) before and after laser photocoagulation (LPC). A 21-year-old Japanese woman underwent LSFG evaluations. Right eye showed an orange-colored tumor consistent with JRCH on the papillomacular bundle, where LSFG showed a mild warm-color blood flow signal. Eight months after the first examination, JRCH in the right eye increased redness with vasodilatation, and the size enlarged, where LSFG showed a stronger warm-color blood flow signal. She underwent direct yellow laser ablation for the JRCH lesion. One week after LPC, JRCH became paler and LSFG eventually depicted a weakened blood flow signal at the same site. In conclusion, non-invasive and reproducible LSFG is a useful tool for assessing not only JRCH activity but also therapeutic effect.

A Case of Metastatic Iris Tumor Observed With Anterior Segment Optical Coherence Tomography Before and After Radiation Therapy.

BACKGROUND/AIM: Metastatic iris tumors are relatively rare entities that are often treated with radiation therapy; however, it remains difficult to evaluate the effectiveness of treatment. Anterior segment optical coherence tomography (AS-OCT) provides high-resolution cross-sectional images with no dye injection, and may thus be useful for evaluating iris tumors. Herein, we report a case of metastatic iris tumor examined with AS-OCT before and after radiation therapy. CASE REPORT: A 56-year-old Japanese woman complained of ocular hyperemia and pain in her right eye, and was referred to our hospital because of uveitis and an iris mass. One year before the initial visit, the patient was diagnosed with lung adenocarcinoma, and treated with chemotherapy. She also underwent radiation therapy 4 months ago due to multiple bone metastases. Slit-lamp microscopy revealed a 2×2 mm elevated lesion with partial depigmentation on the lower iris. AS-OCT examination revealed that the mass was located in the iris stroma with an irregular anterior surface. Based on fine needle aspiration cytology, she was diagnosed with metastatic iris tumor derived from lung adenocarcinoma, leading to local radiotherapy. Seven months later, AS-OCT showed shrinkage and atrophy of the iris tumor with peripheral anterior synechia formation. CONCLUSION: We report a case of cytology-proven metastatic iris tumor observed with AS-OCT before and after radiation therapy. This study highlights the potential usefulness of AS-OCT for monitoring changes in tumor size and configuration following radiotherapy.

Multimodal imaging in sclerochoroidal calcification: a case report and literature review.

BACKGROUND: Sclerochoroidal calcification (SCC), a rare condition found in elderly people, is idiopathic or occasionally secondary to disorders affecting calcium metabolism. Findings of multimodal imaging including choroidal circulation are, however, largely unknown. We present a patient of SCC with systemic background, who underwent multimodal imaging evaluations. CASE PRESENTATION: A 70-year-old Japanese man was referred to our clinic because of bilateral fundus lesions. He had a history of chronic kidney disease (CKD) and secondary hyperparathyroidism. Fundus photography showed a cluster of choroidal folds in the superotemporal extra-macular region OS. Swept-source optical coherence tomography demonstrated ellipsoid zone disruption OD, retinal pigment epithelium undulation OS, dilated Haller layer veins OU, and central choroidal thickening OU and thinning of the overlying choroid due to scleral elevation OS. Fluorescein angiography detected macular hyperfluorescence OD. Indocyanine green angiography demonstrated choroidal vascular hyperpermeability together with numerous scattered hypofluorescent lesions OU. Fundus autofluorescence showed multiple hypoautofluorescent spots surrounded by hyperautofluorescent areas OD. Laser speckle flowgraphy exhibited choroidal blood flow reduction represented by a cold color pattern OU. B-mode echography displayed hyperechoic solid lesions with acoustic shadowing and orbital computed tomography revealed high density areas in the sclera, both of which were consistent with calcification. The patient was diagnosed with SCC, and these imaging findings remained unchanged 7 months after the diagnosis. CONCLUSIONS: We reported a case of SCC with the background of CKD. Our detailed multimodal observations indicated choroidal hypoperfusion possibly caused by mechanical compression due to calcium deposition in the sclera.