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.

Effects of age and axial length on choroidal stratified structure in normal eyes.

To quantify the choroidal structures of normal eyes by optical coherence tomography (OCT)-based binarization and evaluate the relationships among age, refractive power, and ocular axial length. This was a retrospective observational study. One hundred and eighty nine eyes of 189 subjects without ocular diseases were examined by enhanced depth imaging (EDI)-OCT. A choroidal OCT horizontal image with a width of 1500 µm centered on the fovea was binarized. The lumen, stroma, and total choroidal area in the choriocapillaris (CC), Sattler's layer (SL), and Haller's layer (HL) were measured, and the ratio of the luminal area to total choroidal area (L/C ratio) was calculated. Multiple regression analysis was performed for choroidal parameters in each choroidal layer and for age, refractive power, and ocular axial length. Multiple regression analysis showed that an older age was significantly correlated with a lower choroidal area and the L/C ratio in all choroidal layers (each P < 0.05). A Long axial length was significantly associated with lower SL and HL (P < 0.05), but not with refractive power. In the choroid of normal eyes, age-related decreases in the choroidal area and L/C ratio were associated with all choroidal layers, and elongation of the axial length was associated with thinning of SL and HL.

Clinicopathological findings in refractory diabetic macular edema: A case report.

The present study describes the case of a patient with refractory diabetic cystoid macular edema who underwent vitrectomy with en bloc removal of the cystoid lesion component. The current study also performed histopathological and immunohistochemical analyses of the cystoid lesion component to assess fibrin/fibrinogen and advanced glycation end-products (AGEs) immunoreactivity. A 69-year-old Japanese man presented with visual loss in the left eye due to residual cystoid macular edema (CME) refractory to anti-vascular endothelial growth factor therapy. Best-corrected visual acuity was 1.2 in the right eye (OD) and 0.5 in the left eye (OS). Fundus examination showed dot hemorrhages and hard exudates in the peri-macular region with pan-retinal photocoagulation scars in both eye. Swept-source optical coherence tomography revealed CME with slight hyperreflectivity in the cyst OS. A total of 3 months after the initial visit, pars plana vitrectomy was performed, and the translucent solidified component within the cystoid lesion was isolated. Histopathologically, the excised component was elliptical in shape, measuring 0.7x0.4 mm and exhibited homogeneous eosinophilic material without cellular components. No membranous structure was observed surrounding the component. Immunohistochemistry demonstrated that the tissue was positive for fibrin/fibrinogen and weakly positive for AGEs, but was negative for glial fibrillary acidic protein, type 1 collagen and receptor for AGEs. To the best of our knowledge, the present case report is the first to histopathologically examine the contents of refractory CME, and to immunohistochemically demonstrate that fibrin in diabetic CME may be post-translationally modified by AGEs. These results suggested that fibrin in CME may escape degradation by plasmin due to post-translational modifications.

CLINICOPATHOLOGICAL ANALYSIS OF SECONDARY RETINAL VASOPROLIFERATIVE TUMOR/REACTIVE RETINAL ASTROCYTIC TUMOR SUCCESSFULLY TREATED BY ENDORESECTION.

PURPOSE: To report the clinicopathological findings of retinal vasoproliferative tumor/reactive retinal astrocytic tumor (VPT/RRAT) with retinal vasculitis, treated by tumor resection. METHODS: A retrospective single case report. PATIENT: A 29-year-old Japanese woman was referred with cystoid macular edema and retinal vasculitis in the right eye. Best-corrected visual acuity was 0.9. Results of fundus examination, optical coherence tomography, and fluorescein angiography demonstrated VPT/RRATs in the temporal retina surrounded by a subretinal exudate, serous retinal detachment and macular edema, and retinal vasculitis. Despite 3 months of oral prednisolone treatment, a full-thickness macular hole developed. Pars plana vitrectomy and endoresection of the VPT/RRATs were performed. Pathologic and immunohistochemical analyses with anti-CD34 antibody, antiglial fibrillary acidic protein antibody, anti-Ki67 antibody, and anti-vascular endothelial growth factor antibody were performed on the excised tissue. Inflammation was evaluated by immunohistological staining with leukocyte common antigen (LCA), anti-CD3 antibody, and anti-CD20 antibody. RESULTS: After surgery, the macular hole closed, best-corrected visual acuity improved to 1.2, retinal vasculitis was ameliorated, and retinal exudate disappeared. There was no recurrence of VPT/RRAT or retinal vasculitis. Pathologic examination showed that antiglial fibrillary acidic protein and anti-vascular endothelial growth factor were widely expressed, irrespective of the distribution of blood vessels. Ki67-positive proliferating cells were detected in the perivascular area. Leukocyte common antigen-positive leukocytes and CD3-positive T cells were detected throughout the samples, whereas CD20-positive B cells were rarely detected. CONCLUSION: Endoresection of VPT/RRAT could be a good treatment option for secondary VPT/RRAT accompanied by retinal vasculitis. Pathologic findings revealed for the first time that inflammatory cells infiltrate the tissue in secondary VPT/RRAT.

Clinicopathologic features of conjunctival MALT lymphomas refractory to radiation therapy.

OBJECTIVE: Clinicopathologic features of patients with limited-stage mucosa-associated lymphoid tissue (MALT) lymphoma refractory to radiotherapy have not been fully elucidated. This study aimed to elucidate clinicopathologic features of localized conjunctival MALT lymphoma concerning radiosensitivity by analyzing cell proliferation and expression of mismatch repair proteins. METHODS: We enrolled 26 patients with localized conjunctival MALT lymphoma treated with radiotherapy from November 2007 to March 2020. Monoclonal immunoglobulin H gene rearrangement was tested in addition to histopathologic evaluation. Thirty-six specimens were immunostained with antibodies to Ki-67 and MutL protein homologue 1 (MLH1), MutS protein homologue 2 (MSH2), and MutS protein homologue 6 (MSH6). Positive rates under a high-power field at a hot spot were counted manually. RESULTS: After radiotherapy, 21 patients showed clinical disappearance of the tumour without recurrence (effective group). Three patients showed temporary disappearance of the tumour, which later recurred (relapse group). Two patients did not show disappearance of the tumour (ineffective group). The 2 ineffective patients were young, had bilateral lesions, and received x-ray beam therapy. The mean positive rates of Ki-67, MLH1, MSH2, and MSH6 were higher in tumours with complete remission (CR) than in those without CR (23.4% ± 4.0% and 18.7& ± 4.7%, 14.7% ± 2.3% and 7.1% ± 3.7%, 23.9% ± 4.7% and 14.4% ± 5.2%, and 11.5% ± 3.2% and 5.4% ± 2.2%; p > 0.05 for each, respectively). CONCLUSIONS: A few patients could not achieve CR following radiotherapy, whereas there were no significant differences in proliferation activity and mismatch repair proteins between tumours with and without CR.

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.

Phosphorylation of αB-Crystallin Involves Interleukin-1ß-Mediated Intracellular Retention in Retinal Müller Cells: A New Mechanism Underlying Fibrovascular Membrane Formation.

Purpose: Chronic inflammation plays a pivotal role in the pathology of proliferative diabetic retinopathy (PDR), in which biological alterations of retinal glial cells are one of the key elements. The phosphorylation of αB-crystallin/CRYAB modulates its molecular dynamics and chaperone activity, and attenuates αB-crystallin secretion via exosomes. In this study, we investigated the effect of phosphorylated αB-crystallin in retinal Müller cells on diabetic mimicking conditions, including interleukin (IL)-1ß stimuli. Methods: Human retinal Müller cells (MIO-M1) were used to examine gene and protein expressions with real-time quantitative PCR, enzyme linked immunosorbent assay (ELISA), and immunoblot analyses. Cell apoptosis was assessed by Caspase-3/7 assay and TdT-mediated dUTP nick-end labeling staining. Retinal tissues isolated from the Spontaneously Diabetic Torii (SDT) fatty rat, a type 2 diabetic animal model with obesity, and fibrovascular membranes from patients with PDR were examined by double-staining immunofluorescence. Results: CRYAB mRNA was downregulated in MIO-M1 cells with the addition of 10 ng/mL IL-1ß; however, intracellular αB-crystallin protein levels were maintained. The αB-crystallin serine 59 (Ser59) residue was phosphorylated with IL-1ß application in MIO-M1 cells. Cell apoptosis in MIO-M1 cells was induced by CRYAB knockdown. Immunoreactivity for Ser59-phosphorylated αB-crystallin and glial fibrillary acidic protein was colocalized in glial cells of SDT fatty rats and fibrovascular membranes. Conclusions: The Ser59 phosphorylation of αB-crystallin was modulated by IL-1ß in Müller cells under diabetic mimicking inflammatory conditions, suggesting that αB-crystallin contributes to the pathogenesis of PDR through an anti-apoptotic effect.

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.

Changes in choriocapillaris structure occurring in idiopathic macular hole before and after vitrectomy.

PURPOSE: The aim of this study was to analyze the anatomical choroidal vascular layers and the changes in idiopathic macular hole (IMH) eyes over time after vitrectomy. METHODS: This is a retrospective observational case-control study. Fifteen eyes from 15 patients who received vitrectomy for IMH and age-matched 15 eyes from 15 healthy controls were enrolled in this study. Retinal and choroidal structures were quantitatively analyzed before vitrectomy and 1 and 2 months after surgery using spectral domain-optical coherence tomography. Each choroidal vascular layer was divided into the choriocapillaris, Sattler's layer, and Haller's layer, and then, the choroidal area (CA), luminal area (LA), stromal area (SA), and central choroidal thickness (CCT) were calculated using binarization techniques. The ratio of LA to CA was defined as the L/C ratio. RESULTS: The CA, LA, and L/C ratios were 36.9 ± 6.2, 23.4 ± 5.0, and 63.1 ± 7.2 in the choriocapillaris of IMH and were 47.3 ± 6.6, 38.3 ± 5.6, and 80.9 ± 4.1 in that of control eyes, respectively. Those values were significantly lower in IMH eyes than in control eyes (each P < 0.01), whereas there was no significant difference in total choroid, Sattler's layer, and Haller's layer or CCT. The ellipsoid zone defect length showed a significant negative correlation with the L/C ratio in total choroid and with CA and LA in the choriocapillaris of IMH (R = - 0.61, P < 0.05, R = - 0.77, P < 0.01, and R = - 0.71, P < 0.01, respectively). In the choriocapillaris, the LA were 23.4 ± 5.0, 27.7 ± 3.8, and 30.9 ± 4.4, and the L/C ratios were 63.1 ± 7.2, 74.3 ± 6.4, and 76.6 ± 5.4 at baseline, 1 month, and 2 months after vitrectomy, respectively. Those values showed a significant increase over time after surgery (each P < 0.05), whereas the other choroidal layers did not alter consistently with respect to changes in choroidal structure. CONCLUSIONS: The current OCT-based study demonstrated that the choriocapillaris was exclusively disrupted between choroidal vascular structures in IMH, which may correlate with the ellipsoid zone defect. Furthermore, the L/C ratio of choriocapillaris recovered after IMH repair, suggesting an improved balance between supply and demand of oxygen that has collapsed due to temporary loss of central retinal function by IMH.

A Case of Intraocular Metastasis of Lung Cancer Diagnosed Using Cell Block Preparation of the Vitreous Humor.

BACKGROUND/AIM: Masquerade syndrome is characterized by uveitis-like manifestations caused by tumor cell infiltration into the ocular tissues. The aim of the study was to report a lung cancer patient with persistent unilateral vitreous opacity, who was eventually diagnosed with masquerade syndrome using cell block preparation. CASE REPORT: An 82-year-old female complained of blurred vision in her left eye (OS). Because of pulmonary adenocarcinoma, she had previously received anticancer drug treatment at another Hospital and achieved partial remission. Ophthalmic examinations revealed anterior chamber inflammation and vitreous opacity OS. Corticosteroid eye drops were administered, but the inflammation did not improve, and was referred to the Hokkaido University Hospital. The left best-corrected visual acuity was 0.1 with normal intraocular pressure. Anterior chamber inflammation was 2+ cells, and vitreous haze was 4+ OS. B-mode ultrasonography showed diffuse heterogeneous high echoic changes in the vitreous cavity. She underwent vitrectomy, and cell block preparation of the vitreous infusion fluids was performed. Cytopathology revealed adenocarcinoma cells with a high nuclear/cytoplasmic ratio and glandular formation. The immunocytochemical study showed that tumor cells were positive for thyroid transcription factor-1 (TTF-1), napsin A, and CK7, therefore diagnosis of masquerade syndrome due to intraocular metastasis of pulmonary adenocarcinoma was reached. Chemoradiotherapy was administered, and the eye got phthisis bulbi after irradiation 2 years after diagnosis. CONCLUSION: Cell block preparation using vitreous humor may be useful in the diagnosis and management of intraocular metastasis of pulmonary adenocarcinoma in patients with prolonged vitreous opacity.

Two-year clinical outcomes of triple therapy with photodynamic therapy, anti-vascular endothelial growth factor agent, and triamcinolone acetonide for neovascular age-related macular degeneration.

PURPOSE: To analyze the 2-year treatment outcomes of triple therapy with standard-fluence photodynamic therapy (PDT), intravitreal injection of ranibizumab (IVR)/aflibercept (IVA), and sub-tenon injection of triamcinolone acetonide (STTA) for neovascular age-related macular degeneration (nAMD) in Japanese patients. STUDY DESIGN: A retrospective, clinical case-series study. METHODS: Forty-four eyes of 44 patients with treatment-naïve nAMD followed for more than 24 months were evaluated. Initial treatment was given with triple therapy and retreatment with IVR/IVA as a pro re nata regimen. Best-corrected visual acuity (BCVA), central retinal thickness (CRT), the number of treatments, and intraocular pressure elevation were analyzed. RESULTS: The mean age was 73.3 ± 10.0 years. The mean BCVA significantly improved from 0.61 ± 0.30 at baseline to 0.50 ± 0.46 at 24 months (p = 0.034). CRT significantly improved from 373 ± 162 µm at baseline to 200 ± 107 µm at 24 months (p < 0.001). The number of treatments given during the 2-year treatment period was 2.7 ± 1.8. No retreatments were necessary in 18 of 44 eyes (40.9%), with no significant difference between IVR (46.4%) or IVA (31.3%) used during the 2-year follow-up of triple therapy (p = 0.51). Four eyes (9.1%) temporarily required glaucoma eye drop treatments. CONCLUSION: In nAMD patients, induction treatment with triple therapy resulted in approximately 40% of the patients requiring no retreatment for 2 years. The type of anti-VEGF agents used made no difference in the results.

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.

Clinical significance of serum and vitreous soluble interleukin-2 receptor in patients with intraocular lymphoma.

BACKGROUND: Intraocular lymphoma (IOL) is a masquerade syndrome that mimics uveitis, making diagnosis difficult. The serum soluble interleukin-2 receptor (sIL-2R), which is cleaved by matrix metalloproteinase (MMP) -2 and MMP-9, has been recognized as a tumor-related biomarker of malignant lymphomas. The aim of this study was to review the reliability of serum and vitreous sIL-2R for distinguishing IOL from uveitis. METHODS: Patients who underwent diagnostic vitrectomy for marked vitreous haze at Hokkaido University Hospital between April 2014 and June 2019 were enrolled. The patients were divided into an IOL group and a uveitis group, according to the pathology of their vitreous samples. The IOL group was further divided at the time of vitrectomy into patients who already had extraocular involvement (IOL with extraocular involvement group) and patients with no evidence of having extraocular involvement (IOL without extraocular involvement group). Serum sIL-2R, and intravitreal sIL-2R, MMP-2, and MMP-9 levels were assessed. RESULTS: Twenty-five eyes of 25 patients, and 15 eyes of 15 patients were included in the IOL group and uveitis group, respectively. The serum sIL-2R levels were significantly lower in the IOL group than in the uveitis group (P < 0.05), and 20.0% and 66.7% in the IOL and the uveitis group showed high sIL-2R value above the normal range. Vitreous sIL-2R tended to be higher in the IOL group than in the uveitis group (P = 0.80). Serum sIL-2R was significantly lower in the IOL without extraocular involvement group than in the IOL with extraocular involvement group (P < 0.05); 5.9% in the IOL without extraocular involvement group and 50.0% in the IOL with extraocular involvement group showed high sIL-2R value above the normal range. Vitreous sIL-2R, MMP-2, and MMP-9 tended to be higher in the IOL with extraocular involvement group than in the IOL without extraocular involvement group (P = 0.30, < 0.05, 0.16). CONCLUSIONS: Serum sIL-2R is often within the normal range in IOL patients. Even if it is within the normal range, the possibility of IOL should be considered. Serum sIL-2R is not a reliable biomarker for IOL, whereas vitreous sIL-2R may be useful for the diagnosis of IOL.

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.