Primary vitreoretinal lymphoma (PVRL) is a rare and aggressive high-grade non-Hodgkin lymphoma. PVRL always with non-specific symptoms and has therefore been called as masquerade syndrome. Thus, the early and correct diagnosis of PVRL is a difficulty. For PVRL, the imaging findings can be specific but variety, histological diagnosis is still the gold standard. Its diagnosis needs to be combined with clinical manifestations, imaging features, pathological diagnosis and molecular biology. With the advancement of technology, especially in the field of the cytokines detection and the gene expression profiling research, molecular biology diagnosis of PVRL is becoming a research hotspot and an important auxiliary diagnostic method.
The etiology of uveitis is complex and the clinical manifestations are varied. Diagnosis and treatment are often very difficult. Detailed medical history, thorough ocular examination and laboratory examination are important basis for diagnosis. In recent years, with the appearance of microincision vitrectomy surgery, it has been applied widely in the diagnosis and treatment of uveitis. It can confirm diagnosis, manage the fundus complications of uveitis, and has certain therapeutic effects. But it is worth emphasizing that the indications of vitrectomy must be strictly controlled. Suitable indications and surgical timing are the key to the effective diagnosis and treatment of uveitis.
ObjectiveTo observe and analyze the image characteristics of eyes with choroidal osteoma using swept-source OCT (SS-OCT).MethodsSeventy-three eyes of 61 patients with choroidal osteoma were enrolled in the study, including 17 males (20 eyes) and 44 females (53 eyes) with an average age of 33.5±12.7 years. Single tumor was found in 71 eyes and multiple tumors were found in 2 eyes. All patients received examinations of slit lamp ophthalmoscope, color fundus photography, fundus autofluorescence, fluorescein angiography and/or indocyanine green angiography, B-scan ultrasonography and/or computerized tomography. Tumor features were characterized using SS-OCT.ResultsAmong 75 tumors of 73 eyes, the osteoma was completely calcified in 7 tumors, completely decalcified in 5 tumors and all the other tumors were composed of calcified and decalcified portions. SS-OCT revealed normal inner retina in all the calcified areas of tumors and abnormal outer retina including external limiting membrane, ellipsoid zone, interdigitation zone and RPE in some cases. Whereas the outer retina and RPE showed abnormality in the decalcified areas of all cases. The choriocapillaris was invisible in all cases and most of the medium and large caliber vessels showed thinning or nonvisibility. Choroidal osteoma revealed a sponge-like appearance (n=39), loofah sponge appearance (n=11), lamellar pattern (n=10), mixed (n=12) and irregular patterns (n=5). Other unique features included horizontal lamellar lines (n=71), hyperreflective horizontal lines (n=47) and hyporeflective tubules (horizontal:n=39, vertical:n=42, circular:n=41) within choroidal osteoma. Hyperreflective tumor-like tissues were also detected above the disrupted Bruch’s membrane. The sclero-choroidal junction was detectable in all tumors and a posterior ciliary vessel penetrating the sclera was also seen.ConclusionsSS-OCT clearly reveals characteristic appearances of choroidal osteoma as sponge-like, loofah sponge, lamellar pattern, mixed and irregular patterns, with unique features of horizontal lamellar lines, hyperreflective horizontal lines and hyporeflective tubules within osteoma. Hyperreflective tumor-like tissues were detected to grow above the disrupted Bruch’s membrane.
ObjectiveTo investigate the clinical features and prognosis of patients with choroidal tuberculoma. MethodsA retrospective and observational study. From 2011 to 2022, 15 patients (16 eyes) with choroidal tuberculoma diagnosed and treated in Department of Ophthalmology, Shanghai Eye & ENT Hospital of Fudan University were included. The patients underwent examinations including best corrected visual acuity (BCVA), fundus color photography, optical coherence tomography (OCT), fluorescein fundus angiography (FFA), and indocyanine green angiography (ICGA). Chest CT examination, purified protein derivative test and interferon-γ release test were also performed. BCVA was performed using the Snellen visual acuity chart, which was converted to Logarithm of the minimum angle of resolution (logMAR) visual acuity when recorded. All the patients received antitubercular therapy and the duration was 12-15 months. Thirteen patients were combined with oral corticosteroids. The average follow-up time was 36 months. Rank sum test was used to compare logMAR BCVA before and after treatment. ResultsAmong the 15 patients, 5 were male and 10 were female; 1 case was bilateral involvement; 7 patients had a strong positive tuberculin skin test; 8 patients had a positive interferon-γ release assay. Six patients had pulmonary tuberculosis. One patient had peritoneal tuberculosis. Lesions were located in the posterior pole in 12 eyes, 1 eye with papillary tuberculoma. Peripheral lesions were found in 4 eyes. There were 9 eyes each with anterior segment inflammation or vitreous inflammation; 7 eyes were with exudative retinal detachment. OCT examination revealed a hyporeflective thickening of the choroidal stroma, the corresponding elevation of the retina, and often accompanied by subretinal fluid. FFA revealed hyperfluorescence of the mass and fluorescence accumulation with subretinal fluid at the late stage. ICGA revealed hypofuorescence of the lesion. The lesions subsided after treatment, and there was no recurrence during follow-up period. Cataract surgery was performed in 4 eyes due to complicated cataract. One eye undergone vitrectomy due to secondary rhegmatogenous retinal detachment. Anti-vascular endothelial growth factor therapy was performed in 1 eye due to secondary choroidal neovascularization. Ocular wall perforation occurred in 1 eye, and the condition was stable after treatment. Before treatment, the average logMAR BCVA was 1.02±0.57, which improved to 0.31±0.35 after treatment. The difference between the mean logMAR BCVA before and after treatment was statistically significant (P＜0.05). ConclusionsCombination of medical history, clinical manifestations, laboratory tests, and imaging can assist in the diagnosis of choroidal tuberculoma. The main manifestations are subretinal yellowish-white lesions in posterior pole. Standardized anti-tuberculosis therapy can effectively improve the prognosis of vision.
ObjectiveTo observe and analyze the clinical features and prognosis of proliferative diabetic retinopathy (PDR) with chronic myeloid leukemia.MethodsA retrospective case series study. From May 2011 to December 2020, 5 patients (10 eyes) were included in this study in Eye-ENT Hospital of Fudan University. Basic information about the patient's age, gender, diabetes history and CML history were collected. The endocrine and hematological indexes of all patients were evaluated. All the patients were undertaken visual acuity, intraocular pressure, slit lamp and fundus examination and other examinations to observe the eye conditions. Ophthalmic treatments included panretinal laser photocoagulation, intravitreal injection of anti-vascular endothelial growth factor, vitrectomy. During the follow up period from 5 months to 6 years, prognosis was observed at each office visit. During the follow up period, patients' vision, intraocular pressure, anterior segment and retinal status were observed.ResultsThere were 4 males and a female in 5 patients. The ages were from 27 to 49 years, with the mean age of 39 years. All patients were bilateral. All patients suffered type 2 diabetes for 3 months to 13 years. Four of them were diagnosed as chronic myeloid leukemia before visiting to ophthalmologists, while the other visited to ophthalmology first due to poor vision. The initial visual acuity ranged from light perception to 0.4 and 6 eyes were less than 0.1. In addition to the typical manifestations of diabetic retinopathy, such as venous tortuous dilation, exudation, microaneurysm and neovascularization, patients also presented with Roth spot as leukemic fundus manifestations. All eyes developed to PDR stage. Abnormal thickening of the neovascular membranes may occur in the lower part of the retina, with secondary traction retinal detachment. All the eyes were treated with pan retinal photocoagulation and 9 eyes underwent pars plana vitrectomy. After treatment, retina of 8 eyes kept flat. The best corrected visual acuity ranged from no light perception to 1.0, and only 4 eyes reached more than 0.2. Unfortunately, one eye lost vision because of secondary neovascular glaucoma.ConclusionsPDR patients with CMLof fundus not only have venous tortuous dilation, exudation, microaneurysm and neovascularization, also present with Roth spot as leukemic fundus manifestations. Diabetic retinopathy combined with CML could progress rapidly, and its aggravating complications such as hyperplastic membrane, vitreous hemorrhage and traction retinal detachment may result in poor visual prognosis. Early screening and treatment can help improve the prognosis of patients.
Stargardt disease (STGD) is one of the most prevalent inherited macular dystrophy, and most often occurs in child or adolescence. Irreversible vision loss is observed in almost all cases. Type 1 (STGD1) is one of the most common type. It is an autosomal recessive condition, caused by mutations in the Abca4 gene. In recent years, encouraging progress has been made in the treatment of STGD1. C20-D3-retinyl acetate (ALK-001), fenretinide and ICR-14967 (A1120) as visual cycle modulators, StarGen as gene supplementation therapies, and the stem cell transplantation of human embryonic stem cell-derived retinal pigment epithelium cells are the most promising therapies. With the development of studies and clinical trials, the clinical application of various treatments of STGD1 are expected in the near feature, which are expected to save the vision of most patients.
ObjectiveTo investigate macular microvascular abnormalities in eyes with subfoveal fibrotic nodules secondary to Coats' disease. MethodsA cross-sectional study. From January 1, 2018 to July 30, 2021, 45 eyes of 45 patients diagnosed with Coats' disease with or without subfoveal fibrotic nodules in Eye and ENT Hospital, Shanghai Medical College of Fudan University were included in this study. There were 40 eyes in 40 males and 5 eyes in 5 females. All were under 21 years old. According to the presence or absence of subfoveal fiber nodules, the patients were divided into fibrotic group (26 cases, 26 eyes) and non-fibrotic group (19 cases, 19 eyes). Optical coherence tomography angiography was used to scan 3 mm×3 mm or 6 mm×6 mm macular area of both eyes. The software of the device automatically processed the images. The presence of FAZ edge anastomotic vascular arch ring breakage and abnormal microvascular branch (AMB) in the foveal avascular zone (FAZ) were observed. ResultsIn 26 eyes of fibrosis group, AMB originating from the parafoveal retinal capillary network was observed, which grew into and destroyed the integrity of the vascular arch ring at the edge of FAZ. AMB was crisscrossing and winding, and its curvature expands. B-scan images showed the blood flow signal in the subfoveal fiber nodule, and the blood flow signal traversed between the inner retina and the fiber nodule in 23 eyes (88.46%, 23/26). In the non-fibrosis group, all the vascular abnormalities were characterized by capillary dilation and defect, and no breakage of FAZ anastomotic vascular arch ring or AMB was observed. ConclusionsIn Coats' disease with subfoveal fiber nodules, staggered and dilated AMBs emerge from the parafoveal vascular network, grow into and destroy the integrity of the vascular arch ring at the edge of FAZ, and grow down longitudinally into the fiber nodules.
ObjectiveTo observe the changes of varicella zoster virus (VZV)-DNA load in aqueous humour samples in VZV-induced acute retinal necrosis (ARN) in the early stages of antiviral treatment. MethodsA retrospective observational clinical study. From April 2016 to April 2018, 24 patients with 24 eyes of VZV-induced ARN who were diagnosed by Department of Ophthalmology, Eye and ENT Hospital of Fudan University and received complete aqueous humor sampling were included in the study. Among them, there were 13 males with 13 eyes, 11 females with 11 eyes; 12 left eyes and 12 right eyes; the age was 52.0±9.5 years old (39-71 years old). The time from the onset of ocular symptoms to the diagnosis of ARN was 16.6±6.1 days (7-30 days). Best-corrected visual acuity (BCVA) and ultra-wide-field fundus imaging were performed in all affected eyes. The BCVA examination was carried out using the Snellen visual acuity chart, which was converted into the logarithm of the minimum angle of resolution (logMAR) visual acuity. All patients were given intravitreal injection of 40 mg/ml ganciclovir 0.1 ml (including 4 mg of ganciclovir), 2 times a week, until the active necrotizing retinal lesions subsided, at most after the diagnosis 4 weeks, with a maximum of 9 injections. The follow-up period was 12.8±5.6 months. The aqueous humor samples were collected at presentation and 4, 7, 14, 21, 28 days after the initiation of antiviral therapy, and the VZV-DNA load was detected by real-time quantitative polymerase chain reaction. A plateau phase and a logarithmic reduction phase of the DNA load changes were observed after antiviral treatment began. Wilcoxon rank sum test was used to compare and analyze the differences in BCVA between the eyes at baseline and last follow-up. ResultsThe mean viral load at presentation was 8.6×107±1.3×108 copies/ml. The initial plateau phase last for an average of 7.4±2.4 days. In the following logarithmic reduction phase, the mean slope of the decline in viral load was －0.13±0.04 log/day, and the expected time for half reduction of the initial viral load was 2.5±0.7 days. After 28 days antiviral treatment, the viral load decreased to 1.7×105±1.8×105 copies/ml. In the course of the disease, rhegmatogenous retinal detachment occurred in 16 eyes. Before treatment and at the last follow-up, the logMAR BCVA of the affected eye was 1.1±0.6 and 0.8±0.7, respectively. The results of correlation analysis showed that the logMAR BCVA at the last follow-up was correlated with the initial VZV-DNA load (r=0.467, P=0.033). ConclusionThe VZV-DNA load in the aqueous humor of eyes with VZV-induced ARN is significantly decreased after antiviral treatment, which is closely related to the clinical process of ARN.
ObjectiveTo investigate the clinical characteristics, treatment and prognosis of primary vitreoretinal lymphoma (PVRL) diagnosed and treated in our hospital during the past 10 years. MethodsA retrospective clinical study. From 2011 to 2021, 126 eyes of 67 patients with PVRL who were diagnosed and treated in Department of Ophthalmology, Eye-ENT Hospital, Fudan University were included in the study. Among them, there were 23 males (34.3%, 23/67) and 44 females (65.7%, 44/67); the average age was 57.1 years. There were 59 cases with both eyes (88.1%, 59/67) and 8 cases with one eye (11.9%, 8/67). At the initial eye diagnosis, 22 cases had a clear history of primary central nervous system lymphoma (PCNSL); 5 cases were found to have intracranial lesions by head imaging examination; 40 cases had no central nervous system involvement. Twenty cases were treated with glucocorticoids due to misdiagnosed uveitis. All patients received intravitreal injection of methotrexate (IVM) treatment. The treatment regimen was twice a week in the induction period for 2 weeks, once a week in the consolidation period for 1 month, and once a month in the maintenance period. Patients with PCNSL or both eyes received concurrent systemic chemotherapy (chemotherapy), and some in combination with radiation therapy to the brain (radiotherapy). The mean follow-up time was 39.3 months. The clinical manifestations, treatment and prognosis of the patients were retrospectively analyzed. The visual acuity before and after treatment was compared by t test. ResultsAmong the 22 cases with a clear history of PCNSL at the initial eye diagnosis, the average time from intracranial diagnosis to eye diagnosis was 22.9 months. Among the 40 cases without central nervous system involvement at first, 14 cases (20.9%, 14/67) developed central nervous system lesions during follow-up period. The mean time from ocular diagnosis to intracranial diagnosis was 9.9 months. Among the 126 eyes, 42 eyes (33.3%, 42/126) had anterior segment inflammation. vitreous inflammation type, retinal type, and vitreous retinal type were 58 (46.0%, 58/126), 7 (5.6%, 7/126), and 61 (48.4%, 61/126) eyes, and 9 of them (7.1%, 9/126) had optic nerve involvement at the same time. Patients received an average of 12 IVM treatments. IVM combined with systemic chemotherapy in 59 cases (88.1%, 59/67), of which 16 cases were combined with brain radiotherapy. All patients achieved complete remission after completing the treatment cycle (100.0%, 67/67). After treatment, 21 eyes (16.7%, 21/126) had ocular recurrence; 22 (32.8%, 22/67) had intracranial recurrence; 8 cases (11.9%, 8/67) died. The mean progression-free survival of patients was 23.7 months; the mean survival time was 43.6 months; the 5-year overall survival rate was 72.5%. ConclusionsThe manifestations of PVRL are complex and diverse, and most of them are accompanied by involvement of the central nervous system. It can be divided into vitreitis type, retinal type and vitreoretinal type, and the optic nerve can be involved at the same time; IVM combined with systemic treatment can completely relieve the disease.
ObjectiveTo observe the clinical and multimodal imaging features of eyes with acute macular neuroretinopathy (AMN) associated with the coronavirus disease 2019 (COVID-19). MethodsA retrospective study. From December 20, 2022 to January 17, 2023, a total of 29 patients (58 eyes) with COVID-19-associated AMN admitted to Department of Ophthalmology of Eye and ENT Hospital, Shanghai Medical College were included in the study. All the affected eyes underwent the best corrected visual acuity, color fundus photography, infrared fundus photography (IR), short-wavelength autofluorescence (SW-AF), near-infrared autofluorescence (NIL-AF), optical coherence tomography (OCT), and OCT angiography (OCTA). All patients were administered microcirculation-improving oral medication with 12 cases receiving adjunctive low-dose corticosteroid therapy. Follow-ups were conducted 1 to 3 months after the initial diagnosis, with a total of 19 cases (38 eyes) completing the one-month follow-up. ResultsOut of the 29 cases, there were 9 males (18 eyes) and 20 females (40 eyes), all of whom experienced bilateral eye involvement. The age of the patients ranged from 12 to 47 years, with an average age of (29.9±9.5) years. The time from the onset of fever to the appearance of ocular symptoms was (2.52±2.01) days. Among the 58 affected eyes, there were 5 cases with retinal cotton wool spots, 2 cases with optic disc edema, and 1 case with parafoveal branch retinal vein occlusion. All affected eyes exhibited deep reddish-brown macular dark spots. IR revealed wedge-shaped, wedge-like, or "petaloid-like" dark areas involving the fovea and parafovea. SW-AF examination showed no obvious abnormality in 39 eyes. Weak autofluorescence dark area were consistent with IR in 19 eyes. NIR-AF examination showed spot-like or flaky self-fluorescent dark areas. OCT examination showed strong reflex lesions spreading vertically upward from the retinal pigment epithelium (RPE) layer in the macular area in the acute stage, showing typical "bean seedling" sign. OCTA revealed reduced blood flow density in the deep capillary plexus (DCP) of 50 eyes. En-face OCT displayed lesion areas that corresponded to the dark areas seen in IR. One month after the initial diagnosis, the condition improved in 18 eyes (47.4%, 18/38). Among the 5 eyes with cotton wool spots, regression of these spots was accompanied by loss of nerve fiber layer in 4 eyes. In cases with optic disc edema, the edema subsided. The "bean sprout" sign disappeared in all affected eyes, and the lesions became localized. The ellipsoid zone and/or interdigitation zone in the lesion areas were discontinuous. ConclusionsCOVID-19-related AMN is characterized by distinctive features. IR fundus reveals wedge-shaped, wedge-like, or petaloid dark areas involving the fovea and parafovea. OCT displays strongly reflective lesions with vertical spread above the RPE. OCTA shows reduced blood flow density in the DCP of the retina.