Objective To observe the clinical manifestation and ophthalmoscopic image characteristics of uveal metastatic carcinoma. Methods Thirty-six uveal metastatic carcinoma patients (43 eyes) were enrolled in this study. The patients included 21 males and 15 females. The patientsprime; ages ranged from 28 to 71 years, with a mean age of (47.3plusmn;10.2) years. Seven patients had bilateral carcinoma and 29 patients had unilateral carcinoma. There were 30 patients with lung cancer, three patients with breast cancer, one patient with gastric cancer and two patients without primary tumors. There were 20 patients with known primary cancer, 16 patients visited the Department of Ophthalmology first. All the patients were examined documenting visual acuity, intraocular pressure, slit-lamp microscopy and mydriatic fundus examination. Meanwhile, 22 patients (26 eyes) were examined using B-type ultrasound and/or color Doppler flow imaging (CDFI). Twelve patients (12 eyes) were examined using fundus fluorescein angiography (FFA) and/or indocyanine green angiography (ICGA). Seventeen patients (22 eyes) were examined using MRI and/or CT. The clinical manifestation and ophthalmoscopic image characteristics of uveal metastatic carcinoma patients were observed. Results Among 43 eyes, four lesions were in the iris, three lesions in the ciliary body and 32 lesions were in the choroid. Fundus examination showed an isolated mass in 26 eyes and more than two masses in nine eyes. Metastatic tumors of the iris and ciliary body often showed irregular cauliflower-like mass with gray-white or meat-red color and abundant vessels. The choroidal metastasis usually demonstrated flat rounded or irregular intraocular masses with gray-yellow or gray-white color in the posterior pole. B-type ultrasound showed ill-defined, flat, and irregular-shaped masses with uneven internal reflectivity. CDFI showed rich blood flow within the tumor. FFA and (or) ICGA showed pinpoint and mottled leaks against hyperfluorescence background. MRI revealed low or middle signal using T1WI and low signal intensity using T2WI. Conclusions The uveal metastatic carcinoma usually occurs in one eye with an isolated mass. Most of them show a flat gray-yellow mass in posterior choroids and have the primary cancer sites of the lung. FFA and/or ICGA show pinpoint and mottled leaks against hyperfluorescence background. B-type ultrasound and (or) CDFI show ill-defined, flat, and irregular-shaped mass with rich blood flow within the tumor. MRI reveals low signal intensity on T2WI.
Acute zonal occult outer retinopathy (AZOOR) is an acquired retinal diseases. The majority of patients who develop AZOOR are women characterized by an acute onset of visual blurred and scotoma with photopsias. The fundus examination is often normal or appeared mild abnormal. The RPE atrophy of fundus is similar with white syndrome. Although FFA and ICGA features are either unremarkable or unrelated to AZOOR, there are still important in differential diagnosis. The characteristic abnormalities appearance of FAF (complicated and varied), OCT (regional anomaly of ellipsoid zone), visual field (visual field defect) and ERG (decreased amplitude and prolonged latency of rod reaction, maximum reaction, cone reaction and scintillation reaction) are considered critical examinations to the diagnosis of AZOOR. Although there is no effective therapy for AZOOR, it has some self-limitation.
Objective To observe the ocular features and analyze the cause of misdiagnosis of intraocular nonHodgkin's lymphoma (IONHL). Methods A retrospective study was performed in 6 patients and related literatures were reviewed. The age of those patients was 46 to 68 yrs with an average of 562 yrs. Four cases were female, two cases were male. Five patients were unilateral and one case was bilateral case. All patients were investigated by Goldmann tonometer, fundus fluorescein angiography (FFA) and ophthalmic Bscan. Pathological samples come from enucleation and vitrectomy, and were analyzed histologically and immunohistochemically. Results Ophthalmological signs of IONHL included decrease visual acuity, red eye, aqueous flare; keratic precipitates (Kp), vitreous opacity, retinal detachment and multiple whiteyellow retinal lesions of different sizes and fuzzy boundaries. IONHL patients were always misdiagnosed as diverse and nonspecific symptoms and signs, two cases were misdiagnosed as retinitis or chorioiditis, one case as intermediate uveitis, one case as secondary glaucoma in uveitis. The duration from onset of symptom and sign to a final diagnosis was from 6 months to 24 months with an average of 13.3 months. Conclusions The clinical manifestation of IONHL was diverse and non-specific，and this disease was unknown to lots of doctors and easily be misdiagnosed.
ObjectiveTo observe the clinical features and visual prognostic factors of ethambutol-induced optic neuropathy (EON).MethodsA cohort study. Twenty-four inpatients (46 eyes) identified as EON in Neuro-Ophthalmology Department of Chinese PLA General Hospital from January 2014 to December 2017 were enrolled, including 14 males (26 eyes) and 10 females (20 eyes) with a ratio of 1.4/1 male/female. The average age was 42.79±15.12 years and the average weight was 62.46±12.31 kg. The average time duration between oral administration of ethambutol and occurrence of EON was 9.94±16.49 months. The average time of ethambutol duration was 7.06±11.68 months, with an average accumulative dose of 156.7±1 779.0 g and the average daily dose of 15.07±8.95 mg/(kg·d). All patients were tested with visual acuity, fundus photos, colour vision, OCT, visual field, VEP, orbital MRI and the gene of OPA1 and mitochondrial deoxyribonucleic acid (mtDNA). All the patients accepted drug withdrawal immediately after diagnosis, and were given the treatment of systemic nerve nutrition and improvement of microcirculation for 2 weeks. The time of follow-up was more than 12 months. According to whether the visual acuity (VA) in any of eyes was over than 0.1 at the last follow-up, all the patients were divided into two groups: the bad VA group (VA less than or equal to 0.1) and the better VA group (VA over than 0.1) group. The χ2 test and Fisher's exact probabilistic method test were used to compare the counting data between groups, and the Wlincox rank sum test was used to compare the measurement data. Multiple factors of VA outcome between the patients with bad or better va were analyzed by logistic regression.ResultsThirty eyes (65.2%) had VA less than or equal to 0.1 and 5 eyes (10.9%) had VA over than 0.5 at EON onset. The VA of the rest 11 eyes (23.9%) was higher than 0.1 and lower than 0.5. At the last follow-up, 20 eyes (43.5%) had VA less than or equal to 0.1 and 9 eyes (19.6%) had VA over than 0.5, the VA of the rest 17 eyes (36.9%) was higher than 0.1 and lower than 0.5. Fundus examination revealed 7 eyes (15.3%) with optic disc edema. OCT revealed significant loss of the retinal nerve fiber layer (RNFL) in the affected eyes, mainly in the temporal RNFL of the optic disc. All patients had dyschromasia, mainly in distinguishing the color of red and green. The types of visual field defect was as following: central dark spot (52.2%), diffuse visual acuity decreased (30.4%), temporal hemianopsia (17.4%). Orbital MRI revealed that 12/24 (50.0%) patients had T2 lesions with T1 enhancement in 6/24 patients (25.0%). Genetic test showed that 4 patients (16.7%) had gene mutation. Among them, there were 2 patients with OPA1 mutation, 1 with mtDNA 14340 point mutation and 1 with the mtDNA 11778 point mutation. Thirteen patients showed better VA outcomes (over than 0.1) while 11 showed bad VA outcomes after discontinuation of ethambutol. Between the better VA group and the bad VA group, there were statistically significant differences in the daily dose of ethambutol and gene mutation (P=0.031, 0.023). The daily dose was related to visual prognosis of EON while only the daily dose of more than 18 mg/(kg·d) may lead to bad VA outcomes according to the logistic analysis (95% CI 0.007-0.736, OR=0.069, P=0.027).ConclusionsEON may have OPA1 and mtDNA mutation with more bilateral eyes involved and less optic edema, which about 43.5% of the patients showed irreversible visual impact. The daily dose of ethambutol is related to the vision recovery.
ObjectiveTo assess the fundus characteristics and their associations with refractive error, best corrected visual acuity (BCVA) of highly myopic eyes in Chinese teenagers. MethodsThis is a cross-sectional and retrospective study. 544 teenagers (1050 eyes) with refraction more than -6.00 D were recruited from Tongren Eye Care Center. All participants underwent examinations including cycloplegic auto-refractometry and retinoscopy, BCVA, slit lamp and 45℃olor funds photography centered in macular. BCVA was recorded with logarithm of the minimum angle of resolution (logMAR) acuity. 988/1050 (94.1%) fundus photographs with clearly visible optic disc and fovea were selected for analysis. Degree of tessellation in optic disc and macular was defined by the exposure of choroidal vessel. Area of beta parapapillary atrophy (PPA), maximal and minimal diameter of optic disc, degree of fundus tessellation were measured by Image J software. Optic disc ovality was calculated by maximal diameter/minimal diameter. Associations between degree of tessellation, beta PPA area, optic disc ovality and refractive error, BCVA were analyzed. Presence of high myopic retinopathy, including chorioretinal atrophy, lacquer crack and Fuchs spot were also observed. ResultsMean spherical equivalent was (-10.66±2.63) D. Mean logMAR BCVA was 0.11±0.22. Tessellation was in 66.9% eyes. Mean degree in macular and peripapillary region was 0.83±0.96 and 1.04±1.00 (r=0.875, P=0.000). Beta PPA was in 97.3% eyes and mean area was (0.45±0.57) mm2. Mean ovality factor was 1.25±0.18 and Tilted optic disc was in 28.5% eyes. Refractive error, logMAR BCVA, beta PPA area, tilted optic disc and ovality factor were related with the degree of optic disc and macular tessellation (P < 0.05). Highly myopic retinopathy was found in 28 eyes, with older age, larger area of PPA, higher presence of tilted optic disc and degree of tessellation, worse BCVA. ConclusionsBeta PPA was the main fundus characteristics in teenagers. Visual acuity can be seriously impaired by highly myopic retinopathy, such as chorioretinal atrophy.
Objective To observe the clinical features of acute macular neuroretinopathy (AMN). Methods Six patients (11 eyes) with AMN were included in this study, with every 2-week follow-ups till six months. Among them, five had preceding dengue fever (83.3%), one had history of head trauma (16.7%). All patients received routine examination, fundus photography, infrared reflectance (IR) imaging, spectral-domain optical coherence tomography (SD-OCT) scanning and fluorescein fundus angiography (FFA) initially, and fundus photography, IR, SD-OCT during follow-up. Results Sudden onset of central/paracentral scotoma in one eye or both eyes was the main visual symptom. There were 1 eye with normal fundus, 2 eyes with wedge-shape lesions, 8 eyes with yellow-white or brown sheet lesion. IR imaging demonstrated localized areas of hypo-reflection in the macula. SD-OCT scanning through these areas revealed hyper-reflection in the photoreceptor layer and disruption of its normal reflective structures. Subsequent SD-OCT demonstrated that the hyper-reflection of the photoreceptor layer regressed gradually, followed by thinning of the outer nuclear layer. The external limiting membrane and ellipsoid zone became continuous; however, the interdigitation zone was not restored. There was no remarkable findings of the AMN lesions on FFA. The scotomas persisted in all 6 patients (11 eyes) by the last visit. Conclusions IR imaging demonstrated localized areas of hypo-reflection in the macula. SD-OCT revealed hyper-reflection in the photoreceptor layer in acute stage and the interdigitation zone was not restored in late stage. AMN has a relative poor prognosis with persistent scotomas through at least 6 months.
Objective To investigate the characteristics of fundus photography and fundus fluorescein angiography (FFA) of IRVAN (idiopathic retinal vasculitis, aneurysms, and neuroretinitis) syndrome and Eales disease. Methods The fundus photography and FFA data of 4 cases (8 eyes) with IRVAN syndrome and 43 cases (68 eyes) with Eales disease were retrospectively analyzed. All patients received ophthalmic routine examinations, including visual acuity, intraocular pressure, slit-lamp microscope and indirect ophthalmoscope. All patients had taken fundus photography and FFA for both eyes, except 4 patients of Eales disease who had vitreous hemorrhage in one eye. All 4 cases(1 male/3 female )with IRVAN syndrome were bilateral and aged 1643 years old( mean age 2700plusmn;1293 years old). 43 cases (32 male/11 female) of Eales disease aged 6-59 years old( mean 30.79plusmn;11.46 years old), 29 cases were bilateral and 14 cases were unilateral. Both diseases had retinal vascular whitesheath or white threadlike changes, exudative retinal hemorrhage and vitreous hemorrhage. Results Both arteries and veins of posterior pole of all eyes with IRVAN syndrome were involved and shown multiple retinal macroaneurysms. Other signs of IRVAN syndrome included capillary occlusion and nonperfusion (7/8 eyes, 87.5%),fluorescein leakage and edema of optic disc (5/8 eyes,62.5%), optic atrophy(2/8 eyes,25%), vitreous hemorrhage(1/8 eyes,12.5%), neovascularization of optic disc(2/8 eyes,25%), retinal neovascularization(4/8 eyes,50%) and macular edema(4/8 eyes,50%). The signs of Eales disease included fluorescein leakage of peripheral retinal vein (68/68 eyes, 100%), fluorescein leakage of posterior retinal vein (32/68 eyes, 47.06%), artery involvement (5/68 eyes, 7.35%), peripheral capillary occlusion and nonperfusion (38/68 eyes, 55.88%), fluorescein leakage of optic disc(29/68 eyes, 42.65%), neovascularization of optic disc(4/68 eyes,5.88%), retinal neovascularization(26/68 eyes,38.2%) and macular edema(15/68 eyes,22.06%). Compared IRVAN syndrome with Eales disease, the difference of artery inflammation, vein inflammation, retinal macroaneurysms in posterior area had statistics significance(all P=000,Plt;005), and that of edema of optic disc, retinal vascular nonperfusion area, neovascularization of optic disc, neovascularization elsewhere, and macular edema had no statistics significance(chi;2=0.479，P＞0.05；P=0.131，P＞0.05；chi;2=1.449，P＞0.05；chi;2=0.068，P＞0.05；chi;2=1.676，P＞0.05). Conclusions Both IRVAN syndrome and Eales disease may have vein and artery inflammation in posterior pole of the eye, and may result in neuroretinitis. IRVAN syndrome has much more vein and artery inflammation in posterior pole than Eales disease. Posterior retinal macroaneurysms is the most important sign for the diagnosis and differential diagnosis of IRVAN syndrome and Eales disease.
Objective To observe the clinical characteristics and fundus imaging features of unilateral acute idiopathic maculopathy (UAIM). Methods Retrospective cases series. Six eyes of 6 patients with UAIM were included in this study. There were 4 males and 2 females. All patients underwent best corrected visual acuity (BCVA), fundus color photography, spectral domain optical coherence tomography (SD-OCT) and fundus fluorescein angiography (FFA) examinations. 3 patients underwent indocyanine green angiography (ICGA) examination. No intervention was given in all patients after diagnosis. The mean follow-up was 6.3 months. BCVA, fundus color photography, SD-OCT and FFA were performed in follow-up. Results The ocular symptoms included unilateral sudden decreased vision, metamorphopsia and central scotoma. All patients had a yellow-white lesion involving the macular and irregular small bleeding was seen in 3 eyes. Irregular hyper-fluorescence in macular area was seen in all patients, some with the hemorrhages showed blocked fluorescence at the early stage of FFA and fluorescence storage at the late stage. ICGA showed that hypo-fluorescence in macular area continually. SD-OCT showed that subretinal fluid in the macular and lost the ellipsoid zone. During the follow-up period, the subretinal fluid was absorbed spontaneously in one month. The ellipsoid zone structure recovered gradually and the visual acuity were normal. At the time of the latest follow-up, the BCVA was 0.8 in 4 eyes, 1.0 in 2 eyes. Conclusions UAIM is featured with a unilateral, acute, transient and exudative lesion in the macular, which can be spontaneously resolved; It mainly affects outer retina.
Autosomal recessive Best disease (ARB) is a rare clinical fundus disease caused by BEST1 mutation. The critical features of ARB are the presence of multifocal subretinal yellowish lesions, which corresponding to the hyperfluorescent spots on FAF, scattered over the posterior pole of the retina, absent of typical vitelliform lesions in the macula. Imaging of OCT is often manifested as subretinal or intraretinal fluid, and cystoid macular edema, and hypereflective focus at RPE level. EOG shows an absent or severely reduced light rise (decreased value of Arden), which often accompanied by reduction and delay of the rod and cone ERG. Some patients with ARB show hyperopia, short axial length and shallow anterior chambers, with a corresponding high incidence of angle-closure glaucoma. Though there isn't any effective therapeutic methods of ARB at present, prevention and treatment for its complications such as angle-closure glaucoma and choroidal neovascularization should be considered. Present study about ARB mainly focus on some retrospective cases, and ARB is often misdiagnosed with Best vitelliform macular dystrophy, central serous chorioretinopathy and other fundus diseases in clinic. A detailed understanding of the clinical features and genetic characteristics of ARB might be helpful in clinical diagnosis and treatment. Research with larger sample size are expected to further investigate the different stages of ARB and its developing process, the potential pathological mechanism, the relationship between genotype and phenotype, so as to improve the understanding of the disease.
ObjectiveTo analyze the clinical character of uveitis in second hospital of Jilin university. MethodsRetrospectively analyze the clinical data of uveitis patients referred to from Second Hospital of Jilin University from September 2009 to September 2014. According to anatomical location, the manifestation of these uveitis patients were divided into anterior uveitis, panuveitis, intermediate uveitis and posterior uveitis. To discuss the possible causes of these patients according to the general information and relevant clinical laboratory examinations results. ResultsThere were 1215 cases in this study, which included 587 male, accounting for 48.31%; and 628 female, accounting for 51.69%. The ratio of male-to-female was 0.93:1. The range of the age of these patients was from 4 to 91 years old. The mean age of these patients at the onset of these disease was (41.43±14.20) years. Of the 1215 cases, 40 male and 43 female were younger than 20 years. The ratio of male-to-female was 0.93:1; 412 male and 396 female were between 21 and 50 years old. The ratio of male-to-female was 1.04:1; 135 male 189 female were older than 50 years. The ratio of male-to-female was 0.71:1. There were 572 cases of anterior uveitis, accounting for 47.08%; 527 cases of panuveitis, accounting for 43.37%; 52 cases of intermediate uveitis, accounting for 4.28%; 64 cases of posterior uveitis, accounting for 5.27%. 703 cases had etiological diagnosis according to the clinical character and the auxiliary results, accounting for 57.68%. Vogt-koyanagi Haradal (VKH) syndrome, ankylosing spondylitis associated with uveitis and Behçet's disease were the common entity, accounting for 30.44%, 19.77% and 14.22% respectively. ConclusionsThe mean age of these patients in this study was older, compared to other reports. Female patients were more than male, especially in these patients older than 50 years. VKH syndrome, ankylosing spondylitis associated with uveitis and Behçet's disease were the common entities.