ObjectiveTo observe the clinical characteristics of retinoblastoma (RB) in Southwest China.MethodsA retrospective clinical study. From January 2010 to December 2017, 66 RB patients diagnosed in Ophthalmology Department of West China Hospital of Sichuan University were included in the study. All the patients underwent ocular B-ultrasound, orbital CT or MRI examination. Ten patients underwent RetCam examination at the same time. Twenty-nine patients were diagnosed by histopathological examination, and 37 patients were diagnosed by clinical symptoms and imaging examination. According to whether the tumor invaded the orbit and optic nerve, it could be divided into extraocular stage and intraocular stage. Intraocular tumors were divided into A-E stages according to the international intraocular RB classification. Treatments were performed according to different stages. The general information, age at diagnosis, course of diseases (the time between onset symptoms and diagnosis), causes of visiting a doctor, classification, treatment methods and eyeball preservation rate were retrospectively analyzed.ResultsPatients all came from Southwest China (56 patients from Sichuan Province, 2 patients from Yunnan Province, 2 patients from Guizhou Province, and 6 patients from Tibet). The permanent residence were identified in 43 patients, including 27 patients (62.8%) from rural areas. There were 38 males (57.6%); 50 unilateral tumors (75.8%) and 16 bilateral tumors (24.2%); 51 first-visiting patients (77.3%) and 15 re-visiting patients (22.7%). The average diagnostic age of first-visiting patients was 20.9±14.4 months, with 23.2±14.7 and 11.2±7.6 months for unilateral and bilateral tumors, respectively. There were 41 patients had definite course and causes, of whom the average course was 90.6±115.2 days. The most common cause was leucocoria in 32 patients (62.7%), followed by redness and swelling in 4 patients (9.8%), and other causes in 5 patients (12.2%). Among the 15 re-visiting patients, the average diagnostic age was 63.6±46.8 months, the average course was 32.8±45.5 months. Recurrence was occurred in 5 patients (33.3%), leucocoria in 4 patients (26.7%), postoperative complication in 3 patients (20.0%), protrusion in 2 patients (13.3%) and redness in 1 (6.7%) patient, respectively. Fifty out of 82 eyes were admitted to hospital, including 37 eyes of first-visiting patients and 13 eyes of re-visiting patients. Among 37 first-visiting eyes, there were 5 eyes (13.5%) in stage A-C, 26 eyes (70.3%) in stage D-E, 6 eyes (16.2%) in extraocular stage. Five eyes in stage A-C were treated with laser photocoagulation and (or) cryotherapy combined with systemic chemotherapy. Four eyes in stage D were treated with intraocular arterial chemotherapy. Nineteen eyes (51.3%) were performed with enucleation, 2 eyes (5.4%) with evisceration and 7 eyes (18.9%) abandoned treatment. Among 13 re-visiting eyes, 6 eyes (46.2%, with 5 eyes of recurrence) had been enucleated before, 4 eyes (30.8%) were in extraocular stage and 3 eyes (23.1%) in stage D-E. Five eyes (38.5%) were treated with evisceration, 4 eyes (30.8%) with enucleation, 1 eye with oculoplastic surgery and 3 eyes (23.1%) abandoned treatment. The rate of eye preservation was 18.0%, 29.0% for intraocular stage and 0% for extraocular stage, respectively.ConclusionRB patients in Southwest China have a longer course between onset symptoms and diagnosis, more advanced classification and lower rate of eye preservation.
Objective To observe the clinical characteristics of demyelinating optic neuritis (DON) in Chinese children under the age of 16. Methods A retrospective review of the medical charts of 42 pediatric patients with DON was conducted in this study. Twenty-two patients (52.4%) were male, and 20 patients (47.6%) were female. The patients aged from 3 to 15 years, with the mean age of (9.5±2.3) years. There were 35 bilateral patients and 7 unilateral patients. Twenty-seven patients (64.3%) had prodromal symptoms before onset. All patients underwent visual function and imaging tests, such as best corrected visual acuity (BCVA), fundus photography, visual evoked potential (VEP), visual field, MRI. The patients were tested for serum levels of antibodies for aquaporin 4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) with a cell-based assay. All patients were received corticosteroid therapy. The mean follow-up was (1.17±0.42) years. The children who had coordination ability and with BCVA≥0.3 were received examination of Humphery automatic perimeter. Data were collected on the age, gender, clinical features, neuroimaging, serological specific antibodies, treatment and vision prognosis. Results 23.8% of the children were bilateral optic neuritis in onset stages. 64.2% were recurrent optic neuritis and 83.3% exhibited bilateral diseases eventually. BCVA had decreased to ≤0.1 in 87.0%% eyes and disc swelling was observed in 77.9% eyes during the onset stages. All eyes had visual field defects and abnormal VEP exam results, with delayed latency of P100 and P2, and varying degrees of amplitude reduction. Serum AQP4 antibody and MOG antibody were tested by cell-based assay, 2/42 children (4.7%) were positive for AQP4 antibody and 5/24 children (20.8%) were positive for MOG antibody. All of anti-AQP4+ and anti- MOG+ cases relapsed. All children underwent orbital magnetic resonance imaging (MRI), 40 cases (95.2%) showed demyelination features of optic nerve, and 5 cases (11.9%) showed long segments lesion (more than 1/2 length of the optic nerve). There were 2 anti-AQP4+ cases and 3 anti- MOG+ cases from the 5 cases with long segments lesion. MRI also showed brain demyelinating lesions in 4 children (3 of them were anti- MOG+) or spinal cord demyelinating lesions in 3 children (2 of them were anti- MOG+). After treatment with glucocorticoid, visual acuity improved in all eyes, of which 84.4% with BCVA≥0.5. Forty-eight eyes of 26 children accept dynamic visual field during the course of treatment, showed the vision abnormalities associated with optic nerve damage. Conclusions Children under the age of 16 with DON can experience severe visual impairment, higher recurrence tendencies, and higher rate of disc involvement, but good response to glucocorticoid therapy. AQP4 or MOG antibodies positive might be concurrent with brain and (or) spinal cord demyelinating lesions and indicated a poorer prognosis.
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 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.
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.
ObjectiveTo observe the clinical characteristics of adult patients with ocular toxoplasmosis (OT).MethodsThis study was a retrospective clinical trial. Among the 83 consecutive OT patients diagnosed in the Zhongshan Eye Center of Sun Yat-Sen University, 14 patients (16.9%, adult group) aged ≥18 years were included in the study from January 2017 to December 2019. A total of 14 OT patients (children group) aged less than 18 years who had consecutive visits from July to December 2019 were selected as controls. All patients underwent BCVA, slit lamp microscope, fundus color photography, and B-mode ultrasound examination. Eighteen cases underwent UBM examination, including 6 and 12 cases in the adult group and child group respectively; 23 cases underwent FFA examination, including 12 and 11 cases in the adult group and child group respectively. Snellen visual acuity chart was used for BCVA examination, which was converted into logMAR visual acuity in statistics. We compared the two groups of patients' residence (urban or rural), history of contact with dogs and cats, main symptoms, reasons for treatment, course of disease, and clinical and imaging characteristics and so on. For measurement data conforming to normal distribution, t-test was performed for comparison between groups; for Skewness distribution measurement data, rank sum test was performed for comparison between groups.ResultsCompared with the adult group and the child group, there was no significant difference in the canine-cat contact history (Z=2.661, P=0.257) and the time from first diagnosis to diagnosis (t=−0.186, P=0.351); compared with the children group and the adult group, the patient's living environment was mainly urban, and the course of disease was significantly shorter (Z=−2.005), and the difference was statistically significant (P=0.047). The logMAR BCVA of the adult group and the child group were 0.81±1.08 and 2.08±1.30, respectively, and the difference was statistically significant (Z=−2.811, P=0.004); compared with the child group, the adult group had lighter vitreous opacities, but the difference was statistical significance (Z=7.847, P=0.048). FFA examination revealed 20 cases of "fern-like" leakage of retinal capillaries. Among them, the adult group and child group were 10 (83.3%, 10/12) and 10 (90.9%, 10/11) cases respectively.ConclusionsAdult patients account for 16.9% of OT patients. Compared with children, adult patients mostly live in towns or cities, have a short course of disease, good vision at first diagnosis, and mild inflammation or hyperplasia of the vitreous cavity. FFA is helpful for the diagnosis of adult OT.
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.
ObjectiveTo observe the fundus image characteristics of macular telangiectasia type 2 (MacTel type 2) patients. MethodsA total of 8 patients (16 eyes) diagnosed of MacTel type 2 were included in this study. There were 4 males and 4 females, age ranged from 44 to 69 years old with a median age of (59.88±7.85) years. All patients received examination of best-corrected visual acuity (BCVA), slit lamp microscope, indirect ophthalmoscopy, fundus color photography, fundus autofluorescence (AF), fundus fluorescein angiography (FFA), spectral domain optical coherence tomography (OCT) and macular pigment optical density (MPOD). Four eyes of 2 patients received OCT angiography examination at the same time. Classification was made according to the Gass and Blodi's criteria. The follow-up time was from 1 to 19 months with the average time of (11.00±8.91) months. The clinical characteristics were observed and analyzed. ResultsThe BCVA was 0.07-0.8. There were 1 eye in stage 1, 1 eye in stage 2, 6 eyes in stage 3, 8 eyes in stage 4. The disease showed a bilateral appearance with a low progression. Fundus features included loss of retinal transparency (14 eyes, 87.5%), blunted retinal venule (15 eyes, 93.75%), pseudo-lamellar hole (5 eyes, 31.25%), pigment proliferation (9 eyes, 56.25%). FFA findings were telangiectatic capillaries predominantly temporal to the foveola in the early phase and a diffuse hyperfluorescence in the late phase. Spectral domain OCT features included depletion of the retinal inner, outer structures, cavity (7 eyes, 43.75%), and atrophy of the neurosensory retina (9 eyes,56.25%). On AF, reduced foveal masking due to loss of macular pigment can be observed. The loss of macular pigment could also be seen on MPOD. OCTA showed the increased intervascular spaces, broken regular network of foveal avascular zone (FAZ), right-angled vessel dipping, dilatations, traction of superficial and deep capillary layers in both the superficial and deep layers, especially in the deep layer. Only one eye of one patient proceeded from stage 4 to stage 5. ConclusionsCharacteristic features of MacTel type 2 included cavities and outer retina atrophy on OCT; loss of MP on MPOD; broken regular network of FAZ, morphological and structural abnormalities of superficial and deep layers of perifoveal capillary network on OCTA.
Objective To compare neovascular age-related macular degeneration (nAMD) and polypoidal choroidal vasculopathy (PCV) in age at diagnosis, gender and disease laterality. Methods One hundred fourteen nAMD patients (114 eyes) and 145 PCV patients (186 eyes) diagnosed by fundus fluorescein angiography (FFA) and indocyanine green angiography were enrolled in this retrospective study. The age at diagnosis, gender and disease laterality of all the patients were collected. Independent sample t-test, chi;2 test and Fisher's exact test were used to compare the age at diagnosis, gender and disease laterality between nAMD and PCV patients. Results The mean age at diagnosis of nAMD group and PCV group were (68.30plusmn;9.86), (65.67plusmn;9.04) years respectively, the difference was statistically significant (t=-2.168, P=0.031). The patients under 70 years old accounts for 50.88% in nAMD group, which is lower than that in PCV group (63.45%), the difference was statistically significant (chi;2 =4.138, P=0.042). The male/female ratio of nAMD group and PCV group were 3.56∶1 and 2.02∶1 respectively, the difference was statistically significant (chi;2 =3.937, P=0.047). Thirty patients (26.32%) and 41 patients (49.46%) were affected unilaterally in nAMD and PCV group, respectively. The difference of bilateral incidence between two groups was not statistically significant (chi;2 =0.123, P=0.726). There were 69 right eyes (47.92%) and 75 left eyes (52.08%) in nAMD group, 92 right eyes (49.46%) and 94 left eyes (50.54%) in PCV group. The difference of disease laterality between two groups was not statistically significant (chi;2 =0.078，P=0.637). Conclusions PCV patients present at younger age than nAMD. nAMD is more prone to affected males than PCV. Nearly a quarter patients are bilateral in nAMD or PCV, there is no difference in bilateral incidence between these two diseases.
ObjectiveTo analyze the clinical features and prognosis of adult optic neuritis patients with positive serum myelin oligodendrocyte glycoprotein antibody (MOG-ON) or aquaporin 4 antibody (AQP4-ON).MethodsA retrospective study. From December 2015 to February 2018, in the Beijing Chaoyang Hospital of Capital Medical University and Chinese PLA General Hospital, 162 eyes of 132 patients with positive serum MOG antibody and AQP4 were included in the study. There were 42 MOG-ON patients (49 eyes, 31.8%), 90 AQP4-ON patients (113 eyes, 68.2%). The clinical features of optic neuritis (annual recurrence frequency, incidence of optic disc edema), brain and optic nerve enhanced MRI, serum autoimmune antibodies and cerebrospinal fluid test results were compared between MOG-ON and AQP4-ON patients. All patients were treated with intravenous methylprednisolone sodium succinate in the acute phase and then switched to oral prednisone acetate tablets. The average follow-up time was 15 months. The glucocorticoid dependence, visual prognosis, spinal cord symptoms, and myelitis at the last follow-up were comparatively analyzed between MOG-ON and AQP4-ON patients. The comparison of the count data was performed by χ2 test, and the measurement data were compared by t test.ResultsCompared with AQP4-ON patients, MOG-ON patients had higher annual recurrence frequency (t=3.760, P=0.005), higher incidence of optic disc edema (χ2=14.777, P＜0.001), higher incidence of hormone dependence (χ2=25.496, P＜0.001), and better visual prognosis (χ2=28.759, P＜0.001). MOG-ON patients were more likely to involve the optic nerve, AQP4-ON patients were more likely to involve the optic chiasm and the optic tract. There was a significant difference in the location of lesions between MOG-ON and AQP4-ON patients (χ2= 5.447, P= 0.015). The proportion of AQP4-ON patients with autoimmune antibodies was significantly higher than that of MOG-ON patients (χ2 = 20.453, P＜0.001). The results of cerebrospinal fluid test showed that the white blood cell count of patients with MOG-ON and AQP4-ON were within the normal range, but the IgG level of AQP4-ON patients was significantly higher than that of MOG-ON patients (t=8.669, P＜0.001). At the last follow-up, there were 7 and 29 patients of myelitis in MOG-ON and AQP4-ON patients respectively (χ2=3.494, P=0.046).ConclusionsThe clinical characteristics of MOG-ON were different from AQP4-ON. The incidence of optic disc edema and recurrence rate were higher, but the proportion of autoimmune antibodies was lower. MOG-ON was more likely to show hormone dependence, but the visual prognosis was better. AQP4-ON was easily involved in optic chiasm and optic tract, and the incidence of myelitis was higher.