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 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.
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.
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 Beh?et'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 Beh?et's disease were the common entities.
Objective To analyze the onset, clinical manifestation, causation, complications of pediatric uveitis. Methods One hundred and two patients with uveitis under 16 year-old were retrospectively studied. They visited the clinic in Peking University First Hospital from November 1979 to December 2008. Their age ranged from 2.5 to 16 years old, with a mean of 11.9 years. Routine exam was carried out, including visual acuity, slit lamp, fundus, and laboratory workup. The diagnosis and classification were made by the anatomic location according to the standard of The International Uveitis Working Group. The data of disease history, age of onset, manifestation, recurrence, causation, systemic diseases, complications, and lab examination were analyzed.Results A total of 102 patients (170 eyes) with pediatric uveitis were included in this study, 68 patients (66.6%) were bilateral cases. Anterior uveitis represented in 38 patients (37.3%), intermediate uveitis in 19 (18.6%), posterior uveitis in 10 (9.8%), and panuveitis in 35 (34.3%). The disease duration was from five days to 2.4 years, with a mean of 3.6 months. The follow-up time was two weeks to more than ten years. The first three causes of pediatric uveitis were juvenile idiopathic arthritis, Vogt-Koyanagi-Harada disease, and Behccedil;etprime;s disease. 36 patients were found with complications, and among them 19 had complicated cataract, seven had secondary glaucoma, five had corneal band dystrophy, 12 had iris synechia (both anterior and posterior), one had retinal detachment, two had eye atrophy, and one patient with juvenile idiopathic arthritis had bilateral femoral head necrosis because of the use of steroid and hip joint was replaced. There were ten children suffering more than two complications. Conclusions Pediatric uveitis is a possible blindness disease with variety of etiology and manifestations,and tends to cause complications. Early and special attention must be taken to avoid serious consequences.
Objective To observe the characteristics of indocyanine green angiography (ICGA) in inactive polypoidal lesions of polypoidal choroidal vasculopathy (PCV). Methods The clinical data of 36 PCV patients (37 eyes) with inactive polypoidal lesions were retrospectively analyzed. The follow-up of 11 eyes were ranged from nine to 29 months, with a mean of (12.3plusmn;5.5) months. All the patients were examined for visual acuity, intraocular pressure, slit lamp microscope, fundus photography, fundus fluorescein angiography (FFA) and ICGA. According to the ICGA characteristics, PCV lesions were divided into active polypoidal lesions (pocket like hyperfluorescence at early stage and fluorescence leakage or stained with fluorescein at late stage) and inactive polypoidal lesions (pocket like hyperfluorescence and it was gradually faded). According to clinical and ICGA characteristics, inactive polypoidal lesions were divided into asymptomatic group, atrophic and/or cicatricial group and combined (with active polypoidal lesions) group. The visual acuity, fundus, lesions change and image characteristics of three groups were evaluated and analyzed. Results Among the 37 eyes, the time from indocyanine green (ICG) injection to inactive polypoidal lesions begin showing was ranged from 8.2 to 27.0 minutes, with a mean of (15.5plusmn;4.8) minutes. There were five eyes (13.5%), eight eyes (21.6%) and 24 eyes (64.9%) in asymptomatic group, atrophic and/or cicatricial group and combined group, respectively. The results of fundus examination showed that there was no hemorrhage, exudates, retinal pigment epithelium detachment (PED) and/or neural retina detachment in asymptomatic group; atrophy lesions and/or scar lesions were observed in atrophic and/or cicatricial group and there was also no hemorrhage, exudate, PED and/or neural retina detachment; there was no atrophy lesion and/or scar lesion, but there were 10 eyes with subretinal hemorrhage, 15 eyes with retinal exudate, 10 eyes with PED and four eyes with neural retina detachment in combine group. The results of ICGA showed that there were inactive polypoidal lesions in asymptomatic group; inactive polypoidal lesions located at the border of atrophy lesions and/or scar lesions in atrophic and/or cicatricial group; active polypoidal lesions and inactive polypoidal lesions coexisted in combine group. In 11 eyes which completed the follow-up, inactive polypoidal lesions regressed in three eyes (27.3%), partial regressed in two eyes (18.2%), unchanged in six eyes (54.5%). Conclusions The inactive polypoidal lesions of PCV mainly appear in the middle or late stage of ICGA and are manifested in asymptomatic, atrophic and/or cicatricial and combined eyes. The combined type which coexisted with active polypoidal lesions is the main form.
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 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 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 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.
