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 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 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.
Objective To observe the clinical characteristics of ocular albinism type 1 (OA1) in China.Methods Sixteen patients with OA1 and eight female carriers (eight OA1 patients and four carriers were from the same family) were enrolled in this study. All subjects were examined for corrected visual acuity, slit-lamp microscopy and GPR143 gene mutation detection. Some subjects also received indirect ophthalmoscopy, photography of anterior segment and ocular fundus, retinoscopy and optical coherence tomography (OCT). Iris pigmentation was divided into type A, B and C according to the above findings. Type A: slight pigmentation; type B: uneven pigmentation; type C: large patch of hypo-pigmentation with hippus. Result The corrected visual acuity of 16 OA1 patients was from 0.1 to 0.3. All the OA1 patients have bilateral horizontal nystagmus without strabismus. All eight subjects received retinoscopy have medium to low hypermetropia and astigmatism. Type A, B and C iris pigmentation were found in eight (50.0%), seven (43.7%) and one (6.3%) patients, respectively. Two (25.0%) carriers have Type A iris pigmentation, and other six carriers (75.0%) were normal. There is no typical albinism retinopathy, however macular foveal structures were never found in these patients. Four carriers have fundus striate or uneven hypo-pigmentation. The macular foveal structure of all the carriers was normal. The macular foveal structure was not found in three subjects who underwent examination of OCT. GPR143 gene mutation was found in all the OA1 patients (100.0%) and all the carriers.Conclusions The major clinical characteristics of Chinese OA1 patients are foveal hypoplasia and GPR143 gene mutation.
Objective To observe the clinical features of combined central retinal artery and vein occlusion. Methods The clinical data of eight patients of combined central retinal artery and vein occlusion diagnosed by fundus examination and fundus fluorescein angiography (FFA) was analyzed retrospectively, including the causes, fundus manifestations and FFA features. Results 4/8 patients had hypertension and dyslipidemia, 2/8 patients had traumatic retrobulbar hemorrhage, one patient had orbital cellulitis and one patient had systemic lupus erythematosus. All the patients had posterior pole retinal edema, hemorrhage, thin retinal artery, dilated vein, and papilledema. FFA showed delayed arterial filling, and there was no filling of retinal arterial branches until the late stage of FFA. Laminar flow delayed in large retinal veins, and there was no filling or only retrograde filling in retinal vein branches. Large areas with dot-like or patchy weak choroidal fluorescence can be observed in five patients. Conclusions Combined central retinal artery and vein occlusion is rare with complex etiology. The fundus manifestations and FFA features are atypical, but have features of central retinal artery occlusion and central retinal vein occlusion.
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 fundus characteristics of human immunodeficiency virus with acquired immune deficiency (HIV/AIDS). Methods A total of 1041 HIV/AIDS patients were enrolled in this study. The patients included 882 males (88.70%) and 159 females (11.30%). The patientsprime; ages ranged from 12 to 73 years, with a mean age of 41 years. The median time of HIV/AIDS diagnosis was 12 months, which ranged from one month to 10 years. HIV infection was acquired through sexual contact, intravenous drug use, blood transfusion or mother-to-child transmission in 475 patients (45.63%), 508 patients (48.80%), 44 patients (4.25%) and 14 patients (1.34%), respectively. Ocular examinations (vision acuity, slit lamp microscope and fundus examination) were performed on recruited patients with HIV/AIDS. Additional exams (intraocular pressure, fundus photography and fundus fluorescein angiography) were done if abnormal ocular fundus was found. The ocular manifestations were diagnosed according to clinic reference. Results Ocular manifestations of HIV/AIDS were detected in 247 patients (23.73%). Of 247 patients, the most common ocular manifestation was HIV retinopathy, which was present in 132 patients (53.44%); cytomegalovirus retinitis (CMVR) was second place, affecting 70 participants (28.34%). Clinic findings of HIV retinopathy included retina microaneurysm, hemorrhage along the blood vessel with cotton-wool spots, while irregular dry edge, granular appearing border, were present in CMVR, and the optic nerve may be affected. Fluorescein angiogram of HIV retinopathy demonstrated that hemorrhage was shown as sheltered fluorescence, with b fluorescence without leakage in center of hemorrhage. Fluorescein angiogram of CMVR demonstrated significant hemorrhage appearing as sheltered fluorescence with leakage and/or transparent fluorescence. The optic disk and lesioned area were stained with fluorescence. Conclusions There are various HIV/AIDS related ocular manifestation. HIV retinopathy and CMVR are common ocular manifestations. The main clinical findings of HIV retinopathy are hemorrhage and/or cotton-wool spots, while irregular granular appearing edges and hemorrhage were observed in CMVR.
Objective To observe the clinical features and treatment outcomes of presumed tubercular retinal vasculitis. Methods This is a retrospective non-comparative interventional clinical research. A total of nine patients (11 eyes) with major presentation of retinal vasculitis were included in this study. Patients first consulted the eye clinic and were diagnosed presumed tubercular retinal vasculitis. The patients, seven males and two females, aged from 19 to 66 years, with an average of 43.89 years. The time interval from symptoms to diagnosis ranged from two weeks to six months with an average of 76.27 days. Visual acuity, slit lamp ophthalmoscopy, fundus fluorescein angiography (FFA), optical coherence tomography (OCT), hematological and tuberculosis related investigations were examined and analyzed. All patients had standard anti-tuberculosis treatment. Treatment outcomes were followed for six to 37 months with an average of 14.11 months. Results Baseline visual acuity ranged from hand movement to 0.8 with an average of 0.28. Among 11 eyes, six presented mild to moderate vitritis, five presented as retinal vein occlusion with no obvious vitirits. Fundus examination showed six cases with retinal hemorrhage, four cases with macular edema, two with macular epiretinal membrane, and two with vitreous hemorrhage. FFA revealed 11 cases with leakage of vessels, 11 with nonperfusion area, four with macular edema, three with retinal neovascularization, and two with choroidal lesions. OCT of nine eyes suggested six eyes with retinal edema, three with macular edema, three with macular epiretinal membrane. TST of seven patients were all b positive. T-SPOT.TB of four patients were all positive. Three of eight patients who had chest X-ray or chest CT were suggested tuberculosis infection. Four to six weeks after the start of anti-tuberculosis treatment, vitritis, exudates, retinal and macular edema subsided. During follow up, inflammation was stable with no recurrence observed. The visual acuity of last follow-up ranged from 0.15 to 0.8 with an average of 0.51. Conclusions The main presentations of presumed tubercular retinal vasculitis are vitritis, retinal vein occlusion, and retinal hemorrhage. Standard anti-tuberculosis treatment can improve inflammation and retinal hemorrhage.
ObjectiveTo summarize the clinical features and visual outcome of posterior scleritis presented with symptoms involving affected optic nerve.MethodsRetrospective case series study. Twelve eyes of 12 female patients with posterior scleritis were included in this study. The average age was 35.2±14.31 years old. The patients got diagnosed with an average of 24.75±22.91 days. Ocular pain was complained in all patients, and blurred vision in 11 patients. The best corrected visual acuity (BCVA), intraocular pressure (IOP), slit lamp microscope examination, B-scan ultrasound, optical coherence tomography (OCT), fundus photography, fundus fluorescein angiography (FFA) and ocular wall thickness measurement were performed in all patients. Nine eyes received visual field examination. All patients received systemic corticosteroid and steroidal eye drops for 3 months. Clinical features and outcome were retrospectively studied.ResultsBefore treatment, the BCVA was from ＜0.1 to ＞0.8. There were 3 eyes with scleral hyperemia, 3 eyes with anterior chamber flares, 12 eyes with papilledema and different degrees of retinal vein dilatation, 3 eyes with star-shaped macular exudates and 2 eyes with macular retinal pigment epithelium detachment. B-scan ultrasound demonstrated that the ocular walls were thickening in all eyes with typical T-sign, and the average thickness was 2.76±0.68 mm. OCT demonstrated optic disc swelling, and the macular retinal detachment in 2 eyes. In the FFA examination, the fluorescein leakage of the disc was enhanced with time. In the Humphrey test, the value of mean deviation (MD) was 12.56±5.73 dB and pattern standard deviation (PSD) was 8.15±4.23 dB in 9 eyes before the treatment. After treatment for 3 months, the symptoms were attenuated and the visual acuity was obviously improved with BCVA>0.1 in all eyes. Scleral hyperemia and anterior chamber flares were only found in 1 eye. The optic disc edema gradually faded away. The ocular wall thickness in the poster part of the eyeball decreased, and the T-sign disappeared in all eyes, the average thickness was 1.53±0.41 mm. Compared with parameters before the treatment, the difference was statistically significant (t=0.003 5, P＜0.05). OCT demonstrated the recovery of the macular retinal detachment. There was no abnormal leakage evidenced in FFA in the optic disc and macular. After treatment, the value of MD and PSD was 5.19±4.82 dB and (4.33±3.76) dB, respectively. The difference of MD value between before and after the treatment was significant (t=0.026, P＜0.05).ConclusionsPosterior scleritis with an initial symptom of optic nerve was tend to affect middle-aged patients, with clinical manifestations of anterior segment signs in some patients and optic disc swelling with retinal vein dilatation in all patients. B ultrasound examination showed typical T sign. Systemic corticosteroid treatment always obtained remission of the ocular inflammatory activity, and could achieve favorable visual outcome.
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.