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.
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 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.
Objectives To observe the clinical characteristics of patients with posterior scleritis. Methods Ten patients with 16 eyes diagnosed as posterior scleritis were enrolled in this study. Ten patients consisted of seven males and three females. Their age ranged from 18 to 75 years old, with a mean age of 42.0plusmn;14.7 years old. Except for two patients aged 18 and 75 years old, the other eight patients aged 33 to 55 years old. Routine eye examination was performed including visual acuity, slit lamp microscope, ophthalmoscope, B scan ultrasound, color fundus photography, fundus fluorescein angiography (FFA), orbit MRI and chest Xray. According to the B scan ultrasonic examination, these ten posterior scleritis cases were divided into diffused and nodular types. Among them, the diffused type had 8 cases (14 eyes), the nodular type had 2 cases (2 eyes). The visual acuities of ten patients were from light perception to 0.4. The blood laboratory tests were negative in all cases. The diffused posterior scleritis patients received systemic and (or) local glucocorticoid therapy. The nodular posterior scleritis patients could not be ruled out choroidal melanoma in the initial evaluation, and they underwent enucleation operation in other hospitals. Results In the 14 eyes of diffused posterior scleritis, conjunctival congestion and edema were observed in 8 eyes, eyelid edema in 2 eyes, normal eye surface in 4 eyes. In the two eyes of nodular posterior scleritis, there was no conjunctival congestion and edema or eyelids swelling. All 16 eyes performed FFA, 11 eyes had fundus posterior pole early dot-like hyperfluorescence followed by leakage of fluorescence in the late stage, two eyes had hyperfluorescence mixed with hypofluorescence in the nearby retina of the lesion, and the rest three eyes had no abnormality in FFA. In 14 eyes of diffused type posterior scleritis, B scan ultrasound showed diffused scleral thickening more than 2 mm in 10 eyes with a typical quot;Tquot; shape sign. The other 4 eyes did not show typical sign in ultrasound. In 2 eyes of nodular type, ultrasound showed nodular enhanced echo in choroid with medium internal reflection and abundant blood flow. Eleven eyes underwent orbit MRI scan, 9 eyes displayed diffused posterior sclera thickening, 2 eyes showed nodular lesions in choroid. All lesions showed low signal on T1WI, high signal on T2WI. After treatment, six diffused posterior scleritis patients recovered to normal scleral thickness. Two nodular posterior scleritis patients underwent enucleation showed granulomatous posterior scleritis and necrotic posterior scleritis in pathology. Overall, 16 eyes of 10 patients were misdiagnosed as chronic angleclosure glaucoma in 2 eyes, acute iridocyclitis in 1 eye, central serous chorioretinopathy in 2 eyes, retrobulbar neuritis in 2 eyes, and choroidal melanoma in 2 eyes. Conclusions Posterior scleritis occurs mostly in young patients. The diffused posterior scleritis patients usually has anterior segment signs including conjunctival congestion and edema or eyelids swelling, while the nodular posterior scleritis patients has normal anterior segment signs. B scan ultrasonic and MRI examination showed typical image features. Systemic and (or) local glucocorticoid therapy can effectively release the symptoms of these patients.
Objective To observe the hereditary types and clinical characteristics of 137 patients with retinitis pigmentosa (RP) in Ningxia. Methods One hundred and thirty-seven patients with RP who diagnosed by the examinations of visual acuity, optometry, direct or indirect ophthalmoscope, visual field, optical coherence tomography (OCT) and electroretinogram were enrolled. The hereditary types and clinical characteristics were analyzed according to the family history and the Results of ophthalmologic examinations. Results One hundred and thirty-seven patients included 29 autosomal dominant RP (ADRP) patients from 8 families (7.4%), 16 autosomal recessive RP (ARRP) patients from 15 families (13.9%), 10 X-linked RP (XLRP) from 3 families (2.8%), and 82 simplex RP (SRP) patients (75.9%). There were 15 consanguineous marriage families out of 26 families with RP history (57.7%). The patients were classified as typical RP (102 patients, 74.5%) and atypical RP (35 patients, 25.5%). All the ADRP and XLRP patients showed typical clinical features of RP. Ten (62.5%) of ARRP patients and 53 (64.6%) of SRP patients had typical features of RP. Six (37.5%) of ARRP patients and 29 (35.4%) of SRP patients had atypical features of RP. Among atypical RP patients, 17 (48.6%) patients were nonpigmented RP which including 3 patients were misdiagnosed as amblyopia during childhood. The logarithm of minimal angle of resolution (logMAR) best corrected visual acuity (BCVA) of ADRP patients was 1.04plusmn;0.51 at the age older than 51 years, while the BCVA of ARRP and XLRP patients were 0.92plusmn;0.61 and 1.70plusmn;0.02 respectively at 21 to 30 years of age. One hundred and twentythree (89.8%) patients suffered from varying degrees of myopia. OCT showed that the average thickness of macular fovea in ADRP patients was (185.73plusmn;1.23) mu;m at the age older than 51 years, while in ARRP and XLRP patients were (173.21plusmn;0.98) and (170.49plusmn;1.15) mu;m respectively at 21 to 30 years of age. Conclusions ADRP and XLRP are typical RP. All atypical RP are ARRP and SRP. Non-pigmented RP are mainly seen in atypical RP which often misdiagnosed as amblyopia during childhood. The photoreceptors in macula are damaged in the early stage and the decline of visual acuity occurred at 21 to 30 years of age in patients with ARRP and XLRP. The ADRP patients has late slower decline of visual acuity and retain some visual acuity at the age older than 51 years.
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 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 observe and analyze the clinical features, treatment methods and efficacy of patients with retinopathy associated with incontinentia pigmenti (IP).MethodsA retrospective case study. Twelve clinical confirmed IP patients (24 eyes) in Zhongshan Ophthalmic Center of Sun Yat-sen University from January 2015 to December 2018 were included in this study. The best corrected visual acuity and intraocular pressure examination were performed in patients (＞4 years old). All patients were examined on the anterior segment, vitreous body, and fundus under topical anesthesia or general anesthesia. Eight cases underwent genetic testing. Patients with active disease should be given anti-vascular endothelial growth factor (VEGF) drug treatment, retinal laser photocoagulation or vitrectomy, those without active disease should be observed. All patients were followed up for 1 to 3 months, with an average follow-up time of 18.7 months.ResultsAll patients were all female, with an average age of 6.3±9.8 years old at the first ophthalmology visit. According to the recommendations of the pediatrician, 3 cases were actively screened for ophthalmology (referrals), with an average age of 0.4±0.5 years (median age: 2 months). A total of 9 cases were not recommended for referrals (non-referrals), including 3 cases of ophthalmology who were diagnosed for the first time due to visual impairment, and 6 cases of undiagnosed IP before the ophthalmology visit, the average age of their first visit was 8.2±10.8 years (medium age: 3 years old). The age of the first visit for non-referred patients was larger than that of referrals, and the difference was statistically significant (Z=−2.141, P=0.036). Among the 24 eyes of 12 cases, there were no obvious fundus abnormalities in 1 case or 2 eyes, 11 cases of IP-related retinopathy in 22 eyes (91.7%, 22/24), 8 cases of binocular asymmetry (66.7%, 8/12). There were active lesions on the fundus in 7 eyes (29.2%, 7/24). Patients underwent simple retinal laser photocoagulation and/or anti-VEGF drug therapy. During the follow-up, retinal neovascularization recurred in 1 eye. Among the 8 cases that underwent genetic testing, 3 cases (37.5%, 3/8) were deleted in exons 4-10 of the IKBKG gene.ConclusionsIP is more common in women. IP-associated retinopathy is noted with early-onset, asymmetrical retinopathy, which is identified with retinal neovascularization and vitreous proliferation. Early detection and timely treatment are essential.
Objective To observe the clinical features of systemic lupus erythematosus (SLE) with retinopathy.Methods Ninety-seven SLE patients were enrolled in this comparative clinical study. The patients were divided into retinopathy group (positive group, 32 eyes of 23 patients) and non-retinopathy group (negative group, 148 eyes of 74 patients). The age, course of disease, clinical features, laboratory results in these two groups were comparatively analyzed.Results The positive rate of retinopathy in all SLE patients was 23.7%. Seventeen patients (22 eyes, 73.9%) of positive group had retinal cotton-wool spot, retinal hemorrhage, tortuous retinal vein, retinal arterial spasm, microaneurysm and hard exudates. The other six patients (10 eyes, 26.1%) in this group showed retinal main vessel occlusion. The incidence rate of rash, cutaneous vasculitis, elevated erythrocyte sedimentation rate (ESR), decreased complement C3 and positive anti double stranded-DNA (anti-ds-DNA) antibody in the positive group were higher than those in the negative group (chi;2=9.206, 6.987, 7.824, 8.581, 6.599;P＜0.05). There was no significant difference between these two groups in age, course of disease, mucosal ulcers, arthritis, fever, headache, neutropenia, thrombocytopenia, proteinuria, elevated blood urea nitrogen, increased creatinine, positive antinuclear (ANA) and anti-Sm antibodies (t=0.321, 0.063;chi;2=0.135, 0.046, 0.176, 0.002, 0.036, 0.113, 0.053,0.032,0.012,0.000,0.004;P＞0.05). Conclusions Tortuous retinal veins, retinal cotton-wool spots and retinal main vessels occlusion are the three major fundus features of SLE patient with retinopathy. Rash, cutaneous vasculitis, increased ESR, decreased complement C3 and positive anti-ds-DNA antibody are the five major systemic clinical features of SLE patient with retinopathy.