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 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.
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.
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.
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.
ObjectiveTo analyze the clinical features in patients of Purtscher-like retinopathy (PLR) associated with COVID-19. MethodsA retrospective clinical study. Clinical data of 4 patients (7 eyes) of PLR associated with COVID-19 which was firstly diagnosed in Department of Ophthalmology, The Second Hospital of The Army Medical University ranging from December 2022 to January 2023 were included. All patients were examined by best-corrected visual acuity (BCVA), color fundus photography, optical coherence tomography (OCT), OCT angiography, fundus fluorescein angiography (FFA), multifocal electroretinogram (mf-ERG), visual field. Oral vasodilators and neurotrophic agents were applied after definite diagnosis. Three patients were treated by intravenous dexamethasone (10 mg) for 3 days. Follow-up time spans for 4 weeks. The multi-model images, clinical features and treatment response of patients were also retrospectively studied. ResultsAmong 4 patients (7 eyes), 2 cases were male (3 eyes), 2 cases were female (4 eyes), the mean age was (36.00±17.57) years; 3 cases were unilateral, 1 case were unilateral. The time from diagnosis of COVID-19 to the onset of eye symptoms was 2 to 3 days. The BCVA of the affected eye was finger counting for 20 cm to 0.5. Color fundus photography examination revealed that several retinal whitening of varying sizes distributed diffusely on the retina (cotton-wool spots and Purtscher flecken). OCT examination showed that the retinal nerve fiber layer was significantly thickened and the reflex was enhanced in the area corresponding to the gray and white lesions, the inner nuclear layer, internal and external plexus layer segmental and banded strong reflex was observed in 5 eyes. En-face image showed mottled strong reflex in the inner retinal layer and around the deep capillary plexus (DCP). For OCTA, the signal of superficial capillaries and cotton wool spots were lost, and blood flow of deep DCP was poorly distributed. FFA examination showed that the obscured fluorescence of choroid background of Purtscher spot and capillary non-perfusion area. mf-ERG examination showed decreased amplitude of retinal a-wave and b-wave. Visual field examination showed central and paracentral scotoma. During follow-up, the BCVA of diseased eyes were raised, scotomas were shrunk, cotton wool spots and Purtscher flecken became smaller. OCT examination showed that the thickness of the original thickened retinal nerve fiber layer became thinner, the area of the strong reflex area was reduced, and the inner layer of the original segmental strong reflex area was thinner. OCTA examination revealed partial recovery of blood flow signal. ConclusionFor PLR associated with COVID-19, OCT shows thickening of retinal nerve fiber layer, segmental and banding strong reflex in part of the inner layer and inner plexus layers; OCTA is manifested by ischemia in the middle retina and DCP.
ObjectiveTo explore the clinical features, primary lesions and prognosis of optic nerve metastases.MethodsSeven patients (11 eyes) with optic nerve metastatic tumor diagnosed by the examinations of ophthalmology, laboratory and pathology in Chinese PLA General Hospital from April 2015 to September 2017 were included in this study. All patients underwent BCVA, flash VEP, OCT, orbital MRI, serum tumor marker, cerebral spinal fluid detection and PET-CT. Histopathological examination of primary or near superficial metastases was performed. The follow-up period was ranged from 16 to 44 months, with the mean of 23.0±10.9 months. The clinical characteristics, primary tumor, imaging features, treatment and clinical prognosis in the patients were analyzed.ResultsAmong 7 patients, there were 5 males and 2 females, with the mean age of 53.90±14.99 years; 3 patients with unilateral optic nerve involvement, 4 patients with bilateral optic nerve involvement; 5 patients (71.4%) first diagnosed in ophthalmology. Five patients (45.5%) were misdiagnosed as optic neuritis, optic disc edema in 6 eyes (54.5%). All of them appear loss of visual acuity, including 8 eyes (72.7%) with BCVA＜0.1, 2 eyes (18.2%) with BCVA 0.1-0.5, 1 eye (9.1%) with BCVA＞0.5. MRI results show that 1 patient with intraorbital segment, 1 patient with internal segment of optic canal, 4 patients with intracranial segment, 1 patient with intracranial segment and optic chiasma involved simultaneously, 4 patients involving surrounding tissue. There were 4 patients (57.1 %) with lung cancer, 2 patients (28.6%) with kidney cancer, 1 patient (14.3%) with gastric cance; 6 patients (85.7%) with metastasis from other sites, 2 patients with brain metastasis (1 patient with meningitis carcinomatosa). There were 2 patients (28.6%) with previous primary cancer surgery. After diagnosis, 1 patient received chemotherapy, 1 patient received radiotherapy, 5 patients gave up treatment. At the end of follow-up, 1 patient (1 eye) of chemotherapy with BCVA increased by 2 line; 1 patient (2 eyes) of radiotherapy with no change in BCVA; of the 5 patients who gave up treatment, 1 patient died of disease, 1 patient lost follow-up, and 3 patients (4 eyes) had no change in BCVA.ConclusionsWith atypically clinical manifestations, the optic nerve metastases easily misdiagnosed as optic neuritis, and with poor therapeutic effect. Primary lesions are mostly found in lung cancer.
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.
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.
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.