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.
ObjectiveTo observe the blood perfusion changes of peripapillary and macular vessels in patients with nonarteritic anterior ischaemic optic neuropathy (NAION).MethodsRetrospective cohort study. Thirty-six eyes (19 affected eyes and 17 fellow eyes) of 19 patients with NAION diagnosed in People’s Hospital of Wuhan University from November 2017 to January 2019 were included in this study. There were 10 males and 9 females, with the mean age of 55.05±7.11 years. Forty eyes of 20 normal subjects matched with NAION patients were included as controls. BCVA, fundus color photography, SD-OCT and OCT angiography were performed in normal controls and repeated in NAION affected eyes at 1-2 weeks, 1-2 months, 3-5 months intervals. OCT quantitative measurements: average retinal nerve fiber layer thickness (aRNFL) of the disc and its superior values (sRNFL) and the inferior values (iRNFL), average ganglion cell complex thickness (aGCC) in macular region and its superior values (sGCC) and the inferior values (iGCC). OCTA quantitative measurements: average radial peripapillary capillary density (aRPC) and its superior values (sRPC) and the inferior values (iRPC), average vascular density of superficial retina (aSVD) in macular region and its superior values (sSVD) and the inferior values (iSVD), average vascular density of deep layer retina (aDVD), areas of foveal avascular zone (FAZ). The differences of OCT and OCTA quantitative measurements between NAION eyes and the fellow eyes and normal controls were comparatively analyzed. Independent sample t test, paired sample t test or nonparametric rank sum test were performed for comparison among three groups. Pearson or Spearman correlation analysis were used to analyze the correlation between RNFL and RPC, GCC and SVD, RNFL and GCC, RPC and SVD.ResultsAt baseline, the aRNFL, aRPC and aDVD of NAION patients were significantly higher than those of normal controls. Compared with the fellow eyes, the aRNFL increased significantly and the aRPC decreased significantly in NAION affected eyes. The overall differences of aRNFL, aRPC, aGCC and aSVD at four intervals within NAION affected eyes were statistically significant (P＜0.05). The average sRNFL, sRPC, sGCC and sSVD at 1-2 months interval were significantly lower than the average iRNFL, iRPC, iGCC and iSVD (P＜0.05). Correlation analysis: at 1-2 months interval, aGCC was positively correlated with aSVD (r=0.482, P=0.037); at 3-5 months interval, aRNFL was positively correlated with aRPC (r=0.631, P=0.037).ConclusionThere is a sectorial reduction of vascular density of peripapillary RPC and macular SVD with the disease progression of NAION.
ObjectiveTo quantitatively analyze the changes of choroidal capillaries in chronic central serous chorioretinopathy (CCSC) before and after half-dose photodynamic therapy (PDT).MethodsA retrospective cohort study. Nineteen patients (21 eyes) with CCSC were enrolled in this study from November 2017 to September 2018 in People’s Hospital of Wuhan University. Among them, there were 14 males (15 eyes) and 5 females (6 eyes), with diseases course over than 6 months. All patients underwent half-dose PDT. Twenty normal subjects (40 eyes) matched with age and sex in CCSC group were taken as controls. The subfoveal choroidal thickness (SFCT) was measured by Heidelberg depth enhanced imaging-OCT before and after PDT treatment in CCSC patients and in normal subjects. Spectral-domain OCT (Retina map) and Angio-OCT angiography (3 mm×3 mm) were arranged for all subjects at the same time. Macular fovea retinal thickness (CMT) was recorded under OCT-Retina map mode, and Angio-OCT 3 mm×3 mm choroidal capillary images were binarized using Image J software, and calculating the area ratio of low pixel area as flow signal void (FSV). BCVA, spectral-domain OCT and Angio-OCT were performed 1 week and 1, 3 months after PDT with the same equipment and methods before PDT. The changes of CMT, SFCT, FSV and BCVA in CCSC patients before and after PDT treatment were compared. Pearson correlation analysis was used to analyze the correlation between FSV and SFCT, age.ResultsThe average CMT, SFCT and FSV in CCSC patients increased significantly compared with the controls (P＜0.05). The average SFCT and FSV in CCSC patients 3 months after treatment were higher and the average CMT decreased compared with the controls (P=0.000, 0.000, 0.000). Comparison before and after PDT in CCSC patients: there were significant differences in average CMT, SFCT and FSV before and after PDT (P=0.000, 0.000, 0.000). Post Hoc multiple comparisons showed that the average CMT (P=0.000, 0.000, 0.000, 0.000, 0.000) and FSV (P=0.010, 0.000, 0.000, 0.001, 0.000) decreased significantly in all time points except for 1 month and 3 months after treatment, so as the average FSV (P=0.788, 0.702). The average SFCT decreased 1 month and 3 months after treatment compared with the baseline (P=0.024, 0.008), and there was no significant difference between before treatment and 1 week after treatment (P=0.162), and between 1 month and 3 months after treatment (P=0.687). The correlation analysis showed that there was no correlation between FSV and age in CCSC patients (r=0.052, P=0.822), but there was a correlation between FSV and age in controls (r=0.716, P=0.000).ConclusionQuantitative analysis of OCTA showes the degree of choriocapillary ischemia in the form of FSV in CCSC patients decreased after PDT treatment, however, which is still higher than normal controls.
ObjectiveTo observe the changes of macular vascular density of hypertensive patients without obvious hypertensive retinopathy (HRP).MethodsTwenty-three patients (hypertension group) diagnosed as grade 2 or grade 3 essential hypertension in Cardiology Department of Renmin Hospital of Wuhan University from January to April 2019 were enrolled in the study. Among them, there were 13 males and 10 females. The mean age was 61.6 ± 5.6 years, and the mean BCVA was 0.74 ± 0.16. The course of hypertension was more than 7 years; Keith Wagener (K-W) grade was 0 or 1. Fifteen age-matched people without hypertension were selected as the control group, among which included 8 males and 7 females. Their average age was 59.7 ± 4.4 years and the average BCVA was 0.79 ± 0.17, the K-W grades were 0. There was no significant difference (P=0.265, 1.000, 0.563) between the two groups in age (t=1.739), sex ratio (χ2=0.036) or BCVA (t=0.585). All subjects were examined by BCVA, fundus photography and OCTA. OCTA scanned the macular area in the range of 3 mm × 3 mm. The software automatically divided the image into two concentric circles with the macular fovea as the center, which are the inner ring with a diameter of 1 mm (foveal area) and the outer ring with a diameter of 1-3 mm. The blood flow density of the whole, temporal, upper, nasal and lower capillary layers within 3 mm of the macular area, and foveal avascular zone (FAZ) area, central foveal retinal thickness (CFT) were measured.ResultsSignificant differences were observed in the vascular densities of total, temporal, nasal and inferior area of maculas (t=2.188, 2.472, 5.105, 2.734; P=0.037, 0.020, 0.000, 0.010) between the two groups, while no significant differences were evidenced in foveal vascular densities and superior vascular densities (t=0.575, 0.140; P=0.570, 0.889). There was no significant difference in FAZ area or CFT between the two groups (t=0.367, 0.753; P=0.714, 0.457). Macular arches were intact in all hypertension patients.ConclusionsThe vascular densities of total, temporal, nasal and inferior area of maculas in the hypertension patients without HRP decreased. The area of FAZ did not expand, and the structures of macular arch ring were normal.
ObjectiveTo assess changes of blood flow density of idiopathic choroidal neovascularization (ICNV) treated with intravitreal anti-vascular endothelial growth factor (anti-VEGF).MethodsRetrospective case analysis. Sixteen eyes of 16 patients with ICNV diagnosed with FFA and OCT were included in this study. Among them, 12 were female and 4 were male. The mean age was 33.94±9.83 years. The mean course of diseases was 5.13±4.44 weeks. The BCVA, indirect ophthalmoscope, OCT and OCT angiography (OCTA) were performed at the first diagnosis in all patients. The BCVA was converted to logMAR. The macular fovea retinal thickness (CMT) was measured by OCT, and the selected area of CNV (CSA) and flow area of CNV (CFA) were measured by OCTA. The mean logMAR BCVA, CMT, CSA and CFA were 0.336±0.163, 268.500±57.927 μm, 0.651±0.521 mm2, 0.327±0.278 mm2 , respectively. All patients were treated with intravitreal ranibizumab (IVR, 10 mg/ml, 0.05 ml). Follow-up results including the BCVA, fundus color photography, OCT and OCTA were obtained 1 month after treatment. To compare the changes of BCVA, CMT, CSA, CFA of ICNV treated with anti-VEGF. Pearson method was used to analyze the correlation between logMAR BCVA and CMT, CSA and CFA before and after the treatment.ResultsOne month after treatment, the average logMAR BCVA, CMT, CSA and CFA were 0.176±0.111, 232.500±18.910 μm, 0.420±0.439 mm2, 0.215±0.274 mm2. The mean logMAR BCVA (t=5.471, P＜0.001), CMT (t=2.527, P=0.023), CSA (t=4.039, P=0.001), CFA (t=4.214, P=0.001) significantly decreased at 1 month after injection compared to baseline, and the difference had statistical significance. The results of correlation analysis showed that the post-logMAR BCVA was moderately positively correlated with pre-CSA and post-CSA (r=0.553, 0.560; P=0.026, 0.024), and strongly correlated with pre-CFA and post-CFA (r=0.669, 0.606; P=0.005, 0.013), but not correlated with pre-CMT and post-CMT (r=0.553, 0.560; P=0.026, 0.024).ConclusionThe blood flow density of ICNV measured by OCTA were significantly decreased in the treatment of anti-VEGF drugs.
ObjectiveTo observe the imaging features of cystoid macular edema (CME) in multicolor imaging (MC), and to evaluate the value of MC in the diagnosis of CME.MethodsDescriptive case series study. From August 2017 to June 2018, 42 eyes of 37 patients with CME diagnosed in the people's Hospital of Wuhan University were included in the study. Among them, there were 24 males and 13 females, with an average age of 48.51±10.29 years. There were 14 eyes with diabetic retinopathy, 14 eyes with central retinal vein occlusion, 8 eyes with branch retinal vein occlusion, 4 eyes with uveitis, and 2 eyes with Eales disease. The macular color fundus photography (CFP) was performed with Visucam 200 non-mydriatic fundus camera of Zeiss company in Germany. MC, frequnce domainoptical OCT (SD-OCT) and FFA were examined by Spectralis HRA2 + OCT of Heidelberg company in Germany. According to the MC standard method, five images, including 488 nm blue reflection (BR), 515 nm green reflection (GR), 820 nm infrared reflection (IR) imaging and standard MC and blue-green enhancement (BG), were obtained at the same time. Compared with SD-OCT, CFP and MC images were scored. Friedman M test and Wilcoxon signed rank test were used for statistical analysis.ResultsThe standard MC and BG images showed blue-green uplift area or petal-shaped appearance, surrounded by green reflection areas with clear boundaries. BR image can be seen in the low reflexes area. On the GR image, there were patches or cystic low reflection areas, surrounded by a slightly high reflection. On the IR image, patches or cystoid high reflexes can be seen, surrounded by low reflection dark areas with clear boundaries. The average scores of CFP, standard MC, GB, IR, GR and BR were 1.20±0.94, 3.05±0.99, 2.90±1.04, 2.55±1.27, 2.00±0.94, 0.51±0.85 respectively, and the differences were statistically significant (χ2= 151.61, P=0.000). The score of CFP were significantly lower than that of standard MC (Z=－5.421), BG (Z=－5.354), IR (Z=－4.714), GR (Z=－4.438) and higher than that of BR (Z=－3.435). The differences were statistically significant (P=0.000, 0.000, 0.000, 0.000, 0.001).ConclusionsThe quality of MC imaging is better than that of CFP. Combined with SD-OCT, it can be used as an assistant method to diagnose CME.