Objectives To observe the clinical characteristics of Leber congenital amaurosis (LCA) and analyze the features to differentiate LCA from other similar disorders.Methods Prospective study of 15 LCA patients which include 10 males and 5 females, aged from 2 to 31 years with the mean age 13.6 years. Medical history, family history, perinatal conditions, as well as complete ocular evaluations were well documented. Among the subjects, 12 patients underwent optometry check, 10 patients underwent ERG and 8 patients had OCT testing. Results All of the patients had nystagmus and sluggish pupillary reflex. The visual acuity distributed from light perception to 0.1. Fivepatients (33.3%) were presented with photophobia, while 7 patients (46.7%) had nyctalopia. Among 12 cases underwent refraction examination, 6 patients (50%) had spherical equivalent of ge;+5D；1 patient（8.3%）had spherical equivalent of ge;+5D; 2 patients (16.7%) had bilateral mild to moderate hyperopia；1 patient (8.3%) had one emmertropic eye and one mild myopic eye; 2 patients (16.7%) had moderate to high myopia. Eight patients (53.3%) had enophthalmus,4 patients (26.7%) had oculodigital sign. All of the 10 patients underwent ERG showed extinguished waveform. Under OCT assesement, 7 patients had decreased fovea thickness; 1 patient had increased fovea thickness, complicated by epiretinal membrane; mild abnormality of microstructure of the retina with diminished and disrupted IS/OS hyperreflectivity were found in 2 cases；while more pronounced disarrangement of the retinal layers were found in 6 cases，inner layers were better reserved in all patients.Conclusions Severe visual impairment or blindness, nystagmus, pupillary reflex, extinguished ERG and hyperopia are main clinical characteristics of LCA.
Objective Toinvestigate the influence of photocoagulation on macular function and morphous in patients with diabetic retinopathy (DR).Methods Forty eyes of thirty patients with severe nonproliferative diabetic retinopathy (NPDR) were examined by multifocal electroretinogram (mfERG) and optical coherence tomography (OCT) before and 2,7, and 14 days after photocoagulation. The results were statistically analyzed by using analysis of variance and t test; the changes of macular function and macular fovea thickness were detected and observed.Results P1 response densities of ring 1,3,and 5 were 131.79plusmn;50.92，37.50plusmn;17.27，24.07plusmn;11.49，respectively,2 days after photocoagulation; and were 212.96plusmn;53.75，46.70plusmn;15.89，and 30.91plusmn;10.78, respectively, before photocoagulation. The densities before and after photocoagulation differed much（t=7.910, 2.174, 2.205; Plt;0.05）. N1 response density of ring 4 was（60.39plusmn;20.69） and the prephotocoagulation corresponding response density was （107.11plusmn;44.63）; the difference was significant（t=5.375，Plt;0.01）. The latency of P1 of ring 4 was（41.83plusmn;3.41），which had significant statistically difference（t=-2.770，Plt;0.05) with that before photocoagulation（39.52plusmn;2.64); there was no significant changes in the latency of N1 (Pgt;0.05). The most significant changes of P1 and N1 response densities occurred in the central macular 5deg; area. Seven days after photocoagulation, the response density of P1 and N1 in the central macular 5deg; area seemed to be recoverd to some extend and increased to （179.70plusmn;47.10）and (81.11plusmn;34.18) respectively until 14 days after photocoagulation, which was still much lower than that before the photocoagulation（t=3.840, 2.746; P＜0.05）; the response densities of other areas had no significant differences (P＞0.05). Seven days after photocoagulation,the latency of P1 in ring 4 was delayed to（41.78plusmn;3.57）, which had significant difference（t=-3.144，P＜0.01）with that before the photocoagulation（39.52plusmn;2.64) ; but there was no significant difference between 14 days after photocoagulation and prephotocoagulation (t=-1.809，P＞0.05). The latency of N1 in ring 1 was（20.67plusmn;3.85）at seven days after photocoagulation, It had no significant difference (t=-1.171,P＞0.05) with that before the phtocoaguation（18.78plusmn;3.29）. Before and 2 days after photocoagulation, the macular fovea thickness were（224.42plusmn;122.88）and（274.85plusmn;108.20）respectively, and the difference was statistically significant（t=-2.420，P＜0.05）. Forteen days after photocoagulation，the macular fovea thickness was（236.29plusmn;70.45），It had no significant difference with that before the photocoagulation（t=-0.578，P＞0.05）. Before and seven days after photocoagulation, P1 response density had obvious negative correlation with corresponding macular fovea thickness（r=-0.755，Plt;0.01; r=-0.594，Plt;0.05）. Conclusions After photocoagulation in patients with DR，the macular function decreased in a certain degree，and the relationship of macular retinal function and macular morphology changes was close; combination of mfERG and OCT can evaluate macular function and macular morphology structure comprehensively and objectively.
ObjectiveTo evaluate Micron Ⅳ retinal imaging system in three mouse models of retinal diseases. MethodsMouse models of oxygen induced retinopathy (OIR) model (OIR group), N-methyl-N nitrosourea (MNU) model (MNU group) and N-methyl-D-aspartate (NMDA) model (NMDA group) were induced in 24 healthy male C57BL/6J mice. Fundus photograph, fundus fluorescein angiography (FFA) and optical coherence tomography (OCT) and electroretinogram (ERG) were used to evaluate these mice. All the imaging examinations were performed by Micron Ⅳ retinal imaging system. ResultsOIR mice showed tortuous and dilated retinal vessels in fundus photograph, neovascularization plexus and vascular leakage in FFA, and epiretinal fibrovascular tissue and tortuous expansion vascular vessels in OCT. MNU mice showed wax yellow optic disk without retinal pigmentary changes, slight thinning of retinal blood vessels in FFA, and normal structure and thickness in OCT. The a-wave amplitudes of the maximum mixed response decreased significantly, and were (15.38±4.36) μV and (13.78±5.52) μV at 2 or 3 days of modeling, respectively. NMDA mice showed a pale retina with vasospasm. ERG revealed that there was no obvious change in latency of a- and b-wave, but significantly decreased amplitude of b-wave at 12 hours and 24 hours after modeling with (72.28±7.18) μV and (65.35±9.18) μV, respectively. ConclusionMicron Ⅳ retinal imaging system is a real-time, non-invasive tool to study the retinal structure and function in animal models of retinal diseases.
Fundus photograph, angiography, optical coherence tomography, ultrasonography and other image technology and visual electrophysiology can provide a wealth of information for the diagnosis and treatment of pediatric retinal diseases. However, it put forward higher requirements on pediatric retinal imaging equipment and techniques which will be quite different from adult, because of pediatric retinal disease has its own characteristics, such as disease spectrum, pathogenesis, and pathophysiology. The principles and methods of image results interpretation on adult were not quite ready for children. It is necessary to further study the fundus imaging techniques suitable for children, gradually establish standardized examination procedures and clinical interpretation system, to promote the diagnosis of retinopathy in children.
Objective To observe the macular morphology and circumpapillary retinal nerve fiber layer thickness (RNFL) in Parkinson's disease (PD) evaluated by spectral-domain optical coherence tomography (SD-OCT). Methods A total of 37 patients (74 eyes) with PD were in the PD group, 32 age- and sex-matched healthy subjects (64 eyes) in the control group. All subjects underwent SD-OCT examination with 5 line scanning, macular cube 512×128 scanning and optic disc volume 200×200 scanning. The retinal thickness, central foveal thickness (CFT), macular volume and thickness of circumpapillary, superior, inferior, nasal, and temporal of RNFL between two groups were comparatively analyzed. The relationship between SD-OCT parameters and age, disease duration, scores of Hoehn-Yahr and unified PD rating scale (UPDRS) Ⅲ in PD patients was analyzed by Pearson correlation analysis. Results Both of the retinal thickness and macular volume in PD group were significantly reduced than those in control group (t= −2.546, −3.410;P=0.012, 0.001). There was no difference of CFT (t= −0.463,P=0.644) and the thickness of circumpapillary (t= −1.645,P=0.102), superior (t= −0.775,P=0.439), inferior (t=−1.844,P=0.067), nasal (t= −0.344,P=0.732) and temporal (t= −0.541,P=0.590) of RNFL between two groups. The retinal thickness, macular volume, CFT and the thickness of circumpapillary, superior, inferior, nasal, temporal of RNFL had no relationship with age, disease duration and scores of Hoehn-Yahr and UPDRS Ⅲ in PD patients (P＞0.05). Conclusions In PD patients, the retinal thickness and macular volume are decreased, however, the circumpapillary RNFL have no obvious alterations.
ObjectiveTo observe the macular retinal thickness and volume in patients with different degrees of Parkinson's disease (PD).MethodsThirty eyes of 30 patients with primary PD and 20 eyes of 20 healthy subjects (control group) in Xuanwu Hospital of Capital Medical University from October 2016 to October 2017 were enrolled in this study. There were 17 males and 13 females, with the mean age of 63.2±6.4 years and disease course of 3.9±2.4 years. The patients were divided into mild to moderate PD group (15 eyes of 15 patients) and severe PD group (15 eyes of 15 patients). The macular area was automatically divided into 3 concentric circles by software, which were foveal area with a diameter of 1 mm (inner ring), middle ring of 1 to 3 mm, and outer ring of 3 to 6 mm. The middle and outer ring were divided into 4 quadrants by 2 radiations, respectively. The changes of retinal thickness and macular volume of the macular center and its surrounding quadrants were analyzed. SPSS 16.0 software was used for statistical analysis. One-way ANOVA were used to analyze all data.ResultsCompared with the control group, the retinal thickness and volume in macular center and each quadrant of the mild to moderate PD group and severe PD group were reduced. Compared with the mild to moderate PD group, the retinal thickness and volume in macular center and each quadrant of the severe PD group were reduced. The differences of retinal thickness and macular volume among 3 groups were significant (F=5.794, 5.221, 5.586, 5.302, 5.926, 5.319, 5.404, 5.261, 5.603; P=0.001, 0.007, 0.003, 0.005, 0.000, 0.004, 0.004, 0.006, 0.002). In inner ring of the mild to moderate PD group and the severe PD group, the retinal thickness and macular volume in the upper and the nasal were the largest, the inferior was followed, and the temporal was the smallest. In outer ring of the mild to moderate PD group and the severe PD group, the retinal thickness and macular volume in the nasal was the largest, the upper was the second, the temporal and the inferior were the smallest.ConclusionsThe retinal thickness and volume of the macular central fovea and its surrounding areas in PD patients are significantly thinner than that in the healthy subjects. And with the increase of the severity of PD, the macular structure changes obviously, showing macular center and its surrounding macular degeneration thin, macular volume reduced.