Objective To observe the clinical features of acute zonal occult outer retinopathy (AZOOR).Methods Eighteen patients (18 eyes) with AZOOR were enrolled in this study. All the patients were examined for corrected visual acuity, fundus examination, visual field and electroretinography (ERG). For part of these patients, fluorescein angiography (FFA), optic coherence tomography (OCT), visual evoked potential (VEP) and multifocal ERG (mf-ERG) were performed. Clinical features were observed. Comparative analysis was performed on the electrophysiological results between affected eyes and fellow eyes. The consistency of OCT, visual field and mf-ERG results was investigated.Results The patients included three males and 15 females. All patients were unilateral involved. 16 eyes (88.9%) were myopia, two eyes (11.1%) were emmetropia. 11 eyes (61.1%) have normal fundus; single yellow-white punctuate lesion or old RPE lesion near macular were found in three eyes (16.7%); depigmented zones in temporal retina were found in four eyes (22.2%). All central visual field results were abnormal, but peripheral visual field results were normal. OCT findings included irregularity, absence or breaks of the photoreceptor inner segment/outer segment (IS/OS) junction. Amplitudes of full-field ERG were reduced in the affected eyes (t=3.516，2.689，3.103，3.517，2.999，3.309；P＜0.05), and implicit times were delayed in most responses except for rod response (t=1.023,P=0.306). Amplitudes of P100 wave of visual evoked potential were reduced statistically (t=2.04,P=0.041), but the differences of implicit times between the affected eyes and the fellow eyes were not statistically significant (t=1.687,P=0.092). Amplitude reduction of multifocal ERG existed in all affected eyes. Coincidence was found between results of OCT and mf-ERG. But abnormal zones found by central visual field did not always coincide with those by mf-ERG. Conclusions The majority of patients who develop AZOOR are young women with myopia. Most patients have normal fundus. Central visual field and electrophysiological examinations are always abnormal. Irregularity, absence or breaks of the photoreceptor IS/OS junction can be proved by OCT.
Objective To explore the connection between the melanin content of retinal pigment epithelial (RPE) cells and the function of photoreceptors, and the function of melanin on retinal light damage. Methods Agematched old dopachrome tautomerase knockout (DCT-/-) mice and wildtype mice were collected as the DCT-/- group and wildtype group, with 20 mice in each group. Baseline electroretinograms (ERG) in accordance with the international standards for the clinical electrophysiology were performed on all the mice, and the max ERG was recorded. Two mice were randomly selected in each group and were executed，and the removal eyeballs were as the control. The remaining 18 mice in each group were exposed to cold fluorescent light with the quantity of electricity of 20 W for 36 hours with a circle of 12 hours light12 hours dark12 hours light, which was repeated continuously for three times. The light intensity was (5000plusmn;356) lx. Six days after the light illumination, ERG were performed again and the results were recorded. Cervical dislocation methods were used to executed 2 mice which were chosen randomly in each group, and the eyeballs were removed. The tissue sections were observed under the optical and electron microscope.Results The results of ERG showed that the amplitude of a and b wave was lower in DCT-/- group than that in wildtype group before and after light injury （a wave before light injury: t=-7.13，Plt;0.01；b wave before light injury: t=-4.414，Plt;0.01；a wave after light injury: t=-10.162，Plt;0.01；b waveafter light injury: t=-6.772，Plt;0.01）. The decrease of amplitude of a and b wave was much obvious in DCT-/- group than that in wildtype group （a wave:t=4.975,Plt;0.01；b wave:t=2.908,Plt;0.01）. After the light injury, retinal edema and thinning were found in DCT-/- group which wasobvious than that in wildtype group; the photoreceptor layers and melanin were more seriously affected in DCT-/- group than that in wild-type group.Conclusions After the light illumination, the melanin of RPE cells reduces and the function of photoreceptors decreases, which suggests that melanin may play an protective role in the light injury.
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 To observe the changes of visual acuity and multifocal electroretinogram （mfERG）before and after pars plana vitrectomy (PPV) combined with internal limiting membrane（ILM）peeling with ICG on idiopathic macular hole surgery. Methods The clinical data of 19 patients (19 eyes) with idiopathic macular hole（IMH） who had undergone PPV combined with ILM peeling assisted by 0.25% ICG from Zhongshan Ophthalmic Center were retrospectively analyzed. The closure of macular holes was confirmed by OCT two months after surgery. VERIS Science 4.9 visual evoked response image system was used to record the amplitude density of P1 waves according to six concentric rings before and two,six,twelve months after surgery, meanwhile compared and analyzed best corrected visual acuity（BCVA）before and after surgery. Results The results of OCT showed that 16 eyes (84.21%) had anatomic closure of the macular hole two months after the surgery while three eyes (15.79%) failed in the closure. The BCVA after surgery improved significantly which was more obvious at the second month (F=6.389，Plt;0.05). Compared with before surgery, the responses were depressed severely in P1 wave amplitude densities in six rings, the differences were statistically significant (t=6.140，Plt;0.05). Conclusion The postoperative BCVA of IMH patients improved but the amplitude densities of P1 wave depressed compared with before surgery.
Objective To measure the macular function of the fellow eye in patients with unilateral retinal vein occlusion (RVO). Methods A total of 24 cases of unilateral RVO were diagnosed by fundus fluorescein angiography (FFA), and multifocal ERG (mfERG) was recorded by RETI scan. The mfERG data of 24 fellow eyes of those RVO patients, and 18 normal control eyes were analyzed and compared. The parameters included the amplitude density, latency of the P1 and N1 wave in 6 concentric circles and 4 quadrants of the mfERG graphics. Results The amplitude densities of P1 and N1 wave in first and second concentric circles of RVO fellow eyes were significantly lower than normal eyes (t=4.520, 2.147; P＜0.05). There was no significant difference (P＞0.05) of P1/N1 latency in any concentric circles or quadrants between RVO fellow eyes and normal eyes. Conclusion The central fovea of the RVO fellow eyes was functionally impaired.
Objective To observe the mutifocal electroretinogram (mfERG) characteristics of rod and cone cells in patients with retinitis pigmentosa (RP) and to evaluate the function of photosensory cell.Methods The mfERG recording technique for rod cell in eight normal subjects (eight eyes) were established and the influence of different brightness lightstimulus in P1 wave amplitude were analyzed. The cone and rod cells mfERG of 38 eyes in 19 patients were recorded and then calculated positive ratio from local signalnoise ratio. The average visual acuity and P1 wave amplitude density of cone mfERG in different types were compared and statistically analyzed. Meanwhile, the changes in P1 wave amplitude of cone and rod mfERG in four quadrants also compared and analyzed. Results Rod cell mfERG in normal subjects can be recorded stably by using blue flashes with low light intensity as 0.04 cd/m2. In patients with RP, the cone and rod cells mfERG can be detectd 65.79% and 10.51% respectively. P1 wave amplitude density in type I of cone cell mfERG was significantly higher than that in type II (t=5.21,P=0.000). There were no differences in average visual acuity (t=1.15, P=0.612). P1 wave amplitude density in type I was negatively related to logMAR visual acuity （r=-0.48，P=0.04）.The comparison of rod and cone cells mfERG in local wave characteristics showed that P1 wave amplitude densities had spatial relationship in each area. Conclusions The results suggested highly variable central responses in cone cell in RP patients, higher positive recorded ratio in cone cell than rod cell and spatial correspondence between the function of reserved cone and rod cells.
Fullfield electroretinalgraphy (ffERG) is an indispensablemeans in assessment of retinal disease; it is invasive, effective, objective, quantifiable, and reproducible. Currently ffERG has been extensively applied domestically, but it also has disadvantages such as too few detected diseases, nonstandardised methodology, and inaccurate description of the results. It is important to place more emphasis on the internationalization, standardisation, and normalization of the application; obtaining the differences of indication, detection techiniques, and description of the results among ffERG, multifocal ERG and pattern ERG; expanding the new fields and methods of clinical applications. So that ffERG could play an more important role in the diagnosis and management for the patients with retinal disease.
Objective To observe the changes of photopic negative response (PhNR) of electroretinography (ERG) in patients with retinal vein occlusion (RVO). Methods A total of 30 patients (30 eyes) with retinal vein occlusion (RVO) diagnosed by indirect ophthalmoscopy and fundus fluorescein angiography (FFA) were selected; the unaffected fellow eyes of the patients and another 25 healthy agematched individuals (50 eyes) were cllected as the normal control. All of the patients underwent the examination of visual acuity, visual field, and flashERG (FERG); the normal control ones underwent FERG. In the 30 patients with RVO, there were 14 with central RVO (CRVO) and 16 with branch RVO (BRVO). According to the disease history and results of FFA, the patients were divided into 3 time groups: lt;1 month, 1-3 months, and gt;3 months; according to the types of RVO, the patients were divided into ischemic and nonischemic group. The amplitude of PhNR and other parameters were analysed. The relationship among the amplitude of PhNR and RVO types and time course were analyzed.Results The amplitude of PhNR in the CRVO and BRVO eyes was (28.20plusmn;5.8) and (36.96plusmn;4.71) mu;V, respectively; those in the unaffected fellow and control eyes was (61.25plusmn;3.93) and (59.33plusmn;16.92) mu;V, respectively; the amplitude of PhNR was significantly smaller in the CRVO and BRVO eyes than those in the unaffected fellow or control eyes (F=10.69 and 9.80，P＜0.001; F=9.69 and 9.75，P＜0.001). The amplitude of PhNR in ischemic and nonischemic group in CRVO eyes was (22.77plusmn;15.73) and (36.63plusmn;12.91) mu;V, respectively; the difference between the two groups was significant（t=6.54, Plt;0.01）. The amplitude of PhNR in ischemic and nonischemic group in BRVO eyes was (32.39plusmn;13.22) and (46.73plusmn;10.43) mu;V, respectively; there was no significant difference between the two groups（t=2.12, Plt;0.05）. The amplitude of PhNR was (24.58plusmn;14.60) and (27.94plusmn;15.73) mu;V, respectively, in CRVO and BRVO eyes with lt; 1 month disease course; was (50.39plusmn;13.80) and (58.69plusmn;12.43) mu;V in those with 1-3 months disease course; and was (25.40plusmn;19.94) and (34.48plusmn;16.72) mu;V in those with ＞3 months diseases course. Significant difference was found between the 1-3 months group and ＞3 months group in CRVO eyes（F=4.30，Plt;0.01）. Conclusions The amplitude of PhNRs was significantly smaller in RVO eyes than those in the unaffected fellow or control eyes.The amplitude of PhNR amplitude of ischemic type was smaller than that of nonischemic type. The amplitude of PhNR has descending，ascending，and descending tendency during the disease courses.