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Objective To observe the clinical features of late-onset cone dystrophy (LOCD).Methods Eleven patients (15 eyes) of LOCD were enrolled in this study.The patients included 7 males and 4 females.The age was ranged from 50 to 79 years,with a mean age of 60.2 years.There was no obvious photophobia and hemeralopia.The visual acuity was less than or equal to 0.05 in 4 eyes,0.06-0.2 in 5 eyes,0.3-1.0 in 6 eyes.Visual acuity,slit lamp microscope,indirect ophthalmoscopy,flash electroretinogram (FERG) and multifocal electroretinograms (mfERG) were examined for all patients,fundus fluorescein angiography (FFA) for 11 eyes,optical coherence tomography (OCT) and chromoptometry for 6 eyes.Results There were 6 eyes with red/green color blindness,2 eyes with color weakness.Normal fundus was found in 11 eyes,while derangement of macular pigment epithelial in 4 eyes.FFA results showed that there were 5 eyes with normal fundus,4 eyes with blocked fluorescent spots,2 eyes with oval macular atrophy.FERG results showed that in cone response,the amplitude was lower in 6 eyes (including mild decrease in 4 eyes,moderate decrease in 1 eye and severe decrease in 1 eye) ; both in cone and rod response,the amplitude were lower in 9 eyes.mfERG results showed that central part of the cone (less than 7 degree from the center) was damaged in 5 eyes,both central and peripheral part (outside of 7 degree) of the cone were damaged in 10 eyes.OCT results showed that pigment derangement in 3 eyes,fovea was normal in 8 eyes,thinned in 5 eyes (foveal thickness was 83-111 μm).Conclusions The fundus manifestations of LOCD patients are variable,from normal fundus to oval macular atrophy.FERG is abnormal,which mainly in cone response at early stage and both in cone and rod response at late stage.Central part and (or) peripheral part of the cone are abnormal by mfERG.
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Objective To investigate the optical coherence tomography (OCT) characteristics of traumatic maculopathy. Design Retrospective case series. Participants 477 patients (486 eyes) with traumatic maculopathy, who aged from 4 years to 76 years. Method The clinical and OCT data of patients from September 2002 to June 2009 in Beijing Tongren Hospital were reviewed. Main outcome measures Features of the OCT images. Results The major findings by OCT in traumatic maculopathy included: macular hole, sensory retinal detachment, macular hemorrhage, epimacular membrane, choroidal rupture, sensory retinal atrophy, retinal pigment ep-ithelium (RPE) and choroid atrophy. In the early stage after trauma, the common findings with OCT are atrophy of RPE(49.0%), macular hole(24.7%), sensory retinal detachment(26.3%),macular hemorrhage(24.2%) and macular edema(19.2%); in the middle-late stage, atro-phy of RPE (63.0%)and atrophy of sensory retina (36.5%) are the most common changes revealed with OCT. Conclusions OCT is a useful diagnostic modality for imaging traumatic maculopathy. Diverse changes of retina and choroid are usually coexisting by OCT. At-rophy of RPE is the most common change throughout the course. In the early stage, macular hole, sensory retinal detachment, macular hemorrhage and edema are the common changes. In the middle-late stage, atrophy of sensory retina and/or RPE is the dominating change. (Ophthalmol CHN, 2009, 18: 236-238)
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<p><b>INTRODUCTION</b>In this paper, we report a novel VMD2 gene mutation in a Chinese family with Best vitelliform macular dystrophy.</p><p><b>MATERIALS AND METHODS</b>Ophthalmologic examination and optical coherence tomography (OCT) were performed in 2 members of this family. Mutational screening was performed by single-strand conformation polymorphism (SSCP) and direct sequencing of PCR-amplified DNA fragments, corresponding to the 11 exons of the gene.</p><p><b>RESULTS</b>Sequence analysis identified a previously unreported C to G change, predicting a Phe-113-Leu substitution. Both the proband and his sister harboured this novel mutation. Each had bilateral vitelliform lesions.</p><p><b>CONCLUSIONS</b>A novel mutation in the VMD2 gene (C427G) was found in Chinese patients with Best vitelliform macular dystrophy.</p>
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Adult , Female , Humans , Male , Bestrophins , China , Chloride Channels , Eye Proteins , Genetics , Macular Degeneration , Genetics , Mutation , PedigreeABSTRACT
Objective To observe the clinical features of polypoidal choroidal vasculopathy (PCV) in Chinese patients. Methods Nine cases (9 eyes) were examined with fundus examination, fundus fluorescein angiography (FFA) and indocyanine green angiography angiography (ICGA). Results FFA and ICGA showed the branching vascular networks (7 cases) and polyplike dilation at terminals of branches (9 cases), which mainly located in macular area (8 cases) and in peripapillary area (1 case), and which accompanied hemorrhagic or serous pigment epithelial detachment in 7 cases,and 4 of 7 cases had a significant horizontal black-white damarcation line. It definitely differed from fine choroidal neo-vascularization (CNV). Conclusion PCV in Chinese patients has the cardinal clinical features, i.e., polyplike lesions located mainly in macular area and most cases accompanied by hemorrhagic or serous pigment epithelium detachment.
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Objective To evaluated the effect of transpupillary thermotherapy (TTT) on age related macular degeneration (AMD). Methods Sixty two cases (62 eyes) of exudative AMD were managed with TTT. Before treatment, 58 cases underwent fundus fluorescein angiography(FFA),42 cases underwent simultaneous indocyanine green angiography (ICGA), and 56 cases underwent optic coherence tomography (OCT).TTT was delivered using a 810 nm diode laser with variable spot sizes 0.5 3.0 mm and power range 60 40 mW,60 seconds duration. Sixty two cases were followed up for 1 10 months with 4 8 months average. Results The visual acuities of last visit were compared with those before the treatment. The visual acuity was unchanged in 43 cases (69.3%), improved in 15 cases (24.2%), and declined in 4 cases (6.5%). OCT was re done in 51 cases and compared with OCT images before TTT treatment. The height of macular edema was unchanged in 29 cases (56.9%), decreased in 18 cases (35.3%), and increased in 4 cases (7.8%). The amelioration of visual acuity was compatible with that of macular configuration in the majority of cases (74.5%). Only in 13 cases (25.5%) the amelioration of visual acuity lagged behind that of macular configuration. The re treatment was performed in 18 cases (29.1%), probably due to insufficiency of laser power. No side effect was found. Conclusion TTT makes most of the cases of exudative AMD retaining or improving their visual acuity. The employment is secured. Further exploration is needed in order to obtain the parameters of the laser treatment.
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Objective To evaluate the clinical effect of transpupillary thermotherapy (TTT) on circumscribed choroidal hemangioma (CCH). Methods The clinical data of 12 eyes of 12 patients with CCH treated with TTT were retrospectively analysed. Infrared diode laser Iridex, oculight SLX was used in TTT at 810 nm and power between 220 and 1 000 mW with a beam diameter of 1.2 or 2 0 or 3 0 mm, with 1 to 2 minutes of exposure time. The visual acuity , subretinal fluid , complication ,thickness and hyperfluorescence of CCH were observed pre and postoperatively in the treated eyes. The average period of follow up was 10 months (6 16 months). Results Among the 8 eyes with peripheral retinal detachment in 12 cases of CCH the peripheral subretinal fluid was completely absorbed in 6 eyes, and partially absorbed in 2 eyes after TTT treatment. In 8 patients undergone ultrasonography, the mean value of tumor thickness went down by 21.75 % in 7 eyes. The resultant visual acuity after treatment was improved in 3 eyes, maintained no change in 7 eyes and reduced in 2 eye. The fundus fluorescein angiography in 10 eyes revealed a significant decrease of the leakage in tumor. Postoperative complication of TTT in the 12 eyes included retinal hemorrhage (5 eyes) and retinal fold (1 eye). Conclusion TTT is an effective treatment for CCH.
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Objective To verify the pattern characteristics of optical coherance tomography (OCT) images in diabetic maculopathy (DM). Methods OCT were performed in sixty cases of DM and 30 cases of normal control group,and in 54 cases of DM,fundus fluorescein angiography (FFA) was performed simutaneously.The results from 2 kinds of examinations were compared each other. Results The average retinal thickness at the central fovea was (314.54?154.13) ?m in DM group.The macular edema in the cases of DM accounted to 57.1% on OCT examination The rate of retinal swelling associated with cystoid macular edema (CME) was 38.5%,with retinal detachment (RD) 9.2%,and with both CME and RD was 16.9% Those with mormal macular thickness and mild macular edema primarily showed focal leakage in FFA exam,while those with moderate and severe macular edema showed diffuse leakage in FFA exam.The rate of CME was 28.6% presented in FFA and 58.9% in OCT examination (t=3.39,P
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Objective To evaluate the application value of scanning laser angiography with a wide-field contact lens system in the diagnosis of choroidal melanoma. Methods Twenty-four patients with choroidal melanoma were randomly divided into two groups, who underwent fundus fluorescein angiography and indocyanine green angiography scanning with the wide-field contact and non-contact lens system respectively in order to acquire the 150? wide-field and 30? view image data. The quality of the images was comprehensively evaluated. Results Satisfying images were acquired from all of the 24 patients. Wide-field contact lens system indicated the accurate adjacent relation between the lesion position and the other dissection mechanisms, and also provided the general information about the size of the tumor and the perfusion of fluorescien or indocyanine green in the blood vessels. At the same time, it enlarged the view scope 3-5 times, which make for the screening of the peripheral lesions. Conclusions Scanning laser angiography with a wide-field contact lens system has important application value in the diagnosis of choroidal melanoma.
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Objective To evaluate the effect of transpupillary thermotherapy (TTT) on the treatment of intraocular tumors. Methods A total of 50 patients with intraocular tumors, including 37 choroidal hemangioma, 2 retinal capillary hemangioma, 5 choroidal osteoma, 4 choroidal melanoma, and 2 retinoblastoma (RB) underwent TTT and were followed up for 1～20 months. Results In 30 patients with choroidal hemangioma (average follow-up was 5.1 months), 29 (96.7%) had pigment scarring in different levels and the retinal detachemnts were partly or completely recovered; 1 had no obvious improvement. The visual acuity was unchanged in 24 (80.0%) patients, improved in 41 (13.3%) and declined in 2 (6.7%). In 2 patients with retinal capillary hemangioma, no effect was found. In 5 eyes (4 patients) with choroidal osteoma (average follow-up was 6 months), no change of the tumor was found in 1 and the atrophic spots were seen in 4; the visual acuity was unchanged in 3, improved in 1 and declined in 1. In 4 patients with choroidal melanoma (average follow-up was 8 months), the tumor was shrunken in 1, unchanged in 2, and enlarged in 1; the visual acuity was unchanged in 2 and declined in 2. In 2 patients with RB, RB was totally shrunken in 1 and partly shrunken in 1. Visual acuity of one child patient who was followed up for 20 months could not be examined, and was unchanged in another one who was followed up for 3 months. No severe complications were found in the patients during the treatment and the follow-up. Conclusions TTT is effective for the treatment of some intraocular tumors except retinal capillary hemangioma. It is a kind of potential treatment for intraocular tumors with few side-effect.
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Objective To investigate the therapeutic effects of retinal angioma. Methods The clinical data of 16 cases(20 eyes) were retrospectively analyzed, and the patients included 5 males and 11 females of 23.3 yeras old on average. Four cases in this series affected by bilateral retinal angiomas were identified as von Hippel-Lindou disease. The retinal angiomas were divided into 5 stages according to their degrees of developmnet from simple angiom without vessel dilation to feeder vessel dilation and intraretinal exudates, local retinal detachemnt, massive retinal detachment and complication occurrence in proper order. The methods of treatment were laser photocoagulation, trans-scleral cryotherapy and vitreoretinal surgery. The patinets were followed up for 37.8 months on average. Results There were 10 eyes(2 in stage 1, 7 in stage 2, 1 in stage 3)treated with laser photocoagulation, and all of the angiomas were controlled after the treatment. The visual acuity improved in 2 eyes, decreased in 4 eyes, and remained unchanged in 4 eyes. Cryotherapy was performed on 7 eyes(5 in stage 3, 2 in stage 4). The visual deteriortion was found in 5 eyes; and the state of illness was stable in 3 eyes in stage 3 and aggravating in 4 eyes (2 in stage 3, 2 in stage 4). Vitreoretinal surgery was performed on 4 eyes including 2 which had been gi ven photocoagulation, and the visual acuity improved in 2 eyes, decreased in 1 eye, and was unimproved in 1 eye. The visual acuity decreased to no light perception in an untreated eye after 1.5-year follow-up. New angiomas occurred in 3 eyes with Von-Hippel-Lindou disease in the follow-up period. Conclusion Laser photocoagulation is effective in treating the angiomas from stage 1 to 3. Cryotherapy can cause massive exudation and proliferation, and it is only suitable for a few patients in stage 3. The visual prognosis is more favorable in vitreoretinal surgery than other therapies for the patients in stage 4.
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Objective To verifying the characteristics of optical coherence tomography(OCT) in exudative age related macular degeneration (AMD). Methods The patients being investigated in this series included 16 cases (19 eyes) of exudative age related macular degeneration diagnosed by FFA and OCT examinations, among them 4 cases (6 eyes) were examined with ICGA. The color photographs of ocular fundi, FFA, ICGA and OCT were investigated by contrasting each other. Results As compared with the FFA and ICGA examinations, the characteristic findings found in OCT in patients with exudative AMD in this series were as the following:①serous detachment of neurosensory epithelium in 11 eyes,②retinal hemorrhage in 2 eyes,③serous detachment of retinal pigment epithelium in 5 eyes,④hemorrhagic detachment of retinal pigment epithelium in 10 eyes,⑤disciform scar in 4 eyes,⑥fibrovascular pigment epithelial detachment and occult CNV in 12 eyes. Conclusion OCT can supply a comprehensive survey of exudative AMD, in making the diagnosis as an important complementary examination of FFA and ICGA.
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Objective To evaluate the characteristics of two angiographic manifestation of ocular fundus in choroidal metastatic carcinomas. Methods Fundus fluoresein angiography (FFA) and indocyanine green angiography (ICGA) were performed on 17 patients (24 eyes) with choroidal metastatic carcinomas, and the characteristics of these two kinds of angiograms were analyzed and compared each other. Results According to various clinical features, e.g., locations of metastatic tumor in ocular fundus, disease course, and different kinds of original malignancy, the findings of ocular fundus were divided into (1) Isolation type: FFA showed pinpoint and mottled leaks against hypofluorescence background, increased and confluent fluoresecence in later stage. ICGA showed the similar images as in FFA, except later appearance of leaks and the choroidal vesseles could be seen beneath the thin metastatic tumor mass.(2) Diffusion type: Tumors showed hypofluorescence in early stage and uneven ill defined hyperfluorescence both in FFA and in ICGA. (3) Small metastatic carcinomas: Hypofluorescence in early stage and mild mottled hyperfluorescence were found in FFA as well as in ICGA. Conclusion Both FFA and ICGA are helpful in the diagnosis of choroidal metastatic tumors and ICGA may be conducive if there are some troubles in diagnosing the tumors with FFA.
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Objective To evaluate the clinical features of 18 cases of juxtapapillary capillary hemangioma. Methods In 18 cases of juxtapapillary capillary hemangioma, the clinical data of 19 eyes, the results of fundus fluorescein angiography (FFA) of 16 eyes, and the follow-up observation of 7 eyes were retrospectively analyzed. Results In 18 cases, the sex ratio of male and female is 1∶2 with the average age of 28.9 years. Peripheral retinal angioma was found in 3 cases (4 eyes). In 19 eyes, the center of hemangioma was located beyond the rim of optic, most of which was in inferotemporal quadrant of the optic (7/19) with the size of 1-2.5 disc diameter (DD). Most of the tumors were red in color (12/19). In the result of FFA of 16 eyes, wash-out at the late phase was found in 13 eyes. Decreased acuity was found in all 7 cases that were followed up more than 12 months (including 5 cases treated by laser photocoagulation). Retinal detachment didn′t occur in 6 cases without exudative retinal detachment after being followed-up for 54.5 months of the average term. Conclusions Most of the juxtapapillary capillary hemangioma are orange or red oval tumors. The center of hemangioma are located beyond the rim of the optic. The patients often complain gradually decreased visual acuity when they are in prime of their life with exudation and edema in different degree around the tumor. FFA is helpful for the diagnosis and differential diagnosis of this disease.