Adult vitelliform macular degeneration: a clinicopathological study

AIMS/BACKGROUND: The yellow lesions of adult vitelliform macular degeneration (AVMD) slowly fade, progressing to hyperpigmentation or atrophy. This study aims to provide further observations on the location and nature of the vitelliform material. METHODS: This report describes the clinicopathological correlation of four eyes with AVMD. A retrospective histopathological study of a further 526 aged eyes previously graded for the stage of age-related macular degeneration (AMD) found another 10 eyes with similar pathology. RESULTS: The predominant finding was a collection of extracellular material beneath the sensory retina at the fovea. This material was derived internally from photoreceptor outer segments and externally from the retinal pigment epithelium (RPE), the latter first undergoing hypertrophy and then disruption and attenuation. Fallout of foveal cones occurred over these lesions and the inner retina was thinned, which may explain macular hole formation in this condition. All affected eyes showed histopathological evidence of AMD. CONCLUSIONS: This study confirms that the vitelliform lesions of AVMD lie beneath the sensory retina. In contrast to previous reports, however, it is proposed that the lesions comprise mainly extracellular material consisting of photoreceptor debris, possibly the result of faulty phagocytosis by the RPE, mixed with pigment liberated as the RPE undergoes disruption. The vitelliform lesions therefore are a marker for the area of maximal RPE disturbance.     

Adult-onset vitelliform macular dystrophy (AVMD)--case report

PURPOSE: Adult onset vitelliform macular dystrophy (AVMD) was first reported by Gass in 1974. This paper presents rare case of AVMD, in 3 years course of observation. MATERIAL AND METHODS: Visual acuity of R.E. was 0.1 with metamorphopsias in Amsler-test., of L.E. was 1.0 sc. The metamorphopsias in LE were first noticed 3 years later. In ophthalmoscopy in LE typical a vitelliform-like macular lesion was seen, in RE faded vitelliform changes and the RPE atrophy around it. FA of RE showed typical hypofluorescent spot in the center of the macula with small hyperfluorescent spots around it, called corona sign. The same changes in FA of the LE were first noticed 3 years later. The OCT showed central thickening in the reflective band representing RPE in both eyes. The ERG and EOG results were normal. CONCLUSIONS: The ophthalmoscopic appearance of the AVMD is very similar to that in Best's disease. In our case the correlation between appearance of metamorphoses and changes in AF was presented.     

成年人型卵黄样黄斑营养不良的临床特征

目的研究成年人型卵黄样黄斑营养不良的临床和影像学特征。设计回顾性病例系列。研究对象北京同仁医院9例（13眼）成年人型卵黄样黄斑营养不良患者。方法分析患者的眼底表现、荧光素眼底血管造影（FFA）、相干光断层扫描（OCT）和自体荧光检查结果。主要指标FFA及OCT特征。结果所有患者均无家族史。视力在0．3及以上者8／13眼（61．5％）。所有患者均表现为黄斑区圆形卵黄样微隆起、边界清楚的、不超过1PD的视网膜下病变。FFA显示病变处呈遮蔽荧光,其旁无或显现荧光,在吸收过程中,荧光相应增加。OCT显示在视网膜色素上皮（RPE）光带前见一梭形均匀的高反射区,在吸收过程中,高反射区出现不均匀,或有小的无光反射暗区。结论黄斑区圆形卵黄样不超过1PD的视网膜下病变,无明显视网膜脱离及无病变破裂分层是本病的特点。FFA和OCT检查相结合有助于成年人型卵黄样黄斑营养不良的诊断。     

Dome-shaped macula in eyes with myopic posterior staphyloma

PURPOSE: To describe an unusual feature in myopic eyes responsible for visual loss, which we call a dome-shaped macula. DESIGN: Retrospective, observational case series. METHODS: After observing isolated cases of dome-shaped macula, we analyzed optical coherence tomography (OCT) scans of 140 highly myopic eyes present in our OCT database to find similar cases. Fifteen eyes of 10 patients had a dome-shaped macula. These patients all had undergone fluorescein angiography (FA), indocyanine green angiography (ICGA), and B-scan ultrasonography examinations. RESULTS: The mean refractive error of the affected eyes was -8.25 diopters (D; range, -2 to -15 D). Median visual acuity was 20/50. Recent visual impairment was noted in 11 of the 15 eyes studied, and metamorphopsia was noted in eight eyes. Four eyes were asymptomatic. FA showed atrophic changes in the macular retinal pigment epithelium (RPE) in all eyes, combined with focal points of leakage in seven of the 15 eyes. The dome-shaped appearance of the macula was visible on both B-scan ultrasonography and OCT: a characteristic bulge of the macular retina, RPE, and choroid within the concavity of the moderate posterior staphyloma was present in all eyes. In 10 eyes, OCT also showed a shallow foveal detachment at the top of the dome-shaped macula. CONCLUSIONS: A dome-shaped macula within a myopic staphyloma is an unreported type of myopic posterior staphyloma. The dome-shaped macula often is associated with RPE atrophic changes and foveal retinal detachment, which may explain the visual impairment in these eyes.     

Ultrahigh-Resolution Combined Coronal Optical Coherence Tomography Confocal Scanning Ophthalmoscope (OCT/SLO): A pilot study

OBJECTIVE: To evaluate clinical images from a prototype ultrahigh resolution (UHR) combined coronal optical coherence tomography/confocal scanning ophthalmoscope (OCT/SLO) and to compare them to standard-resolution OCT/SLO images on the same patients. DESIGN: Cross-sectional pilot-study. PARTICIPANTS: Sixty-six eyes of 42 patients with various macular pathologies, such as age-related macular degeneration, macular edema, macular hole, central serous retinopathy, epiretinal membrane and posterior vitreous traction syndrome. METHODS: Each subject was first scanned with a standard-resolution OCT/SLO that has an axial resolution of ∼10 micron. Immediately following, patients were scanned with the prototype UHR OCT/SLO device. The UHR system employs a compact super luminescent diode (SLD) with a 150 nm bandwidth centered at 890 nm, which allows imaging of the retina with an axial resolution of 3 microns. Both coronal and longitudinal OCT scans were acquired with each system, and compared side-by-side. Scan quality was assessed for the observer's ability to visualize the vitreo-retinal interface and retinal layers – in particular of the outer retina/RPE/choroidal interface, increased discrimination of pathological changes, and better signal intensity. MAIN OUTCOME MEASURES: Ultrahigh and standard-resolution coronal and longitudinal OCT/SLO images of macular pathologies. RESULTS: In the side-by-side comparison with the commercial standard-resolution OCT/SLO images, the scans in the Ultrahigh resolution OCT/SLO images were superior in 85% of cases. Relatively poor quality images were attributed to lower signal-to-noise ratio, limited focusing, or media opacities. Several images that had a better signal intensity in the standard-resolution OCT/SLO system were found to show more retinal detail in the UHR system. In general, intraretinal layers in the UHR OCT/SLO images were better delineated in both coronal and longitudinal scans. Enhanced details were also seen in the outer retina/RPE/choroidal complex. The UHR OCT/SLO system produced better definition of morphological changes in several macular pathologies. CONCLUSIONS: Broadband SLD-based UHR OCT/SLO offers a compact, efficient, and economic enhancement to the currently available clinical OCT imaging systems. UHR OCT/SLO imaging enhanced the quality of the OCT C-scans, facilitated appreciation of vitreo-retinal pathologies, and improved sensitivity to small changes in the retina, and the outer retina/RPE/choroidal interface.     

外伤性黄斑病变的OCT特征研究

目的研究外伤性黄斑病变的相干光断层扫描（OCT）图像特征,以总结其患病规律。设计回顾性病例系列。研究对象477例（486眼）4-76岁的外伤性黄斑病变患者。方法回顾及分析2002年9月．2009年6月在北京同仁医院眼科门诊就诊的不同类型外伤性黄斑病变患者的门诊病历资料及OCT图像。主要指标OCT图像特征。结果外伤性黄斑病变的OCT表现主要有九种：黄斑裂孔、神经上皮层脱离、黄斑出血、黄斑水肿、黄斑前膜、脉络膜破裂、黄斑部神经上皮层萎缩薄变、色素上皮层萎缩及脉络膜萎缩。在外伤的早期,较常见的OCT表现为黄斑部色素上皮层萎缩（49．0％）、黄斑裂孔（24．7％）、神经上皮层脱离（26．3％）、黄斑出血（24．2％）、黄斑水肿（19．2％）;在外伤的中晚期,较常见的OCT表现为黄斑部色素上皮层萎缩（63．0％）、神经上皮层萎缩薄变（36．5％）。结论外伤性黄斑病变以多种OCT表现并存为多,黄斑部视网膜色素上皮层萎缩是贯穿外伤早期、中晚期最多的表现。外伤性黄斑病变早期以黄斑裂孔、视网膜脱离、黄斑出血、黄斑水肿为主,中晚期以黄斑部神经上皮层及色素上皮层萎缩为主。（眼科,2009,18：236-238）     

Evaluation of peripapillary detachment in pathologic myopia with en face optical coherence tomography

BACKGROUND: A newly recognized lesion in pathologic myopia is peripapillary detachment of the retinal pigment epithelium (RPE) and retina. Recently introduced en face optical coherence tomography (OCT) provides not only cross-sectional but also coronal scans of the retina, and allows lateral extent visualization and thickness measurement of lesions. METHODS: Three patients presenting bilateral peripapillary yellow-orange lobulated area in high myopia have been evaluated with fluorescein angiography (FA), indocyanine green angiography (ICGA), en face OCT (OCT/SLO; Ophthalmic Technologies Inc, Toronto, Canada), and Humphrey visual field analyzer. RESULTS: In all eyes, en face OCT has shown the presence of a peripapillary sub-RPE nonreflective area. The lateral extent of this area was clearly detectable and the measurement of its thickness was obtained. We detected a cleft in the RPE at one edge of the cavitation in two eyes, vascular tractions and vitreoretinal tractions in two eyes, a macular hole with posterior retinal detachment, and small areas of RPE detachment nonconnected with the peripapillary detachment in one eye. In the four eyes presenting a proper central fixation, glaucomatous visual field defects were evident. CONCLUSION: En face OCT has allowed to evaluate the thickness and the lateral extent of the peripapillary detachment. Therefore, its use could be important in determining the size and grading of these lesions at first visit, and to detect minimal changes of width and thickness during follow-up as an alternative to fluorescein angiography.     

病理性近视黄斑部脉络膜新生血管的OCT和FFA表现

目的分析病理性近视（PM）黄斑部脉络膜新生血管（CNV）的光学相干断层扫描（OCT）和眼底荧光素血管造影（FFA）的图像特征。设计临床病例研究。研究对象23例（25眼）伴有黄斑部CNV的PM患者。方法对上述患眼行OCT和FFA检查，对比分析结果。主要指标黄斑部CNV的OCT和FFA图像。结果OCT检查，18眼（72％）CNV呈纺锤形或类圆形团块状，呈强或中等强度反射，自视网膜色素上皮层向上突出，位于视网膜神经上皮层下；7眼（28％）CNV呈不规则增强紊乱的反射信号，位于视网膜色素上皮层平面。9眼伴视网膜色素上皮出血性脱离，6眼伴视网膜前膜形成，2眼伴黄斑裂孔形成，15眼伴不同程度的视网膜增厚和水肿。FFA检查中有19眼显示造影早期黄斑部的盘状或不规则荧光渗漏，6眼仅显示病灶处荧光遮蔽和／或透见荧光。结论PM黄斑部CNV的OCT图像以在色素上皮层增生的类圆形和纺锤形纤维血管膜为特征，其范围较局限，常伴有色素上皮层出血性脱离。     

病理性近视的相干光断层扫描

目的研究病理性近视的相干光断层扫描(OCT)的图像特征。方法对100例(195只眼)高度近视患者行OCT检查。患者年龄16～76岁。屈光度数-6．00～-36．00D。结果OCT检查正常者7只眼(3．6％),视网膜色素上皮及脉络膜毛细血管光带不均匀和薄变者161只眼(82．6％),黄斑全层裂孔21只眼(10．8％),其中合并视网膜脱离10只眼(5．1％),黄斑限局性视网膜浅脱离24只眼(12．3％),其中合并视网膜劈裂7只眼(3．6％),黄斑脉络膜新生血管膜19只眼(9．7％),Fuchs斑9只眼(4．6％),厚的黄斑出血3只眼,黄斑前膜18只眼(9．2％),常合并有视网膜水肿。黄斑视网膜神经上皮薄变5只眼(2．6％),漆裂纹3只眼(1．5％)。结论与裂隙灯全视网膜显微镜检查比较,OCT在观察视网膜色素上皮和脉络膜毛细血管改变,确诊黄斑裂孔,发现小的神经上皮脱离和劈裂,诊断黄斑前膜,神经上皮薄变等方面均有优越性。但对薄的黄斑出血及小色素点不能分辨,对厚出血与色素难以分辨,因而也有其局限性。     

Best's vitelliform macular dystrophy with pseudohypopyon: an optical coherence tomography study

PURPOSE: To report optical coherence tomography (OCT) changes in Best's vitelliform macular dystrophy (BVMD) with pseudohypopyon. DESIGN: Observational case report. METHODS: Both eyes of a patient with BVMD showing pseudohypopyon were examined with OCT. RESULTS: OCT demonstrated the presence of serous retinal elevation with normal appearing retinal pigment epithelium (RPE) superiorly, and broadening of the outer-retina-choroid-complex signal under retinal elevation, inferiorly. CONCLUSIONS: OCT findings in our study suggest the accumulation of material under neurosensory retina in BVMD with pseudohypopyon.     

Acute exudative polymorphous vitelliform maculopathy syndrome

PURPOSE: The authors describe clinical characteristics of acute exudative polymorphous vitelliform maculopathy syndrome, also termed polymorphous maculopathy syndrome (PMS), an unusual tapetoretinal disorder first reported by Gass. METHODS: Detailed ophthalmoscopy, fluorescein angiography, electrophysiology, and optical coherence tomography (OCT) were performed on a patient with PMS. RESULTS: Numerous small, yellowish lesions arranged in a honeycombed pattern at the level of the retinal pigment epithelium (RPE) around the disk and the macula, a shallow macular detachment (documented by OCT), and a perifoveolar yellow ring appeared in both eyes. Initial fluorescein angiography revealed mild early hyperfluorescence and late staining of the perifoveolar rings and the multifocal yellow lesions. Gradually, subretinal yellowish deposits gravitated as a meniscus below the macula with subsequent further changes. Electrophysiology showed reduced amplitudes of the electroretinogram and electrooculogram and abnormal dark adaptometry. A rhegmatogenous retinal detachment in the left eye was successfully repaired, with a visual acuity of 20/30. Antecedent upper respiratory infection and headaches and positive serology for the coxsackie virus suggest the possibility of a virus-induced disorder. Genetic studies of this patient showed normal DNA sequences for the bestrophin and peripherin/RDS genes. CONCLUSION: This is the first report of PMS with the following characteristics: occurrence in a woman, development of a rhegmatogenous retinal detachment, and electroretinogram, visual-evoked response, color vision, and OCT findings. PMS has features similar to but also different from those of Harada disease and Best disease. Further studies are necessary to determine its relationship to other acquired conditions, such as virus-induced disorders, and genetic defects unrelated to abnormalities of the bestrophin and peripherin/RDS genes.     

Optical coherence tomography in diagnosing adult-onset vitelliform macular dystrophy

PURPOSE: To assess usefulness of optical coherence tomography (OCT) in adult-onset vitelliform macular dystrophy (AVMD) diagnosis. To assess retinal pathological changes during all stages of the disease. MATERIAL AND METHODS: Retrospective case-series report. 16 patients (9 men, 7 women, mean age 60.25), with vitelliform macular dystrophy, who underwent ophthalmic examination including optical coherence tomography (OCT Stratus III, Zeiss Meditec, CA, USA) in 2005 and 2006. Retinal Thickness Map and Fast Retinal Thickness Map Acquisition Protocols were used during OCT scanning. Patients were evaluated with ETDRS charts, biomicroscopy, fluorescein angiography and electrophysiological exams. RESULTS: 7 patients (44%) were referred to our outpatient clinic with diagnosis of macular hole, 7 patients (44%) with diagnosis of AMD. In 2 patients Best disease was suspected. Diagnosis of AVMD was possible to establish in all patients including medical history, fundus photography and OCT. CONCLUSIONS: OCT enables retinal morphology assessment and can be treated as the basis of adult-onset vitelliform macular dystrophy early evaluation, diagnosis differentiation and monitoring progression.     

Exudative age-related macular degeneration or Best vitelliform macular dystrophy?--A case report

PURPOSE: To report a case of Best vitelliform macular dystrophy referred to the Department Ophthalmology in Krakow with a diagnosis of exudative age-related macular degeneration (AMD). MATERIALS AND METHODS: 70-years old man was diagnosed in our clinic because of a two years history of slow, progressive visual acuity worsening in both eyes with the presence of metamorphopsia. The basic ophthalmic examination was performed with additional diagnostic methods including: colour vision test (Panel D-15), Amsler grid test, contrast sensitivity test (Pelli-Robson chart), fluorescein angiography (FA), indocyanine green angiography (ICGA), electroretinogram (ERG), electrooculogram (EOG) and optical coherence tomography (OCT). RESULTS: Visual acuity in the right eye was: 0.16 and in the left: 0.25. Amsler grid test revealed the presence of bilateral mild etamorphopsia with the relative central scotoma. Pelli-Robson test showed decreased contrast sensitivity perception in both eyes; PO > LO. On fundoscopy in macula of both eyes the symmetrical round, elevated lesions of 1.5 dd with the meniscus of subretinal creamy-yellow masses were present. The early frames of FA showed the presence of round lesions with distinct borders, unchanged in size and shape throught the examination, hypofluorescent in the lower and hyperfluorescent in the upper half of the lesions. Late frames of FA revealed the irregular hyperfluorescence also in lower aspects of the lesions. ICGA showed: round hypofluorescent lesions with isofluorescence in the upper part of the lesions. ERG--revealed no pathology, EOG--showed decreased light response and depressed Arden ratio in both eyes. OCT demonstrated hiperreflectivity of the retinal pigment epithelium with elevation of retina and deletion of the foveolar depression in both eyes. CONCLUSIONS: Based on the results of performed tests the diagnosis of the Best vitelliform macular dystrophy was established. In some cases various pathologies involving the macula may mimic the exudative AMD. The basic ophthalmic examination supported by additional diagnostic methods allow to establish the definitive diagnosis in most cases of macular disorders.     

Optical coherence tomography identification of occult choroidal neovascularization in age-related macular degeneration

PURPOSE: To evaluate and describe the various optical coherence tomography (OCT) features of occult choroidal neovascularization (CNV) in age-related macular degeneration (AMD) at the time of diagnosis. DESIGN: Prospective, consecutive, observational case series. METHODS: One hundred and fifty-three eyes of 130 consecutive patients with subfoveal occult CNV diagnosed on scanning laser ophthalmoscope (SLO) fluorescein angiography (FA) and SLO indocyanine green angiography (ICGA) were evaluated with OCT. The diagnostic criteria for occult CNV on angiography were heterogeneous hyperfluorescence with late leakage in the macular region associated with pigment epithelial detachment (PED), stippled hyperfluorescent dots, and signs of deterioration. OCT findings were evaluated and described. RESULTS: A PED was observed on OCT in 98% (150 eyes) either as a limited retinal pigment epithelium (RPE) elevation (54 eyes [35.3%]) or a complete detachment (96 eyes [62.7%]). Occult CNV corresponded to zones of hyperreflectivity in contact with the RPE band and was detected in 62.7% of eyes. In fibrovascular PED (63 eyes [65.5%]), the elevated RPE was highlighted posteriorly by a moderately reflective band overlying a hyporeflective cavity. In serous PED, the cavity remained optically empty. The RPE in the detached zone showed changes such as fragmentation (137 eyes [89.5%]). OCT also showed intraretinal (122 eyes [79.7%]) and subretinal (64 eyes [41.8%]) fluid. CONCLUSIONS: Analysis of the various OCT features observed in this study confirms the polymorphic nature of occult CNV in AMD, their exudative reactions, the almost constant presence of PED, and the different changes in the RPE band. OCT examination, therefore, provides valuable data to confirm the features of subepithelial occult CNV.     

Optical coherence tomography study of adult vitelliform macular detachment in a patient with basal laminar drusen

To report the morphological data of adult vitelliform macular detachment in a patient with basal laminar drusen using optical coherence tomography (OCT-3) as an observational case report. A 70-year-old man presented with adult vitelliform macular detachment and basal laminar drusen underwent fundus biomicroscopy, fundus fluorescein angiography and OCT-3. Fundus examination showed bilateral yellow subretinal macula deposits with associated basal laminar drusen. Examination with OCT revealed areas of hyper-reflectivity compatible in size with the subretinal deposits below and elevating the photoreceptor layer. This was accompanied by underlying disruption of the OCT signal from retinal pigment epithelium (RPE) in the more affected left eye. In adult vitelliform macular detachment and basal laminar drusen, OCT may demonstrate that the yellow material is located predominantly below RPE in early disease and between the photoreceptor layer and the retinal pigment epithelium in later disease.     

多模式影像技术在Stargardt病不同病变阶段的临床运用

目的： 观察多模式影像技术在Stargardt病不同病变阶段的影像特征。 方法： 回顾性系列病例研究。 对云南大学附属医院2016 年5 月至2019 年8 月确诊的28 例（ 56 眼） Stargardt患者进行眼底彩色照相、 自发荧光、 眼底荧光血管造影、 光学相干断层扫描（ OCT）、 炫彩眼底成像、 血流成像OCT（ OCTA） 等多模式检查, 总结各类影像检查在疾病不同阶段的特征。 结果： 眼底彩色照相可显示, 随病变进 展黄斑区的萎缩病变逐渐扩大, 伴后极部眼底色泽改变。炫彩成像通过多波长的组合, 可用更好的 对比度和层次感显示出萎缩的细胞和范围。眼底自发荧光通过自发荧光的高低变化提示视网膜色 素上皮（ RPE） 的受损情况, 同时能清楚直观地显示黄斑区萎缩病灶的大小。眼底荧光血管造影在 Stargardt病中的观察可直接反映视网膜色素上皮层、 脉络膜血管的损伤程度。 随着Stargardt病的发展, 黄斑区RPE层萎缩范围扩大, 视网膜及脉络膜毛细血管继发进行性萎缩。 OCT提供了断层眼底的影 像信息, 随病变进展, 中心凹周围神经上皮层变薄, 光感受器不同程度萎缩、 消失, RPE层不同程 度萎缩, 反光减弱, 脉络膜层毛细血管萎缩, 从层次深度和范围的广度都体现了病变的发展。 OCTA 中本研究观察到随着病变的进展, 各层次血管密度呈减少趋势。扫描视网膜深层血管时, 透见了 下方的脉络膜血管, 随着萎缩加重, 透见的脉络膜血管的范围扩大。 结论： 不同的眼科影像技术在 Stargardt病的不同阶段表现有各自特点和优势, 为临床医师提供了更多角度的追踪随访、 病情评估 的手段。     

Optical coherence tomographic features in a case of bilateral macular coloboma with strabismus

 Purpose:To report the optical coherence tomography (OCT) findings in a patient with bilateral macular coloboma with strabismus. Methods:A 21-year-old male presented with macular coloboma in both eyes.Fundus photography, fundus fluorescence angiography (FFA) and OCT were performed. Results:Color fundus photography showed a sharply-demarcated, round macular defect, approximately 4×4 disc diameters with bare sclera at the base and pigment clumping in both eyes. FFA showed hypofluorescence at the macula corresponding to the size of the lesion bilaterally. OCT revealed a crater-like depression at the macula, demonstrating atrophic neurosensory retina, and an absence of retinal pigment epithelium (RPE) and choroid in the lesion. Conclusion:OCT can be a beneficial tool to confirm the diagnosis of macular coloboma.     

Differential macular and peripheral expression of bestrophin in human eyes and its implication for best disease

PURPOSE: Best disease, or vitelliform macular degeneration, is an autosomal dominant form of macular degeneration that is caused by mutations in the gene encoding bestrophin. On clinical examination, Best disease is characterized by an elevated lesion beneath the neurosensory retina, resembling an egg yolk. The lesions in Best disease are primarily restricted to the macula, a small region of the retina responsible for central vision. The nature of the vitelliform material and the reason the development of such lesions is usually restricted to the macula are two unsolved questions in the pathogenesis of this disorder. METHODS: The expression of bestrophin protein and mRNA was evaluated by immunohistochemistry, Western blot, and quantitative PCR in a series of normal human eyes. The ultrastructure of the retinal pigment epithelium and the histopathology of two donors with clinically diagnosed Best disease were also examined. RESULTS: An eye from a Best disease donor with a T6R mutation was found to have deposits containing lipid and glycoconjugates within the central retinal scar. These deposits may be remnants of the vitelliform lesion. Immunohistochemical localization of bestrophin in a series of 22 unaffected eyes revealed a pattern in which macular labeling was less robust than labeling outside the macula in most (18/22) cases. This pattern was confirmed using quantitative PCR and Western blotting. CONCLUSIONS: Topographic differences in the levels of bestrophin protein may in part explain the propensity for the macula to develop lesions in Best disease.     

老年性黄斑变性光学相干断层扫描观察

目的 观察萎缩型，渗出型老年黄斑变性（Age-related macular degeneration AMD）患者的光学相干断层扫描图像特征。比较光学相干断层扫描（Optical Coherence Tomography OCT）和荧光血管造影（Fluorescein angiography FFA）的特点，对脉络膜新生血管（Choroidal neovascularization CNV）进行OCT分型。方法 经FFA确诊的AMD57例76只眼进行OCT检查。结果 AMD患者色素上皮萎缩，软性玻璃膜疣，神经上皮和色素上皮脱离具有特有的OCT特征，OCT图像中视网膜神经上皮增厚、隆起反映视网膜下，视网膜层间积液。神经上皮或色素上皮（Retinal pigment epithelium，RPE）隆起，其下低反射区反映神经上皮或RPE层脱离。CNV的OCT图像分为边界清晰的CNV，边界模糊的CNV，纤维血管性RPE脱离。FFA中的典型性CNV相当于OCT图像中边界清晰CNV，隐匿性CNV相当于OCT图像中边界模糊CNV和纤维血管性RPE脱离。结论 OCT能特征性显示AMD中视网膜神经上皮隆起，视网膜层间积液，出血，神经上皮和RPE的脱离，且显示不同类型CNV的OCT特征。     

Optical coherence tomography on autologous translocation of choroid and retinal pigment epithelium in age-related macular degeneration

PURPOSE: To analyse structural changes after autologous translocation of choroid and retinal pigment epithelium (RPE) in patients with age-related macular degeneration (AMD) using optical coherence tomography (OCT). METHODS: We performed a prospective nonrandomised study in 29 consecutive patients, who underwent submacular surgery with translocation of an autologous full-thickness graft of RPE, Bruch's membrane, and choroid. All patients had recent loss of reading vision due to AMD. OCT was performed before surgery and at 3- and 6- month follow-up to analyse the morphological appearance of the graft and the overlying retina. RESULTS: Maximum retinal thickness decreased from mean 408 microm (standard deviation (SD) 127 microm) preoperative to mean 373 microm (SD 104 microm) at 6-month follow-up (P=0.094). In 11 cases (40%), a nearly physiological shape of the retina was seen at this time point. A macular hole persisted in two eyes after silicone oil removal. In most eyes, the highly reflective band of the graft presumably corresponding to RPE was continuous with the surrounding RPE band in all six OCT scans. Eyes with flat appearance of the graft at 6-month follow-up (<300 microm) showed a significantly better functional outcome than eyes with more prominent grafts. Interestingly, most patients did not complain about metamorphopsia, even though the graft was prominent or wrinkled in some cases. CONCLUSION: OCT is a useful tool in monitoring intra- and subretinal changes after subretinal surgery with graft translocation. We demonstrated that graft translocation may lead to a normalisation of retinal thickness and stabilisation of visual acuity.