Fifty-six globes that had to be enucleated following ruthenium plaque therapy were examined histopathologically. These eyes account for 10% of all uveal melanomas treated at the University Eye Clinic Essen up until 1985. All but one revealed at least some supposedly viable tumor cells. The most prominent findings within the tumors were tumor cell necrosis, vacuolization and balloon cell degeneration, vascular obstruction and fibrosis of the tumor stroma with accumulation of pigmented macrophages. Tumor necrosis was complete or nearly complete in five cases. Tumor regression correlated with cell type and pigmentary characteristics of the tumor, with epithelioid and heavily pigmented tumor cells being more radiosensitive. Tumor regression was inhomogeneous, possibly due to polyclonality, with tumor cells of varying radiosensitivity, or due to patchy areas of vascular obliteration. Among other ocular structures, extensive subretinal gliosis, chorioretinal atrophy and scarring of the sclera within the field of radiation were observed. Scleral necrosis was present in only five cases and was limited to areas in which the tumor had infiltrated the deep scleral layers. The findings described were considered to reflect radiation injury rather than spontaneous tumor regression when compared to 70 control eyes that had been enucleated without prior treatment for uveal melanoma.

目的探讨视网膜母细胞瘤(retinoblastoma,RB)在不同年龄组中的组织病理特点.方法对1985～1995年间摘除的297只RB眼球的病理切片,重新在光镜下观察.比较3个年龄组段(＜18个月,18～60个月,＞60个月)的肿瘤细胞分化程度及对球壁和视神经的浸润情况.结果小于18个月组的肿瘤细胞以分化好为主(59.24%),18～60个月组以及大于60个月组的肿瘤细胞以分化差为主(69.90%～90.00%).肿瘤细胞对球壁和视神经的浸润在小于18个月组和大于60个月组中表现类似,浸润轻而少见,而在18～60个月组中则表现为浸润程度重而多见.结论随着患者年龄的增长,肿瘤细胞逐渐由分化较好转化为以分化较差为主.5岁以内RB患者的肿瘤细胞分化程度越差,对球壁和视神经的浸润越明显.

为探讨视网膜母细胞瘤色素上皮-脉络膜分期与视神经浸润的关系,于光镜下观察297例原发视网膜母细胞瘤摘除眼球的组织病理切片中视网膜色素上皮、玻璃膜和脉络膜的变化及视神经浸润的情况.将视神经浸润分为视神经无浸润(N0),筛板前浸润(N1),筛板浸润(N2),筛板后浸润(N3)4期.结果显示,N0占61.95%,N1占21.21%,N2占10.78%,N3占6.06%.在视神经浸润度为N0～N2时,色素上皮-脉络膜分期也处于Ⅰ期和Ⅱ期,此时视神经和球壁的局部屏障均尚未破坏,肿瘤细胞转移率仅为0.73%,而视神经浸润破坏筛板屏障时(N3),色素上皮-脉络膜分期也达到Ⅳ期,此时肿瘤细胞转移率高达11.76%.因此,随着视神经浸润度的加深,色素上皮-脉络膜分期的程度也加重,肿瘤细胞发生转移的可能性也增大.

目的:探讨视网膜母细胞瘤病理标本的不同取材方法对脉络膜浸润检出率的影响,寻求理想的常规取材方法.方法:复查297只原发摘除的视网膜母细胞瘤眼球的病理切片,比较五平面取材法(151只眼)和单平面取材法(146只眼)对脉络膜浸润的检出率. 结果:五平面取材法对脉络膜浸润的检出率(31%)明显高于单平面取材法(17%),且主要是对玻璃膜在肿瘤细胞影响下发生变化的检出率高.脉络膜浸润的各期都主要分布在五平面取材法的各平面上.结论:五平面取材法能提供判断视网膜母细胞瘤患者预后的较大量病理信息,在日常医疗工作中从人力、物力、财力上能够承受,可作为常规取材方法应用于临床.连续切片取材只作为在特殊情况下对五平面取材法的补充.单平面取材法有可能丢失一些重要的病理信息而应由五平面取材法代替.

BACKGROUND A tapetoretinal dystrophy with crystalline deposits of the retina and limbal cornea was described by Bietti in 1937. To date, only a few cases with long-term follow up have been reported. PATIENTS AND METHODS Two patients are presented including the clinical findings, fluorescein angiography, electrophysiology [electroretinography (ERG); electrooculography (EOG), multifocal electroretinography (MERG)], adaptometry, and transmission electron microscopy (TEM) of peripheral blood lymphocytes. The clinical findings were at least in part documented over a period of 30 years. RESULTS The most striking features were deposits in the retina and cornea associated with crystalloid lysosomal inclusions in peripheral lymphocytes, and choroidal atrophy especially in advanced stages of the disease. The light rise (EOG), rod- and cone-driven responses (ERG), the responses of the MERG and visual fields were affected progressively during the course. These advanced changes of the retinal pigment epithelium and choriocapillaris were observed in the 2(nd) decade already. CONCLUSIONS The findings of deposits in the cornea, retina and lymphocytes may help to differentiate BCD from other chorioretinal dystrophies. The results confirm a variable course in clinical expression of BCD between individuals.

目的:探讨视网膜母细胞瘤(Retinoblastoma,RB)患儿发病年龄与肿瘤细胞分化程度及其与预后的关系.方法:光镜下观察291名患儿的297只眼球的病理切片,并追踪观察患儿的预后.将肿瘤细胞的分化程度分为四级,即肿瘤细胞完全未分化(Do);分化的肿瘤细胞所占的范围<1/3肿瘤面积(D1);1/3～2/3肿瘤面积(D2);>2/3肿瘤面积(D3).结果:年龄小于6个月,肿瘤细胞分化最好(D3),年龄介于6～1 2个月者,肿瘤细胞分化较好(D2),年龄大于36个月,肿瘤细胞分化不良(D1),年龄介于24～30个月者,肿瘤细胞未分化(Do).四名1 5～24个月的患儿死于肿瘤细胞转移,肿瘤细胞分化程度在Do-D1.结论:2岁以前患儿肿瘤细胞的分化程度较高.若在此年龄段患儿的肿瘤细胞分化不良,又同时伴有脉络膜浸润和/或视神经浸润,应视为预后不好的指征.

OBJECTIVE:To find a morphological criterion of classification for choroidal invasion of retinoblastoma in order to further study clinical pathology of retinoblastoma (Rb) under an integrate rule.METHODS:The changes of retinal pigment epithelium, Bruch's membrane and choroid on histopathological sections of primarily enucleated eyes with Rb in 297 cases were observed under a light microscope.RESULTS:The globe wall invasion could be morphologically divided into four stages (briefly pigment epithelium-choroid classification). Stage I: Only retinal pigment epithelium was infiltrated, the Bruch's membrane was intact. Stage 2.: The Bruch's membrane was destructed, but the choriocapillaries were not invaded. Stage 3: The invasion reached middle choroidal blood vessels close to the choriocapillaris in a limited scope. Stage 4: Massive choroidal infiltration of all choroidal layers existed and the sclera could be simultaneously involved. The mortality for stage 4 was 11.5% and for stage 1 to 3 was 0.4%.CONCLUSION:The pigment epithelium-choroid stage (PEC-stage) classification in our study can be used as an integrate rule to compare the invasion degree of retinoblastoma in different individuals and studies.

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In order to inquire into standard for morphologic classification of choroidal invasion of retinoblastoma and to study the tumor clinic--pathologic further under the integrated scale, the changes of retinal pigment epithelium, Bruch's membrane and choroid on the histopathological sectins of 297 cases of primary enucleated eyes of retinoblastoma were retrospectively observed under the light microscopy. The choroidal invasion was morphologically divided into four stages: Stage 1, only retinal pigment epithelium involved, the Bruch's membrane was intact; Stage 2, the Bruch's membrane was destroyed and the choroidal capillaris was not infiltrated; Stage 3, choroial capillary and middle blood vessel layer in small limits were infiltrated; Stage 4, invasion involved in choroid in great limits and involvement of sclera existed simultaneously. This classification (also called pigment epithel--choroid stage, PEC--stage) reflected both infiltrated procedure of tumor cells and preventive mechanism in eye and morphological criterion half--quantitatively. It can be used as a united standard to compare the infiltration degree of retinoblastoma among different individuals and different studies.

Radiotherapy has become increasingly important for the treatment of complicated choroidal hemangiomas. In recent years, a broad spectrum of different radiotherapeutic options that either replace or complement the conventional therapeutic approach by photocoagulation has been recommended. This article reviews the various treatment modalities and their indications with a special focus on the long-term outcome of low-dose external beam irradiation.

Solitary peripheral retinal hemangioma can be ablated effectively by ruthenium-106 brachytherapy. A favorable outcome can be expected if the hemangioma diameter is 5.0 mm or smaller and if there is no preoperative exudative retinal detachment.

Background: Clinical studies have demonstrated the relevance of focal RPE proliferations in early AMD as risk factors for visual loss caused by late AMD. Angiographically these focal RPE proliferations are characterized as small hypofluorescent spots with hy perfluorescent rim without leakage. Corresponding to histological and experimental studies they can be interpreted as small areas of occult choroidal neovascularizations covered by proliferated RPE cells. The characterization of the long-term prognosis of these lesions was the aim of the present study.Patients and methods: Ninety-eight patients (52 female, 46 male) were reexamined clinically and angiographically with a follow-up of 2-12 years (mean 6.5 years).Results: Visual loss of two lines or more could be observed in 64.5 % of patients with final visual acuity less than 20/100 in 24.5 % of patients. Morphologically the changes in visual acuity were related to the progression towards classical choroidal neovascularizations in 32.7 % of patients. In addition 11.2 % of patients demonstrated a regression of the small occult membrane with the development of small areas of RPE atrophy covering the size of the original occult neovascularization. In 10.2 % of the patients enlargement of the lesion was observed, resulting in a large occult choroidal neovascularization without signs of classical membranes, and in 45.9 % of patients the clinical and angiographical situation was unchanged. The most important prognostic factor correlating with visual loss was the presence of a disciform lesion in the fellow eye and of multiple drusen in the examined eye. Other factors like the size or location of the focal RPE proliferation and the duration of follow-up did not correspond with visual loss.Conclusions: Focal RPE proliferations in early AMD interpreted as small occult choroidal neovascularizations are associated with a high risk of visual loss. Especially if these lesions are associated with multiple drusen and a disciform lesion in the fellow eye, nearly all patients are at risk for visual loss. These changes may therefore characterize a special high-risk group for future prophylactic treatments in early AMD, but because of the high risk for the development of classical choroidal neovascularizations in this group, these results are also very important for the planning of prophylactic laser trials for drusen in early AMD.

vention studies (Diabetic Retinopathy Study, [8]; Early Introduction Treatment Diabetic Retinopathy Study, [9 ]). We distinguish the following consecutive stages [10]: The proportion of diabetic patients admitted to renal replacement programs has dramatically increased in recent years. Optimal management of the diabetic 1. Mild non-proliferative retinopathy patient requires interdisciplinary collaboration and Initially isolated microaneurysms are found in the adequate knowledge by the nephrologist of the ophposterior pole of the eye mainly in the vicinity of the thalmological problems of the diabetic patient. The optic discs or along the vascular arcades. This is poorest managed problems are those at the interface occasionally accompanied by small intraretinal haembetween specialties, in this instance ophthalmology, orrhages (Figure 1). diabetology, and nephrology. It is the purpose of this brief summary to provide the necessary background information to the nephrological non-expert. 2. Moderate non-proliferative retinopathy The first description of diabetic retinopathy goes back to Eduard Jäger in Graz, Austria [1] in 1856, i.e. Subsequently the number of microaneurysms and only a few years after the introduction of the ophthalintraretinal haemorrhages increases. In addition, segmoscope. Following the classical descriptions of mental dilatation of veins or venules is observed (so Hirschberg in 1890 [2] and 1891 [3] the specificity of called ‘beading’). Cotton wool lesions are frequently the lesion remained in doubt and it was questioned present. In contrast to past opinion, their prognosis is whether they might not represent only the sequelae of not sinister (Figure 2). hypertension and atherosclerosis. The issue was neglected until the descriptions of Ballantyne [4], Ashton [5], and Cogan [6] who clearly documented the specificity of diabetic retinopathy.

Retinitis pigmentosa bezeichnet eine Gruppe von progredienten Erkrankungen mit Defekten im Pigmentepithel-Photorezeptorkomplex, die jedoch genetisch ausgesprochen heterogen ist. In der vorliegenden Übersicht soll auf den gegenwärtigen Stand klinischer, genetischer und pathophysiologischer Aspekte der Retinitis pigmentosa unter Berücksichtigung der in den letzten Jahren auf diesem Gebiet erschienenen Literatur eingegangen werden. Therapeutische Ansätze bei Degeneration der Photorezeptoren werden genannt.