静脉旁色素沉着性视网膜脉络膜萎缩之视网膜微血管病变

静脉旁色素沉着性视网膜脉络膜萎缩是一无症状性疾病,其特征为:沿视网膜静脉有不一致的色素分布的视网膜脉络膜萎缩,双眼对称,病程缓慢,病因不明。本文报告1例非对称性的静脉旁色素沉着性视网膜脉络膜萎缩,合并局部周边视网膜进行性微血管病变。患者为黑人,女性,51岁,否认夜盲和既往眼病史,家族史无类似记载。双眼视力6/6,外眼和眼球运动正常,眼位正常,裂隙灯查见双眼轻度楔形白内障,眼压16mmHg。右眼底有典型的静脉旁微粒状色素沉着,伴有从视盘延伸至赤道部以外的视网膜脉络膜萎缩,视网膜颞侧周边部可见微动脉瘤和视网膜毛细血管扩张,但无渗出;左眼底仅有一孤立性视网膜脉络膜萎缩斑沿颞下视网膜静脉分布。右眼视野显著缩小,尤其是鼻侧。左眼视野正常。ERG显示     

色素性静脉旁网络膜萎缩

Brown(1937)首先报告一例双眼底视乳头周围萎缩,以及视网膜血管特别在静脉周围出现萎缩和色素沉着,一种稀有的眼底病变称之为放射状视网膜脉络膜炎以来,仅名称一项就有6种之多,及至Franceshetti(1962)系统观察予以报导,定名为“色素性静脉旁视网膜脉络膜萎缩。称世界上不超过30例。现将我科1984年遇到1例,做了眼底荧光血管造影检查,报告如下: 金××女21岁于5岁时发现双眼视力较差,以夜间为甚。近二年来感觉双眼视力明显下降,夜间不能视物,影响正常生活     

静脉旁色素性视网膜脉络膜萎缩

静脉旁色素性视网膜脉络膜萎缩是一种少见的眼底病,其特征是双眼对称性沿视网膜静脉分布的色素沉着及视网膜脉络膜局限性萎缩。本病由Brown(1937)首先报道,称为放射状视网膜脉络膜炎,尔后又以不同名称相继有报道,包括先天性视网膜色素沉着;条纹状脉络膜视网膜炎;视网膜黑变病;静脉旁色素性脉络膜萎缩;假性色素性视网膜炎;静脉旁色素性视网膜脉络膜变性;以及静脉旁色素性视网膜脉络膜萎缩。据笔者所知国外文献迄今共报告48例,其中一例为我国纪秀香(1948)报道于美国眼科杂志,除此之外,国内于1985年眼底病杂志上综合报道7例,为进一步加深了解这一少见病,本文特收集55例,就其病因、发病机理、症状、检查以及诊断、鉴别诊断等方面作一介绍。     

初盘旁脉络膜炎眼底改变及荧光眼底血管造影的特征

目的探讨视盘旁脉络膜炎(Jensen病)的眼底改变及荧光眼底血管造影(FFA)的特征。方法分析11例(12只眼)Jensen病患者的临床特征和FFA表现。结果11例患者均为近视。眼底可见视盘旁的灰白色局限性脉络膜渗出病灶,边界不清,轻度隆起;FFA早期为低荧光,后期荧光渗漏和染色;非活动病灶荧光着染,萎缩区可见大的脉络膜血管。结论Jensen病多发生于近视患者,以青壮年多见,多为单眼发病,可复发。眼底表现有特征性,FFA检查有助于了解病变进展和活动程度,指导临床治疗。     

初盘旁脉络膜炎眼底改变及荧光眼底血管造影的特征

目的 探讨视盘旁脉络膜炎(Jensen病)的眼底改变及荧光眼底血管造影(FFA)的特征.方法 分析11例(12只眼)Jensen病患者的临床特征和FFA表现.结果 11例患者均为近视.眼底可见视盘旁的灰白色局限性脉络膜渗出病灶,边界不清,轻度隆起;FFA早期为低荧光,后期荧光渗漏和染色;非活动病灶荧光着染,萎缩区可见大的脉络膜血管.结论 Jensen病多发生于近视患者,以青壮年多见,多为单眼发病,可复发.眼底表现有特征性,FFA检查有助于了解病变进展和活动程度,指导临床治疗.     

双眼先天性黄斑缺损一例报告

先天性黄斑缺损临床较为少见。一般单眼发病,双眼者罕见。现将我院近期所遇一例双眼先天性黄斑缺损报道如下:患者女13岁因双眼自幼视物不清近半年增重于2005年3月7日来我院就诊。既往无眼病史,父母非近亲结婚,家族无同类患者,发育正常,无指趾畸形。视力:右0.08,左0.04,双眼前节无异常发现。双眼底:视乳头边界清晰,色正常,黄斑区可见直径约4PD大小横椭圆形脉络膜缺损区,边界清晰,缺损区内视网膜血管走行正常,无间断,底部可透见部分脉络膜大血管并见不规则散在色素沉着斑(图1)。荧光血管造影检查:黄斑缺损区无脉络膜背景荧光,可见部分脉络膜…     

色素性静脉旁视网膜脉络膜萎缩一例

色素性静脉旁视网膜脉络膜萎缩一例张瑛，董永章近年来国内文献相继报道了９例罕见的色素性静脉旁视网膜脉络膜萎缩的病例，现将我院发现并经眼底荧光血管造影证实的１例报告如下。患者男，２７岁。无意中发现自己右眼比左眼视物模糊，于１９９１年１月４日来我院眼科门诊...     

无脉络膜症一例

患者男 ,2 1岁。因夜盲 12年 ,于 2 0 0 0年 7月 31日来我院就诊。其父母非近亲结婚 ,家族中无类似患者。全身检查正常。眼部检查 :视力右眼 0 .3,左眼 0 .2 ,双眼前节未见异常 ,散瞳检查眼底 :双眼视盘色腊黄 ,视网膜血管略细 ,除黄斑区外 ,视网膜色素上皮及脉络膜毛细血管变性 ,呈弥漫性萎缩 ,暴露脉络膜大血管 ,视网膜少量散在不规则形色素沉着 (图 1a,b) ,视野呈向心性缩小。双眼荧光素眼底血管造影 (fundus fluoresceinangiograph,FFA)显示 :除黄斑区为片状强荧光外 ,脉络膜始终暗背景 ,毛细血管无灌注 ,可见脉络膜大血管 ;各期均未…     

双眼视乳头周围脉络膜硬化症二例

例1 女性,52岁.因"双眼视力渐降1年,左眼重"于2010年5月10日来我院就诊,视力:VOD 0.3、VOS 0.05,眼前节检查未见异常,眼底检查可见双眼视盘周围视网膜呈灰黄色及散在点片状色素斑,病变已累及黄斑,黄斑结构不清,中心凹光反射消失,视盘边界清楚,色淡红(图1A、2A).荧光素眼底血管造影(FFA)显示:在造影早期暨动脉期双眼视盘周边及其颞侧见脉络膜毛细血管萎缩呈点片状高低荧光柑间改变(图1B、2B),视网膜动脉变细,黄斑区见高低荧光相间改变;右眼视盘见环形斑,左眼视盘边界清楚.造影过程未见荧光素渗漏.眼科诊断:双眼视乳头周围脉络膜硬化症.     

双眼脉络膜骨瘤一例

患者女 ,30岁。因左眼视力下降 1年余 ,右眼视力下降3个月 ,于 1998年 11月 5日来院就诊。体检未见异常。眼部检查 :右眼视力 0 6 ,左眼 0 1;双眼前节检查未见异常。眼底 :双眼黄斑部视网膜隆起 ,视网膜下可见黄白色病灶 ,色素沉着明显 ,呈伪足样改变。疑诊为双眼脉络膜骨瘤。荧光素眼底血管造影 :早期病变处为高荧光 ,晚期呈强荧光 ,并有斑驳状染色。Ｂ超检查 :眶内组织回声均较弱 ,但各部眼底光带仍有回声 ,呈高反射。双眼ＣＴ扫描 :双眼球后部ＣＴ值高 ,并与骨密度相同。诊断 :双眼脉络膜骨瘤。讨论　脉络膜骨瘤多见于 2 6～ 2 9岁的女…     

Macular dysplasia and pigmented paravenous retino-choroidal atrophy

Pigmented paravenous retino-choroidal atrophy (PPRCA) is a rare retinal disease characterized by bilateral patches of pigment and areas of chorioretinal atrophy distributed along the veins. The authors present a 21-year-old male with pigmented paravenous retinochoroidal atrophy and unilateral macular dysplasia. To their knowledge, this is the second reported case of macular involvement. They believe that such association is not occasional, but may be suggestive of a variable expressivity of the disease.     

Hereditary pigmented paravenous chorioretinal atrophy

Pigmented paravenous chorioretinal atrophy PPCRA) is a rare disorder which is diagnosed primarily because of the typical fundoscopic appearance of retinal pigment epithelial (RPE) atrophy and clumping in a paravenous distribution. A mildly affected and asymptomatic 54-year-old mother and her mildly affected daughter and severely affected son presented with pigmented paravenous chorioretinal atrophy. The severely affected (proband) 28-year-old man manifested the characteristic paravenous chorioretinal atrophy with pigment clusters in both eyes with macular involvement. Besides the characteristic fundus picture, he also had chronic angle closure glaucoma. His 23-year-old sister presented with unilateral involvement. Her right eye showed focal perivenular retinal pigment epithelial hyperplasia at the 2 o'clock position and dilated, tortuous retinal veins, while her left eye had only dilated and tortuous retinal veins. Both patients were hyperopic. Their mother had an area of chorioretinal atrophy in one eye near a retinal vein. The scotopic ERG responses were markedly abnormal in the male patient, while his sister had a mild decrease in amplitude of both a and b waves in both eyes. One of the children of an unaffected family member was found to have dilated and tortuous retinal veins and hyperopia (III-12). To our knowledge, this is the fourth report of familial occurrence of pigmented paravenous chorioretinal atrophy. The present pedigree is compatible with X-linked recessive or dominant inheritance.     

Bilateral pigmented paravenous chorioretinal atrophy: a case report

Purpose

To describe a case of bilateral pigmented paravenous chorioretinal atrophy.

Methods

Observational case report.

Results

A 50-year-old female patient complained of right eye pain and decreased near visual acuity. She had a best-corrected visual acuity of 20/20 in both eyes. In the posterior segment, there were atrophic changes along retinal vessels, and bony spicule pigmentation was observed in a paravenous distribution. There were some abnormal changes in the electroretinogram and electrooculogram in both eyes.

Conclusions

A diagnosis of bilateral pigmented paravenous chorioretinal atrophy was made.Key words: Atrophy, Chorioretinal atrophy, Paravenous atrophy     

Pigmented paravenous chorioretinal atrophy

Two patients with pigmented paravenous chorioretinal atrophy are presented. Both patients had chorioretinal atrophy with pigment clusters located in the paravenous areas, and one of the patients had macular affection. This patient had exotropia and juvenile cataract, the other patient had senile cataract. Electroretinography showed decreased, but not totally extinguished potentials. Automatic and manual perimetry revealed relative and absolute scotomas corresponding to the atrophic paravenous areas. To our knowledge this is only the sixth report of pigmented paravenous chorioretinal atrophy with macular affection.     

Unilateral pigmented paravenous retinochoroidal atrophy

We report the case of an asymptomatic unilateral pigmented paravenous retinochoroidal atrophy (PPRCA) in a 43-year-old patient. The right eye showed chorioretinal atrophy with bone-spicule-like pigmentations along the retinal veins. Visual acuity was 20 / 20 and perimetry revealed scotomas correlating to the chorioretinal atrophy. Electrophysiological examination showed decreased signals in ERG and EOG. Fundus autofluorescence and angiography findings are presented. Pathogenetically, a classification as hereditary retinal dystrophy (as in retinitis pigmentosa) as well as a post-inflammatory residuum are discussed.     

Atrofia coriorretiniana pigmentada paravenosa de presentación unifocal y unilateral

Paravenous pigmented chorioretinal atrophy is a generally multifocal, bilateral and symmetric rare entity associated with autoimmune diseases and other ocular complications.We present the clinical case of a patient with rheumatoid arthritis who attended for pain of several days. He presented decreased visual acuity of the left eye, nodular scleritis and chorioretinal atrophy with pigment accumulation in bone spicules in the inferior temporal vascular arcade and lamellar macular hole. The right eye shows no alterations. LE autofluorescence shows a hypoautofluorescence lesion with defined edges. Fluorescein angiography shows hyperfluorescence consistent with retinal pigmentary epithelial degeneration and blockage in pigment areas. The visual field reveals a defect in the superior hemifield.This case describes an atypical unifocal and unilateral paravenous pigmented chorioretinal atrophy. This variant must be known to make a correct differential diagnosis, as well as to provide adequate prognostic information.     

Retinal microangiopathy in pigmented paravenous chorioretinal atrophy.

This report describes an atypical case of pigmented paravenous chorioretinal atrophy, associated with focal progressive peripheral retinal microangiopathy, in a 51-year-old black female. The eyes were asymmetrically involved. Although several cases have been reported with typical features of this uncommon entity, none of them have been known to be associated with retinal microangiopathy. The occurrence of such microangiopathy supports the hypothesis that damage to the retinal photoreceptors may induce retinal microangiopathy, as suggested in other clinical and experimental studies.     

Hereditary pigmented paravenous retinochoroidal atrophy.

A father and son with pigmented paravenous retinochoroidal atrophy demonstrated the classical fundus appearance of bilateral sharply circumscribed patches of retinochoroidal atrophy and pigmentation along the retinal veins. Our patients, and the preponderance of males in previously reported cases of this disease, suggest the possibility of Y-chromosome mediated male-to-male transmission. Electrophysiologic findings in our patients included elevated dark-adaptation thresholds, slightly reduced electroretinographic amplitudes, slightly prolonged a- and b-wave implicit times, and (in Case 1) abnormal electro-oculographic ratios. These findings indicate a diffuse tapetoretinal degeneration, and suggest that pigmented paravenous retinochoroidal atrophy is another incomplete form of retinitis pigmentosa.     

Pigmented paravenous chorioretinal atrophy

Six patients (three men and three women, ranging in age from 19 to 65 years) with pigmented paravenous chorioretinal atrophy, a rare disorder of unknown origin, were studied for three- to 21-year periods. The diagnosis was made on the basis of the distinctive fundus appearance of bilateral, symmetrical bone corpuscular pigment accumulation exclusively along the distribution of the retinal veins. Although the fundus abnormalities can be mild or severe, retinal function tests indicated that this is a geographic and not a generalized disorder. Central visual acuities were normal. Follow-up studies showed no evidence of ophthalmoscopic or functional deterioration in five of the patients. In the sixth (Patient 6, the most severely affected), there was a mild progression of the fundus abnormality during the 21-year follow-up period.     

Pigmented paravenous retinochoroidal atrophy

A patient with paravenous pigmented chorioretinal atrophy is presented. A differential diagnosis and review of the literature are discussed. Fluorescein angiography confirms the presence of a widespread paravenous retinal pigment epithelial defect with minimally affected choroidal vasculature. The visual fields are constricted in proportion to the extent of the pigmentary abnormality. The electro-oculogram and electroretinogram show reduced responses indicative of a more extensive abnormality than is visible ophthalmoscopically. The etiology and natural course of this entity are unknown.