视网膜血管瘤13例诊疗分析

目的通过对13例视网膜血管瘤的诊断、治疗的分析,了解视网膜脱离与视网膜血管瘤的诊断、治疗、预后的关系。方法对13例视网膜血管瘤进行回顾性分析,其中包括一例Von-HippelLindau病。结果13例病例中,11例伴有视网膜脱离,2例单纯视网膜血管瘤患者。在接受治疗的12位患者中,11例可经氩离子激光光凝治疗后视网膜脱离复位,血管瘤萎瘤。1例视网膜隆起程度高、网膜出现机化僵硬,氩离子光凝治疗后仍需冷冻治疗。患者视网膜脱离时间越长,越接近黄斑部,视力预后越差。结论正确、及时地鉴别和处理视网膜血管瘤中的视网膜脱离与视网膜血管瘤的诊断、治疗和预后紧密相关。     

视盘毛细血管瘤合并视网膜毛细血管瘤的 von Hippel 病一例

患者男 ,2 2岁。因右眼视力渐进性下降 5年 ,于 2 0 0 0年 1月 2 0日就诊。既往身体健康 ,无眼外伤及其它眼病史 ,无癫痫发作病史。全身检查 :全身皮肤无脱色斑 ,面部皮肤无丘疹 ,其余亦未见异常。眼部检查 :右眼视力 0 .2 ,矫正无提高 ,左眼视力 0 .6 ,- 2 .0 D矫正 1.0。左眼内外眼正常。右眼前节正常 ,眼底检查 :视盘表面黄白色肿物 ,隆起约 6 D,范围约3DD× 3DD,边界清楚 ,其内可见血管走行 ,无色素 ,视盘周围视网膜呈放射状皱褶 ,黄斑光反射消失 ,向鼻侧轻度移位 ,中央见水平条索状视网膜前出血灶。颞侧近中周部可见 1个视网膜血管…     

视网膜海绵状血管瘤一例

患者男 ,2 0岁。因左眼反复玻璃体积血伴视力下降 6年 ,视力丧失 1个月收入我科。否认其它病史 ,双眼近视 - 6 .5 D。全身检查和实验室检查未发现异常。眼科检查 :视力 :右眼矫正0 .5 ,左眼眼前手动。右眼外眼及眼底检查正常 ,左眼眼前节正常 ,玻璃体内大量血性混浊 ,B型超声检查提示玻璃体内大量不均匀高密度回声 (图 1)。拟诊 :左眼玻璃体积血。行玻璃体切割手术。手术中发现视网膜颞下支静脉旁紫红色葡萄串样隆起物 ,表面有少许白色纤维样物附着 ,隆起物表面有活动性出血。给予电凝止血 ,于隆起物周围氩激光光凝 ,出血缓解但未停止。手…     

氩激光治疗视网膜血管瘤三例

氩激光治疗视网膜血管瘤三例郑文１病例报告例１女，２０岁，右眼视力下降２０多天来诊。既往史无特殊。其姐亦有类似眼疾失明。全身检查：颅脑ＣＴ未见异常，Ｂ超检查提示：右肝后叶８．５ｍｍ血管瘤；双肾及胰脾无异常。眼科检查：右眼视力０．１，玻璃体轻度混浊。视乳...     

视网膜血管瘤病1例

我院１９７１年曾发现１例并追踪观察２５ａ,现将此病例报告如下。１　临床资料患者,女,２２ａ,未婚,于１９７１年４月２８日来诊。主诉双眼时有不适感０－５ａ,右眼较重。否认眼痛、头痛或头收稿　１９９９０８２０本文编辑　张知达作者单位　４５５００４　鞍钢铁西医院白楼门诊眼科晕,无恶心、呕吐及视物不清。眼部检查：远近视力双眼均１－０,无屈光异常。双外眼无异常,屈光间质无混浊,左眼底无异常。右眼底视盘正常,自盘鼻侧发出两支粗大网膜血管,横行达鼻侧网膜周边都。这两支血管颜色暗红,上枝略细似为动脉,下枝略…     

视网膜血管瘤二例

例 1　女 ,38岁 ,因右眼视力下降半年 ,于 1997年来我院就诊。否认家族眼病史、脑病史及肿瘤史。全身检查无异常。颜面部皮肤未见血管瘤改变。视力 :右眼 0 .8,左眼 1.2。双眼眼前节无异常 ,右眼视盘表面见一约 1.5 PD× 1.3PD大小肿物 ,橘红色 ,覆盖视盘 ,突出于玻璃体腔内 ,表面光滑 (图 1)。右眼 B型超声检查 :视神经表面有一突出于玻璃体腔的实性肿块。荧光素眼底血管造影检查 :12 s瘤体内出现强荧光 ,13.5 s视网膜中央动脉开始充盈 ,瘤体内荧光素同时迅速充盈 ,到早期动静脉期瘤体已完全充盈成一强荧光团 (图 2 ) ,晚期瘤体周围有荧…     

Retinal angioma associated with von Hippel‐Lindau disease

Von Hippel‐Lindau disease is a multi‐system disorder that can produce hamartomas (benign tumour‐like nodules) of the eyes, skin and nervous system. Retinal capillary angioma is a common ocular association of this congenital phakomatosis that may result in blinding sequelae, if not managed appropriately. We present a case of retinal angioma associated with von Hippel‐Lindau disease and discuss the ocular and systemic signs, diagnosis and management. The optometrist is of particular importance in screening for this disorder, as it is often first detected in a routine eye examination.     

Verteporfin photodynamic therapy of six eyes with retinal capillary haemangioma

Acta Ophthalmol. 2010: 88: e334–e340

Abstract.

Purpose: Single‐centre consecutive interventional case series by retrospective chart review to evaluate the efficacy of verteporfin (Visudyne?) photodynamic therapy (PDT) of retinal capillary haemangioma (RCH). Methods: Following an initial period of observation, six eyes of five patients with RCH (juxtapapillary 3 and extrapapillary (EP) 3) received 1–3 sessions of standard verteporfin PDT upon the development of progressive vision‐threatening complications. Four of the five patients had von Hippel‐Lindau (VHL) disease. Follow‐up included documentation of best‐corrected Snellen visual acuity (BCVA), tumour regression, and presence or absence of subretinal fluid (SRF) and/or lipid exudation as assessed by dilated fundus examination (DFE), fundus photos, and optical coherence tomography (OCT). These parameters were documented at 1 week, 1 month, and 3 months following each PDT session and up to 32 months following the first PDT. Results: All eyes showed favourable response to PDT as defined by tumour regression or stabilization as well as improvement of SRF and lipid exudation. BCVA improved or stabilized in three eyes. Three eyes required PDT retreatment for recurrent SRF. Epiretinal membrane (ERM) worsened in three eyes, requiring vitreoretinal surgery at a median of 6 months following PDT. Conclusions: PDT is a moderately effective treatment for juxtapapillary and EP RCH. In this series, PDT resulted in tumour regression or stabilization as well as in the improvement of SRF and lipid exudation in all cases. However, stabilization or improvement of visual acuity was observed in only 50 per cent of the cases. The treatment benefits may be limited by pre‐existing macular changes and worsening of ERM. A larger prospective study is necessary to validate these findings.     

Clinical and molecular features of familial and sporadic cases of von Hippel‐Lindau disease from Mexico

Background: von Hippel‐Lindau disease (VHL) is an uncommon autosomal dominant condition predisposing to the development of tumours in a variety of body organs and caused by germline mutations in VHL, a tumour suppressor gene located on 3p. Up to 60% of VHL patients show ocular involvement with retinal hemangioblastoma being the most common observed lesion. In this study, we describe the clinical and genetic characteristics of two familial and one apparently non‐familial case of VHL ascertained at our institution. Methods: Clinical evaluation included ophthalmologic examination and imaging exams for tumours identification; molecular analysis consisted of PCR amplification of the complete VHL gene coding sequence (three exons) and automated nucleotide sequencing. Results: A total of eight affected subjects were demonstrated to carry a causative mutation in VHL. Affected subjects from family #1 had a c.245G > C change, predicting a p.R82P substitution, affected individuals from family #2 were shown to have a c.266T > C change, leading to a p.L89P missense substitution, whereas the apparently non‐familial case had a c.298‐299insA mutation. One subject from family #2 was a non‐penetrant carrier. No ocular anomalies were found in two adult affected subjects carrying the p.L89P mutation. Conclusion: Considerable interfamilial and intrafamilial clinical variability as well as one instance of non penetrance were recorded in these VHL disease cases. Three different mutations were demonstrated, including the c.298‐299insA one base insertion, which has been previously described in two unrelated families from our country. Although additional studies are needed, our data suggest that this insertion could be a ‘founder’ mutation.     

Clinical and genetic screening in patients with capillary retinal angioma

Summary Capillary retinal angiomas are rare vascular tumors that frequently occur in von Hippel-Lindau syndrome (vHL) but may also be sporadic. In all patients presenting with this tumor a thorough search for other vHL-associated lesions must be performed. After identification of the vHL gene on the short arm of chromosome 3 (3p25–26), the diagnosis is supported by molecular genetic analysis. Patients: In 20 patients with retinal angioma a clinical search for other manifestations of vHL was performed. In 5 patients only one angioma was present. In all patients molecular genetic tests for a mutation of the vHL gene were performed by SSCP and direct sequencing. Results: In 16 (80 %) patients vHL was present, and in 15 it could be diagnosed by clinical findings or a positive familiy history. Organ lesions in vHL patients were CNS hemangioblastoma in 10 (63 %), pancreatic cysts in 7 (43 %) and renal cysts in 7 (43 %) patients. In two patients (13 %) renal carcinoma could be detected; in one patient a pheochromcytoma was present. A mutation could be detected in all 15 patients with clinically confirmed vHL. In three patients a new mutation of vHL disease was diagnosed genetically. In one of these patients a single retinal angioma was the only sign of vHL. Conclusion: In patients presenting with capillary retinal angioma a careful search for other vHL lesions has to be performed. A mutation of the vHL gene can be detected in the majority of patients; thus, moleculargenetic testing is a powerful tool for diagnosis and detection of asymptomatic gene- carriers.      

视网膜中央静脉阻塞并发渗出性视网膜脱离的临床特征

目的探讨视网膜中央静脉阻塞并发渗出性视网膜脱离患者的临床特征.方法回顾性分析视网膜中央静脉阻塞并发渗出性视网膜脱离患者的一般情况及视力、眼压、眼底、荧光素眼底血管造影、眼部超声波等检查资料.结果15例患者中,男性8例,女性7例;年龄18～42岁,平均25岁.5例合并新生血管性青光眼.患者就诊时的视力：光感至0.05.眼底检查显示全视网膜散在出血、视网膜静脉迂曲,黄斑区有明显的水肿和出血,在水肿和脱离区的边缘可见大片渗出.坐位时视网膜脱离为2～5个钟点范围.全部患者均未见视乳头血管吻合支的形成.荧光素眼底血管造影显示大片出血遮蔽荧光或可见后极部毛细血管无灌注区.超声波测量有5只眼平均眼轴长为22.98 mm.结论渗出性视网膜脱离是视网膜中央静脉阻塞的少见、严重并发症.多见于青年患者,易并发新生血管性青光眼,其眼底及荧光素眼底血管造影检查具有特征性.     

儿童孔源性视网膜脱离的临床特征和手术疗效分析

目的探讨儿童孔源性视网膜脱离的临床特征和手术疗效。方法为系列病例研究。对近10年首诊眼科≤14岁的33例（36只眼）儿童孔源性视网膜脱离患者临床资料进行回顾性分析。结果33例（36只眼）中,男性26例（78．8％）,女性7例。患儿首诊年龄中位数为12岁。双侧孔源性视网膜脱离10例（30．3％）;合并对侧眼异常22例（66．7％）。31只眼（86．1％）具有至少1种发生孔源性视网膜脱离的危险因素。有眼部钝挫伤史13只眼（36．1％）,合并先天性或发育性眼部结构异常11只眼（30．6％）,有高度近视和其他内眼手术史者各7只眼（19．4％）,合并眼后节炎性反应3只眼（8．3％）。术前发生黄斑脱离28只眼（77．8％）。随诊时间中位数为12个月。每只眼经历1～5次手术,平均1．9次。最终随诊时,硅油仍然在位4只眼,29只眼（90．6％）视网膜复位。预示术后视力差的因素：术前视力为手动或更低（P=0．001）,黄斑脱离（P=0．003）,增生性玻璃体视网膜病变≥C级（P=0．000）,需要行玻璃体切除术（P=0．002）和使用硅油（P=0．005）。结论有眼部钝挫伤史和先天性或发育性眼部结构异常是儿童孔源性视网膜脱离最常见的危险因素。现代玻璃体视网膜手术能使大多数患儿视网膜复位。预示患儿术后视力差的因素与成人相同。对具有发生孔源性视网膜脱离危险因素的儿童应定期随诊。（中华腰科杂志,2008,44：20-24）     

自发性黄斑区出血性视网膜脱离的临床特征

目的 分析自发性黄斑区出血性视网膜脱离的临床特征，提高对本病的诊治水平。 方法 对1998年9月至2003年3月我院眼底病科临床确诊的黄斑区视网膜下出血，范围大于4 DD×5 DD的患者，共23例（23只眼）的连续临床资料进行回顾性分析。经荧光素眼底血管造影 （FFA）、吲哚青绿血管造影（ICGA）及B型超声波检查，进行病因分析，随诊观察视力及眼底变化。 结果 23例患者均表现为视力突然减退至0.2以下或光感。23只眼中7只眼确诊为老年性黄斑变性的脉络膜新生血管（CNV）；5只眼为息肉状脉络膜血管病变（PCV）；2只眼为视网膜大动脉瘤；9只眼病因不明。23只眼中3只眼积血吸收，黄斑中心凹无瘢痕形成，视力恢复0.4～0.8；12只眼黄斑出血区形成机化瘢和色素增生，视力数指/眼前～0.2；另外8只眼并发玻璃体积血，其中3只眼行玻璃体切割术，黄斑区大片机化及瘢痕，视力数指/66 cm～0.2；5例视力丧失。 结论 自发性出血性视网膜脱离多发生于老年人，视力突然下降或丧失。出血形态表现为局灶性或弥漫性，因黄斑区组织严重损害，大部分病例预后差。 (中华眼底病杂志, 2006, 22: 228-231)     

独眼视网膜脱离的临床特征及其治疗观察

目的探讨独眼视网膜脱离的临床特征及其治疗效果。方法复习西京医院眼科2002年1月至2007年5月间住院的、资料完整的、一眼已失明而残存眼(独眼)发生视网膜脱离的病例资料,分析对侧眼失明原因,总结独眼视网膜脱离的临床特点,观察其治疗效果。共包括105例独眼视网膜脱离患者,其中42例首次行巩膜外垫压(包括环扎)术,44例首次接受玻璃体手术,8例行单纯玻璃体内注气(C3F8)术,4例激光光凝术治疗;7例未行特殊治疗而失访。经治疗的98例患者随访6个月至1年。结果本组接受手术治疗的94例患者中一次手术后有72例视网膜复位(76.6%)。其中42例行巩膜外垫压术,一次手术视网膜复位率为74.0%(31/42);44例接受玻璃体切除术,复位率为82.0%(36/44);8例行玻璃体内注气术后,5例视网膜复位(62.5%)。经再次手术后,最终视网复位率为93.6%(88/94只眼)。接受治疗的98例患者中,66.3%(65/98只眼)术后视力提高。结论尽管独眼视网膜脱离患者的疾病特点与一般视网膜脱离无明显差别,但在社会学和人文方面有其特殊性,在选择治疗时,建议采取尽量保守的手术方案更符合患者长远利益;对经选择的病例,巩膜外垫压术的远期效果可靠。     

近视盘视网膜毛细血管瘤的临床特征分析

目的观察近视盘视网膜毛细血管瘤的临床特征方法回顾分析18例近视盘毛细血管瘤19只眼的临床资料、16只眼的荧光素眼底血管造影（FFA）以及7只眼的随访观察资料。结果18例患者中，男女比为1：2，平均年龄为28.9岁。3例4只眼合并周边视网膜瘤。19只眼血管瘤中心均位于视盘的边缘以外，以颞下象限为最多（7/19），血管瘤大小为1.0-2.5个视盘直径（DD），颜色以红色为主（12/19）。16只眼FFA检查显示13只眼造影晚期有“排空”现象。长期随访的7只眼（其中5只眼经过激光光凝治疗）视力均下降。初诊时无渗出性视网膜脱离的6只眼（其中4只眼、未经过治疗的只眼）在平均随访的54.4个月后均未发生视网膜脱离。结论近视盘视网膜毛细血管瘤多呈类似椭圆形、橙黄色或红色包块。瘤体的中心位于视盘的边缘外。患者多在青壮年期出现视力下降的症状，有症状者瘤体周围均有不同程度的渗出、视网膜水肿。视力下降较缓慢，治疗效果差。FFA检查有助于本病的诊断和鉴别诊断。（中华眼底病杂志，2004，20：1-4）     

有裂孔的视网膜变性的临床特征和氩激光治疗

目的 探讨有裂孔的视网膜变性的临床特征和氩激光治疗效果。 方法 回顾性分析本院210例224只眼相应视网膜变性的氩激光治疗资料，并与同期尚无裂孔的视网膜变性氩激光治疗对照。 结果 有裂孔的视网膜变性患者，小于60岁者89.7%，男性53.3%，女性46.7%，格子样变性65.6%，变性范围≤1个象限者87.5%，卵圆形裂孔60.7%，伴有局限性视网膜浅脱离者23.7%。与尚无裂孔的视网膜变性患者相比，≥35岁、囊样变性、视网膜纵向小皱襞、有自觉症状的患眼构成比明显偏高，而氩激光视网膜脱离预防性治疗对已出现局限性孔源性视网膜脱离的视网膜变性患者疗效明显偏低(P<0.01)。 结论 有裂孔的视网膜变性常见于青壮年，多数患者为1个象限内的格子样变性；裂孔多数为卵圆形，多不伴有视网膜脱离；裂孔没有明显的性别差异，多数没有自觉症状。不伴有视网膜脱离的视网膜单纯性裂孔的视网膜变性，氩激光视网膜脱离预防性治疗可获得满意疗效。 (中华眼底病杂志, 2006, 22: 39-41)