Purtscher样视网膜病变的临床观察.pdf

Purtscher样视网膜病变是一种少见的视网膜血管性疾病，该文分析了13例Purtscher样视网膜病变的临床特征。典型的眼底表现包括 Purtscher斑22眼、棉絮斑22眼、视网膜出血13眼、黄斑水肿22眼、视盘水肿12眼和假樱桃红斑10眼。荧光素血管造影异常表现包括毛细血管无灌注区21眼，毛细血管前闭塞22眼。13例患者中有9例使用糖皮质激素、免疫抑制剂，4例使用改善循环、营养神经等治疗。随访2月至5年，4例患者(6眼)在随访期间出现神经上皮萎缩并持续低矫正视力(0.1)。基于文献回顾，讨论了Purtscher样视网膜视力恢复不佳可能与黄斑水肿及无灌注区形成有关。关键词Purtscher样视网膜病变；Purtscher斑；棉绒斑；黄斑水肿；毛细血管无灌注区Clinical observation of Purtscher-like retinopathyWANG Bo,ZHANG Yujie,JIN Rui,YANG Yaojuan,HUA Jiajia,CHANG Hongmin,LI Chuanbao(Affiliated Hospital of Jining Medical University,Jining 272029,China)Contributions:1)Conception and design:LI Chuanbao,WANG Bo;2)Administrative support:LI Chuanbao;3)Provision of study materials:ZHANG Yujie,JIN Rui;4)Collection and assembly of data:WANG Bo,YANG Yaojuan,CHANG Hongmin;5)Data analysis and interpretation:WANG Bo,HUA Jiajia;6)Manuscript writing:WANG Bo;7)Final approval of manuscript:LI ChuanbaoAbstract Purtscher-like retinopathy is a rare retinal vascular disease.In this study,the clinical characteristics of 13 cases of Purtscher-like retinopathy were analyzed.Typical fundus abnormalities included Purtscher flecken(22 eyes),论著论著 电子全文388Keywords Purtscher视网膜病变(Purtscher retinopathy,PuR)于1910年由Otmar Purtscher首次描述1，是非眼部损伤相关的脉络膜视网膜病变，视网膜表现包括棉绒斑、伴或不伴有中低数量视网膜出血、Purtscher斑、黄斑水肿2，50%病例会出现特征性的Purtscher斑，多分布在黄斑区和视盘鼻侧，为边界清楚、位于小动脉周围约50 m范围内的视网膜内层白斑，形态不一，大小从1/4 PD到数个PD，黄斑周围Purtscher斑较多时，黄斑呈假性樱桃红斑样改变，其与毛细血管前小动脉的阻塞有关3。如果在非创伤的情况下出现类似表现，被称为Purtscher样视网膜病变(Purtscher-like retinopathy,PLR)。其与多种临床疾病相关，包括系统性红斑狼疮4、急性胰腺炎5、肾功能衰竭6、先兆子痫7、结缔组织病8、脂肪栓塞综合征9、骨科手术10、淋巴增生性疾病和骨髓移植11、眼眶和鼻道内及周围注射类固醇12、溶血性尿毒症综合征13、婴儿摇晃综合征9,新型冠状病毒感染等14。PLR的发病机制及治疗仍存在争议，现对本院2014年1月至2021年9月诊断的13例(22只眼)PLR的临床资料进行回顾性分析，探讨其与视力预后相关的高危因素及相应治疗cotton-wool spots(22 eyes),retinal hemorrhages(13 eyes),macular edema(22 eyes),swelling of optic disk(12 eyes),and falsecherry red spots(10 eyes).The abnomal manifestations of fluorescein angiography included non-perfusion area of capillaries(21 eyes),and precapillary occlusion(22 eyes).Among 13 patients,9 patients were treated with glucocorticoids and immunosuppressive drugs,and 4 patients were treated with circulation-improving and neurotrophicdrugs.During the follow-up period of two months to five years,four patients(six eyes)experienced neuroepithelial atrophy and persistent low vision(20 PD)，5.2(520 PD)，5.3(20 PD),5.2(520 PD),5.3(5PD),6 hyperreflectivity of the inner retinal layer;OD oculus dexter;OS oculus sinister;OU oculus uterque;SLE Systemic Lupus Erythematosus;AP Acute Pancreatitis;ARF Acute Renal Failure.390FFA检查：可见视盘荧光渗漏，视盘周围及黄斑区广泛毛细血管无灌注区(图1B)。诊断：双眼Purtscher样视网膜病变。给予改善循环、营养神经等治疗。3个月后患者视力无明显改善，眼底可见视盘色淡，视盘周围有部分小动脉白线，视网膜浅层见少许出血灶，视网膜色素紊乱。FFA检查：可见右眼视盘周围小动脉阻塞，后极部广泛无灌注区，视盘荧光素渗漏(图1C)。左眼眼底视盘边界欠清，血管壁荧光素着染，周围散在无灌注区，黄斑区中心凹周围残留部分毛细血管(图1E)。图1 眼底彩色图像和FFA图像Figure 1 Fundus photographs and fuorescein angiography of the posterior poles(A)右眼底彩色图像，视盘周围大量棉絮斑(黑色箭头)及小片状出血(白色箭头)，黄斑区有黄白色Purtscher斑(红色箭头)，假樱桃红斑；(B)右眼FFA图像,可见视盘强荧光渗漏(红色箭头)，视盘周围及黄斑区广泛毛细血管无灌注区(白色箭头)；(C)随访3月后右眼FFA图像，可见视盘强荧光渗漏(红色箭头)，视盘周围及黄斑区广泛无灌注区，血管壁荧光素着染。(D)左眼底彩色图像，视盘周围大量棉絮斑(黑色箭头)，视盘周围及黄斑区可见黄白色Purtscher斑(红色箭头)。(E)随访3月后左眼FFA静脉期图像，可见视盘强荧光(视盘水肿)，血管壁荧光素着染(红色箭头)，周围散在无灌注区(白色星号)，黄斑区中心凹周围残留部分毛细血管,血管末端可见较多点状强荧光区(白色箭头)。(A)Fundus photographs of the oculus dexter,peri-papillary cotton-wool spots(black arrows),purtscher fleken(red arrows),scattered hemorrhage(white arrows),false cherry red spots.(B)Fluorescein angiography of the oculus dexter,papillary hyper fuorescence(red arrow)and peri-papillary non-perfusion areas(white arrows).(C)Fluorescein angiography of the oculus dexter afer follow-up 3 months,papillary hyper fluorescence(red arrow)and peri-papillary non-perfusion areas,fluorescein staining of the vessel wall.(D)Fundus photographs of the oculus sinister,peri-papillary coton-wool spots(black arrows),Purtscher feken(red arrows).(E)Fluorescein angiography of the oculus sinister afer follow-up 3 months,papillary hyper fuorescence(papillary edema),capillary staining(red arrows),peri-papillary non-perfusion areas(white asterisks),punctate high-fuorescence areas were seen at the end of the capillaries vessels(white arrows).病例2：患者女性，32岁，因“系统性红斑狼疮”收入风湿免疫科，双眼视力下降5d请眼科会诊，眼部检查：矫正视力右眼指数/鼻侧20 cm，左眼0.02，双眼眼压正常，结膜无充血，角膜透明，瞳孔圆，直径约4 mm，对光反射迟钝，眼底视盘界清，色可，视网膜动脉细，后极部视网膜见片状出血及棉绒斑(图2A、B)，FFA检查：可见视盘荧光渗漏，视盘周围及黄斑区广泛毛细血管无灌注区，未见新生血管(图2C、D)。OCT显示 Purtscher 斑和棉绒斑处的内层视网膜呈高反射(图2E、F)。诊断：双眼Purtscher样视网膜病变。给予糖皮质激素、免疫抑制剂、抗凝、改善循环、营养神经等治疗，针对眼部症状给予眼底激光治疗。完善全视网膜激光光凝术3个月后患者矫正视力提高至0.04，眼底可见视盘色淡，视盘周围有部分小动脉白线，视网膜浅层见少许斑片状出血(图2G、H)。病例3：患者男性，42岁，因高强度体力劳动后左眼视力下降1 d，就诊我院眼科，右眼自幼视力差。眼部检查：矫正视力右眼光感，左眼0.1，双眼眼压正常，眼前节未见明显异常，左眼眼底可见后极部视网膜片状Purtscher斑及棉绒斑(图3A)。FFA检查：后极部未见明显无灌注区(图3B)。OCT：黄斑区内层视网膜Purtscher样视网膜病变的临床观察 王波，等391图2 眼底彩色图像、OCT及FFA图像Figure 2 Fundus photographs,OCT and fuorescein angiography of the posterior poles(A，B)眼底彩色图像，视盘界清，色可，视网膜动脉细，后极部黄斑区视网膜见片状出血(白色箭头)、棉绒斑(黑色箭头)及Purtscher斑(红色箭头)。(C，D)双眼FFA可见视盘荧光渗漏，广泛毛细血管无灌注区。(E，F)双眼OCT：棉絮斑对应视网膜神经纤维层的高反射(白色箭头)和Purtscher斑对应内核层增厚(白色箭头)。(G，H)双眼全视网膜激光光凝后出血较前吸收，棉絮斑和Purtscher斑较前部分消退。(A,B)Fundus photographs of the oculus uterque,optic disc was mildly pale with sharp margins and proximal occlusion of the retinal arteries，scattered hemorrhage(white arrows)，peri-papillary cotton-wool spots(black arrows),purtscher fleken(red arrows).(C,D)Fundus angiography of the oculus uterque,papillary hyper fuorescence and peri-papillary non-perfusion areas.(E,F)Macular OCT，coton-wool spots:hyperrefectivity in the retinal nerve fber layer(white arrow).purtscher feken:thickening of the inner nuclear layer(white arrows).(G,H)Afer panretinal laser photocoagulation,scatered hemorrhage was absorbed and coton-wool spots and purtscher feken were partially resolved.结构紊乱，呈高反射(图3C)。诊断：左眼Purtscher样视网膜病变。给予皮质糖皮质激素、改善循环、营养神经等治疗，2个月后患者视力提高至1.0，眼底可见Purtscher斑及棉绒斑均较前明显减少(图3D)。2 讨论PLR是一种罕见的视网膜血管性疾病，发病率约为每百万人口0.24例，其中60%为双眼发病4，其发病机制、诊断及治疗仍存在争议，结合其特征性临床表现目前公认的诊断标准为：1)有可能导致PLR的相关全身疾病；2)黄斑区Purtscher斑；3)视盘周围棉絮斑；4)后极部火焰状或点状视网膜出血；5)视盘和(或)黄斑水肿；6)FFA见视网膜毛细血管闭塞及无灌注区。至少符合1)2)项再加上后四项中任何一项可诊断6。PLR的病理生理机制尚不明确，已有的报道包括淋巴漏学说、栓塞学说、血管痉挛学说、轴浆流阻断学说、补体介导的白细胞凝集等9，其中公认392的理论一是栓塞学说，即中等大小的栓子如空气、脂肪、白细胞聚集体、血小板和纤维蛋白等导致毛细血管前小动脉栓塞和视网膜神经纤维层微血管梗死6。由于视网膜后极部存在毛细血管吻合较少，因此病变部位多位于后极部的内丛状层及内核层中15(如典型病例3的OCT所示)，多见于球周注射、羊水栓塞等情况时发生。二是补体介导的白细胞凝集，原发疾病导致部分或完全视网膜血管闭塞，出现棉绒斑及Purtscher斑，表现为视野缺损和视力下降等症状，进而激活一系列凝血级联反应，阻碍毛细血管流动，激活补体C5通路，引起白细胞、血小板和纤维蛋白血管内聚集导致内皮细胞破坏，表现为视网膜血管闭塞白线样改变、视网膜内层水肿，严重时出现视网膜出血10,14,16(如典型病例2所示)，多在系统性红斑狼疮、胰腺炎、肾功能衰竭时发生。其他代表性理论还有血管内容量激增，例如视网膜静脉阻塞、高黏滞症、颅内压升高，导致筛板水平的毛细血管闭塞，毛细血管内皮损伤和失调，诱导血管病变17。影响PLR预后的可能因素仍不明确，包括视盘水肿、黄斑水肿、视网膜血管渗漏、脉络膜灌注不足、视网膜内层受损和严重的毛细血管灌注缺失等18。FFA、OCT及OCTA可作为诊断及随访的重要手段，OCT可显示此类患者的视网膜神经纤维层的棉绒斑以及内核层的Purtscher斑15，OCTA可在早期识别视网膜缺血性损伤，并有助于定位血管闭塞的水平19，FFA中可显示视网膜毛细血管灌注状态14有助于评估病情的严重程度及预后。由于诱发因素的多样化，PLR的治疗尚无共识，目标是识别和控制原发疾病，积极治疗眼部病变，挽救视力，发病2周内是最佳干预时机20。研究表明，急性期视盘及黄斑水肿是视力下降的首要原因。随着黄斑水肿的加剧和持续时间的延长，会导致晚期黄斑神经纤维层的萎缩4。在相关文献中报道了糖皮质激素治疗可改善视力预后(如典型病例3)，糖皮质激素可以稳定受损神经元膜和微血管通道、加速神经纤维恢复和防止继发于补体激活的粒细胞聚集18。本文13例病例中7例患者出现黄斑区视网膜水肿，4例使用糖皮质激素治疗的患者较其他3例随访中最佳矫正视力明显改图3 眼底彩色图像、OCT及FFA图像Figure 3 Fundus photographs,OCT and fuorescein angiography of the posterior poles (A)左眼视盘界清，色可，视盘周边大量棉绒斑(黑色箭头)，视盘及黄斑周边大量Purtscher斑(红色箭头)；(B)FFA检查可见后极视网膜未见毛细血管无灌注区；(C)OCT可见棉絮斑(白色箭头)和Purtscher斑(红色箭头)对应处的视网膜内层高反射；(D)随访2月后，右眼后极部的棉绒斑及Purtscher斑较前明显减少。(A)Fundus photographs，optic disc was mildly pale with sharp margins，peri-papillary coton-wool spots(black arrows),purtscher feken(red arrows).(B)Fundus angiography,posterior retina without peri-papillary non-perfusion areas.(C)Macular OCT，coton-wool spots:hyperrefectivity in the retinal nerve fber layer(white arrow).purtscher feken:thickening of the inner nuclear layer(red arrows).(D)Afer follow-up 2 months,coton-wool spots and purtscher feken were obviously resolved.Purtscher样视网膜病变的临床观察 王波，等393善。推测合并严重黄斑水肿的病例采用糖皮质激素的短期治疗，可能改善预后。长期随访中发现后极部毛细血管无灌注区的形成及范围，是远期视力预后不佳的重要原因(如典型病例1、2)，应积极给予激光治疗。综上所述，PLR是一种罕见的血管闭塞性视网膜疾病，其视力预后与病程中是否出现黄斑水肿或毛细血管无灌注区有关，及早干预可改善视力预后。利益冲突 所有作者均声明不存在利益冲突开放获取声明本 文 适 用 于 知 识 共 享 许 可 协 议(C re at i v e Commons)，允许第三方用户按照署名(BY)-非商业性使用(NC)-禁止演绎(ND)(CC BY-NC-ND)的方式共享，即允许第三方对本刊发表的文章进行复制、发行、展览、表演、放映、广播或通过信息网络向公众传播，但在这些过程中必须保留作者署名、仅限于非商业性目的、不得进行演绎创作。详情请访问：https:/creativecommons.org/licenses/by-nc-nd/4.0/。参考文献1.Purtscher O.Noch unbekannte befunde nach schadeltrauma.Ber Dtsch Ophthalmol Ges,1910,36:294-301.2.Stroh I,Nguyen A,Channa R.Purtscher-like retinopathy in hemolytic uremic syndromeJ.JAMA Ophthalmol,2019,137(1):e183911.3.Miguel AI,Henriques F,Azevedo LF,et al.Systematic review of purtscher s and purtscher-like retinopathiesJ.Eye(Lond),2013,27(1):1-13.4.Ishibashi T,Wakabayashi T,Nishida K.Purtscher-like retinopathy associated with systemic lupus erythematosus observed using wide-feld OCT angiographyJ.Ophthalmol Retina,2019,3(1):76.5.Narang S,Aggarwal P,Bhattacharyya A,et al.Acute pancreatitis presenting as purtscher-like retinopathyJ.Pancreatology,2022,22(2):333-334.6.Pinto C,Fernandes T,Gouveia P,et al.Purtscher-like retinopathy:ocular fndings in a young woman with chronic kidney 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