Pang Claudine E, Shields Carol L, Jumper J Michael, Yannuzzi Lawrence A
Vitreous Retina Macula Consultants of New York, New York, New York; LuEsther T. Mertz Retinal Research Center, Manhattan Eye, Ear and Throat Hospital, New York, New York.
Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.
Am J Ophthalmol. 2014 Dec;158(6):1253-1261.e2. doi: 10.1016/j.ajo.2014.08.031. Epub 2014 Aug 28.
To describe the nature and evolution of paraneoplastic cloudy vitelliform submaculopathy in patients with primary vitreoretinal lymphoma and propose a mechanism for its development and course.
Retrospective, observational case series.
Three patients presenting with unilateral cloudy vitelliform submaculopathy based on clinical examination, fundus autofluorescence, fluorescein angiography, and spectral-domain optical coherence tomography (SD OCT) imaging and ultimately diagnosed with primary vitreoretinal lymphoma and/or primary central nervous system lymphoma were analyzed.
In all 3 patients, cloudy vitelliform submaculopathy appeared with hazy indistinct yellow subretinal material resembling the vitelliform lesions found in acute exudative paraneoplastic polymorphous vitelliform maculopathy, although with less distinct appearance and without intense hyper-autofluorescence. In all 3 patients, cloudy vitelliform submaculopathy was transient, showed spontaneous regression within 3 months, and preceded the diagnosis of lymphoma, suggestive of a paraneoplastic process. The diagnosis of primary vitreoretinal lymphoma and/or primary central nervous system lymphoma was made within 6 months with classic features of new intraretinal or sub-retinal pigment epithelium infiltration of lymphoma in the peripheral retina (n = 2) and hyperintense lesions on brain magnetic resonance imaging (n = 2). With SD OCT imaging, the cloudy vitelliform subretinal lesions appeared as hyperreflective debris above the retinal pigment epithelium band in all 3 eyes, and were associated with an irregularly thickened and rippled retinal pigment epithelium band in 2 eyes. Resolution of the cloudy submacular lesions resulted in outer retinal atrophic changes in all 3 eyes.
Paraneoplastic cloudy vitelliform submaculopathy, a form of lymphoma-associated retinopathy, can precede the diagnosis of primary vitreoretinal lymphoma or primary central nervous system lymphoma and can regress spontaneously, leaving outer retinal abnormalities.
描述原发性玻璃体视网膜淋巴瘤患者副肿瘤性混浊卵黄样黄斑下病变的性质和演变，并提出其发生发展及病程的机制。
回顾性观察病例系列。
分析3例基于临床检查、眼底自发荧光、荧光素血管造影及光谱域光学相干断层扫描（SD OCT）成像表现为单侧混浊卵黄样黄斑下病变，最终诊断为原发性玻璃体视网膜淋巴瘤和/或原发性中枢神经系统淋巴瘤的患者。
在所有3例患者中，混浊卵黄样黄斑下病变表现为视网膜下模糊不清的黄色物质，类似于急性渗出性副肿瘤性多形性卵黄样黄斑病变中的卵黄样病灶，但其外观不那么清晰，且无强烈的高自发荧光。在所有3例患者中，混浊卵黄样黄斑下病变是短暂性的，在3个月内自发消退，且先于淋巴瘤的诊断，提示为副肿瘤性过程。原发性玻璃体视网膜淋巴瘤和/或原发性中枢神经系统淋巴瘤的诊断在6个月内作出，其具有典型特征，即周边视网膜有新的视网膜内或视网膜色素上皮下淋巴瘤浸润（2例），以及脑磁共振成像上有高信号病变（2例）。通过SD OCT成像，所有3只眼中混浊的卵黄样视网膜下病变表现为视网膜色素上皮带上方的高反射性碎屑，2只眼中伴有视网膜色素上皮带不规则增厚和波纹。混浊的黄斑下病变消退后，所有3只眼均出现外层视网膜萎缩性改变。
副肿瘤性混浊卵黄样黄斑下病变是淋巴瘤相关性视网膜病变的一种形式，可先于原发性玻璃体视网膜淋巴瘤或原发性中枢神经系统淋巴瘤的诊断出现，且可自发消退，遗留外层视网膜异常。
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