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多发性复发性浆液性视网膜色素上皮脱离综合征的临床病理相关性

Clinicopathologic correlation of the multiple recurrent serosanguineous retinal pigment epithelial detachments syndrome.

作者信息

MacCumber M W, Dastgheib K, Bressler N M, Chan C C, Harris M, Fine S, Green W R

机构信息

Eye Pathology Laboratory, Johns Hopkins Medical Institutions, Baltimore, Maryland.

出版信息

Retina. 1994;14(2):143-52. doi: 10.1097/00006982-199414020-00007.

DOI:10.1097/00006982-199414020-00007
PMID:8036324
Abstract

PURPOSE

Ocular clinicopathologic correlation in a patient with bilateral multiple recurrent hemorrhagic detachments of the sensory retina and retinal pigment epithelium (RPE), which eventually led to rubeosis and loss of useful vision in one eye, is described. The clinical appearance was most consistent with multiple recurrent serosanguineous RPE detachments syndrome (which may be similar to posterior uveal bleeding, or PUB, syndrome).

METHODS

Light microscopic examination of 450 serial sections through a 3.7-mm area including the macula, optic nerve head, and superior and inferior peripapillary region of the eye that underwent diagnostic enucleation was performed. One fresh cap was used for immunohistochemical staining. Tissue from the posterior aspect of the other cap was prepared for electron microscopy.

RESULTS

Microscopic examination disclosed extensive fibrovascular proliferation in the subretinal space and within Bruch's membrane, and the presence of at least 23 choroidal blood vessels that traversed defects in Bruch's membrane. Immunohistochemical studies of inflammatory infiltrates within the choroid and fibrovascular tissue demonstrated both a B- and T-cell response.

CONCLUSION

In this case, recurrent serosanguineous detachments of the sensory retina and RPE were secondary to choroidal neovascularization with extensive fibrovascular proliferation in the subretinal space and within Bruch's membrane. Marked lymphocytic infiltration was present throughout the choroid and the fibrovascular tissue. Clinical improvement in the fellow eye after immunosuppressive therapy suggested that an immunologic component might be a causative factor, although the waxing and waning course in this condition precludes any definitive evaluation for its use in multiple recurrent serosanguineous RPE detachments syndrome.

摘要

目的

描述一名患有双侧多发性复发性感觉视网膜和视网膜色素上皮(RPE)出血性脱离的患者的眼部临床病理相关性,该疾病最终导致一只眼睛出现虹膜新生血管并丧失有用视力。临床外观与多发性复发性血清血性RPE脱离综合征(可能类似于后葡萄膜出血或PUB综合征)最为相符。

方法

对一只接受诊断性眼球摘除术的眼睛进行了光镜检查,该眼睛包括黄斑、视神经乳头以及乳头上下周边区域的3.7毫米区域的450个连续切片。使用一块新鲜组织帽进行免疫组织化学染色。另一块组织帽后部的组织用于电子显微镜检查。

结果

显微镜检查发现视网膜下间隙和布鲁赫膜内有广泛的纤维血管增生,并且至少有23条脉络膜血管穿过布鲁赫膜的缺损。对脉络膜和纤维血管组织内炎性浸润的免疫组织化学研究显示了B细胞和T细胞反应。

结论

在该病例中,感觉视网膜和RPE的复发性血清血性脱离继发于脉络膜新生血管形成,伴有视网膜下间隙和布鲁赫膜内广泛的纤维血管增生。整个脉络膜和纤维血管组织均有明显的淋巴细胞浸润。免疫抑制治疗后对侧眼的临床改善表明免疫成分可能是一个致病因素,尽管这种情况下病情的起伏变化使得无法对其在多发性复发性血清血性RPE脱离综合征中的应用进行任何明确评估。

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