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儿童角膜双侧瘢痕疙瘩样肌成纤维细胞增生

Bilateral keloid-like myofibroblastic proliferations of the cornea in children.

作者信息

Holbach L M, Font R L, Shivitz I A, Jones D B

机构信息

Department of Ophthalmology, Cullen Eye Institute, Baylor College of Medicine, Houston, TX 77030.

出版信息

Ophthalmology. 1990 Sep;97(9):1188-93. doi: 10.1016/s0161-6420(90)32437-5.

Abstract

Two 6-month-old white boys developed localized multifocal white nodules of the anterior corneal stroma without evidence of antecedent trauma, inflammatory disease, or familial occurrence. The first patient had bilateral lesions initially; the second patient initially had unilateral involvement followed by a corneal nodule in the second eye at 11 years of age. Light and electron microscopic studies of the lesions in both patients were similar and disclosed stromal nodules composed of proliferating myofibroblasts, activated fibroblasts, and haphazardly arranged fascicles of collagen. Immunohistochemical studies revealed that the spindle cells within the nodules expressed immunoreactivity for vimentin and alpha-smooth muscle actin. Management options for these slowly progressive corneal lesions include lamellar versus penetrating keratoplasty and topical corticosteroids.

摘要

两名6个月大的白人男孩出现角膜前基质局限性多灶性白色结节,无前驱创伤、炎症性疾病或家族发病证据。首例患者最初为双侧病变;第二例患者最初为单侧受累,11岁时第二只眼出现角膜结节。两名患者病变的光镜和电镜研究结果相似,显示基质结节由增殖的肌成纤维细胞、活化的成纤维细胞和排列紊乱的胶原束组成。免疫组化研究显示,结节内的梭形细胞对波形蛋白和α-平滑肌肌动蛋白呈免疫反应性。对于这些进展缓慢的角膜病变,治疗选择包括板层角膜移植术与穿透性角膜移植术以及局部使用皮质类固醇。

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