彼得斯异常患者后弹力层的一种新的组织病理学发现：病例报告及文献综述
A novel histopathologic finding in the Descemet's membrane of a patient with Peters Anomaly: a case-report and literature review.
作者信息
Ni Wei, Wang Wei, Hong Jing, Zhang Pei, Liu Cong
机构信息
Department of Ophthalmology, Peking University Third Hospital, Beijing, 100191, China.
Key Laboratory of Vision Loss and Restoration, Ministry of Education, Beijing, China.
出版信息
BMC Ophthalmol. 2015 Oct 23;15:139. doi: 10.1186/s12886-015-0131-y.
BACKGROUND
Peters anomaly is a rare developmental abnormality of the anterior segment of the eye and is one of the main causes of congenital corneal opacities. Typically, histopathology of Peters anomaly shows immature or absent Descemet's membrane and attenuated endothelial cells in the area of the corneal opacity, in addition to thinning or absence of Bowman's membrane and defects in the posterior stroma. In this report, we present a novel histopathological finding, which has not been previously reported, in the Descemet's membrane of a patient who is clinically diagnosed with Peters anomaly.
CASE PRESENTATION
A 7-years old female child with developmentally delayed was born of a normal pregnancy, labor, and delivery. Apparent bilateral corneal opacifications were present at birth. On ophthalmologic examination, the child had a visual acuity of FC/20 cm in the right eye and that of FC/10 cm in the left one. Horizontal nystagmus and congenital cataract were found in both eyes. Slit-lamp examination revealed bilateral central corneal opacities which covered the iris and pupils. High-frequency UBM and AS-OCT both showed a shallow anterior chamber with multiple areas of iridocorneal adhesions and no corneal lenticular touch in each eye. A corneal specialist performed a penetrating keratoplasty with extra-capsular cataract extraction and intraocular lens implantation. Histopathologic procedures were conducted on the host corneal button, including Hematoxylin-Eosin stain and Periodic Acid-Schiff stain. All the sections were examined by light microscopy.
CONCLUSION
The "multiple-layer" structure of the Descemet's membrane described in our case has not been reported before as in association with abnormalities of the cornea tissues in Peters anomaly. Such pathological finding need to be reported to enhance further understanding of the special structure of Descemet's membrane as an abnormality during embryogenesis and neural crest cell differentiations.
背景
彼得斯异常是一种罕见的眼前段发育异常，是先天性角膜混浊的主要原因之一。通常，彼得斯异常的组织病理学表现为角膜混浊区域的Descemet膜不成熟或缺失，内皮细胞变薄，此外还有Bowman膜变薄或缺失以及后基质缺陷。在本报告中，我们展示了一例临床诊断为彼得斯异常患者的Descemet膜中一种以前未报道过的新的组织病理学发现。
病例介绍
一名7岁发育迟缓女童，足月顺产。出生时即出现明显的双侧角膜混浊。眼科检查显示，患儿右眼视力为FC/20 cm，左眼视力为FC/10 cm。双眼均发现水平性眼球震颤和先天性白内障。裂隙灯检查显示双侧中央角膜混浊，覆盖虹膜和瞳孔。高频超声生物显微镜（UBM）和眼前节光学相干断层扫描（AS-OCT）均显示前房浅，有多个虹膜角膜粘连区域，且每只眼均无角膜晶状体接触。一名角膜专科医生进行了穿透性角膜移植术，同时行白内障囊外摘除和人工晶状体植入术。对宿主角膜植片进行了组织病理学检查，包括苏木精-伊红染色和过碘酸-希夫染色。所有切片均通过光学显微镜检查。
结论
我们病例中所描述的Descemet膜的“多层”结构，以前尚未报道与彼得斯异常中的角膜组织异常相关。需要报告这种病理发现，以进一步加深对Descemet膜在胚胎发生和神经嵴细胞分化过程中作为一种异常的特殊结构的理解。
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