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一例科茨病的临床-组织病理学相关性

Clinical-histopathological correlation in a case of Coats' disease.

作者信息

Fernandes Bruno F, Odashiro Alexandre N, Maloney Shawn, Zajdenweber Moyses E, Lopes Andressa G, Burnier Miguel N

机构信息

Department of Ophthalmology and Pathology, The McGill University Health Center & Henry C. Witelson Ocular Pathology Laboratory, Montreal, Canada.

出版信息

Diagn Pathol. 2006 Aug 30;1:24. doi: 10.1186/1746-1596-1-24.

Abstract

BACKGROUND

Coats' disease is a non-hereditary ocular disease, with no systemic manifestation, first described by Coats in 1908. It occurs more commonly in children and has a clear male predominance. Most patients present clinically with unilateral decreased vision, strabismus or leukocoria. The most important differential diagnosis is unilateral retinoblastoma, which occurs in the same age group and has some overlapping clinical manifestations.

CASE PRESENTATION

A 4 year-old girl presented with a blind and painful right eye. Ocular examination revealed neovascular glaucoma, cataract and posterior synechiae. Although viewing of the fundus was impossible, computed tomography disclosed total exudative retinal detachment in the affected eye. The eye was enucleated and subsequent histopathological evaluation confirmed the diagnosis of Coats' disease.

CONCLUSION

General pathologists usually do not have the opportunity to receive and study specimens from patients with Coats' disease. Coats' disease is one of the most important differential diagnoses of retinoblastoma. Therefore, it is crucial for the pathologist to be familiar with the histopathological features of the former, and distinguish it from the latter.

摘要

背景

科茨病是一种非遗传性眼部疾病,无全身表现,于1908年由科茨首次描述。它在儿童中更常见,男性明显居多。大多数患者临床上表现为单眼视力下降、斜视或白瞳症。最重要的鉴别诊断是单侧视网膜母细胞瘤,其发生在相同年龄组且有一些重叠的临床表现。

病例介绍

一名4岁女孩因右眼失明且疼痛就诊。眼部检查发现新生血管性青光眼、白内障和后粘连。尽管无法观察眼底,但计算机断层扫描显示患眼有完全性渗出性视网膜脱离。该眼被摘除,随后的组织病理学评估证实了科茨病的诊断。

结论

普通病理学家通常没有机会接收和研究科茨病患者的标本。科茨病是视网膜母细胞瘤最重要的鉴别诊断之一。因此,病理学家熟悉前者的组织病理学特征并将其与后者区分开来至关重要。

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