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色素失禁症中的黄斑血管病变及其演变

Macular vasculopathy and its evolution in incontinentia pigmenti.

作者信息

Goldberg M F

机构信息

Wilmer Ophthalmological Institute, Johns Hopkins University School of Medicine and Hospital, Baltimore, MD 21287-9278, USA.

出版信息

Trans Am Ophthalmol Soc. 1998;96:55-65; discussion 65-72.

PMID:10360282
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1298388/
Abstract

PURPOSE

To describe macular vasculopathy in incontinentia pigmenti.

METHODS

Twelve baby girls with incontinentia pigmenti were examined under general anesthesia by fluorescein angiography of the macula. Nine eyes of 9 patients had sufficient detail to allow evaluation of capillary changes. Angiography was initiated as early as 3 months of age and was repeated in 7 eyes at 3- to 12-month intervals. Changes in capillary patterns were identified.

RESULTS

Irregularly enlarged or distorted foveal avascular zones were noted in all 9 maculas. Sparseness of the perifoveolar capillary bed was a characteristic finding. Sequential macular angiography demonstrated non-progressive (stable) capillary closure in 2 eyes; progressive closure in another macula; progressive closure plus addition or reopening of macular capillaries in 3 eyes; and central retinal artery occlusion, with cherry-red spot formation, in 1 eye at 12 days of age. In addition, progressive tractional detachment of the macula occurred in 2 of these eyes, and progressive macular neovascularization occurred in 1 eye.

CONCLUSIONS

Macular ischemia is characteristic of incontinentia pigmenti and is often progressive. It is the initiating event of a typical vasculopathy, characterized by capillary remodelling and, occasionally, by neovascularization and tractional detachment of the retina.

摘要

目的

描述色素失禁症中的黄斑血管病变。

方法

对12名患有色素失禁症的女婴在全身麻醉下进行黄斑荧光素血管造影检查。9名患者的9只眼有足够的细节可用于评估毛细血管变化。血管造影最早在3个月大时开始，7只眼每隔3至12个月重复进行一次。确定了毛细血管模式的变化。

结果

在所有9个黄斑中均观察到中央凹无血管区不规则扩大或变形。中央凹周围毛细血管床稀疏是一个特征性表现。连续的黄斑血管造影显示，2只眼中有非进行性（稳定）的毛细血管闭合；另一只黄斑出现进行性闭合；3只眼中有进行性闭合加黄斑毛细血管增加或重新开放；1只眼在12日龄时发生视网膜中央动脉阻塞并伴有樱桃红斑形成。此外，其中2只眼发生了黄斑的进行性牵拉性脱离，1只眼发生了黄斑进行性新生血管形成。

结论

黄斑缺血是色素失禁症的特征，且常为进行性。它是典型血管病变的起始事件，其特征为毛细血管重塑，偶尔伴有新生血管形成和视网膜牵拉性脱离。

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Retinal and other manifestations of incontinentia pigmenti (Bloch-Sulzberger syndrome).色素失禁症（布洛赫-苏尔茨贝格综合征）的视网膜及其他表现。
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