Goldberg M F
Wilmer Ophthalmological Institute, Johns Hopkins University School of Medicine and Hospital, Baltimore, MD, USA.
Ophthalmic Genet. 1998 Sep;19(3):141-8.
To describe macular vasculopathy in incontinentia pigmenti.
Twelve baby girls with incontinentia pigmenti were evaluated under general anesthesia by fluorescein angiography of the macula. Nine eyes of nine patients had sufficient detail to allow evaluation of capillary changes. Angiography was initiated as early as three months of age, and was repeated in seven eyes at 3-12 month intervals. Changes in capillary patterns were identified.
Irregularly enlarged or distorted foveal avascular zones were noted in all nine maculas. Sparseness of the perifoveolar capillary bed was a characteristic finding. Sequential macular angiography demonstrated nonprogressive (stable) capillary closure in two eyes; progressive closure was noted in another macula; progressive closure plus addition or reopening of macular capillaries occurred in three eyes; and central retinal artery occlusion, with cherry red spot formation, was observed in one eye at 12 days of age. In addition, progressive tractional detachment of the macula, associated with bleeding pre-retinal neovascularization, occurred in two of these eyes, and progressive macular neovascularization also occurred in one eye.
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.
描述色素失禁症中的黄斑血管病变。
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评估。9名患者的9只眼睛有足够的细节以评估毛细血管变化。血管造影最早在3个月大时开始，7只眼睛每隔3 - 12个月重复进行。确定毛细血管模式的变化。
在所有9个黄斑中均观察到不规则扩大或扭曲的黄斑无血管区。黄斑周围毛细血管床稀疏是一个特征性发现。连续的黄斑血管造影显示，2只眼睛有非进行性（稳定）的毛细血管闭合；另一个黄斑出现进行性闭合；3只眼睛出现进行性闭合并伴有黄斑毛细血管的增加或重新开放；1只眼睛在12日龄时观察到视网膜中央动脉阻塞并伴有樱桃红斑形成。此外，其中2只眼睛出现与视网膜前新生血管出血相关的进行性黄斑牵拉性脱离，1只眼睛也出现进行性黄斑新生血管形成。
黄斑缺血是色素失禁症的特征，且通常呈进行性。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。
