目的:研究家族性腺瘤性息肉病(familial adenomatous polyposis,FAP)患者中双眼多灶性先天性视网膜色素上皮肥厚(congenital hypertrophy of the retinal pigment epithelium,CHRPE)患者的眼底荧光素血管造影(fundus fluoresceinangiography,FFA)的特点。方法:我们对22例有65处CHRPE病灶的FAP患者前瞻性进行FFA和眼底检查。结果:发现86%CHRPE病损面积>0.5个视盘直径,74%的CHRPE病变接近视网膜血管,视网膜血管有以下变化:46%CHRPE病变中出现毛细血管无灌注区,8%穿过病灶的视网膜血管部分阻塞,6%出现脉络膜视网膜吻合支,3%病变有毛细血管微血管瘤,5%可观察到脉络膜毛细血管,20%的脉络膜毛细血管出现在脱色素边缘晕环中。约10%的CHRPE病变眼底检查未发现,只能通过FFA观测到。结论:虽然通过眼底检查能诊断CHRPE,但有些病变仍需通过FFA确诊,其对此病变仍是非常有用的诊断方法。

AIM:In order to investigate the features of fundus fluorescein angiography(FFA) in congenital hypertrophy of the retinal pigment epithelium (CHRPE) in patients with familial adenomatous polyposis(FAP). METHODS: Prospective FFA and ophthalmoscopy were carried out in 22 patients with 65 CHPRE lesions.RESULTS: A total of 86% were over half the optic disc diameter in size. About 74% of CHRPE lesions were close to retinal vessels. The retinal vascular changes were: with capillary non-perfusion area (46%), partly block of retinal vessels (8%), chorioretinal anastomoses (6%), capillary microaneurysms (3%), choriocapillary vessels can be observed (5%) and in the depigmented marginal halo (20%). About 10% of the lesions were not seen on ophthalmoscope and could only be detected by angiography.CONCLUSION: Even though the diagnosis of CHRPE is clinical, FFA may be useful in confirming the diagnosis, as well as detecting additional lesions not seen by means of ophthalmoscopic examination.

R774.1