PDF(Pubmed)
Abstract：
Retinal angiomatous proliferation (RAP) and other types of choroidal neovascularization (CNV) are very rarely reported in patients with retinitis pigmentosa (RP). We present a case report of a 91-year-old patient with an obvious RP phenotype, who presented with a sudden onset of vision worsening and metamorphopsia in the left eye. Genetic testing on the UK inherited retinal disease panel did not identify a pathogenic variant. Multimodal imaging comprising optical coherence tomography (OCT), OCT angiography, and fluorescein and indocyanine green angiography showed a RAP lesion in the left macula. The patient received three treatments of monthly injections of aflibercept, with excellent morphological and functional outcomes. Taking into account the patient\'s age at presentation of the RAP lesion, it is not clear whether the RAP was associated or coincidental with RP. This case report highlights the importance of possessing an awareness that RAP lesions can occur in RP. Moreover, due to a good response and potential safety concerns with continuous anti-VEGF injections in RP patients, a pro re nata (PRN) regimen might be the safest option.
摘要：
视网膜血管瘤增生（RAP）和其他类型的脉络膜新生血管（CNV）在视网膜色素变性（RP）患者中很少报道。我们提供了一个91岁患者的病例报告，该患者具有明显的RP表型，左眼突然出现视力恶化和变形视。对英国遗传性视网膜疾病小组进行的基因检测未发现致病性变异。包括光学相干断层扫描（OCT）的多模态成像，OCT血管造影,荧光素和吲哚菁绿血管造影显示左侧黄斑有RAP病变。患者接受了三次每月注射阿柏西普的治疗，具有优异的形态和功能结果。考虑到患者出现RAP病变时的年龄，尚不清楚RAP是否与RP相关或巧合.此病例报告强调了认识到RP中可能发生RAP病变的重要性。此外，由于RP患者持续注射抗VEGF的良好反应和潜在的安全性问题，prorenata(PRN)方案可能是最安全的选择.
