Huang Hou-Bin, Zhang Yi-Xin
Department of Ophthalmology, The General Hospital of Chinese PLA, Beijing 100853, P.R. China.
Department of Ophthalmology, Hainan Branch of General Hospital of PLA, Sanya, Hainan 572013, P.R. China.
Exp Ther Med. 2014 Jun;7(6):1439-1445. doi: 10.3892/etm.2014.1648. Epub 2014 Mar 28.
Pigmented paravenous retinochoroidal atrophy (PPRCA) is an uncommon disease characterized by perivenous aggregations of pigment clumps associated with peripapillary and radial zones of retinochoroidal atrophy that are distributed along the retinal veins. Patients are usually asymptomatic and the disease process is non-progressive or slow and subtly progressive. It is commonly bilateral and symmetric. The cause of the condition may be unknown or idiopathic, although a dysgenetic, degenerative, hereditary etiology or even an inflammatory cause has been hypothesized. A non-inflammatory cause is referred to as primary, while inflammation-associated PPRCA is referred to as secondary or pseudo PPRCA. The present study reviewed and summarized the features of PPRCA.
色素性静脉旁视网膜脉络膜萎缩(PPRCA)是一种罕见疾病,其特征为色素团块沿视网膜静脉呈静脉旁聚集,并伴有视乳头周围及视网膜脉络膜萎缩的放射状区域。患者通常无症状,疾病进程为非进行性或缓慢且隐匿性进展。该病通常为双侧对称。尽管有人提出其病因可能是发育异常、退行性变、遗传性病因甚至炎症,但该病病因可能不明或为特发性。非炎症性病因称为原发性,而与炎症相关的PPRCA称为继发性或假性PPRCA。本研究回顾并总结了PPRCA的特征。