[关键词]
[摘要]
远达性视网膜病变(Purtscher retinopathy)是指与创伤相关的闭塞性视网膜微血管病变。无直接眼部外伤史,常见于头、胸、腹、四肢等处的严重挤压伤,产生视网膜棉绒斑、后极部视网膜多发的灰白色斑块(Purtscher斑)、视网膜内出血等一系列视网膜病理表现。类远达性视网膜病变(Purtscher-like retinopathy)则用于描述胰腺炎、结缔组织病等无外伤情况下的远达性视网膜病变。随着影像学辅助下的超微结构研究的不断发展,远达性视网膜病变的发病机制经历了从创伤导致的损伤及损伤修复产物形成的级联反应,到类远达性视网膜病变的全身病变致脂肪酶、游离脂肪酸或补体激活等诱发视网膜小血管内皮损伤,最终形成血管栓塞机制理论。远达性视网膜病变的发病机制并不单纯存在,目前的主流学说为视网膜微血管通透性改变导致的毛细血管前小动脉栓塞。栓塞之外的学说包括视网膜血管淋巴外渗、血管痉挛、内皮损伤、补体激活等机制也具有重要意义。文章从外伤与非外伤病理过程中存在的炎症与血管级联反应出发,阐述疾病的病理机制从而指导临床诊治,以期在罕见病的研究中发现诊疗新思路。
[Key word]
[Abstract]
Purtscher retinopathy is an occlusive retinal microangiopathy typically associated with trauma. It is characterized by a series of retinal pathological manifestations, such as cotton wool spots, multiple gray plaques(Purtscher spots)in the posterior pole, and intraretinal hemorrhage. Notably, there is no history of direct ocular trauma, as this condition is commonly observed in cases of severe crush injuries to the head, chest, abdomen, limbs, and other regions. Purtscher-like retinopathy, on the other hand, describes extensive retinopathy occurring in the absence of trauma, often associated with conditions such as pancreatitis and connective tissue diseases. With advancements in imaging-assisted ultrastructure research, the understanding of the pathogenesis of this retinopathy has evolved. Initially, it was attributed to trauma-induced injury and the subsequent cascade of damage repair processes. However, current theories suggest that systemic lesions involving lipase, free fatty acids, or complement activation play a significant role in inducing endothelial damage to small retinal blood vessels, ultimately leading to vascular occlusion. The pathogenesis of Purtscher retinopathy is not isolated; it is now widely believed to involve anterior capillary arteriole embolism resulting from changes in retinal microvascular permeability. In addition to embolization, other mechanisms such as retinal vascular-lymphatic extravasation, vasospasm, endothelial injury, and complement activation are also considered crucial contributors to the development of this condition. This paper starts from the inflammation and vascular cascade reaction in the pathological process of trauma and non-trauma, and expounds the pathological mechanism of the disease so as to guide the clinical diagnosis and treatment, in order to find new ideas of diagnosis and treatment in the research of rare diseases.
[中图分类号]
[基金项目]
联勤保障部队第九四○医院院内基金项目(No.2021yxky032)