Hittner H M
Cullen Eye Institute, Baylor College of Medicine, Houston, Texas.
Metab Pediatr Syst Ophthalmol (1985). 1985;8(2-3):5-10.
Recently, the mechanism of the development of severe retrolental fibroplasia has been shown to occur by gap junction formation between adjacent mesenchymal spindle cells in the nerve fiber layer. Multiple syndromes with oculo-cerebral malformations give rise to retinal dysplasia (disturbances of neuroectodermal migration and/or differentiation) or retinal vascular abnormalities (disturbances of mesenchymal differentiation) which resemble clinically retrolental fibroplasia. Case 1 is an example of retinal dysplasia (disturbed neuroectodermal migration and/or differentiation) with normal retinal vascularization simulating superficially an active peripheral retinal detachment of retrolental fibroplasia in the preterm infant. Case 2 is an example of normal retinal differentiation with abnormal retinal vascularization (disturbed mesenchymal differentiation) simulating identically cicatricial dragging of the retinal vessels temporally of retrolental fibroplasia in the preterm infant. Thus, cases of central nervous system malformation should prompt an early ophthalmologic examination to document retinal abnormalities in term infants which occur without the administration of oxygen congenitally, from true retrolental fibroplasia in preterm infants which occurs following changes in oxygen tension approximately 8 weeks postnatally.
最近研究表明，严重晶状体后纤维增生症的发病机制是神经纤维层中相邻间充质梭形细胞之间形成缝隙连接。多种伴有眼脑畸形的综合征可导致视网膜发育异常（神经外胚层迁移和/或分化障碍）或视网膜血管异常（间充质分化障碍），临床上类似于晶状体后纤维增生症。病例1是视网膜发育异常（神经外胚层迁移和/或分化受阻）的一个例子，其视网膜血管化正常，表面上类似于早产儿晶状体后纤维增生症的活动性周边视网膜脱离。病例2是视网膜分化正常但视网膜血管化异常（间充质分化受阻）的一个例子，同样类似于早产儿晶状体后纤维增生症时视网膜血管的瘢痕性牵拉。因此，中枢神经系统畸形病例应促使进行早期眼科检查，以记录足月儿先天性未吸氧情况下出现的视网膜异常，以及早产儿出生后约8周氧张力变化后发生的真正晶状体后纤维增生症。
