Clemente-Tomas Rodrigo, Ruíz-Del Río Noemí, Gargallo-Benedicto Amparo, García-Ibor Francisca, Hervas-Hernándis José M, Duch-Samper Antonio M
Departament of Ophthalmology, Hospital Clínico, School of Medicine, Universidad de Valencia, Spain.
Departament of Ophthalmology, Hospital Clínico; Associated Professor of Ophthalmology, School of Medicine, Universidad de Valencia, Spain.
Indian J Ophthalmol. 2020 Jul;68(7):1473-1475. doi: 10.4103/ijo.IJO_1895_19.
A 13-year-old child diagnosed with neurofibromatosis type 1 who on a routine control presented with rhegmatogenous retinal detachment associated to dialysis of the ora serrata in the left eye (OS). There were no clinical signs or history of contuse ocular trauma. Neurofibromatosis produces alterations in fibroblasts of the cortex of the vitreous base. This results in deficient production of the collagen fibers that anchor the vitreous base to the pars plana and the peripheral neurosensory retina. Thus, suboptimal function of the fibroblasts explains spontaneous avulsion of the vitreous base. Such avulsion in turn is related to dialysis of the ora serrata.
一名13岁被诊断为1型神经纤维瘤病的儿童,在常规检查时出现左眼锯齿缘离断相关的孔源性视网膜脱离。无眼部钝挫伤的临床体征或病史。神经纤维瘤病会导致玻璃体基底部皮质的成纤维细胞发生改变。这会导致将玻璃体基底部固定于睫状体平坦部和周边神经感觉视网膜的胶原纤维产生不足。因此,成纤维细胞功能欠佳解释了玻璃体基底部的自发性撕脱。而这种撕脱又与锯齿缘离断有关。