Mosier M A, Lieberman M F, Green W R, Knox D L
Arch Ophthalmol. 1978 Aug;96(8):1437-42. doi: 10.1001/archopht.1978.03910060185017.
Premorbid ophthalmoscopic and histopathologic findings were correlated in a case of bilateral optic nerve hypoplasia in a 9-month-old infant with bilateral hydranencephaly. The double-ring sign was due to an extension of retina and retinal pigment epithelium (RPE) over the outer portion of the lamina cribrosa. The outer ring was the junction between sclera and lamina cribrosa, and the inner ring was the termination of the RPE. The center of the inner ring was the hypoplastic nerve head, which appeared whitish because of fibroglial tissue surrounding the central retinal vessels where they entered the optic nerve head. We speculate that an in utero vascular insult, after the third month of development, led to cystic cavitation of the anterior cerebral hemispheres, with subsequent retrograde degeneration of developing retinal ganglion cells.
在一名患有双侧积水性无脑畸形的9个月大婴儿的双侧视神经发育不全病例中，对病前的检眼镜检查和组织病理学发现进行了相关性分析。双环征是由于视网膜和视网膜色素上皮（RPE）延伸至筛板外侧部分所致。外环是巩膜与筛板的交界处，内环是RPE的终止处。内环中心是发育不全的视乳头，由于围绕进入视乳头的视网膜中央血管的纤维胶质组织，视乳头呈现白色。我们推测，在发育第三个月后，子宫内血管损伤导致大脑前半球囊性空洞形成，随后发育中的视网膜神经节细胞发生逆行性变性。
