Abstract
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.
Original language | English (US) |
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Pages (from-to) | 1437-1442 |
Number of pages | 6 |
Journal | Archives of ophthalmology |
Volume | 96 |
Issue number | 8 |
DOIs | |
State | Published - Aug 1978 |
Externally published | Yes |
ASJC Scopus subject areas
- Ophthalmology