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August 1978

Hypoplasia of the Optic Nerve

Author Affiliations

From the Eye Pathology Laboratory, Wilmer Institute, and Department of Pathology, The Johns Hopkins Medical Institutions, Baltimore. Dr Mosier is currently with the Department of Ophthalmology, University of California, Irvine.

Arch Ophthalmol. 1978;96(8):1437-1442. 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.

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