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July 1993

Optic Nerve Hypoplasia, Big Discs, Large Cupping, and Vascular Brain Malformation Embolized: 22 Years of Follow-up

Author Affiliations

Petah Tikva, Israel

Arch Ophthalmol. 1993;111(7):901-902. doi:10.1001/archopht.1993.01090070019007

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Ragge et al1 recently described optic nerve hypoplasia with big optic discs and large cupping. In their case "... late prenatal injury with significant transsynaptic loss of nerve fibers did not alter the genetically predetermined size of the scleral canals, but it did alter the shape."1

We have observed the case of a 35-year-old man with left temporal lobe vascular malformation since it was embolized 22 years ago with Luessenhop pearls2 (Figure 1). He had had repeated subarachnoidal hemorrhages until the embolization; afterward, there were no further neurological events. Visual acuity was 20/25 in each eye, with corrected myopic astigmatism. The intraocular pressure was 12 mm Hg. There were right homonymous hemianopia, big optic discs with large cupping unchanged since 1971, and hemiopic pattern of nerve fiber loss (Figure 2).

Intrauterine lesion of the postgeniculate visual pathway may result in transsynaptic degeneration of the anterior visual pathways on

Ragge NK, Hoyt WF, Lambert SR.  Big discs with optic nerve hypoplasia . J Clin Neuro Ophthalmol . 1991;11:137-138.
Luessenhop AJ.  Artificial embolization for cerebral arterio-venous malformations . Prog Neurol Surg . 1969;3:320-324.
Hoyt WF, Rio-Montenegro CN, Behrens MM, Eckelhoff RJ.  Homonymous hemiopic hypoplasia: fundoscopic features in standard and red-free illumination in three patients with congenital hemiplegia . Br J Ophthalmol . 1972;56:537-545.Article