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Optic Nerve Aplasia

Optic Nerve Aplasia Abstract • Optic nerve aplasia is a rare congenital defect invariably associated with other ocular or systemic disorders. We examined a 3-year-old girl with monocular microphthalmos who had optic nerve aplasia on histopathologic examination of the eye after enucleation. Magnetic resonance imaging verified the presence of unilateral optic nerve aplasia, and demonstrated hemichiasmal hypoplasia on the affected side and bilateral optic tracts. The visually evoked cortical response revealed increased signals over the occipital cortex ipsilateral to the aplastic nerve, suggesting misdirection of axons from the temporal retina of the normal eye. The visual pathway in unilateral optic nerve aplasia may assume a primitive form of neuronal organization characterized by an increase in contralateral retinogeniculostriate projection. References 1. Scheie HG, Adler FH. Aplasia of the optic nerve . Arch Ophthalmol . 1941;26:61-70.Crossref 2. Smith HE. Aplasia of the optic nerve: report of three cases . Am J Ophthalmol . 1954;37:498-504. 3. LaFranchi SH. Sexual precocity with hypothalamic hypopituitarism . AJDC . 1979;133:739-742. 4. Jeffreys DA, Axford JG. Source location of pattern specific components of human visualevoked potentials, I: component of striate cortical origin . Exp Brain Res . 1972;16:1-19. 5. Hotchkiss ML, Green WR. Optic nerve aplasia and hypoplasia . J Pediatr Ophthalmol Strabismus . 1979;16:225-240. 6. Weiter JJ, McLean IW, Zimmerman LE. Aplasia of the optic nerve and disk . Am J Ophthalmol . 1977;83:569-576. 7. Storm RL, PeBenito R. Bilateral optic nerve aplasia associated with hydroencephaly . Ann Ophthalmol . 1984;16:988-992. 8. Ginsberg J, Bove KE, Cuesta MG. Aplasia of the optic nerve with aniridia . Ann Ophthalmol . 1980;12:433-439. 9. Awan KJ. Ganglionic neuroretinal aplasia and hypoplasia: aplasia and hypoplasia of optic nerve . Ann Ophthalmol . 1976;8:1193-1202. 10. Yanoff M, Rorke LB, Allman MI. Bilateral optic system aplasia with relatively normal eyes . Arch Ophthalmol . 1978;96:97-101.Crossref 11. Little LE, Whitmore PV, Wells TW Jr. Aplasia of the optic nerve . J Pediatr Ophthalmol . 1976;13:84-88. 12. Barry DR. Aplasia of the optic nerves . Int Ophthalmol . 1985;7:235-242.Crossref 13. Margo CE, Hamed LM, McCarty J. Congenital optic tract syndrome . Arch Ophthalmol . 1991;109:1120-1122.Crossref 14. Rogalski T. The visual paths in a case of unilateral anophthalmia with special reference to the problem of crossed and uncrossed visual fibers . J Anat (Lond) . 1946;80:153-159. 15. Hoff J, Winestock D, Hoyt WF. Giant suprasellar aneurysm associated with optic stalk agenesis and unilateral anophthalmos . J Neurosurg . 1975;43:495-498.Crossref 16. Brodsky MC, Glasier CM, Pollock SC, Angtuago EJC. Optic nerve hypoplasia: identification by magnetic resonance imaging . Arch Ophthalmol . 1990;108:1562-1567.Crossref 17. Slamovits TL, Kimball GP, Frieberg TB, Cortin HD. Bilateral optic disc colobomas with orbital cysts and hypoplastic optic nerves and chiasm . J Clin Neuro-ophthalmol . 1989;9:172-177. 18. Lambert SR, Hoyt CS, Narahara MH. Optic nerve hypoplasia . Surv Ophthalmol . 1987;32:1-9.Crossref 19. Creel D, Witkop CJ, King RA. Asymmetric visually evoked potential in human albinos: evidence for visual system anomalies . Invest Ophthalmol . 1974;13:430-440. 20. Guillery RW, Okoro AN, Witkop CJ Jr. Abnormal visual pathway in the brain of an albino . Brain . 1975;96:373-377.Crossref 21. Apkarian P, Reits D, Spekreijse H. A decisive electrophysiological test for human albinism . Electroencaphalogr Clin Neurophysiol . 1983;55:513-531.Crossref 22. Apkarian P. Methodology of testing for albinism with visual-evoked cortical potentials . In: Heckenlively J, Arden G, eds. Principles and Practice of Clinical Electrophysiology of Vision . St Louis, Mo: Mosby-Year Book; 1991:433. 23. Guillery RW, Kass JH. A study of normal and congenitally abnormal retinogeniculate projections in cats . J Comp Neurol . 1971;143:73-100.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Ophthalmology American Medical Association

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References (25)

Publisher
American Medical Association
Copyright
Copyright © 1992 American Medical Association. All Rights Reserved.
ISSN
0003-9950
eISSN
1538-3687
DOI
10.1001/archopht.1992.01080230110033
Publisher site
See Article on Publisher Site

Abstract

Abstract • Optic nerve aplasia is a rare congenital defect invariably associated with other ocular or systemic disorders. We examined a 3-year-old girl with monocular microphthalmos who had optic nerve aplasia on histopathologic examination of the eye after enucleation. Magnetic resonance imaging verified the presence of unilateral optic nerve aplasia, and demonstrated hemichiasmal hypoplasia on the affected side and bilateral optic tracts. The visually evoked cortical response revealed increased signals over the occipital cortex ipsilateral to the aplastic nerve, suggesting misdirection of axons from the temporal retina of the normal eye. The visual pathway in unilateral optic nerve aplasia may assume a primitive form of neuronal organization characterized by an increase in contralateral retinogeniculostriate projection. References 1. Scheie HG, Adler FH. Aplasia of the optic nerve . Arch Ophthalmol . 1941;26:61-70.Crossref 2. Smith HE. Aplasia of the optic nerve: report of three cases . Am J Ophthalmol . 1954;37:498-504. 3. LaFranchi SH. Sexual precocity with hypothalamic hypopituitarism . AJDC . 1979;133:739-742. 4. Jeffreys DA, Axford JG. Source location of pattern specific components of human visualevoked potentials, I: component of striate cortical origin . Exp Brain Res . 1972;16:1-19. 5. Hotchkiss ML, Green WR. Optic nerve aplasia and hypoplasia . J Pediatr Ophthalmol Strabismus . 1979;16:225-240. 6. Weiter JJ, McLean IW, Zimmerman LE. Aplasia of the optic nerve and disk . Am J Ophthalmol . 1977;83:569-576. 7. Storm RL, PeBenito R. Bilateral optic nerve aplasia associated with hydroencephaly . Ann Ophthalmol . 1984;16:988-992. 8. Ginsberg J, Bove KE, Cuesta MG. Aplasia of the optic nerve with aniridia . Ann Ophthalmol . 1980;12:433-439. 9. Awan KJ. Ganglionic neuroretinal aplasia and hypoplasia: aplasia and hypoplasia of optic nerve . Ann Ophthalmol . 1976;8:1193-1202. 10. Yanoff M, Rorke LB, Allman MI. Bilateral optic system aplasia with relatively normal eyes . Arch Ophthalmol . 1978;96:97-101.Crossref 11. Little LE, Whitmore PV, Wells TW Jr. Aplasia of the optic nerve . J Pediatr Ophthalmol . 1976;13:84-88. 12. Barry DR. Aplasia of the optic nerves . Int Ophthalmol . 1985;7:235-242.Crossref 13. Margo CE, Hamed LM, McCarty J. Congenital optic tract syndrome . Arch Ophthalmol . 1991;109:1120-1122.Crossref 14. Rogalski T. The visual paths in a case of unilateral anophthalmia with special reference to the problem of crossed and uncrossed visual fibers . J Anat (Lond) . 1946;80:153-159. 15. Hoff J, Winestock D, Hoyt WF. Giant suprasellar aneurysm associated with optic stalk agenesis and unilateral anophthalmos . J Neurosurg . 1975;43:495-498.Crossref 16. Brodsky MC, Glasier CM, Pollock SC, Angtuago EJC. Optic nerve hypoplasia: identification by magnetic resonance imaging . Arch Ophthalmol . 1990;108:1562-1567.Crossref 17. Slamovits TL, Kimball GP, Frieberg TB, Cortin HD. Bilateral optic disc colobomas with orbital cysts and hypoplastic optic nerves and chiasm . J Clin Neuro-ophthalmol . 1989;9:172-177. 18. Lambert SR, Hoyt CS, Narahara MH. Optic nerve hypoplasia . Surv Ophthalmol . 1987;32:1-9.Crossref 19. Creel D, Witkop CJ, King RA. Asymmetric visually evoked potential in human albinos: evidence for visual system anomalies . Invest Ophthalmol . 1974;13:430-440. 20. Guillery RW, Okoro AN, Witkop CJ Jr. Abnormal visual pathway in the brain of an albino . Brain . 1975;96:373-377.Crossref 21. Apkarian P, Reits D, Spekreijse H. A decisive electrophysiological test for human albinism . Electroencaphalogr Clin Neurophysiol . 1983;55:513-531.Crossref 22. Apkarian P. Methodology of testing for albinism with visual-evoked cortical potentials . In: Heckenlively J, Arden G, eds. Principles and Practice of Clinical Electrophysiology of Vision . St Louis, Mo: Mosby-Year Book; 1991:433. 23. Guillery RW, Kass JH. A study of normal and congenitally abnormal retinogeniculate projections in cats . J Comp Neurol . 1971;143:73-100.Crossref

Journal

Archives of OphthalmologyAmerican Medical Association

Published: Nov 1, 1992

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