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Ganglioneuromelanocytosis of the Choroid: With Bilateral Juvenile Glaucoma

Ganglioneuromelanocytosis of the Choroid: With Bilateral Juvenile Glaucoma Abstract Congenital overabundance of ganglion cells was found all through the choroid of one eye of a patient with bilateral absolute glaucoma and buphthalmus. Associated excess of choroidal Schwann cells, melanocytes and nerves make this case very similar to choroidal neurofibromatosis. However, there are no history and general findings to support this diagnosis and some of the typical histological findings of choroidal neurofibromatosis are absent in the present patient. Report of Case Case History. —This 42-year-old white woman was first seen in 1946 at age 24 complaining of poor vision in both eyes. The family history revealed her living four sisters and two brothers as well as her parents to be without any known eye disease and without signs of neurofibromatosis. The patient stated that she had had poor vision as long as she could remember.The examination in 1946 revealed her vision to be OD: light perception, and OS: 20/30. References 1. Scharenberg, K., and Zeman, W.: Zur Leistungsfaehigkeit und zur Technik der Hortega'schen Silberkarbonatmethoden , Z Psychiat 188:430, 1952. 2. Vrabec, F.: Sur les rapports des fibres nerveuses régénérées de la cornée humaine avec les kératoblastes , Ophthal. mologica 130:24, 1955.Crossref 3. Wolter, J.R.: Hyperregeneration of Corneal Nerves in Bullous Keratopathy , Amer J Ophthal 58:31, 1964. 4. Fabritius, R.: Hypertrophy of Corneal Nerves in the Human Eye , Amer J Ophthal 58:21, 1964. 5. Wolter, J.R.; Gonzales-Sirit, R.; and Mankin, W.J.: Neurofibromatosis of the Choroid , Amer J Ophthal 54:217, 1962. 6. Wolter, J.R.: Nerve Fibrils in Ovoid Bodies, With Neurofibromatosis of the Choroid , Arch Ophthal 73:696, 1965.Crossref 7. Collins, E.T., and Batten, R.D.: Neurofibroma of the Eyeball and Its Appendages , Trans Ophthal Soc UK 25:248, 1905. 8. Wheeler, J.M.: Plexiform Neurofibromatosis (von Recklinghausen's Disease) Involving the Choroid, Ciliary Body, and Other Structures , Amer J Ophthal 20:368, 1937. 9. Davis, F.A.: Plexiform Neurofibromatosis (Recklinghausen's Disease) of Orbit and Globe , Arch Ophthal 22:761, 1939.Crossref 10. Friedenwald, J.S., in Penfield, W.: Cytology and Cellular Pathology of the Nervous System , New York: Hoeber, Medical Division of Harper & Row, 1932, p 1079. 11. Francois, J., and Katz, C.: Association homolatérale d'hydrophtalmie de neurome plexiforme de la paupière supérieme et d'hémihypertrophie faciale dans la maladie de Recklinghausen , Ophthalmologica 142:549, 1961.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Ophthalmology American Medical Association

Ganglioneuromelanocytosis of the Choroid: With Bilateral Juvenile Glaucoma

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References (12)

Publisher
American Medical Association
Copyright
Copyright © 1965 American Medical Association. All Rights Reserved.
ISSN
0003-9950
eISSN
1538-3687
DOI
10.1001/archopht.1965.00970040355012
Publisher site
See Article on Publisher Site

Abstract

Abstract Congenital overabundance of ganglion cells was found all through the choroid of one eye of a patient with bilateral absolute glaucoma and buphthalmus. Associated excess of choroidal Schwann cells, melanocytes and nerves make this case very similar to choroidal neurofibromatosis. However, there are no history and general findings to support this diagnosis and some of the typical histological findings of choroidal neurofibromatosis are absent in the present patient. Report of Case Case History. —This 42-year-old white woman was first seen in 1946 at age 24 complaining of poor vision in both eyes. The family history revealed her living four sisters and two brothers as well as her parents to be without any known eye disease and without signs of neurofibromatosis. The patient stated that she had had poor vision as long as she could remember.The examination in 1946 revealed her vision to be OD: light perception, and OS: 20/30. References 1. Scharenberg, K., and Zeman, W.: Zur Leistungsfaehigkeit und zur Technik der Hortega'schen Silberkarbonatmethoden , Z Psychiat 188:430, 1952. 2. Vrabec, F.: Sur les rapports des fibres nerveuses régénérées de la cornée humaine avec les kératoblastes , Ophthal. mologica 130:24, 1955.Crossref 3. Wolter, J.R.: Hyperregeneration of Corneal Nerves in Bullous Keratopathy , Amer J Ophthal 58:31, 1964. 4. Fabritius, R.: Hypertrophy of Corneal Nerves in the Human Eye , Amer J Ophthal 58:21, 1964. 5. Wolter, J.R.; Gonzales-Sirit, R.; and Mankin, W.J.: Neurofibromatosis of the Choroid , Amer J Ophthal 54:217, 1962. 6. Wolter, J.R.: Nerve Fibrils in Ovoid Bodies, With Neurofibromatosis of the Choroid , Arch Ophthal 73:696, 1965.Crossref 7. Collins, E.T., and Batten, R.D.: Neurofibroma of the Eyeball and Its Appendages , Trans Ophthal Soc UK 25:248, 1905. 8. Wheeler, J.M.: Plexiform Neurofibromatosis (von Recklinghausen's Disease) Involving the Choroid, Ciliary Body, and Other Structures , Amer J Ophthal 20:368, 1937. 9. Davis, F.A.: Plexiform Neurofibromatosis (Recklinghausen's Disease) of Orbit and Globe , Arch Ophthal 22:761, 1939.Crossref 10. Friedenwald, J.S., in Penfield, W.: Cytology and Cellular Pathology of the Nervous System , New York: Hoeber, Medical Division of Harper & Row, 1932, p 1079. 11. Francois, J., and Katz, C.: Association homolatérale d'hydrophtalmie de neurome plexiforme de la paupière supérieme et d'hémihypertrophie faciale dans la maladie de Recklinghausen , Ophthalmologica 142:549, 1961.Crossref

Journal

Archives of Ophthalmology – American Medical Association

Published: Sep 1, 1965

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