Abstract • A 24-year-old woman was seen with painless, progressive, unilateral visual acuity loss, optic nerve swelling, and opticociliary shunt vessels. Results of a skull series and conventional tomography showed enlargement and erosion of the optic canal. In addition, computed tomography (CT) disclosed a thickened optic nerve consistent with optic nerve sheath meningioma. A preoperative diagnosis of granulomatous optic neuropathy was based on the presence of mild posterior uveitis, "snowball" opacities in the vitreous ("string of pearl" sign), and a slightly elevated angiotensin-converting enzyme level. A specimen from a biopsy by means of a craniotomy indicated granulomatous optic nerve involvement with chiasmal invasion. The systemic effects of steroids for six months resulted in a modest improvement of visual acuity and restoration of normal optic nerve structure on repeated CT. References 1. Statton R, Blodi F, Hanigan J: Sarcoidosis of the optic nerve . Arch Ophthalmol 1964;71:834-836.Crossref 2. Frisen L, Lindgen S, MacGregor BJL, et al: Sarcoid-like disorder of the intracranial optic nerve . J Neurol Neurosurg Psychiatry 1977;40:702-707.Crossref 3. Anderson WB, Parker JJ, Sondheimer FK: Optic foramen enlargement caused by sarcoid granuloma . Radiology 1966;86:319-322.Crossref 4. Sanders MD, Moseley IF: CT scanning in neuro-ophthalmology-optic nerve compression , in Thompson HS (ed): Topics in Neuro-Ophthalmology . Baltimore, Williams & Wilkins Co, 1979, pp 334-335. 5. Wright JE: Primary optic nerve meningiomas: Clinical presentation and management . Trans Am Acad Ophthalmol Otol 1977;85:617-624. 6. Rodriguez MM, Savino SJ, Schatz NJ: Spheno-orbital meningioma with optociliary veins . Am J Ophthalmol 1976;81:666-670. 7. Frisen L, Hoyt WF, Tengroth BM: Optociliary veins, disc pallor, and visual loss: A triad of signs indicating spheno-orbital meningioma . Acta Ophthalmol 1973;51:241-249.Crossref 8. Lax F, Tabaddor K: Extraparenchymal sarcoid mass: A case report . Neurosurgery 1979;5:604-606.Crossref 9. Skillicorn SA, Garrity RW: Intracranial Boeck's sarcoid tumor resembling meningioma . J Neurosurg 1955;12:407-413.Crossref 10. Goodman SS, Marguiles ME: Boeck's sarcoid simulating a brain tumor . Arch Neurol 1959;81:419-423.Crossref 11. Zakka KA, Summerer RW, Yee RD, et al: Opticociliary veins in a primary optic nerve sheath meningioma . Am J Ophthalmol 1979;81:91-95. 12. Miller N, Green R: Arachnoid cysts involving a portion of the intraorbital optic nerve . Arch Ophthalmol 1975;93:1117-1121.Crossref 13. Elschnig A: Cilioretinale Gefasse . Albrecht Von Graefes Arch Klin Exp Ophthalmol 1897;44:144-171.Crossref 14. Hayreh SS: Occlusion of the central retinal vessels . Br J Ophthalmol 1965;49:626-645.Crossref 15. Karel I, Otradovec J, Peleska M: Fluorescein angiography in circulatory disturbances in drusen of the optic disc . Ophthalmologica 1972;164:449-462.Crossref 16. Henkind P, Benjamin JV: Vascular anomalies and neoplasms of the optic nerve head . Trans Ophthalmol Soc UK 1976;96:418-423. 17. Hollenhorst RW Jr, Hollenhorst RW Sr, MacCarty CS: Visual prognosis of optic nerve sheath meningiomas producing shunt vessels on the optic disc . Mayo Clin Proc 1978;53:84-92. 18. Iwata K, Nanba K, Sobue K, et al: Ocular sarcoidosis: Evaluation of intraocular findings . Ann NY Acad Sci 1976;278:445-454.Crossref 19. Turner RG, Geraint JD, Friedmann AI, et al: Neuro-ophthalmic sarcoidosis . Br J Ophthalmol 1975;59:657-663.Crossref 20. Lieberman J: A new confirmatory test for sarcoidosis . Am Rev Respir Dis 1974;109:743-747. 21. Silverstein E, Friedland J, Lyons H, et al: Serum angiotensin converting enzyme activity in sarcoidosis , abstracted. Clin Res 1975;23:352. 22. Romer FK: Angiotensin converting enzyme in sarcoidosis . Acta Med Scand 1979;206:27-30.Crossref 23. Lieberman J, Nosal A, Schlessner LA, et al: Serum angiotensin-converting enzyme for diagnosis and therapeutic evaluation of sarcoidosis . Am Rev Respir Dis 1979;120:329-335. 24. Shultz T, Miller WC, Bedrossian CWM: Clinical application of measurement of angiotensin converting enzyme level . JAMA 1979;242:439-441.Crossref 25. Weinreb RN, O'Donnell JJ, Sandman R, et al: Angiotensin-converting enzyme in sarcoid uveitis . Invest Ophthalmol Vis Sci 1979;18:1285-1287. 26. Weinreb RN, Kimura SJ: Uveitis associated with sarcoidosis and angiotensin converting enzyme . Trans Am Ophthalmol Soc 1979;77:280-293. 27. Gupta RG, Oparil S, Szidon JP: Clinical significance of serum angiotensin-converting enzyme levels in sarcoidosis . J Lab Clin Med 1979;93:941-948. 28. Silverstein E, Friedland J, Kitt M, et al: Increased serum angiotensin converting enzyme activity in sarcoidosis . Isr J Med Sci 1977;13:995-1000.
Archives of Ophthalmology – American Medical Association
Published: Jun 1, 1981
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