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Show ]. Clin. Neuro-ophthalmol. 3: 13-18, 1983. An Unusual Presentation of Isolated Optic Nerve Sarcoidosis JACQUELINE S. LUSTGARTEN, M.D. JOEL S. MINDEL, M.D., Ph.D. MICHAEL E. YABLONSKI, M.D., Ph.D. ALAN H. FRIEDMAN, M.D. Abstract A 32-year old diabetic female had unilateral reduced vision (20/30) in an eye having the ophthalmoscopic appearance of papillophlebitis of the optic nerve head. After an apparently benign course, with recovery, the optic disc became edematous again and a central retinal vein occlusion blinded the eye. Thereafter, an avascular bilobed mass progressively protruded from the optic nerve head. Computerized tomography demonstrated uniform thickening of the optic nerve from the globe through the optic canal. The radiographic appearance was consistent with an optic nerve glioma or meningioma. Spinal fluid, chest x-ray, and serum angiotensin converting enzyme activity were normal. Tuberculin skin test was positive. Optic nerve biopsy demonstrated a noncaseating granuloma and a subsequent Kveim test was positive. Isolated optic nerve sarcoidosis may exist without other systemic manifestations. The appearance on computerized tomography may be similar to that of an optic nerve glioma or meningioma. Introduction Unilateral optic nerve involvement by a noncaseating granulomatous process has rarely been reported as being the only manifestation of presumed sarcoidosis. I. 2 In these cases an optic nerve glioma or meningioma was suspected prior to the biopsy. Computerized tomography of the orbits and head was not performed in these patients but it would be hoped that this modality would be of value in differentiating tumor from granuloma. To the contrary, the present report describes a patient in whom computerized tomography appeared to support a preoperative diagnosis of optic nerve glioma. From the Departments of Ophthalmology (jSL, JSM, MEY, AHFJ, and Pharmacology (jSM) of The Mount Sinai School of Medicine, New York, and the Ophthalmology Section (jSL, JSM) of the Bronx Veterans Hospital, New York. March 1983 Case History A 32-year-old, black woman complained of recent onset of blurred vision in her left eye. Her acuity was 20/20, right eye and 20/30, left eye. The left eye had a moderate afferent pupillary defect to light and ophthalmoscopic examination revealed an edematous optic disc with dilated veins and peripapillary hemorrhages (Fig. 1). Visual fields demonstrated a relative central defect to a 2mm red test object at 1 meter distance and enlargement of the physiologic blind spot. The right eye was normal in all parameters. Ten years earlier, she had converted her tuberculin skin test and received a I-year course of isoniazid. The patient's father was diabetic and the patient was known to have an abnormal glucose tolerance test. Ten months earlier, she had developed a transient left abducens nerve paresis that resolved within 4 weeks and was attributed to diabetes mellitus. Computerized tomography of the head and orbits using intravenous contrast dye was unremarkable at that time and her serum fasting glucose level was mildly elevated, 127 mg %. Because of the recent loss of left eye vision, the patient was admitted to the hospital. Her fasting serum glucose level was 144 mg %. The following tests were within normal limits: x-rays of the chest (Fig. 2), skull, and optic foramina; repeat computerized tomography of the head and orbits with intravenous contrast dye enhancement; spinal fluid analysis, hemoglobin, hematocrit, white cell count and blood sedimentation rate; latex fixation, antinuclear antibody, LE, and VORL tests; serum electrolytes, including calcium; and serum protein and globulin electrophoresis. A tentative diagnosis of diabetic papillophlebitis was made and she was placed on oral prednisone, 60 mg/day for 2 weeks. There appeared to be little therapeutic effect so the drug was tapered and discontinued. During the next 2 weeks, the disc edema and hemorrhages resolved, leaving a somewhat pale nerve head. Acuity returned to 20/20. Three weeks later, the left eye vision again decreased to 20/40 and disc 13 Optic Nerve Sarcoidosis Figure 1. Marked unilateral edema and hyperemia of the left optic disc with dilated veins and multiple peripapillary hemorrhages, yet good visual acuity (20/30) was consistent with the diagnosis of diabetic papillophlebitis. Figure 2. Roentgenogram of the chest without evidence of sarcoidosis. edema was present especially inferiorly, but without the previous florid venous vasculopathy (Fig. 3). The left eye vision continued to deteriorate over the next week to 20/200 and the patient complained of mild periocular discomfort on adduction. The eye was neither proptotic nor inflamed. Prednisone 60 mg orally every other day was begun; after 2 weeks without visual improvement, 14 Figure 3. Recurrent edema of the left optic disc especially inferiorly, but without the previous florid vasculopathy. The acuity was 20/40. Figure 4. Central retinal vein occlusion producing a blind left eye. however, the medication was rapidly tapered and discontinued. Two weeks later all vision was suddenly lost in the left eye. Fundus examination revealed an appearance compatible with a central vein occlusion (Fig. 4). Over the next 4 weeks, the venous vasculopathy resolved but a raised (7 diopter) bilobed mass progressively protruded from the inferior disc (Fig. 5) and several small white masses appeared in the adjacent vitreous. Computerized Journal of Clinical Neuro-ophthalmo!ogy Figure 5. Bilobed mass projecting 7 diopters from the inferior medial aspect of the left optic disc. Multiple small white opacities can be seen in the vitreous. Lustgarten, Mindel, Yablonski, Friedman Figure 6. Computerized tomography demonstrating uniform thickening of the left optic nerve from the globe through the optic canal. (Reprinted by permission from the Journal of Computer Assisted Tomography 6: 614-616. 1982. Raven Press Publishers, New York.) Figure 7. External appearance of the patient at the time of optic nerve biopsy. The blind left eye is slightly exotropic. The eye movements are full and there is no proptosis. The lids are retracted in the central and inferior photographs to demonstrate that the conjunctiva is normal and without inflammation. tomography was performed again. A uniform enlargement of the optic nerve was found (Fig. 6). It extended from the sclera through the optic canal. Carotid arteriography indicated a relatively avascular optic nerve lesion. The radiologic diagnosis was optic nerve glioma. The external appearance of the eye and the ocular movements appeared normal (Fig. 7). Mammography and repeat chest xrays were normal. An optic nerve biopsy was performed using a lateral orbital approach. The tissue diagnosis was noncaseating granuloma consistent with sarcoidosis (Fig. 8). A subsequent Kveim test was positive. Serum angiotensin converting en- March 1983 zyme activity, 14 nmole/minute/ml, was within normal limits. Tuberculin skin test (intermediate strength) was positive. Pulmonary function tests were normal. Discussion Optic nerve involvement in sarcoidosis can take many forms. Intraocular granulomas of the nerve head may produce neovascularization or be seen as nodular masses with the ophthalmoscope.:l . ~ Unilateral or bilateral granulomas of the retrobulbar nerve may produce edema of one or both 15 Optic Nerve Sarcoidosis Figure 8. PhotomicrogrJph of the left optic nerve demonstrating a noncaseating granulomatous reaction. (Hematoxylin & eosin, x 500) discs."-7 Increased intracranial pressure may cause papilledema. Compression by suprasellar, hypothalamic, or chiasmal granulomas may produce optic atrophy.~· 9 However, isolated optic nerve involvement is rare in sarcoidosis. Only 5% of patients have central nervous system involvement, and in these the most commonly impaired cranial nerve is the facial nerve. In addition, only 5% of patients have a normal chest roentgenogram at the time the diagnosis of sarcoidosis is made. III The present report may be the first in which a Kveim test confirmed the diagnosis in a patient having no other manifestations of sarcoidosis except optic nerve involvement. There are a few scattered reports of isolated noncaseating granulomas of the optic nerve but Kveim tests were not performed. In this situation some authors have hesitated to make the diagnosis of sarcoidosis while others have not. I. 2. II False-positive Kveim tests occur with a 23% incidence12 . \:3 Perhaps the present authors should be more circumspect in accepting this diagnosis in their patient. It is interesting to speculate on the cause of the transient abducens nerve paresis 10 months before the visual loss. In retrospect, this may have been a manifestation of sarcoidosis and not diabetes mel- 16 litus, as was assumed at that time. If so, this too would have been a rare presentation. Obenauf et al. In reported only one instance of an isolated abducens muscle paresis in 532 patients with sarcoidosis. Kendall and Tatler14 reported abducens muscle pareses in five patients who presented with neurosarcoidosis but in all of them other cranial nerve abnormalities were simultaneously present. Direct infiltration of the extraocular muscles has never been documented in sarcoidosis and it is assumed the abducens nerves were infiltrated or compressed. The present patient had an abnormally high fasting blood glucose level, a prior elevated glucose tolerance test, and a family history of diabetes mellitus. Palsies of the oculomotor and abducens nerves have occurred in young diabetics without retinopathy or other neuropathy. IS Perhaps the initial episode of left optic disc swelling was also a manifestation of diabetes. Her presentation with minimal visual involvement despite a florid vasculopathy of the nerve head (Fig. 1) was compatible with the diagnosis of papillophlebitis, which has been reported in young diabetics. 16 . 17 If so, this would explain why the computerized tomography at that time was unremarkable, why the response to corticosteroids was unimpressive, and Journal of Clinical Neuro-ophthalmology why the condition initially resolved in an apparently benign manner. According to this scenario, it was only the second episode of disc edema (Fig. 3), which was not accompanied by vasculopathy (until the central retinal vein occlusion intervened) that was due to sarcoidosis. The absence of systemic manifestations of sarcoidosis and the presence of normal chest roentgenograms have already been mentioned as contributing to the delay in diagnosing this disease in this patient. In addition, her serum angiotensin converting enzyme activity was within normal limits, her tuberculin skin test remained positive, and the abnormal appearance of the optic nerve on computerized tomography was compatible with an optic nerve glioma or meningioma. Approximately 80 % of patients with active sarcoidosis have elevated serum angiotensin converting enzyme activities if they are not receiving corticosteroid therapy. 18. 19 At the time this test was performed, more than 8 weeks had elapsed since discontinuing prednisone. With regard to the tuberculin skin test, Siltzbach and co-workers20 and James et a1. 21 found approximately one-third of sarcoidosis patients demonstrated positive responses. They pointed out that the insensitivity to tuberculin was relative, not absolute. Positive responses tended to be weakly so. Most misleading of all was the apparent optic nerve tumor seen on computerized tomography (Fig. 6). The uniform thickening of the nerve, its poor enhancement after contrast dye injection, and the avascularity demonstrated on arteriography were compatible with this impression. It has been said that computerized tomography is of value in diagnosing neurosarcoidosis both because the granulomas can be seen and because they tend to enhance after contrast dye injection. 14 The relatively rapid rate with which the nerve thickening developed and the intravitreal small white masses (Fig. 5) did suggest that other diagnoses such as lymphoma, metastatic malignancy, tuberculosis, and sarcoidosis be considered. The histologic diagnosis of noncaseating granuloma was, therefore, not entirely unexpected. The postoperative management of this patient has presented another problem. The process may extend back along the intracranial portion of the left optic nerve toward the chiasm and involve the optic nerve of the remaining useful eye. Monitoring such a progression prior to visual loss in the right eye has proven difficult. The authors have been reluctant to perform a craniotomy and remove the remainder of the left optic nerve in order to prevent contiguous spread. Computerized tomography performed 2 months after the optic nerve biopsy did not show the chiasmal area in sufficient detail to rule in or out a progressive granulomatous infiltration. Perhaps intrathecal injection of contrast material in conjunction with computerized tomography would better delineate March 1983 Lustgarten, Mindel, Yablonski, Friedman the chiasmal area but how often could this be repeated in a patient requiring long-term followup? Prophylactic systemic corticosteroids could be administered. It has been stated that this treatment is more effective earlier in the disease. 22 However, there are no parameters by which to judge the appropriate dosage and duration of treatment in this diabetic female. No therapy has been administered in the 13 months following the optic nerve biopsy. The visual acuity and visual field of the right eye continue to be frequently monitored and as yet no deficit has occurred. References 1. Papo, I., Beltrami, CA., Salvolini, U., and Caruselli G: Sarcoidosis simulating a glioma of the optic nerve. Surg. Neural. 8: 353-355, 1977. 2. Frisen, L., Lindgren, S., MacGregor, B.J. L, and Statten, S: Sarcoid-like disorder of the intracranial optic nerve. ]. Neural. Neurasurg. Psychiatry 40: 702-707, 1977. 3. Laties, AM., and Scheie, H.G.: Evolution of multiple small tumors in sarcoid granuloma of the optic disc. Am. ]. Ophthalmol. 74: 60-66, 1972. 4. Noble, K.G.: Ocular sarcoidosis occurring as a unilateral optic disc vascular lesion. Am.]. Ophthalmol. 87: 490-493, 1979. 5. Hart, W.M., Jr., and Burde, R.M.: Optic disc edema in sarcoidosis. Am. ]. Ophthalmol. 88: 769-771, 1979. 6. Jampol, L.M., Woodfin, W., and McLean, LB.: Optic nerve sarcoidosis. Arch. Opthalmol. 87: 355-360, 1972. 7. Slatton, R., Blodi, F.C, and Hanigan, J.: Sarcoidosis of the optic nerve. Arch. Ophthalmol. 71: 834-836, 1964. 8. Ingestad, R., and Stigmar, G.: Sarcoidosis with ocular and hypothalamic-pituitary manifestations. Acta. Ophthalmol. 49: 1-10, 1971. 9. Stuart, CA, Neelon, F.A, and Lebovitz, H.E.: Hypothalamic insufficiency: The cause of hypopituitarism in sarcoidosis. Ann. Intern. Med. 88: 589-594, 1978. 10. Obenauf, CD., Shaw, H.E., Sydnor, CF., and Klentworth, GX.: Sarcoidosis and its ophthalmic manifestations. Am. ]. Ophthalmol. 86: 648-655, 1978. 11. Krohel GB, Charles, H., and Smith, R.S.: Granulomatous optic neuropathy. Arch. Ophthalmol. 99: 1053-1055,1981. 12. Chapman, J: The Kveim test. Arch. PathoI. Lab. Med. 101: 515-51~ 1977. 13. Douglas, A.C: Kveim and Siltzbach. Mt. Sinai l. Med. 44: 706-708, 1977. 14. Kendall, B.L, and Tatler, G.L.V.: Radiological findings in neurosarcoidosis. Br. ]. Radiol. 51: 81-92, 1978. 15. Grunt, ].A, Destra, R.L., Hamtil, L. W., and Baska, R.E.: Ocular palsies in children with diabetes mellitus. Diabetes 25: 459-462, 1976. 16. Barr, CC, Glaser, ].S., and Blankenship, G: Acute disc swelling in juvenile diabetes. Arch. Ophthalmol. 98: 2185-2192,1980. 17 18 Optic Nerve Sarcoidosis 17. Pavan, P.R., Aiello, L.M., Wafai, M.Z., Briones, I.e., Sebestyen, J.G., and Bradbury, M.J.: Optic disc edema in juvenile-onset diabetes. Arch. Ophthalmol. 98: 2193-2195, 1980. 18. Lieberman, J., Nosal, A., Schlessner, L.A., and Sastre-Foken, A.: Serum angiotensin-converting enzyme for diagnosis and therapeutic evaluation of sarcoidosis. Am. Rev. Respir. Dis. 120: 329-335, 1979. 19. DeRemee, R.A., and Rohrbach, M.5.: Serum angiotensin- converting enzyme activity in evaluating the clinical course of sarcoidosis. Ann. Intern. Med. 92: 361-365, 1980. 20. Siltzbach, L.E., James, D.G., Neville, E., Turiaf, J., Battesti, J.P., Sharma, O.P., Hosoda, Y., Mikami, R., and Odaka, M.: Course and prognosis of sarcoidosis around the world. Am. f. Med. 57: 847-852, 1974. 21. James D.G., Anderson R., Langley, D., and Ainslie, D.: Ocular sarcoidosis. Br. f. Ophthalmol. 48: 461469, 1964. 22. Colp e.: Sarcoidosis-Course and treatment. Med. Clin. North Am. 61: 1267-1278, 1977. Write for reprints to: Joel S. Mindel, M.D., Ph.D., Department of Ophthalmology, The Mount Sinai Hospital, Annenberg Bldg. 22-14, 1 Gustave Levy Place, New York, New York 10029. Journal of Clinical Neuro-ophthalmology |
