Vol. 113, No. 3
Letters to The Journal
5. Hughes, W. T., Kennedy, W., Shenep, J. L., Flynn, P. M., Hetherington, S. V., Fullen, G., Lancas ter, D. J., Stein, D. S., Palte, S., Rosenbaum, D., Liao, S. H. T., Blum, M. R., and Rogers, M. D.: Safety and pharmacokinetics of 566C80, a hydroxynaphthoquinone with anti-Pneumocystis carinii activity. A phase I study in human immunodeficiency virus (HIV)infected men. J. Infect. Dis. 163:843, 1991.
Branch Retinal Vein Occlusion Associated With a Sarcoid Choroidal Granuloma P h i l i p p e D e n i s , M.D., Jean-Philippe N o r d m a n n , M.D., Laurent Laroche, M.D., and Henry Saraux, M.D. Department of Ophthalmology, Hopital Saint Antoine. Inquiries to Philippe Denis, M.D., Department of Oph thalmology, Hopital Saint Antoine, 184, rue du Fau bourg Saint Antoine, F-75012 Paris, France. Sarcoidosis is a chronic, inflammatory dis ease of unknown cause, which is characterized by the development of noncaseating epithelioid granulomas in miscellaneous tissues including the eye and its adnexa. The most common manifestation of ocular sarcoidosis is acute or chronic iridocyclitis. Ocular findings may also include dacryoadenitis, dacryocystitis, iris nod ules, retinal perivasculitis with perivenous sheathing, and superficial exudative candlewax lesions. 1 We encountered a peculiar mani festation of the disease appearing as a branch vein occlusion associated with a characteristic sarcoid subretinal granuloma. A 32-year-old black woman with a history of chronic fever and dyspnea was referred to our institution for examination. The patient com plained of transient episodes of blurred vision in her left eye, but best-corrected visual acuity was 20/20 in both eyes. Results of anterior segment examination were normal. The right fundus was normal. Fundus examination of her left eye disclosed a yellow-white granulomatous lesion (about Vs disk diameter) covered by the superotemporal retinal vein appearing ir regularly dilated and tortuous, with scattered retinal hemorrhages and cotton-wool spots. The retinal vessels were free of sheathing and did not produce the classic appearance of can dle-wax drippings. Fluorescein angiography
333
demonstrated incomplete occlusion of the in volved vein (with an increase of retinal circula tion time) and showed initial relative hypofluorescence (Fig. 1) with progressive dye accumulation in late-phase angiography from the subretinal nodule. The diagnosis of sarcoid osis, suggested by the presence of bilateral hilar lymphadenopathy, increased erythrocyte sedi mentation rate, and increased angiotensin-converting enzyme levels, was confirmed by a high ratio of T-helper to T-suppressor lymphocytes in the bronchoalveolar lavage, salivary gland, and transbronchial lung biopsy specimens demonstrating noncaseating granulomas. Re sults of a detailed systemic evaluation, includ ing blood cell count, hemoglobin level, glucose tolerance, serum protein electrophoresis, and antinuclear antibody, were unremarkable. Hemoglobin electrophoresis showed a normal percentage of hemoglobin A. The patient was treated with prednisolone, 30 mg orally, daily. Six months later, the left subretinal lesion had dramatically flattened and decreased in size with total resolution of the accompanying venous obstruction (Fig. 2). Granulomatous involvement of the choroid has been reported in patients with biopsyproven sarcoidosis. 2 Franck and Weiss8 de scribed the histopathologic features of a preretinal neovascular membrane in presumed
Fig. 1 (Denis and associates). Fluorescein angiogram of the left eye shows an incomplete occlusion of the superotemporal retinal vein with an increase of retinal circulation time. The involved vein was free of perivascular sheathing and did not produce the ap pearance of candle-wax drippings. Subretinal nodule showed relative hypofluorescence in initial transit frames.
334
AMERICAN JOURNAL OF OPHTHALMOLOGY
March, 1992
Visual Loss in Pseudotumor Cerebri From Branch Retinal Artery Occlusion Byron L. Lam, M.D., R. Michael Siatkowski, M.D., Gregory M. Fox, M.D., and Joel S. Glaser, M.D. Bascom Palmer Eye Institute, University of Miami. Dr. Lam is a Heed Ophthalmic Foundation Fellow (1991).
Fig. 2 (Denis and associates). Six months of oral treatment with prednisolone produced a marked re duction of the subretinal granuloma with total reso lution of the retinal branch vein occlusion.
sarcoidosis, suggesting a previous venous oc clusion. Retinal branch vein occlusion in ocular sarcoidosis has also been documented as a complication of the periphlebitis at a site of arteriovenous crossing. 4,5 The presence of cot ton-wool patches in our patient indicates a significant reduction of blood flow in the area of the obstruction. The venous vasculature re turned to normal after the flattening of the granuloma with corticosteroid treatment, thus emphasizing that the cause of the venous ob struction was the vascular compression by the underlying choroidal nodule. """
References 1. Obenauf, C. D., Shaw, H. E., Sydnor, C. F., and Klintworth, G. K.: Sarcoidosis and its ophthalmic manifestations. Am. J. Ophthalmol. 86:648, 1978. 2. Campo, R. V., and Aaberg, T. M.: Choroidal granuloma in sarcoidosis. Am. J. Ophthalmol. 97:419, 1984. 3. Franck, K. W., and Weiss, H. W.: Unusual clini cal and histopathological findings in ocular sarcoido sis. Br. J. Ophthalmol. 67:8, 1983. 4. Kimmel, A. S., McCarthy, M. J., Blodi, C. F., and Folk, J. C : Branch vein occlusion in ocular sar coidosis. Am. J. Ophthalmol. 107:561, 1989. 5. Gass, J. D. M., and Olson, C. L.: Sarcoidosis with optic nerve and retinal involvement. A clinicopathologic case report. Trans. Acad. Ophthalmol. Otolaryngol. 77:OP739, 1973.
Inquiries to Joel S. Glaser, M.D., Bascom Palmer Eye Institute, P.O. Box 016880, Miami, FL 33101. Chronic papilledema in pseudotumor cerebri may cause optic nerve fiber attrition, which will result in visual field contraction and nerve fiber bundle field defects. 1 Visual loss is usually insidious but may occur suddenly from ischemic optic neuropathy, 2 subretinal neovascular hemorrhage, 3 and central retinal artery occlu sion. 4 Recently, we evaluated a case of pseudo tumor cerebri with chronic papilledema where acute visual loss occurred with a branch retinal artery occlusion. A healthy, 16-year-old obese (200 lbs, 5 ft. 5 in.) black girl had suffered occasional frontal headache as well as orthostatic transient visual obscurations since the age of 12 years. Two weeks before examination while taking no medications, she had sudden visual loss in the right eye, followed two days later by subjective visual loss in the left eye. Visual acuity was R.E.: 20/200 and L.E.: 20/70. There was no anisocoria and no relative afferent pupillary defect. Intraocular pressures were 11 mm Hg bilaterally. Automated visual fields showed marked visual field contractions in both eyes and an inferior foveal-splitting defect in the right eye (Fig. 1). Both optic disks were swollen, and a branch retinal artery occlu sion with geographic retinal edema was visible in the right fundus (Fig. 2). Echography documented distended optic nerve sheaths, 5.3 mm in the right eye and 5.5 mm in the left eye (normal, 2.2 to 3.3 mm), with positive 30-degree abduction tests, confirming the presence of subarachnoid fluid.5 Results of computed brain tomography with contrast were normal. Lumbar puncture disclosed an opening pressure of 400 mm H 2 0 with normal cerebrospinal fluid cell count, protein, glucose, cytolo gy, and culture. Complete blood cell count, electrolytes, glucose, prothrombin time, and partial thromboplastin times were normal. Lu-
Letters to The Journal
5. Hughes, W. T., Kennedy, W., Shenep, J. L., Flynn, P. M., Hetherington, S. V., Fullen, G., Lancas ter, D. J., Stein, D. S., Palte, S., Rosenbaum, D., Liao, S. H. T., Blum, M. R., and Rogers, M. D.: Safety and pharmacokinetics of 566C80, a hydroxynaphthoquinone with anti-Pneumocystis carinii activity. A phase I study in human immunodeficiency virus (HIV)infected men. J. Infect. Dis. 163:843, 1991.
Branch Retinal Vein Occlusion Associated With a Sarcoid Choroidal Granuloma P h i l i p p e D e n i s , M.D., Jean-Philippe N o r d m a n n , M.D., Laurent Laroche, M.D., and Henry Saraux, M.D. Department of Ophthalmology, Hopital Saint Antoine. Inquiries to Philippe Denis, M.D., Department of Oph thalmology, Hopital Saint Antoine, 184, rue du Fau bourg Saint Antoine, F-75012 Paris, France. Sarcoidosis is a chronic, inflammatory dis ease of unknown cause, which is characterized by the development of noncaseating epithelioid granulomas in miscellaneous tissues including the eye and its adnexa. The most common manifestation of ocular sarcoidosis is acute or chronic iridocyclitis. Ocular findings may also include dacryoadenitis, dacryocystitis, iris nod ules, retinal perivasculitis with perivenous sheathing, and superficial exudative candlewax lesions. 1 We encountered a peculiar mani festation of the disease appearing as a branch vein occlusion associated with a characteristic sarcoid subretinal granuloma. A 32-year-old black woman with a history of chronic fever and dyspnea was referred to our institution for examination. The patient com plained of transient episodes of blurred vision in her left eye, but best-corrected visual acuity was 20/20 in both eyes. Results of anterior segment examination were normal. The right fundus was normal. Fundus examination of her left eye disclosed a yellow-white granulomatous lesion (about Vs disk diameter) covered by the superotemporal retinal vein appearing ir regularly dilated and tortuous, with scattered retinal hemorrhages and cotton-wool spots. The retinal vessels were free of sheathing and did not produce the classic appearance of can dle-wax drippings. Fluorescein angiography
333
demonstrated incomplete occlusion of the in volved vein (with an increase of retinal circula tion time) and showed initial relative hypofluorescence (Fig. 1) with progressive dye accumulation in late-phase angiography from the subretinal nodule. The diagnosis of sarcoid osis, suggested by the presence of bilateral hilar lymphadenopathy, increased erythrocyte sedi mentation rate, and increased angiotensin-converting enzyme levels, was confirmed by a high ratio of T-helper to T-suppressor lymphocytes in the bronchoalveolar lavage, salivary gland, and transbronchial lung biopsy specimens demonstrating noncaseating granulomas. Re sults of a detailed systemic evaluation, includ ing blood cell count, hemoglobin level, glucose tolerance, serum protein electrophoresis, and antinuclear antibody, were unremarkable. Hemoglobin electrophoresis showed a normal percentage of hemoglobin A. The patient was treated with prednisolone, 30 mg orally, daily. Six months later, the left subretinal lesion had dramatically flattened and decreased in size with total resolution of the accompanying venous obstruction (Fig. 2). Granulomatous involvement of the choroid has been reported in patients with biopsyproven sarcoidosis. 2 Franck and Weiss8 de scribed the histopathologic features of a preretinal neovascular membrane in presumed
Fig. 1 (Denis and associates). Fluorescein angiogram of the left eye shows an incomplete occlusion of the superotemporal retinal vein with an increase of retinal circulation time. The involved vein was free of perivascular sheathing and did not produce the ap pearance of candle-wax drippings. Subretinal nodule showed relative hypofluorescence in initial transit frames.
334
AMERICAN JOURNAL OF OPHTHALMOLOGY
March, 1992
Visual Loss in Pseudotumor Cerebri From Branch Retinal Artery Occlusion Byron L. Lam, M.D., R. Michael Siatkowski, M.D., Gregory M. Fox, M.D., and Joel S. Glaser, M.D. Bascom Palmer Eye Institute, University of Miami. Dr. Lam is a Heed Ophthalmic Foundation Fellow (1991).
Fig. 2 (Denis and associates). Six months of oral treatment with prednisolone produced a marked re duction of the subretinal granuloma with total reso lution of the retinal branch vein occlusion.
sarcoidosis, suggesting a previous venous oc clusion. Retinal branch vein occlusion in ocular sarcoidosis has also been documented as a complication of the periphlebitis at a site of arteriovenous crossing. 4,5 The presence of cot ton-wool patches in our patient indicates a significant reduction of blood flow in the area of the obstruction. The venous vasculature re turned to normal after the flattening of the granuloma with corticosteroid treatment, thus emphasizing that the cause of the venous ob struction was the vascular compression by the underlying choroidal nodule. """
References 1. Obenauf, C. D., Shaw, H. E., Sydnor, C. F., and Klintworth, G. K.: Sarcoidosis and its ophthalmic manifestations. Am. J. Ophthalmol. 86:648, 1978. 2. Campo, R. V., and Aaberg, T. M.: Choroidal granuloma in sarcoidosis. Am. J. Ophthalmol. 97:419, 1984. 3. Franck, K. W., and Weiss, H. W.: Unusual clini cal and histopathological findings in ocular sarcoido sis. Br. J. Ophthalmol. 67:8, 1983. 4. Kimmel, A. S., McCarthy, M. J., Blodi, C. F., and Folk, J. C : Branch vein occlusion in ocular sar coidosis. Am. J. Ophthalmol. 107:561, 1989. 5. Gass, J. D. M., and Olson, C. L.: Sarcoidosis with optic nerve and retinal involvement. A clinicopathologic case report. Trans. Acad. Ophthalmol. Otolaryngol. 77:OP739, 1973.
Inquiries to Joel S. Glaser, M.D., Bascom Palmer Eye Institute, P.O. Box 016880, Miami, FL 33101. Chronic papilledema in pseudotumor cerebri may cause optic nerve fiber attrition, which will result in visual field contraction and nerve fiber bundle field defects. 1 Visual loss is usually insidious but may occur suddenly from ischemic optic neuropathy, 2 subretinal neovascular hemorrhage, 3 and central retinal artery occlu sion. 4 Recently, we evaluated a case of pseudo tumor cerebri with chronic papilledema where acute visual loss occurred with a branch retinal artery occlusion. A healthy, 16-year-old obese (200 lbs, 5 ft. 5 in.) black girl had suffered occasional frontal headache as well as orthostatic transient visual obscurations since the age of 12 years. Two weeks before examination while taking no medications, she had sudden visual loss in the right eye, followed two days later by subjective visual loss in the left eye. Visual acuity was R.E.: 20/200 and L.E.: 20/70. There was no anisocoria and no relative afferent pupillary defect. Intraocular pressures were 11 mm Hg bilaterally. Automated visual fields showed marked visual field contractions in both eyes and an inferior foveal-splitting defect in the right eye (Fig. 1). Both optic disks were swollen, and a branch retinal artery occlu sion with geographic retinal edema was visible in the right fundus (Fig. 2). Echography documented distended optic nerve sheaths, 5.3 mm in the right eye and 5.5 mm in the left eye (normal, 2.2 to 3.3 mm), with positive 30-degree abduction tests, confirming the presence of subarachnoid fluid.5 Results of computed brain tomography with contrast were normal. Lumbar puncture disclosed an opening pressure of 400 mm H 2 0 with normal cerebrospinal fluid cell count, protein, glucose, cytolo gy, and culture. Complete blood cell count, electrolytes, glucose, prothrombin time, and partial thromboplastin times were normal. Lu-
