Choroidal Neovascular Membrane and Other Chorioretinal Complications of Acquired Syphilis

Choroidal Neovascular Membrane and Other Chorioretinal Complications of Acquired Syphilis Lawrence S. H a l p e r i n , M . D . , H i l e l L e w i s , M . D . , Mark S. B l u m e n k r a n z , M . D . , J. D o n a l d M . G a s s , M . D . , R. Joseph O i k , M . D . , a n d Stuart L. Fine, M . D . We reviewed ten patients who had posterior segment complications of acquired syphilis. Five of these patients had a neovascular membrane of the choroid associated with secondary or tertiary syphilis. Vision was stabilized after laser photocoagulation in one patient who had a choroidal neovascular membrane. Five additional patients had ocular manifestations including uveitis, optic neuritis, neuroretinitis, chorioretinitis, retinal hemorrhages, arterial and venous occlusion, vasculitis, and retinitis. Treatment of chorioretinal complications of syphilis with intravenous penicillin, if initiated early in the course of the disease, may result in excellent visual recovery. O C U L A R FINDINGS in acquired syphilis may

include posterior uveitis, chorioretinitis, perivasculitis, optic neuritis, and vascular occlusion. Since the first reports of syphilitic eye disease around 1858 by Baeder' and Hutchinson, 2 there have been many reports about the ocular complications of this infectious disease. Over the past ten years, the incidence of primary and secondary syphilis has increased twofold to fourfold, 35 with a shift in incidence toward homosexual men. 6 The number of syphilis cases was 25% greater in 1987 than 1986, which is the largest one-year increase ever reported. 7 We describe ten cases of posterior segment complications of secondary and tertiary syphi-

Accepted for publication July 26, 1989. From the Departme'nt of Ophthalmology, Washington University School of Medicine, St. Louis (Drs. Halperin and Oik); Jules Stein Institute (Dr. Lewis); Wilmer Ophthalmological Institute, Baltimore (Dr. Fine); Kresge Eye Institute, Royal Oak, Michigan (Dr. Blumenkranz); and Bascom Palmer Eye Institute, Miami (Dr. Gass). Reprint requests to R. Joseph Oik, M.D., Suite 17413 East Pavilion, 4949 Barnes Hospital Plaza, St. Louis, MO 63110. 554

lis. Five patients had a neovascular membrane of the choroid, which is a condition that has been rarely reported. 8

Case Reports Case reports were collected from the Bascom Palmer Eye Institute in Miami, the Retina Consultants in St. Louis, and the Wilmer Ophthalmological Institute in Baltimore. The diagnosis of syphilis in each patient was based on serologic studies, which included VDRL, fluorescent treponemal antibody absorption test (FTAABS), and rapid plasma reagin. Cerebrospinal fluid analysis was performed in selected patients. Case 1 A 34-year-old black woman had decreased vision in the right eye. At 9 years of age, an episode of vomiting and photophobia was treated with penicillin in the absence of a specific diagnosis. At 19 years of age, vision was lost in the left eye for unknown reasons. Visual acuity was R.E.: 20/125 and L.E.: no light perception. The right eye demonstrated iritis, vitritis, attenuated retinal vessels, and atrophic chorioretinal lesions in the periphery. The retinal pigment epithelium was mottled, and there was a white fibrotic placoid subretinal lesion in the macula (Fig. 1, left). Fluorescein angiography showed diffuse hyperfluorescence, especially on the temporal edge, consistent with a choroidal neovascular membrane (Fig. 1, right). The patient had a positive FTA-ABS. A lumbar puncture or serum VDRL was not performed. Because the choroidal neovascular membrane was subfoveal, laser treatment was not performed. One year later, vision was unchanged and the right eye was quiet. The chorioretinitis was diffuse but stable, and the large neovascu-

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lar membrane was unchanged. Seven years later, visual acuity had spontaneously improved to R.E.: 20/80. Case 2 A 33-year-old white woman complained of two spots in front of the left eye. During a pregnancy at 17 years of age, she developed active syphilis and was treated with penicillin. At 22 years of age, she was found to be blind in the right eye and to have normal vision in the left eye. She underwent strabismus surgery at that time for a right exotropia. Visual acuity was R.E.: light perception and L.E.: 20/200. The right eye had mild vitritis, optic atrophy, chorioretinitis, and retinal necrosis. There were no active lesions. The left eye showed a quiet anterior chamber, early posterior subcapsular cataract, moderate vitritis, peripapillary myelinated nerve fibers off the optic disk, and chorioretinal atrophy along the vascular arcades. There was an active choroidal lesion inferotemporal to the macula and a serous macular detachment with subretinal hemorrhage (Fig. 2, top left). A fluorescein angiogram disclosed a choroidal neovascular membrane, inferotemporal to the fovea and outside the foveal avascular zone (Fig. 2, top right). There was a second choroidal neovascular membrane adjacent to the optic disk. Chest x-ray, and results of complete blood cell count, chemistries, urinalysis, and serum immunoglobulin studies were normal. The serum VDRL test result was positive. A lumbar puncture was not performed. Oral tetracycline and prednisone were initiated (the patient was allergic to penicillin). The argon laser was used to photocoagulate the perifoveal choroidal neovascular membrane (Fig. 2, bottom left). Over several months, the subretinal fluid disappeared, and the treated membrane developed into a scar (Fig. 2, bottom right). At seven years of follow-up, visual acuity was L.E.: 20/200, and the treated choroidal membrane remained unchanged. The other retinal lesions were stable. Case 3 A 40-year-old white man had decreased vision in the right eye. Six years earlier, he had a visual acuity of R.E.: 20/200 and L.E.: 20/70, with vitritis and cystoid macular edema. Serum and cerebrospinal fluid VDRL test results were Positive, and he was treated with penicillin and prednisone with resultant improvement in vi-

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sion. One year earlier, he had recurrent cystoid macular edema, but his lumbar puncture showed a nonreactive VDRL. The serum VDRL was positive at 1:1. He received prednisone with no effect on the macular edema. (This case was previously reported. 9 ) At examination, visual acuity was R.E.: 20/ 100 and L.E.: 20/15. A right afferent pupillary defect was present. The right eye showed mild iritis, vitritis, optic atrophy, large areas of retinal pigment epithelium dropout, and cystoid macular edema. There was a gray subretinal macular lesion surrounded by hemorrhage consistent with a choroidal neovascular membrane. Ophthalmoscopic examination indicated that the left eye was normal. Fluorescein angiography disclosed large retinal pigment epithelial window defects. The view of the macula was blocked by blood from an underlying choroidal neovascular membrane. Results of the rapid plasma reagin and FTAABS tests were positive. Because the patient had received a course of penicillin before examination for decreased vision, administration of penicillin was not repeated. The patient was lost to follow-up. Case 4 A 40-year-old white man had floaters in his left eye. He had a history of uveitis complicated by vitritis, chorioretinitis, and progressive loss of vision in both eyes. Ten years earlier, he had had primary syphilis treated with one dose of penicillin. After this treatment, his rapid plasma reagin became negative, whereas the FTAABS remained positive. He had recently had a urethral discharge and a maculopapular rash on both legs. Visual acuity was R.E.: 20/200 and L.E.: 20/ 20. Both eyes showed moderate vitritis, chorioretinitis, and perivenous sheathing. The right eye showed a gray, elevated subretinal macular lesion associated with local hemorrhage consistent with a choroidal neovascular membrane in the fovea. Fluorescein angiography showed a hyperfluorescent lesion in the macula in the same area as the gray subretinal mass, confirming the diagnosis of subfoveal neovascular membrane. Serum VDRL and FTA-ABS test results were positive, and the cerebrospinal fluid VDRL test result was negative. The cerebrospinal fluid had 7 white blood cells/ml and increased protein. The patient received 18 MU/day of intravenous penicillin for ten days. Four years later, visual acuity was R.E.: 20/

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TABLE CLINICAL FINDINGS* VISUAL ACUITY PATIENT

1

SEROLOGIC

BEFORE

AFTER

TESTS

ANTIBIOTICS

ANTIBIOTICS

R.E.: 20/125 L.E.: NLP R.E.: LP L.E.: 20/200

R.E.: 20/125 L.E.: NLP R.E.: LP L.E.: 20/200

R.E.:: 20/100 L.E.:: 20/15 R.E.:: 20/200 L.E.:: 20/20 R.E.:: 20/400 L.E.:: 20/25 R.E.: 1/200 L.E.: 6/200 R.E.: 20/20 L.E.: 20/20 R.E.: 20/50 L.E.: 2/200 R.E.: 20/70 L.E.: 20/200 R.E.: 20/60 L.E.: 20/20

R.E.: NA L.E.: NA R.E.: 20/300 L.E.: 20/20 R.E.: 20/400 L.E.: 20/25 R.E.: 20/200 L.E.: 20/80 R.E.: 20/20 L.E.: 20/20 R.E.: NA L.E.: 6/200 R.E.: 20/50 L.E.: 20/70 R.E.: 20/20 L.E.: 20/20

FINDINGS

6

Vitrttis, attenuated vessels, chorioretinal lesions, choroidal neovascular membrane (L.E.) vitritis, chorioretinal atrophy, serous macular detachment, and choroidal neovascular membrane (treated with argon laser photocoagulation) Iritis, vitritis, optic atrophy, choroidal neovascular membrane, cystoid macular edema Vitritis, chorioretinitis, choroidal neovascular membrane Vitritis, chorioretinitis, venous sheathing, disk edema, choroidal neovascular membrane Disk edema, vitritis, gray macular lesions

7

Disk hyperemia, chorioretinal lesions

RPR, FTA-ABS

8

Homosexual, vitritis, disk edema, vasculitis, arteriole occlusion, retinal nodules Vitritis, disk edema, venous engorgement, periphlebitis Homosexual, vitritis, disk edema, acute retinitis

RPR, FTA-ABS

2

3 4 5

9 10

FTA-ABS VDRL

VDRL VDRL VDRL, FTA-ABS RPR

VDRL, FTA-ABS VDRL

CEREBROSPINAL FLUID VDRL

NP NP

•FTA-ABS, fluorescent treponemal antibody absorption test; NP, not performed; NLP, no light perception; LP, light perception; NA, not available; RPR, rapid plasma reagin. 'Cerebrospinal fluid VDRL was positive many years ago. Recent testing not performed.

300 and L.E.: 20/20. Both eyes had chronic iritis, keratic precipitates, and vitritis. The right eye had a disciform macular lesion, and the left eye had mild cellophane maculopathy. Serologic tests for syphilis were not performed again. Case 5

A 48-year-old black man had decreased vision in the right eye for one year. He had a palmar rash six months before examination. At the age of 9 years, the patient had syphilis and was treated for four weeks with an unknown medication. In the 1950s, he was treated with several intramuscular shots for venereal disease. Visual acuity was R.E.: 20/400 and L.E.: 20/ 25. Both eyes showed episcleritis, mild iritis, and vitritis. The right eye showed disk edema, venous sheathing, and multifocal chorioretinitis. Temporal to fixation, there was a white

geographic placoid lesion at the level of the pigment epithelium associated with subretinal hemorrhage and a large circinate ring of lipid (Fig. 3, left). The left eye showed minimal venous sheathing, drusen, and an old chorioretinal scar. Fluorescein angiography disclosed a large area of hyperfluorescence temporal to the fovea with leakage of dye late in the study (Fig. 3, right). The patient refused laser photocoagulation to the extrafoveal choroidal neovascular membrane. The serum VDRL was reactive at 1:4, and the FTA-ABS was 4+ reactive. The cerebrospinal fluid had 10 white blood cells (lymphocytes and monocytes)/ml and a negative VDRL. The patient was treated with intravenous penicillin for ten days followed by 2.4 MU of Bicillin intramuscularly every week for three weeks. Visual acuity remained unchanged. No follow-up is available.

Fig. 1 (Halperin and associates). Patient 1. Left, White fibrotic choroidal neovascular membrane with an active temporal edge. The retinal pigment epithelium is diffusely mottled. Right, Fluorescein angiogram in midphase demonstrates the choroidal neovascular membrane with leakage.

Fig. 2 (Halperin and associates). Patient 2. Top left, Two gray-white subretinal lesions and retinal striae. Top fight, Fluorescein angiogram shows choroidal neovascular membranes with leakage of dye late in the study. Bottom left, Lesion immediately after treatment with argon laser photocoagulation. Bottom right, Mature scar six months after treatment.

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Case 6 A 56-year-old black man had bilateral blurred vision. He had a sore on his penis for four weeks previous to the examination. The patient disclosed that one of his sexual partners (Patient 10 of the present study) had been treated for ocular syphilis. Visual acuity was R.E.: 1/200 and L.E.: 6/200. Both eyes showed bilateral vitritis, disk hyperemia and edema, and gray, ill-defined, outer retinal lesions in the maculae. A thick urethral discharge with maceration of the glans skin was present, but a urethral culture was negative. The rapid plasma reagin was positive. The cerebrospinal fluid had increased protein, normal glucose, 12 mononuclear cells/ml, and a nonreactive VDRL. Intravenous penicillin, 24 MU/day and oral prednisone, 60 mg/day were administered. Two days later, the optic disk edema decreased and prednisone was discontinued. In six days, the retinal lesions resolved. After 14 days of penicillin therapy, visual acuity was R.E.: 20/200 and L.E.: 20/80. The optic disks had mild swelling, and the maculae showed retinal pigment epithelial mottling without active lesions. Follow-up is not available. Case 7

A 24-year-old black man complained of redness and photophobia in the right eye. He had a chancre on his penis four months previously and a rash on his trunk IViz months previously. Visual acuity was 20/20 in both eyes. The pupils reacted poorly to light. The right eye showed ciliary injection, large keratic precipitates, iritis, mild disk hyperemia with a mottled appearance of the retina, and discrete, yellowish choroidal spots with overlying retinal thickening. The left eye showed disk swelling and hyperemia, retinal thickening, and small choroidal spots. The serum rapid plasma reagin and FTA-ABS were reactive. A lumbar puncture disclosed a positive VDRL and 12 mononuclear cells/ml. Results of an electroretinogram were normal. Intravenous penicillin, 24 MU/day, was administered. Five days later, the choroidal lesions developed hypopigmented centers, and a Bussaca nodule was detected in the right eye. After ten days of intravenous penicillin, vision was unchanged, but the iritis had resolved. The retina appeared normal.

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Six-month follow-up demonstrated small choroidal atrophic spots and a slightly pale, flat left optic disk. The cerebrospinal fluid VDRL was nonreactive. The eye remained quiet. Case 8 A 35-year-old homosexual white man had decreased visual acuity of R.E.: 20/50 and L.E.: 20/200. The patient denied any previous history or use of illicit drugs. A left afferent pupillary defect was present. The left eye showed a mild granulomatous iritis, severe optic disk swelling, occlusion of the superotemporal retinal arteriole, perivasculitis, a diffuse yellow-white opacification of the retina, focal nodular infiltrates of the inner retina with sparing of the macular region and vitritis. The right fundus showed discoloration of the posterior pole. The serum rapid plasma reagin and FTA-ABS were highly reactive. H u m a n immunodeficiency virus titer was not performed. The cerebrospinal fluid VDRL was nonreactive. A ten-day course of intravenous penicillin was administered, and a dramatic decrease in optic nerve and retinal changes occurred within 48 hours. The patient's vision was unchanged. Two weeks later, the rapid plasma reagin titer decreased and visual acuity was L.E.: 6/200 despite complete resolution of the fundus changes. Follow-up is not available. Case 9

A 63-year-old black woman had bilateral blurred vision and redness for one month. Visual acuity was R.E.: 20/70 and L.E.: 20/200. The right eye showed keratic precipitates, and the left had numerous mutton fat keratic precipitates, iritis, a 2% hyphema, and extensive posterior synechiae. Both eyes showed moderate vitritis, elevated and hyperemic optic disks with peripapillary hemorrhages, engorged veins, and segmental periphlebitis. Neurologic examination showed dementia, flapping tremor, frontal release signs, hyperreflexia, and bilateral Babinski signs. Serum VDRL and FTA-ABS were positive. Lumbar puncture showed a nonreactive VDRL and negative cultures. Computed tomography of the head disclosed cortical atrophy. Late neurosyphilis (general paresis of the insane) with recurrent active ocular syphilis was treated with 12 MU/day of intravenous

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Fig. 3 (Halperin and associates). Patient 5. Left, White geographic placoid lesion at the level of the pigment epithelium with a circinate ring of lipid. Right, Late frame of the fluorescein angiogram shows extensive leakage from a choroidal neovascular membrane. penicillin. After six days, the ocular findings did not change, so oral prednisone was started but was discontinued because of side effects. After 12 days of treatment, visual acuity was R.E.: 20/50 and L.E.: 20/70, the iritis resolved, and the vitritis and optic disk swelling decreased. No follow-up is available.

Case 10 A 24-year-old homosexual white man had decreased vision and floaters in the right eye. Three years earlier, a rash on his forearms and thighs was treated with topical corticosteroids. The VDRL was negative. One year later, he was exposed to syphilis through a sexual partner,

Fig. 4 (Halperin and associates). Patient 10. Left, Active retinitis with yellow-white retina. Note the blurriness of fundus details secondary to vitritis. Right, Same area after ten days of intravenous penicillin. Note pigment clumping in the retina, chorioretinal scar formation, lack of active retinitis, and clearing of the view because of resolving vitritis.

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had a positive VDRL, and was treated with an unknown dose of penicillin. At that time, he had his first-known episode of iritis associated with bilateral optic disk edema. Computed tomography disclosed that the head was normal. Topical corticosteroids were administered. Visual acuity was R.E.: 20/60 and L.E.: 20/20. The right pupil was fixed. Both eyes had moderate iritis and vitritis. The right eye showed keratic precipitates, a yellow-white retinitis with white clumps in the cortical vitreous, and vitreous haze (Fig. 4, left). The left eye had demarcated areas of retinitis temporally and small vitreous infiltrates. The disks were swollen in both eyes. Results of a computed tomography of the head were normal. Physical examination disclosed diffuse lymphadenopathy and a rectal fissure. A presumptive diagnosis of acute retinal necrosis syndrome was made, and intravenous acyclovir was given. The white blood cell count was 15,500/ml. Serum VDRL was positive at 1:256. Human immunodeficiency virus titer was not performed. Cerebrospinal fluid had a glucose level of 30 mg/dl, a protein level of 123 mg/dl, 5 lymphocytes/ml, and the VDRL was positive at 1:8. Intravenous penicillin, 24 MU/day, was administered for ten days. Repeat lumbar puncture showed improvement. One year later the patient had visual acuity of 20/20 in both eyes, no iritis, mild vitritis, and pigmented chorioretinal scars without retinitis (Fig. 4, right).

Discussion Common findings in the ten patients are uveitis in all patients; disk edema in five patients; chorioretinal lesions in five patients; vascular narrowing or occlusion in three patients; and vasculitis and perivasculitis in three patients (Table). Because the incidence of syphilis is increasing, ophthalmologists are seeing more ocular complications of untreated or partially treated secondary and tertiary syphilis. Initial tests include the VDRL or the rapid plasma reagin tests. The FTA-ABS test is more sensitive. All patients who have positive syphilis serologic test results and ocular findings must have their cerebrospinal fluid investigated for neurosyphilis. The cerebrospinal fluid is tested for VDRL

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titers that confirm a diagnosis of neurosyphilis. Syphilis must be considered in any differential diagnosis of chorioretinal and optic nerve lesions. The main clinical features of acquired ocular syphilis involving the posterior segment are uveitis 1011 ; chorioretinitis 10,1217 ; vasculitis 18 ; perivasculitis 1920 with associated arterial occlusion21; disciform detachment of the macula22; macular stellate figure23; macular edema1924,25 and cystoid macular edema 911 ; neuroretinitis with optic neuritis, vitritis, retinal edema and retinal vasculitis1726"30; pseudoretinitis pigmentosa associated with vasculitis and vascular occlusion15171831'35; central retinal artery and vein occlusion 24 ; and retinal detachment and uveal effusion. 36 Acute retinitis and retinal necrosis have been associated with syphilis. 37,38 The patients in these two reports were homosexual men, and one had neurosyphilis. Human immunodeficiency virus titer should be performed in a patient with ocular syphilis and acute retinitis. Ocular syphilis occurs in patients with human immunodeficiency virus infection. 39 Prompt treatment may lead to improved vision. Not only is it important to establish an early diagnosis of syphilis, but it is also imperative to prescribe proper treatment. Patients 3, 4, and 5 in this study had treatment that may have been inadequate. Patients 2 and 10 had positive VDRL tests even after treatment, demonstrating the importance of follow-up serologic testing after treatment. Treatment guidelines are recommended by the United States Public Health Service. 40 Choroidal neovascular membrane appears in age-related maculopathy, trauma, tumors, hereditary diseases, idiopathic disorders, and inflammatory diseases including histoplasmosis, toxoplasmosis. Behest's syndrome, sarcoidosis, serpiginous choroidopathy, and chronic uveitis. Choroidal neovascular membrane associated with ocular syphilis has been reported rarely. 8 The cause of a choroidal neovascular membrane in inflammatory conditions must be related to changes in Bruch's membrane and the retinal pigment epithelium. Blodi and Harvouet 34 reported the pathologic findings in four cases of syphilitic chorioretinitis with breaks in Bruch's membrane, though none demonstrated a choroidal neovascular membrane. These breaks may be the source of the subretinal neovascular proliferation. It is believed that the white geographic placoid-like lesions at the level of the retinal

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pigment epithelium, usually associated with p r o f o u n d v i s u a l l o s s , m a y b e p a t h o g n o m o n i c of s e c o n d a r y or t e r t i a r y o c u l a r s y p h i l i s . A n extrafoveal m e m b r a n e m a y b e a m e n a b l e to laser photocoagulation. Laser photocoagulation s h o u l d c a u s e scar f o r m a t i o n a n d d e c r e a s e d exu d a t i o n , b o t h of w h i c h m a y stabilize v i s i o n . S y p h i l i s is k n o w n as " t h e g r e a t i m i t a t o r " a n d therefore m u s t be considered w h e n visual loss, uveitis, chorioretinitis, optic disk e d e m a , vascular o c c l u s i o n , a n d p e r i v a s c u l i t i s are p r e s e n t . Early d i a g n o s i s of syphilitic I n f e c t i o n allows a p p r o p r i a t e a n t i b i o t i c t r e a t m e n t t h a t m a y imp r o v e v i s i o n . T h e c e r e b r o s p i n a l fluid m u s t b e c h e c k e d in a n y p a t i e n t w i t h p o s i t i v e s y p h i l i s serologic findings a n d positive ocular findings. C h o r o i d a l n e o v a s c u l a r m e m b r a n e is a s e q u e l a to m a n y i n f l a m m a t o r y d i s e a s e s , i n c l u d i n g syphilis.

References 1. Sidler-Huguenin: Uber die hereditar-syphilitischen Augenhintergrundsveranderungen, nebst einigen allgemeinen Bemerkungen uber Augenkrankungen bei angeborener Lues. Beitr. Augenheilkd. 51:1, 1904. 2. Hutchinson, J.: A Clinical Memoir of Certain Diseases of the Eye and Ear Consequent to Inherited Syphilis. London, J. Churchill, 1863, p. 130. 3. Syphilis—United States, 1983. M.M.W.R. 33:433, 1984. 4. Leads from the MMWR: Early syphilis— Broward County Florida. JAMA 257:2565, 1987. 5. JAMA 1987, STD fact sheet, ed. 34. Atlanta, U.S. Department of Health, Education, and Welfare, 1979, p. 19. 6. Fichtner, R. R., Aral, S. O., Blount, J. H., Zaidi, A. A., Reynolds, G. H., and Darrow, W. W.: Syphilis in the United States. 1967-1979. Sex. Transm. Dis. 10:77, 1983. 7. Syphilis and congenital syphilis—United States, 1985-1988. M.M.W.R. 37:486, 1988. 8. Kawona, Y.: A case of syphilitic chorioretinitis. Folia Ophthalmol. 35:80, 1984. 9. Martin, N. F., and Fitzgerald, C. R.: Cystoid macular edema as the primary sign of neurosyphilis. Am. J. Ophthalmol. 88:28, 1979. 10. Schlaegel, T. F., and Kao, S. F,: A review (1970-1980) of 28 presumptive cases of syphilitic uveitis. Am. J. Ophthalmol. 92:412, 1982. 11. Ross, W. H., and Sutton, H. F.: Acquired syphilitic uveitis. Arch. Ophthalmol. 98:496, 1980. 12. deSouza, E. C , Jalkh, A. E., Trempe, C. L., Cunha, S., and Schepens, C. L.: Unusual central

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chorioretinitis as the first manifestation of early secondary syphilis. Am. J. Ophthalmol. 105:271, 1988. 13. MacFaul, P. A., and Caterall, R. D.: Acute chorioretinitis in secondary syphilis. Br. J. Vener. Dis. 47:159, 1971. 14. Luxen, L., Lees, A. J., and Greenwood, R. J.: Neurosyphilis today. Lancet 1:90, 1979. 15. Rice, N. S. C , Jones, B. R., and Wilkinson, A. E.: Study of late ocular syphilis. Demonstration of treponemes in aqueous humor and cerebrospinal fluid. Ocular findings. Trans. Ophthalmol. Soc. U.K. 88:257, 1969. 16. Eide, N., and Skjeldal, O. J.: Juxtapapillary chorioretinitis in neurosyphilis. Acta Ophthalmol. 62:351, 1984. 17. Duke-Elder, S.: Diseases of the Retina. In System of Ophthalmology, vol. 10. St. Louis, C. V. Mosby, 1967, pp. 252-261. 18. Morgan, C. M., Webb, R. M., and O'Connor, G. R.: Atypical syphilitic chorioretinitis and vasculitis. Retina 4:225, 1984. 19. Savir, H., and Kurz, O.: Fluorescein angiography in syphilitic retinal vasculitis. Ann. Ophthalmol. 8:713, 1976. 20. Crouch, E. R., and Goldberg, M. F.: Retinal periarteritis secondary to syphilis. Arch. Ophthalmol. 93:384, 1975. 21. Smith, J. L.: Acute blindness in early syphilis. Arch. Ophthalmol. 90:256, 1973. 22. Saari, M.: Disciform detachment of the macula. Acta Ophthalmol. 56:510, 1978. 23. Fewell, A. G.: Unilateral neuroretinitis of syphilitic origin with a striate figure at the macula. Arch. Ophthalmol. 8:615, 1932. 24. Belin, M. W., Baltch, A. L., and Hay, P. B.: Secondary syphilitic uveitis. Am. J. Ophthalmol. 92:210, 1981. 25. Lobes, L. A., and Folk, J. C : Syphilitic phlebitis simulating branch vein occlusion. Ann. Ophthalmol. 13:825, 1981. 26. Folk, J. C , Weingeist, T. A., Corbett, J. J., Lobes, L. L., and Watzke, R. C : Syphilitic neuroretinitis. Am. J. Ophthalmol. 95:480, 1983. 27. Arruga, J., Valentines, J., Mauri, F., Roca, G., Salom, R., and Rufi, G.: Neuroretinitis in acquired syphilis. Ophthalmology 92:262, 1985. 28. Graveson, G. S.: Syphilitic optic neuritis. J. Neurol. Neurosurg. Psychiatry 13:216, 1950. 29. Earl, C. J., and Zilkha, K. J.: Syphilitic visual failure. Br. J. Ophthalmol. 48:630, 1964. 30. Verhoeff, F. A.: A case of syphilitic retinochoroiditis juxtapapillaris with microscopic examination. Arch. Ophthalmol. 45:352, 1916. 31. Spoor, T. C , Wynn, P., Hartel, W. E., and Bryan, C. S.: Ocular syphilis. J. Clin. NeuroOphthalmol. 3:197, 1983. 32. Volpi, U.: Pseudo-retinitis pigmentosa caused by acquired syphilis. Ann. Ottalmol. Clin. Ocul. 92:408, 1966. 33. Lorentzen, S. E.: Syphilitic optic neuritis. A case report. Acta Ophthalmol. 45:769, 1967.

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34. Blodi, F. C , and Harvouet, F.: Syphilitic chorioretinitis. Arch. Ophthalmol. 79:294, 1968. 35. Skalka, H. W.: Asymmetric retinitis pigmentosa, luetic retinopathy and the question of unilateral retinitis pigmentosa. Acta Ophthalmol. 576:351, 1979. 36. Deluise, V. P., Clark, S. W., Smith, J. L., and Collart, P.: Syphilitic retinal detachment and uveal effusion. Am. J. Ophthalmol. 94:757, 1982. 37. Stoumbos, V. D., and Klein, M. L.: Syphilitic

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retinitis in a patient with acquired immunodeficiency syndrome-related complex. Am. J. Ophthalmol. 103:103, 1987. 38. Mendelsohn, A. D., and Jampol, L. M.: Syphilitic retinitis. Retina 4:221, 1984. 39. Passo, M. S., and Rosenbaum, J. T.: Ocular syphilis in patients with human immunodeficiency virus infection. Am. J. Ophthalmol. 106:1, 1988. 40. 1985 STD treatment guidelines. M.M.W.R. 34(suppl. 4):94S, 1985.

O P H T H A L M I C MINIATURE

He was quite y o u n g , s o m e w h e r e between twenty and thirty years of age, a n d of normal height and build, but o t h e r w i s e his a p p e a r a n c e was s t r a n g e . His skin was as white as the snow on the roofs, he had p r o t u b e r a n t eyes of startling bright green, a n d his hair was the color of a peeled carrot. The maids t h o u g h t he was ugly; the old women felt sorry for him, a n d the small boys laughed until they fell d o w n . Ken Follett, The Pillars of the Earth New York, William Morrow and C o m p a n y , Inc., 1989, p. 13