19. Syphilis


Fig. 19.1

This 59-year-old woman, born in 1923, had lifelong poor vision with chorioretinal scarring and optic atrophy. Serological testing with a treponemal-specific test was positive, and she was felt to have had congenital syphilis infection



Acquired Syphilis


Acquired syphilis is usually transmitted sexually. It is subclassified by its stage in the disease to primary, secondary, latent, and tertiary types. The primary infection appears as a chancre, which may be undetected, with associated painless lymphadenopathy. A delayed hypersensitivity reaction resolves the lesions, but some organisms survive and may cause a persistent infection. Secondary syphilis manifests as a cutaneous eruption with lymphadenopathy, along with fever, malaise, sore throat, and joint pain (Wilhelmus and Lukehart 1996). This stage remits even if not treated, but relapses can occur in up to 25% of people, usually during the first year of infection but up to decades later during the phase of latent syphilis.


Seventy-two percent of patients with latent syphilis will develop no further complications or relapses, but 28% will progress to tertiary syphilis (Kolker et al. 1997). This is an advanced disease state with cardiovascular and neurological conditions involving the meninges, brain vessels, brain parenchyma, optic nerve, and posterior column in the spinal cord. The most common ocular manifestation of secondary and tertiary syphilis is uveitis (Wilhelmus and Lukehart 1996). Ocular syphilis can occur in any stage of disease (Fig. 19.2).

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Fig. 19.2

This 56-year-old non-HIV-positive man had been treated for 3 years for a smoldering intermediate uveitis with mild vision loss in the right eye. There was a history of bicillin penicillin treatment for syphilis within the last 5 years. Lumbar puncture revealed a positive VDRL. (a) Late stage fluorescein angiogram of the right eye at the time of presentation showing diffuse retinal leakage, optic nerve leakage, and pigmentary changes. The vessels are emptied of dye. Vision was 20/30. (b) Late stage fluorescein angiogram of the left eye at presentation showing similar retinal and optic nerve leakage and pigmentary changes. Retinal perfusion was somewhat better in this eye, which was 20/20. (c) Late stage fluorescein angiogram of the right eye 2 years after two courses of intravenous penicillin. Optic nerve leakage has resolved, the retina is better perfused. There is persistent leakage in the center of the macula and extensive pigmentary change. (d) Late stage fluorescein angiogram of the left eye 2 years after treatment. Retinal perfusion is improved, with resolved optic nerve leakage and residual central leakage. Pigmentary changes are noted


Clinical Presentations


Anterior Segment Findings in Ocular Syphilis


Compared to other infectious and inflammatory etiologies affecting the eye, syphilis is highly variable in presentation with the ability to involve all structures of the eye (Davis 2014) (Table 19.1). Although there are some characteristic features, syphilis can also present as a nonspecific anterior, intermediate, posterior, or panuveitis (Cunningham et al. 2014). Interstitial keratitis typically occurs in children and adolescents with congenital syphilis and is often associated with conjunctival hyperemia and iritis (Aldave et al. 2001; Margo and Hamed 1992). Dilated iris vessels known as roseola are rare but specific (Margo and Hamed 1992). Large granulomatous keratic precipitates, hypopyon, posterior synechiae, and elevated intraocular pressure can accompany iridocyclitis (Reddy et al. 2007). Episcleritis/scleritis has been reported (Fenolland et al. 2016).


Table 19.1

Ocular features of syphilis by site and stage of disease






























Site/stage

Congenital

Secondary

Tertiary

Uveal tract

Acute iritis


Secondary cataract or glaucoma

Iridocyclitis


Iris nodules


Isolated vitritis

Iridocyclitis


Single or multiple gummas


Retina

Retinal pigmentary mottling in “salt-and-pepper” pattern


Retinal vasculitis

Focal or multifocal chorioretinitis


Multifocal choroidal infiltrates


Necrotizing retinitis


Neuroretinitis


Retinochoroiditis


Retinal vasculitis


Serous retinal detachment


Cystoid macular edema

Focal or multifocal chorioretinitis


Necrotizing retinitis


Neuroretinitis


Retinochoroiditis


Retinal vasculitis


Serous retinal detachment


Cystoid macular edema


Optic nerve

Optic atrophy

Inflammatory disc edema


Papilledema

Inflammatory disc edema


Papilledema


Optic atrophy


Gumma of optic disc


Posterior Segment Findings in Ocular Syphilis


Posterior segment findings of acquired syphilis are varied. Rather than necrosis, the infection produces retinal edema, often with subretinal fluid. The inflamed retina usually has an opacified appearance that differs from the classical whitening of herpetic necrotizing retinitis or toxoplasma chorioretinitis. The edema can be focal (Fig. 19.3) or diffuse (Fig. 19.4). If there is substantial subretinal fluid, there can be actual serous detachment, although this is usually shallow (Fig. 19.5). Fluorescein angiography is important in detecting retinal vasculitis, which is often nonspecific and confused with a noninfectious uveitis (Fig. 19.6). Cystoid macular edema is common in eyes with retinal vascular leakage (Fig. 19.7). Necrotizing retinitis and branch vein occlusion have been described (Jumper et al. 2000; Villanueva et al. 2000; Venkatesh et al. 2002; Yokoi and Kase 2004). Optic nerve manifestations include inflammatory disc edema (Fig. 19.8), neuroretinitis, pallor, and optic nerve gumma, a solid inflammatory lesion (Margo and Hamed 1992).

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Fig. 19.3

This 43-year-old HIV-positive man complained of cloudy vision in the right eye for a few months. Fundus examination showed a region of retinal swelling above the superotemporal arcade with vitreous inflammation. The photograph depicts white, focal preretinal opacities. There is some irregularity of the vascular caliber in the region of greatest retinal whitening. He was serologically positive by both treponemal and non-treponemal tests, confirming the diagnosis of syphilitic uveitis


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Fig. 19.4

Left fundus photograph of a 36-year-old HIV-positive man who presented with subacute vision loss in both eyes in the setting of secondary syphilis. Diffuse retinal edema with possible shallow subretinal fluid was present in both eyes. Focal preretinal vitreous opacities are visible at the edge of the frame. Sheathing of vessels is seen inferonasally


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Fig. 19.5

Color photograph of the right fundus of a man presenting with syphilitic uveitis. The inferotemporal quadrant shows retinal whitening and vascular sheathing. There is substantial subretinal fluid and retinal edema around the nerve


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Fig. 19.6

A nonspecific diffuse small and large retinal vasculitis with optic nerve hyperfluorescence and cystoid macular edema is depicted in this late stage fluorescein angiogram of a left eye in a patient with ocular syphilis


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Fig. 19.7

The OCT scan of the macula of the eye depicted in Fig. 19.6. There is typical cystoid macular edema with subfoveal fluid. Although the outer retina can be disrupted in syphilitic uveitis, it appears mostly intact in this patient


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Fig. 19.8

Relatively isolated syphilitic optic nerve edema is depicted in this fluorescein angiogram of a left eye. The OCT scan through the nerve shows diffuse thickening of the perineural tissue. The macular contour is normal. The amount of retinal leakage is small although there is some vascular tortuosity


Some distinctive posterior segment features may help support the diagnosis of syphilis. Superficial retinal precipitates may occur in syphilitic panuveitis, often associated with other inflammatory signs (Fu et al. 2010; Wickremasinghe et al. 2009) (Fig. 19.9). These small, creamy white focal opacities appear to migrate over the surface of inflamed retina during the course of disease and treatment, but do not float. Other focal opacities line up along the retinal arteries in some cases, similar to the so-called Kyrieleis plaques described in toxoplasma chorioretinitis (Krishnamurthy and Cunningham 2008). The involved retina in cases of syphilitic retinitis usually heals with milder alteration of the retinal pigment epithelium and less retinal scarring than in herpetic retinitis, but chronic cases can experience substantial scarring (Baglivo et al. 2003) (Figs. 19.10 and 19.11).

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Fig. 19.9

White focal preretinal opacities overlying inflamed retina are virtually pathognomonic for syphilitic uveitis. They are migratory and transient, occurring only in the acute stages of the disease. In this photograph of the superonasal region of a right eye, there are also many sheathed and occluded retinal vessels. In some areas, there appear to be retinal infiltrates (arrowhead)


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Fig. 19.10

Follow-up photograph of the patient in Fig. 19.3 after treatment of syphilitic uveitis with intravenous penicillin. There is extensive destruction of the retinal pigment epithelium, retinal thinning, and a permanent scotoma


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Fig. 19.11

Follow-up photograph of the patient in Fig. 19.5 after treatment of syphilitic uveitis with intravenous penicillin. There is a leopard spot pattern of RPE destruction in the region of subretinal fluid. Retinal vascular closures are present in the areas of pigmentary change. There is residual staining of large retinal veins in the peripheral and hyperfluorescence of the optic nerve


Another distinctive finding is acute syphilitic posterior placoid chorioretinitis (Gorovoy and Desai 2013; Gass et al. 1990). This finding is attributed to a uniform inflammatory process involving the outer retina and inner choroid in a circular fashion on the posterior pole (Eandi et al. 2012) (Fig. 19.12). On spectral domain optical coherence tomography (OCT), there may be subretinal fluid and disruption of the ellipsoid zones (Pichi et al. 2014) (Fig. 19.13). With treatment, the subretinal fluid resolves with longer persistence of the disruption of the outer retina. Deposits may develop at the level of the RPE, which may resolve in time. Angiography may also help in distinguishing placoid syphilis from other etiologies, such as viral retinitis, lymphoma, and sarcoidosis. On indocyanine green (ICG) angiography, placoid lesions typically show diffuse early and late hypofluorescence, and fluorescein angiography shows a progressive diffuse leakage (Eandi et al. 2012) (Fig. 19.14). This pattern is specific to syphilitic uveitis compared to other types of infectious retinitis. The placoid lesion is hyperautofluorescent, probably due to the loss of the masking effect of the outer retina (Jumper and Randhawa 2012; Knecht et al. 2013).

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Fig. 19.12

Dense retinal swelling and pigment change affecting the temporal macula. This may be a preferential site of involvement in syphilitic uveitis


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Fig. 19.13

Outer retinal disruption in a man complaining of recent vision loss in the left eye. The outer retina is diffusely abnormal without cystoid macular edema or, in this case, subretinal fluid. Other clinical findings of this case are depicted in Fig. 19.14


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Mar 22, 2020 | Posted by in OPHTHALMOLOGY | Comments Off on 19. Syphilis

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