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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 2  |  Issue : 3  |  Page : 708-710

Multimodal imaging in syphilitic retinitis with vasculitis in an immunocompetent patient: A case report


1 Department of Uvea, Sankara Netralaya, Chennai, Tamil Nadu, India
2 Department of Uveitis and Ocular Pathology, Sankara Netralaya, Chennai, Tamil Nadu, India

Date of Submission30-Nov-2021
Date of Acceptance07-Mar-2022
Date of Web Publication16-Jul-2022

Correspondence Address:
Dr. Jyotirmay Biswas
Department of Uveitis and Ocular Pathology, Sankara Netralaya, 41 College Road, Nungambakkam, Chennai - 600 006, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_2992_21

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  Abstract 


A 47-year-old male reported a sudden diminution of vision in the left eye for 1 month. Fundus examination showed dense vitritis, retinal hemorrhages around the disc with subretinal precipitates. Peripheral examination revealed snowball opacities. Multimodal imaging indicated syphilitic retinitis with vascular leakage. A complete systemic workup was performed to support the diagnosis. Intramuscular benzathine penicillin injection along with oral corticosteroids showed an excellent response as confirmed by the multimodal imaging. Syphilitic retinitis with vasculitis is uncommon and can occur in immunocompetent patients. Multimodal imaging can be used to demonstrate the extent of involvement and to monitor treatment response.

Keywords: Bacterial infection, immunocompetent patient, posterior uveitis, sexually transmitted, syphilitic retinitis


How to cite this article:
Ramdoss J, Jain A, Biswas J. Multimodal imaging in syphilitic retinitis with vasculitis in an immunocompetent patient: A case report. Indian J Ophthalmol Case Rep 2022;2:708-10

How to cite this URL:
Ramdoss J, Jain A, Biswas J. Multimodal imaging in syphilitic retinitis with vasculitis in an immunocompetent patient: A case report. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Aug 11];2:708-10. Available from: https://www.ijoreports.in/text.asp?2022/2/3/708/351163



Syphilis is a sexually transmitted infection caused by spirochete treponema pallidum.[1] Mode of transmission is primarily sexual or vertically from a mother to baby. Men are mostly affected and young men are disproportionately affected.[2] Ocular syphilis has protean manifestation and can occur at the secondary or tertiary stage of the disease. It can infect any part of the eye.[3] Posterior uveitis most commonly presents as chorioretinitis with vitritis involving the posterior pole and mid periphery.[4] It can be unilateral or bilateral with a unique ground glass appearance.[5] We here report a 47-year-old male, with unilateral syphilitic retinitis, confirmed by multimodal imaging with positive  Treponema pallidum Scientific Name Search magglutination assay (TPHA) and negative Rapid plasma reagin (RPR).


  Case Report Top


A 47-year-old male presented to the tertiary eye care center with a complaint of sudden diminution of vision in the left eye (LE) associated with on and off flashes of light for 1 month. No history of any systemic disease was noted. His best-corrected visual acuity was 6/9 and N6 in the right eye (RE) and 6/12 and N12 in the LE. Extraocular movements were full and the pupil was of normal size, round, react to light in the RE, fixed, and mid dilated in the LE. Intraocular pressure in both eyes was within normal limits. On slit-lamp examination, RE was normal, while in LE, there was a flare and few cells. On indirect ophthalmoscopy, RE was within normal limits, while in LE, there was dense vitritis (grade 3) along with retinal hemorrhages in the superonasal and inferonasal quadrant around the disc. Retinal peripheral examination revealed snowball opacities. All systemic workups which include RA factor, antinuclear antibody, serum angiotensin-converting enzyme, Mantoux, QuantiFERON TB gold, c-ANCA, p-ANCA to rule out infectious and noninfectious etiology were negative. Reactive plasma reagin (RPR) was done which was negative. TPHA was positive with a titer of 1:160. HIV 1 and HIV 2 testing was negative. CD4 helper T cell count was within normal range.

Ancillary testing swept-source optical coherence tomography (SS-OCT), swept-source optical coherence tomography angiography (SS-OCTA), fundus fluorescein angiography (FFA), Humphrey visual field (HVF) analysis was performed. RE was normal, while LE showed bumpy retinal pigment epithelium (RPE) with an ellipsoidal layer defect with hard exudates in the nasal quadrant. In FFA, capillary nonperfusion (CNP) areas in the superonasal quadrant were noted, while HVF [Figure 1] showed enlargement of blind spot SS-OCTA [Figure 2] also demonstrated CNP areas. The patient was consulted by an infectious disease specialist and was given intramuscular benzathine penicillin three doses at a 1-week interval after a test dose. The patient was started on systemic steroid 1 mg/kg body weight dosing with weekly taper by 10 mg/week and reviewed after 4 weeks. A cerebrospinal fluid tap was not advised by an infectious disease specialist. Contact tracing was done. The patient's history showed unprotected sex with a sex worker.
Figure 1: Montage fundus photograph and FFA at the time of presentation showing vitritis with vasculitis with subretinal precipitates along with vascular leakage with capillary nonperfusion

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Figure 2: SS-OCTA showing superficial and deep plexus with evident vasculitis and capillary nonperfusion at presentation

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At review, the patient was symptomatically better. On examination, RE was normal, in LE there were 2+ vitreous cells with resolving retinitis. Repeat ancillary testing was performed, SS-OCT showed few cystic spaces located temporally to the fovea, FFA revealed neovascularization in superonasal quadrant along with CNP areas, and a similar finding was noted in SS-OCTA [Figure 3] and [Figure 4]. TPHA along with RPR was repeated, TPHA was positive, and RPR was negative.
Figure 3: Montage fundus photograph and FFA at follow-up showing resolution of vitritis with resolved vasculitis and neovascularization

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Figure 4: FFA with montage at follow-up showing vascular leakage confirming neovascularization

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As vitritis and inflammation subsided with complete resolution of snowball exudates, the patient was advised sectoral laser photocoagulation along with slow tapering of steroid, with a 2-month follow-up. At 2-month follow-up RPR remained negative; TPHA was positive.


  Discussion Top


Acquired syphilis is a sexually transmitted infectious disease caused by the spirochete Treponema pallidum.[1] It affects multiorgan like skin, heart, bone, nervous system, and eye. Common ocular involvement includes iridocyclitis, papillitis, retinochoroiditis, retinal pigment epithelitis, serous retinal detachment, cystoid macular edema, vitritis, retinal vasculitis, and neuroretinitis.[4],[6] Most common presentation is retinochoroiditis. The clinical signs in our case were very specific of syphilitic retinitis especially the presence of superficial retinal precipitates. Other manifestations in the form of one or more yellowish-white patches of retinal necrosis, along with vasculitis, vitreous inflammation, and anterior segment inflammation were present. SS-OCT was performed to see the lesion at the outer retina and subretinal fluid, in cases of the placoid chorioretinitis, as if left untreated, it may develop geographic atrophy with hyperpigmentation of RPE. Shen et al.[7] reported FFA findings of CNP areas, neovascularization, vascular staining, disc hyper fluorescence, and macular edema. FFA in our patient showed vascular leakage indicating vasculitis with neovascularization. SS-OCTA demonstrated the presence of CNP with neovascular fronds. Diagnosis and staging of syphilis are done by various nonspecific tests [Venereal Disease Research Laboratory (VDRL) and RPR] and specific tests [Fluorescent Treponemal Antibody Absorption Test, T. pallidum particle agglutination]. Nonspecific tests quantify the amount of serum anticardiolipin antibodies by flocculation, while specific tests measure the amount of serum antibodies specifically directed against treponemal antigens.[8] RPR and VDRL test being nonspecific can be false positive or false negative depending upon the stage of the disease. Syphilitic uveitis shows an excellent response to injection penicillin; the adult regimen is intravenous aqueous crystalline penicillin G 18-24 million units per day for 10–14 days. The alternative is procaine penicillin 2.4 million units IM per day and probenecid 500 mg PO four times per day, for 10–14 days. Three doses of long-acting Benzathine penicillin G (2.4 million units administered intramuscularly) at weekly intervals are also recommended for patients with late latent syphilis or latent syphilis of unknown duration.[9]

Adjunctive treatment is corticosteroids (topical, periocular, and systemic) and other immunosuppressives. However, chances of Jarrisch Herxheimer reaction after the initiation of treatment is there which can be controlled with oral corticosteroids.


  Conclusion Top


Our case shows that syphilis can present as retinal vasculitis leading to neovascularization. Injection penicillin with corticosteroids can resolve retinal vasculitis; neovascularization can be treated with laser photocoagulation. Also, both RPR and TPHA should be considered in cases of retinal vasculitis to rule out syphilis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Yu HG. ed. Inflammatory and Infectious Ocular Disorders. Springer Nature, 2019.  Back to cited text no. 1
    
2.
Koundanya VV, Tripathy K. Syphilis Ocular Manifestations. In: StatPearls. StatPearls Publishing; 2021.  Back to cited text no. 2
    
3.
Kiss S, Damico FM, Young LH. Ocular manifestations and treatment of syphilis. Semin Ophthalmol 2005;20:161-7.  Back to cited text no. 3
    
4.
Dutta Majumder P, Chen EJ, Shah J, Ching-Wen Ho, D, Biswas J, See Yin L, et al. Ocular syphilis: An update. Ocul Immunol Inflamm 2019;27:117-25.  Back to cited text no. 4
    
5.
Fu EX, Geraets RL, Dodds EM, Echandi LV, Colombero D, McDonald HR, et al. Superficial retinal precipitates in patients with syphilitic retinitis. Retina 2010;30:1135-43.  Back to cited text no. 5
    
6.
Doris JP, Saha K, Jones NP, Sukthankar A. Ocular syphilis: The new epidemic. Eye (Lond) 2006;20:703-5.  Back to cited text no. 6
    
7.
Shen J, Feng L, Li Y. Ocular syphilis: An alarming infectious eye disease. Int J Clin Exp Med 2015;8:7770-7.  Back to cited text no. 7
    
8.
Singh AE, Romanowski B. Syphilis: A review with emphasis on clinical, epidemiologic, and some biologic features. Clin Microbiol Rev 1999;12:187-209.  Back to cited text no. 8
    
9.
Davis JL. Ocular syphilis. Curr Opin Ophthalmol 2014;25:513-8.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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