Grand Rounds The Great Imitator Patrick Burchell, PGY-3

Grand Rounds The Great Imitator Patrick Burchell, PGY-3

Grand Rounds The Great Imitator Patrick Burchell, PGY-3 January 11, 2019 Department of Ophthalmology and Visual Sciences Patient Presentation CC Sudden decreased vision in left eye HPI 26 yo AAM presents with a 1 week history of sudden decreased vision in his left eye. Noticed

upon awakening. Non-progressive. Associated flickering in periphery. No recent illness. History (Hx) Past Ocular Hx: Denied Past Medical Hx: Denied Fam Hx: Denied Meds: None Allergies: NKDA Social Hx: Everyday smoker, denied illicits ROS: +Headache External Exam OD

VA sc 20/20 Refraction OS 20/60-2 No improvement Pupils 43mm

43mm IOP 16 mmHg 15 mmHg EOM full full

CVF full full Anterior Segment Exam PLE or SLE OD OS External/Lids

WNL WNL Conj/Sclera WNL WNL Cornea Clear

Clear Ant Chamber Deep and quiet Deep and quiet Iris WNL WNL

Lens Congenital Cataract (lamellar) Congenital Cataract (lamellar) Posterior Segment Exam Fundus OD

OS Optic Nerve Elevation nasally, hyperemic Elevation nasally, hyperemic Vitreous Pigment cells in anterior vitreous

Pigment cells in anterior vitreous WNL Intraretinal creamy dots, blunted foveal reflex Normal caliber Normal caliber White without

pressure nasal to disc Few white dots along arcade Macula Vessels Periphery Color Fundus Photos OD OS

SD-OCT OD OS FAF 12/5 OD OS FA/ICG OS

56.52 1.19.12 FA/ICG OS 2.04.95 5.27.54 Assessment 26 yo AAM with a multifocal choroiditis OS Differential Diagnosis Syphilis

Sarcoidosis Tuberculosis White dot syndrome Idiopathic CBC, CXR, ACE, RPR, TP-PA, Quantiferon, HIV, HSV, CMV Course Pt was lost to follow up for 2 months but returned after an acute decrease in vision OD Stated that vision had improved OS Did not obtain lab work

Exam OD OS VA sc 20/100; PHNI 20/40-2; PH 20/25-2 Pupils

43mm 43mm IOP 7 mmHg 12 mmHg AC 1+ cell

quiet Posterior Segment Exam Fundus OD OS Optic Nerve Elevation nasally, hyperemic

Elevation nasally, hyperemic Vitreous Pigment cells in anterior vitreous Pigment cells in anterior vitreous 3 dot heme surrounding fovea, blunted foveal reflex

Intraretinal creamy dots, blunted foveal reflex Normal caliber Normal caliber White without pressure nasal to disc Few white dots along arcade

Macula Vessels Periphery SD- OCT OD 10/18 OD12/5 SD-OCT OS 10/18 OS 12/5

FAF 10/18 12/05 Plan Pt left clinic prior to full workup Later called with progression of vision loss and obtained lab studies Outpatient Lab Results RPR+, titer 1:512, TP-PA+ HIV+ CMV IgG+, IgM EBV IgG+, IgM HSV IgG+, IgM Normal CXR

Hospital Course Pt was instructed to go to the ED, where he was admitted for treatment Started on IV Penicillin G 24 million units Neurosyphilis dosing Lumbar Puncture VDRL-, no pleocytosis, normal protein & glucose, Cryptococcus Discharged on IV Penicillin G X 2 weeks Syphilis Multisystem, chronic bacterial infection

caused by spirochete Treponema pallidum Transmission Transplacental (After 10th week) Sexual (Most common) Incidence 9.7/100.000 Men who have sex with men (MSM) African Americans https://labtestsonline.org/ tests/syphilis-tests Congenital Syphilis Hepatosplenomegaly, desquamating rash,

bone abnormalities, Hutchinson teeth, Mulberry molars, deafness (CN VIII), cardiac abnormalities, ocular signs Congenital Syphilis Ocular Findings Panuveitis Salt & Pepper fundus Multifocal chorio-retinitis Retinal Vasculitis Optic Neuritis Argyll-Robertson pupil Interstitial Keratitis Hutchinson Triad

Acquired Syphilis Primary painless chancre Secondary lymphadenopathy, rash on palms/soles Tertiary gummas, neurological/cardiac involvement Ocular involvement at any stage Acquired Syphilis

Ocular involvement 5 - 8% of cases Usually secondary & tertiary stages Great Masquerader Iris Roseola Can involve all structures including pupillomotor pathways and optic nerve Posterior uveitis most

common Multifocal chorioretinitis Posterior Placoid Chorioretinitis Workup and treatment Serologic testing Non-treponemal (RPR, VDRL) + Treponemal (TP-PA) Include HIV Lumbar puncture Ocular syphilis = Neurosyphilis

IV Penicillin G 18-24 million units per day for 10-14 days IM Procaine penicillin 2.4 million units daily + probenecid 500 mg QID for 10-14 days Conclusions Syphilis should always be on your differential With prompt diagnosis it is curable with penicillin! Ocular syphilis = neurosyphilis Commonly coinfected with HIV Thank You

Dr. Wang Dr. Fleissig Dr. Piri References 1. 2. 3. 4. 5. 6. BCSC Section 9, Intraocular Inflammation and Uveitis

Wells J, Wood C, Sukthankar A, Jones NP. Ocular syphilis: the reestablishment of an old disease. Eye (2018) 32: 99-103. Lapere S, Mustak H, Steffen J. Clinical Manifestations and Cerebrospinal Fluid Status in Ocular Syphilis. Ocular Immunology and Inflammation (2018) 00: 1-5. Pichi F, Ciardella AP, Cunningham ET, et al. Spectral domain optical coherence tomography findings in patients with acute syphilitic posterior placoid chorioretinopathy. Retina (2014): 34; 373-384. Davis J. Ocular Syphilis. Ocular Manifestations of Systemic Disease (2014) 25; 513-518. Lima LH, Costa de Andrade G, et al. Multimodal imaging analyses of hyperreflective dot-like lesions in acute syphilitic posterior placoid chorioretinopathy. Journal of Ophthalmic Inflammation and Infection (2017) 7: 1-6.

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