Fall 2017 Newsletter

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Fall 2017 Newsletter
7th Residency Announcement

Exciting Residency Announcement!

ACGME approves seventh resident complement; first increase in over 30 years!

Grand Rounds

Grand Rounds and Case Studies

Check out our weekly presentations

OTEP

OTEP

Ophthalmic Technician Education Program

LARGEST STUDY ON CHINESE AMERICANS PUBLISHED

LARGEST STUDY ON CHINESE
AMERICANS PUBLISHED

USC Ophthalmology Researchers Find More
Effective Treatments For Blinding Eye Diseases

EDUCATION

Case Study: As Good as Mercury

Greer Patel
Presenter: Christine Greer, MD Discussant: Vivek Patel, MD
 

History

  • 44-year-old male with a history of optic neuropathy OD in 2011
  • Presents to LAC+USC Medical Center in May 2016 with pain OS associated with superotemporal injection
    • Pain is intermittent, 10/10, shoots from the periorbital area across the left temple to the left side of the head
    • No tenderness to palpation of the left temple; no jaw claudication
    • No photophobia
  • In May 2016 patient was diagnosed with idiopathic scleritis; started on topical and oral steroids with taper
  • September 2016 patient presented with decreased vision OS with persistent (though improved) pain and injection

Exam Findings

  • VAsc
    • OD: 20/100
    • OS: 20/50 PH 20/40
  • Pupils: Round and Reactive OU, 2+ RAPD OD
  • Ishihara color plates: OD 1/8 , OS 6/8 (decreased OS from prior exam of 8/8)
  • IOP OD: 12, OS: 12
  • External exam
    • Violaceous injection of the scleral vessels (non-blanching with phenylephrine), more predominate in the superotemporal quadrant
    • No scleral thinning
  • Slit Lamp Exam (pertinent positives and negatives)
    • K: clear OS
    • AC: deep and quiet OS
    • Iris: flat, round, no nodules
    • Vitreous: syneresis, no cell OS
  • Posterior segment
    • Media: clear
    • ON: OD Pale; OS CDR 0.1, hyperemic with increased vascularity temporally and temporal swelling, no obscuration of the vessels coursing over the nerve, no hemorrhages
    • Macula: flat OU, no exudates OS
    • Vessels: normal, no vascular sheathing
    • Periphery: normal, no holes, tears or detachments

Figure 1
Figure 1: Color disc photograph of the right eye demonstrates optic disc atrophy (CDR 0.1).

 
Figure 2
Figure 2: Color disc photograph of the left eye, cup-to-disc ratio of 0.1, the disc appears hyperemic with increased vascularity temporally with associated temporal swelling. There is no obscuration of the vessels coursing over the nerve. No hemorrhages.

 

Differential Diagnosis

  • Elevated ICP
  • Inflammation
  • Infection
  • Demyelination
  • Sarcoidosis
  • Vasculitis
  • Compression (Foster-Kennedy syndrome)
  • Infiltration

Additional Investigations

  • Fluorescein angiogram
    • Demonstrated early disc leakage with late staining OS
Figure 4
Figure 3: Fluorescein angiogram of the left eye demonstrates in A. arterial phase early temporal disc leakage and in B. venous phase late temporal disc staining.
  • Visual fields (Humphrey Visual Field 24-2)
    • OD: reliable, inferior altitudinal defect, enlargement of the blind spot
    • OS: reliable, normal

Figure 4
Figure 4: Humphrey Visual Field 24-2 of the right eye: reliable, notable for an inferior altitudinal defect and enlargement of the blind spot, classic findings in optic neuropathy.

 
Figure 5
Figure 5: Humphrey Visual Field 24-2 of the left eye: reliable exam, normal visual field exam.

  • Laboratory testing September 2016
    • RPR 1:2
    • FTA ABS +
    • CSF 5 cells, protein 30, Glucose 191 (serum 298)
    • CSF VDRL negative
    • HIV negative
    • Additional infectious/inflammatory workup negative
  • History
    • In 2011 the patient had tested positive for a “venereal disease” and was treated with 2-3 intramuscular injections

Diagnosis

  • Ocular syphilis (Neurosyphilis)

Pathophysiology

  • Syphilis is an infection of the spirochete T. Pallidum. It is spread through direct contact with a chancre; transmission occurs during sexual contact. Pregnant women can also transmit the disease through the placenta within the first 16 weeks of pregnancy.
  • Syphilis is characterized by the phases of infectious process:
    • Primary syphilis: chancre at site of inoculation
    • Secondary syphilis: fever, malaise, lymphadenopathy, rash
    • Early latent (year) and late latent (decades)
    • About 1/3 progress to tertiary syphilis
    • 2/3 of patients have subclinical disease or the infection clears
  • Tertiary syphilis: Neurosyphilis, Cardiac, Benign (gummas)
  • Ocular syphilis is considered Neurosyphilis from a management standpoint and its manifestations vary widely through the anterior and posterior segments
    • Anterior segment
      • Interstitial keratitis (congenital > acquired)
      • Posterior synechiae
      • Lens dislocation
      • Iris roseola
      • Vascularized papules (iris papulosa)
      • Large red iris nodules (iris nodosa)
      • Gummata
      • Iris atrophy
    • Posterior segment
      • Vitritis/posterior and panuveitis
      • Chorioretinitis
      • Focal retinitis
      • Retinal vasculitis
      • Exudative RD
      • Isolated papillitis
      • Neuroretinitis
    • Neuro-ophthalmologic findings of syphilis
      • Argyll Robertson pupil
      • Oculomotor nerve palsies (meningovascular syphilis)
      • Optic neuropathies
      • Retrobulbar optic neuritis
  • HIV may modify the natural course of syphilis by modulating the immunologic response to T. pallidum
    • Evidence demonstrates that the ophthalmologic manifestations significantly differ between HIV+ patients and HIV- patients
    • In addition, significant difference in patient serology:
      • Higher RPR titers in HIV+ patients
      • More likely to test positive for VDRL in CSF if HIV+

Diagnostics

  • Nontreponemal Tests: cardiolipin-cholesterol-lecithin antigen
    • Rapid plasma reagin (RPR)
    • Venereal Disease Research Laboratory (VDRL)
      • Toluidine Red Unheated Serum Test (TRUST)
    • Changes in titer are followed after treatment to detect therapeutic response
    • 1 to 2 percent False Positives
  • Treponemal Tests
    • Fluorescent treponemal antibody absorption (FTA-ABS)
    • Microhemagglutination test for antibodies to T. pallidum (MHA-TP)
    • T. pallidum particle agglutination assay (TPPA)
    • T. pallidum enzyme immunoassay (TP- EIA)
    • Chemiluminescence immunoassay (CLIA)
    • Positive for life
  • Negative Non-Treponemal Test
    • For most patients this precludes a diagnosis of active syphilis
    • If symptomatic consider:
      • Early empiric treatment (seroreversion)
      • In early syphilis: Testing could be prior to Antibody formation, or prozone reaction (2 percent)
      • Late syphilis: advanced immunosuppression (B cell failure), natural history
  • Interpretation of CSF
    • VDRL in CSF = highly specific; poor sensitivity
      • If positive establishes the diagnosis
    • FTA-ABS can be ordered = sensitive; not specific
    • CSF-VDRL false+: traumatic tap when serum nontreponemal titer high
    • Pleocytosis >5 and Protein >45 is consistent with the diagnosis, but is also non specific
    • Non-specific pleocytosis also occurs in setting of HIV infection and makes interpretation difficult

Treatment

  • Treat ocular syphilis as neurosyphilis
    • Aqueous crystalline penicillin G (18 to 24 million units per day)
    • 3 to 4 mil units IV q 4 H 10 to 14 days
    • Desensitization in patients with severe allergy
    • May consider doxycycline and ceftriaxone as alternatives in patients with severe allergy (excluding pregnancy)

References

  • Gajula V, Kamepalli R, Kalavakunta JK. A star in the eye: cat scratch neuroretinitis. Clinical Case Reports. 2(1):17.
  • Hicks, CB and M Clement. Syphilis: Screening and diagnostic testing, Post TW (Ed), UpToDate, Waltham, MA. (Accessed on January 08, 2017)
  • Marra CM. Neurosyphilis. In: UpToDate, Post TW (Ed), UpToDate, Waltham, MA. (Accessed on January 15, 2017)
  • Lee SY, Cheng V, Rodger D, Rao N. Clinical and laboratory characteristics of ocular syphilis: a new face in the era of HIV co-infection. J Ophthal Inflamm Infect. 2015 Dec; 5(1):56
  • Liu L, Lin L, Tong M, Zhang H, Huang S, Chen Y, et al. Incidence and Risk Factors for the Prozone Phenomenon in Serologic Testing for Syphilis in a Large Cohort. Clinical Infectious Diseases (2014);59(3):384-389.
  • Read RW, Acharya N, Levinson RD, Rao PK, Sen HN, Walker JD, et al. Section 09, Intraocular Inflammation and Uveitis. In: Cantor LB, Rapuano CJ, Cioffi GA, 2016-17 AAO, Basic and Clinical Science Course. Section 9: Intraocular Inflammation and Uveitis, 2016:359.
  • Weisenthal, RW, Afshari, NA, et al. Cantor LB, Rapuano CJ, Cioffi GA. 2016-2017 AAO, Basic and Clinical Science Course. Section 8: External Disease and Cornea, 2016:359.

Contact

Section Editors

  • Vivek Patel, MD, Associate Professor of Clinical Ophthalmology, Program Director, vivek.patel@med.usc.edu
  • Jesse Berry, MD, Assistant Professor of Clinical Ophthalmology, Associate Program Director, jesse.berry@med.usc.edu
  •  

    Produced by: Monica Chavez, John Daniel, Joseph Yim and Dr. Vivek Patel
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