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Fall 2017 Newsletter

Follow our latest updates in our Summer 2019 Newsletter

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: Snakes on an Eye

Greer Ameri
Presenter: Jonathan Lu, MD Discussant: Hossein Ameri, MD, PhD
 

History

  • 59 year-old woman with history of diabetes, hypertension, cervical cancer, and choroidal lesion of right eye three years ago presents through ER with eye discomfort

Exam Findings

  • Vision 20/100 right eye, 20/20-1 left eye
  • Pupils round and reactive without RAPD
  • Pressures 10 right eye, 14 left eye
  • Anterior exam unremarkable other than dry corneas and mild cataracts
  • Posterior exam: chorioretinal lesions branching out from peripapillary region. The lesions reveal underlying choroidal vessels and sclera
Figure 1: Color fundus photos of left and right eye, demonstrating chorioretinal lesions restricted to the posterior pole, emanating from the peripapillary region.
Figure 2: Close-up color fundus photo, revealing choroid and choroidal vessels. Pigmentary changes evident at fovea.

 

Differential Diagnosis

  • Serpiginous choroiditis
  • Multifocal serpiginoid choroiditis (MSC), previously tubercular serpiginous-like choroiditis
  • Ampiginous choroiditis
  • Toxoplasmosis
  • Syphilis
  • Age-related macular degeneration / other retinal dystrophy
  • Herpetic choroiditis
Figure 3: Fundus autofluorescence photo, demonstrating areas of hypofluorescence due to RPE loss and areas of autofluorescence correlating to exposed sclera from retinal atrophy.
Figure 4: Fundus auto-fluorescence: demonstrating bright signal from exposed sclera. In the left eye superior to the nerve, there is a wavefront of hyperautofluorescence surrounded by hypoautofluorescence, suspicious for activity of disease.
Figure 5: Fluorescein angiography demonstrating transmission window defects.

 

Additional Investigations

  • OCT macula imaging: outer retina and RPE atrophy correlating in areas of lesion.
  • Fluorescein angiography: at various stages, with all showing choroidal and scleral signal due to transmission defect from retinal atrophy.
  • Labs
    • QuantiFERON gold: negative x2
    • RPR/FTA: negative
    • HIV: negative
    • CBC: unremarkable
Figure 6: OCT and corresponding areas on infrared fundus photo. Blue line: RPE and ONL atrophy. Red line: hyperreflectivity in ONL. (Carreno et al)

 

Diagnosis

  • Serpiginous choroiditis
    • Rare (0.3%-5% of uveitis cases)
    • Inflammatory disorder of the outer retina and inner choroid
      • Chronic, progressive
      • Recurrent inflammation, months/years of inactivity between periods of activity
      • Extending from the nerve
      • Usually bilateral, but asymmetric. Average lag time five years
  • Symptoms
    • Metamorphopsia, floaters, flashes, scotomas, visual field deficits, blurred vision, or even asymptomatic
  • Demographic
    • Middle age
    • Without clear gender or race predilection
    • Possibly increased HLA-B27 association
  • Variants
    • Classic (~80%)
    • Patches of creamy yellow/gray sub-retinal infiltrates
    • Extending from the nerve
    • Active lesions resolve 6-8 weeks +/- treatment, with residual atrophy
    • Chronically recurrent, typically at edges of prior
  • Macular (5.9% to 30%)
    • Lesions starting at macula
    • Worse prognosis
    • Can be mis-diagnosed as age-related macular degeneration, toxoplasmosis, and macular dystrophy
  • Atypical
    • Periphery or multifocal
  • Techniques
    • Activity and reactivation
      • Fundus autofluorescence
        • Particularly great for detecting subtle peripheral lesions
    • Color fundus photography
    • Fluorescein angiography
    • Indocyanine green angiography
    • Amsler grid
  • Monitor for choroidal neovascularization (CNV)
    • 13-35% rate of occurrence
    • Clinical exam, OCT, fluorescein angiography

Pathophysiology

  • No established etiology
    • Infectious, inflammatory, and autoimmune theories

Treatment

  • Corticosteroids
    • Can shorten duration of active disease
    • PO prednisone 60-80 mg or IV methylprednisone 1 gm/day
  • Immunomodulatory agents (eg Cyclosporine A/azathioprine/mycophenolate mofetil)
    • Typically needed for serpiginous and to spare steroids
  • Alkylating agents (eg cyclophosphamide and chlorambucil) and antimetabolites
    • Limited data but appears effective. Last resort and thorough discussion with patient
  • Biologicals (eg interferon alpha-2a)

Prognosis and Future Directions

  • Serpiginous choroidopathy is a rare inflammatory condition of outer retina and choroid with poor visual prognosis due to permanent retinal damage
  • Thorough history and exam are necessary to distinguish it from other “white dot syndromes” and infectious etiologies such as tuberculosis, as these require different management and depending on the etiology may have better prognosis
  • Close monitoring with fundus auto-fluorescence and OCT are necessary. Fluorescein angiography is also important to monitor for CNV. Management focuses on catching and limiting recurrent flares as well as preventing recurrence
  • Research is ongoing about the exact etiology of serpiginous choroidopathy

References

  • Rodriguez A, Calonge M, Pedroza-Seres M, et al: Referral patterns of uveitis in a tertiary eye care center. Arch Ophthalmol 1996;114:593.
  • Blumenkranz MS, Gass JD, Clarkson JG: Atypical serpiginous choroiditis. Arch Ophthalmol 100:1773-5, 1982.
  • Carreño E, Fernandez-Sanz G, Sim DA, et al. Multimodal Imaging of Macular Serpiginous Choroidopathy From Acute Presentation to Quiescence. Ophthalmic Surg Lasers Imaging Retina. 2015;46(2):266-270. doi:10.3928/23258160-20150213-04.
  • Carreño E, Portero A, Herreras HM, and Lopez MI. Assesment of fundus autofluorescence in serpiginous and serpiginous-like choroidopathy. Eye (2012) 26, 1232-1236.
  • Christmas NJ, Oh KT, Oh DM, et al: Long-term follow-up of patients with serpinginous choroiditis. Retina 22:550-6, 2002.
  • EdelstenC, StanfordMR, GrahamEM. Serpiginouschoroiditis: anunusualpre- sentation of ocular sarcoidosis. Br J Ophthalmol. 1994;78(1):70-71.
  • Erkkila ̈ H, Laatikainen L, Jokinen E. Immunological studies on serpiginous choroiditis. Graefes Arch Clin Exp Ophthalmol 1982;219:131-4.
  • Chisholm IH, Gass JD, Hutton WL: The late stage of serpigi- nous (geographic) choroiditis. Am J Ophthalmol 82:343- 51, 1976.
  • Hooper PL, Kaplan HJ: Triple agent immunosuppression in serpiginous choroiditis. Ophthalmology 98:944-51; discus- sion 951-2, 1991.
  • Kawali A, Emerson GG, Naik NK, Sharma K, Mahendradas P, Rao NA. Clinicopathologic Features of Tuberculous Serpiginous-like Choroiditis. JAMA Ophthalmol. 2018;136(2):219. doi:10.1001/jamaophthalmol.2017.5791.
  • Lim W-K, Buggage RR, Nussenblatt RB. Serpiginous Choroiditis. Survey of Ophthalmology. 2005;50(3):231-244. doi:10.1016/j.survophthal.2005.02.010
  • Mahendradas P, Kamath G, Mahalakshmi B, Shetty KB. Serpiginous Choroiditis-Like Picture Due to Ocular Toxoplasmosis. Ocular Immunology and Inflammation. 2007;15(2):127-130. doi:10.1080/09273940701244202
  • Nazari Khanamiri H, Rao NA. Serpiginous choroiditis and infectious multifocal serpiginoid choroiditis. Surv Ophthalmol. 2013;58(3):203-232. doi:10.1016/j.survophthal.2012.08.008
  • Rifkin LM, Munk MR, Baddar D, Goldstein DA. A New OCT Finding in Tuberculous Serpiginous-like Choroidopathy. Ocular Immunology and Inflammation. 2015;23(1):53-58. doi:10.3109/09273948.2014.964421
  • Vasconcelos-Santos DV. Clinical Features of Tuberculous Serpiginouslike Choroiditis in Contrast to Classic Serpiginous Choroiditis. Arch Ophthalmol. 2010;128(7):853. doi:10.1001/archophthalmol.2010.116
  • Weiss H, Annesley WH, Shields JA, et al: The clinical course of serpiginous choroidopathy. Am J Ophthalmol 87:133- 42, 1979

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