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.
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
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.
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.
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
Contact
Hossein Ameri, MD, PhD, Assistant Professor of Clinical Ophthalmology, ameri@med.usc.edu
