Case Presentation
- 59-year-old man with 2-week history of acute-onset, progressive vision loss and central scotomas in both eyes.
- No systemic symptoms, eye redness, pain, or photophobia.
- Medical history: prostate cancer (post-cryotherapy), osteoarthritis, migraines.
- Social history: active smoker, immigrated from a Middle Eastern country (TB-endemic region).
- Medications: naproxen (500 mg PRN), tamsulosin (0.4 mg daily), aspirin (81 mg daily).
Examination Findings
- Best-corrected visual acuity (BCVA): 20/40 OD, 20/25 OS.
- Normal pupils, extraocular motility, and intraocular pressure (16 mm Hg OU).
- Unremarkable anterior segment (no cell/flare in anterior chamber or vitreous).
- Fundus photography: Yellow-gray, curvilinear, puzzle piece-shaped subretinal macular lesions bilaterally.
- Fundus autofluorescence (FAF): Hyperfluorescent lesions with hypofluorescent borders.
- Optical coherence tomography (OCT): Disruption of outer retinal layers (outer nuclear layer and deeper), hyperreflectivity of outer retina, retinal pigment epithelium (RPE), and choroid; no inner retina involvement.
- Negative lab results for syphilis and tuberculosis (TB).
Diagnosis
- Macular serpiginous choroiditis—a variant of serpiginous choroiditis.
Key Features of Serpiginous Choroiditis
- Bilateral, asymmetric, recurrent, progressive inflammatory condition affecting RPE, choriocapillaris, and choroid.
- Typically affects healthy young to middle-aged adults, more common in men.
- Pathogenesis: Likely organ-specific autoimmune inflammation, no clear infectious/systemic autoimmune link.
- Symptoms: Decreased vision, metamorphopsia, scotomas, floaters; anterior segment usually quiet (minimal/no cell/flare).
- Three patterns:
- Classic (80%): Grayish/yellowish serpentine subretinal infiltrates starting juxtapapillary, extending centrifugally; may have serous retinal detachments or edema.
- Macular: Serpentine lesions in macula, not contiguous with juxtapapillary area (as in this case).
- Ampiginous (relentless placoid chorioretinitis): Multifocal lesions in periphery and posterior pole.
- Lesion evolution: Inactive over 6-8 weeks, leaving RPE/choriocapillaris atrophy; recurrences in ~50% within 5 years, often at atrophic patch borders.
- Complications: Macular vision loss, chorioretinal atrophy, choroidal neovascularization (CNV), subretinal fibrosis, retinal ischemia, vascular occlusion, neovascularization, serous/pigment epithelial detachments.
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Imaging Characteristics
- Fluorescein Angiography (FA):
- Early hypofluorescence (atrophic choriocapillaris).
- Late hyperfluorescence at lesion borders.
- Indocyanine Green Angiography (ICGA): Hypofluorescence in areas of choriocapillaris nonperfusion (early and late phases).
- OCT: Hyperreflectivity of outer retina, choriocapillaris, choroid; disruption of ellipsoid zone and outer layers.
- FAF: Varies by lesion stage—hyperfluorescence (active), hypofluorescence (inactive); useful for monitoring.
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Differential Diagnosis
- Must distinguish from infectious multifocal serpiginoid choroiditis (e.g., TB, herpes, syphilis)—requires antimicrobial therapy.
- Patient’s TB-endemic origin necessitated TB testing before corticosteroids.
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Management
- Best next step: Discuss risks/benefits of oral corticosteroid therapy (Option C).
- Treatment options: Systemic corticosteroids (e.g., prednisone 1 mg/kg) ± immunomodulatory therapy (e.g., methotrexate).
- Rationale: Reduces inflammation, prevents progression; limited definitive evidence of benefit, so weigh systemic risks.
- No role for:
- Multivitamins (Option A): No evidence for inflammatory choroiditis.
- Intravitreal anti-VEGF (Option B): Only if CNV or macular edema present (not seen here).
- Observation alone (Option D): Less prudent given risk of significant vision loss.
- Patient outcome: High-dose prednisone (1 mg/kg) tapered weekly, methotrexate (25 mg) added; stable VA (20/30 OD, 20/25 OS) at 8 months, no significant progression.
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High-Yield Takeaways for Exams
- Macular serpiginous choroiditis presents with acute vision loss, central scotomas, and characteristic macular lesions (yellow-gray, curvilinear, puzzle piece-shaped).
- Imaging hallmarks: FA (early hypo → late hyper at borders), ICGA (persistent hypofluorescence), OCT (outer retinal disruption/hyperreflectivity), FAF (hyper/hypofluorescence by stage).
- Recurrence risk (~50% in 5 years) and complications (e.g., CNV, atrophy) drive treatment decisions.
- Rule out infectious mimics (TB, syphilis) before immunosuppression, especially with relevant history (e.g., TB-endemic region).
- Corticosteroids ± immunomodulators are mainstay therapy; observation risks vision loss, anti-VEGF reserved for CNV.