Case Series Overview
- Cohort: 3 men, ages 55-68 years, all with bilateral persistent placoid maculopathy (PPM).
- Median time to presentation: 3 months (range 2-24 months); follow-up: 8 months (range 3-24 months).
- Features: Recurrence and choroidal neovascularization (CNV) in 1 patient; novel findings of far-peripheral lesions and optic nerve hyperfluorescence.
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Clinical Presentation
- Symptoms: Central scotomas (Case 1), bilateral vision loss (Cases 2 and 3).
- Initial BCVA: 20/20-20/30 (Case 1), 20/125-20/300 (Case 2), 20/125 OU (Case 3).
- Fundus: Whitish geographic plaques in deep macular layers, ± mottled RPE pigmentary changes, ± peripheral lesions (Case 3).
- Associated findings: Trace anterior vitreous cells (Case 2), post-flu-like illness (Case 3).
Imaging Characteristics
- Fundus Autofluorescence (FAF):
- Case 1: Largely unremarkable.
- Cases 2 & 3: Stippled hyperfluorescence corresponding to pigmentary deposits.
- Fluorescein Angiography (FA):
- Early hypofluorescence of lesions (blockage).
- Late-phase filling, ± stippled hyperfluorescence/staining around pigmentary changes.
- Unique: Optic nerve hyperfluorescence (Case 2).
- Indocyanine Green Angiography (ICGA):
- Persistent hypofluorescence in early and late phases.
- Early phase: Faintly visible deep choroidal vessels, obscured later.
- Spectral Domain OCT (SD-OCT):
- Baseline: Homogeneous reflectivity/thickening of inner choroid, attenuation of outer retina (ellipsoid zone), sub-RPE deposits, flecks of hyperreflectivity.
- Post-treatment: Near-complete resolution of choroidal and outer retinal abnormalities (Cases 1 & 3); recurrence in Case 2.
Disease Course and Management
- High-dose corticosteroids (0.75-1 mg/kg/day):
- Rapid improvement (days-weeks) in scotomas, lesion whitening, ICG hypofluorescence, SD-OCT abnormalities, and microperimetry.
- Case 1: IV methylprednisolone + oral prednisone; stable 20/25 OU at 8 months.
- Case 2: Prednisone → methotrexate → mycophenolate mofetil + cyclosporine; recurrence at 6 months, bilateral CNV treated with bevacizumab, final VA 20/200 OD, 20/500 OS at 24 months.
- Case 3: Prednisone after doxycycline (for weakly positive Bartonella); 20/63 OU at 3 months.
- CNV: Developed in Case 2, responsive to bevacizumab but poor final VA.
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Pathogenesis Insights
- Inflammatory origin in inner choroid, with secondary RPE and outer retina changes.
- Evidence:
- SD-OCT: Primary homogenous reflectivity/thickening in inner choroid, minimal outer retinal changes initially (Case 1).
- ICGA: Persistent hypofluorescence, possibly due to cellular infiltrate masking (not just hypoperfusion).
- Rapid corticosteroid response supports inflammation.
- Progression: Delayed presentation (Cases 2 & 3) linked to worse VA and irreversible RPE/outer retina damage.
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Differential Diagnosis
- PPM vs. APMPPE:
- PPM: Older patients (50-68), lesions persist months, CNV common.
- APMPPE: Younger patients, resolves weeks, rare CNV.
- PPM vs. Relentless Placoid Chorioretinitis (RPC):
- PPM: Posterior pole, slow resolution, no significant scarring.
- RPC: >50 lesions across equator, relentless new crops, severe scarring.
- PPM vs. Macular Serpiginous Choroiditis:
- PPM: No contiguous spread, minimal scarring.
- Serpiginous: Peripapillary/macula, contiguous recurrences, chorioretinal atrophy.
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High-Yield Takeaways for Exams
- PPM: Bilateral, idiopathic, whitish macular plaques, older patients (50-60s), high CNV risk.
- Imaging hallmarks: FA (early hypo → late filling), ICGA (persistent hypo), SD-OCT (inner choroid reflectivity, outer retina attenuation).
- Novel findings: Far-peripheral lesions (Case 3), optic nerve hyperfluorescence on FA (Case 2)—expands PPM spectrum.
- Management: High-dose corticosteroids (0.75-1 mg/kg/day) for rapid short-term benefit; bevacizumab for CNV.
- Pathogenesis: Inner choroid inflammation → secondary RPE/outer retina damage; delay in treatment may worsen outcomes.
- Distinguish from mimics: APMPPE (acute, self-limiting), RPC (relentless, widespread), serpiginous (progressive, scarring).