What is the recommended treatment for a patient with diffuse chorioretinal atrophy and disc edema, potentially related to underlying hypertension or diabetes?

Diffuse Chorioretinal Atrophy with Disc Edema

This presentation requires immediate systemic workup to exclude inflammatory choroiditis, particularly multifocal choroiditis with panuveitis, Vogt-Koyanagi-Harada disease, or sarcoidosis, as these conditions mandate urgent systemic corticosteroids combined with immunosuppressive therapy to prevent irreversible vision loss. 1, 2

Immediate Diagnostic Workup Required

Multimodal imaging is essential before initiating any treatment:

• Optical coherence tomography (OCT) to identify subretinal fluid, outer retinal disruption, cystoid macular edema, posterior cystoid retinal degeneration, and subretinal fibrosis 1, 3
• Fluorescein angiography (FA) to detect focal leakage points, diffuse leakage patterns, retinal vasculitis, and choroidal neovascularization 1, 2
• Indocyanine green angiography (ICGA) to assess choroidal hyperperfusion, hypofluorescent changes characteristic of white dot syndromes, and exclude pachychoroid spectrum disorders 1, 3
• OCT-angiography to detect silent neovascular networks that may not be visible on conventional imaging 1, 3
• Fundus autofluorescence (FAF) to characterize atrophic patterns and differentiate inflammatory from degenerative processes 4

Critical Differential Diagnoses to Exclude

Inflammatory Choroiditis (Most Urgent)

Multifocal choroiditis with panuveitis presents with multiple yellow-white focal lesions, vitritis, and can progress to diffuse chorioretinal atrophy despite treatment over 8 years of follow-up 5. This requires immediate systemic therapy, never observation 3.

Vogt-Koyanagi-Harada disease presents with rapid onset, bilateral involvement in 95% of cases, numerous central leakage points on FA, optic disc edema, and requires aggressive initial corticosteroid therapy 1, 2. The American Academy of Ophthalmology recommends starting high-dose oral prednisolone at 1 mg/kg/day (maximum 60 mg/day) immediately without waiting for diagnostic workup completion 2.

Sarcoidosis presents with nodules, retinal vasculitis, small round atrophic granulomas in the inferior peripheral fundus, and optic disc edema 1. This requires systemic corticosteroids and immunosuppressive medication 1.

Birdshot chorioretinitis presents with disc edema, vitritis, chorioretinal lesions, and retinal vasculitis 6. Delayed treatment leads to optic disc atrophy and permanent vision loss of 20/100 or worse 6.

Severe Chronic Central Serous Chorioretinopathy

Severe chronic CSC should be considered if the patient has at least 5 optic disc areas of diffuse atrophic RPE alterations on mid-phase FA, diffuse fluorescein leakage >1 disc area, or posterior cystoid retinal degeneration on OCT 1. However, disc edema is not a typical feature of CSC and should raise suspicion for inflammatory disease 1.

Diabetic or Hypertensive Retinopathy

If the patient has underlying diabetes, severe nonproliferative or proliferative diabetic retinopathy with macular edema must be excluded 1. The International Council of Ophthalmology recommends referral to an ophthalmologist for any level of macular edema, severe NPDR, or any PDR 1.

Treatment Algorithm Based on Diagnosis

If Inflammatory Choroiditis is Confirmed:

Immediate systemic therapy (never observation):

• High-dose oral prednisolone 1 mg/kg/day (maximum 60 mg/day) started immediately without waiting for complete diagnostic workup 2
• Maintain initial high dose for at least 4-5 weeks before beginning taper, targeting 10-15 mg/day by 3 months if inflammation is controlled 2
• Add steroid-sparing immunosuppressive therapy early (within 4-8 weeks) rather than waiting for steroid-related complications 2
• Mycophenolate mofetil is preferred for most cases due to favorable tolerability profile and efficacy 2, 3
• Azathioprine is particularly effective for Behçet's disease-associated retinal vasculitis with 90% inflammation control rate 2
• Methotrexate demonstrates comparable efficacy to mycophenolate mofetil 2

Local corticosteroids may be used adjunctively for acute inflammation control (topical drops, periocular injections), but never as monotherapy 3.

If choroidal neovascularization is present: Intravitreal anti-VEGF injections are the primary treatment 3.

If Severe Chronic CSC is Confirmed:

Treatment should be recommended for patients with chronic CSC complicated by posterior cystoid retinal degeneration due to likelihood of progressing to severe vision loss 1.

• ICGA- and FA-guided half-dose or half-fluence photodynamic therapy (PDT) is the primary treatment 1
• However, both half-dose PDT and standard PDT have relatively low efficacy in severe chronic CSC with posterior cystoid retinal degeneration (44% complete resolution, 48% reduction) 1
• Rule out macular neovascularization using OCT, OCT-A, FA, and/or ICGA, as MNV can be present in up to 45% of severe chronic CSC cases 1
• If MNV is present: Combination therapy using both PDT and intravitreal anti-VEGF injections may be viable 1

If Diabetic Retinopathy with Macular Edema:

• Optimal glycemic control can substantially reduce risk and progression 1
• Optimal blood pressure control can reduce risk and progression 1
• Laser photocoagulation for clinically significant macular edema reduces doubling of visual angle from 20% to 8% 1
• Promptly refer to ophthalmologist knowledgeable in diabetic retinopathy management 1

Common Pitfalls to Avoid

Do not confuse inflammatory choroiditis with central serous chorioretinopathy - disc edema strongly suggests inflammatory disease, not CSC 1, 2.

Do not delay treatment if inflammatory choroiditis is suspected - start high-dose systemic corticosteroids immediately as delay risks irreversible vision loss 2.

Do not use corticosteroids as monotherapy for inflammatory choroiditis - systemic immunosuppression is required for the underlying inflammatory process 2, 3.

Do not ignore underlying systemic conditions - optimize glycemic and blood pressure control if diabetes or hypertension is present 1.

Do not miss silent neovascular networks - up to 23% of patients with chronic CSC have vascular networks visible only on OCT-A 1.

Long-term Monitoring

Regular ophthalmologic surveillance is mandatory for at least 3 years after achieving remission to detect recurrence early in inflammatory conditions 4, 3.

For chronic CSC, follow-up OCT imaging at 1-3 month intervals is recommended to assess for complete subretinal fluid resolution and detect potential complications 4.