Decreased central vision; may be sudden or gradual. Central or paracentral scotomas.

Critical

Subretinal red-orange, polyp-like lesions of the choroidal vasculature. Can be macular (more symptomatic) or peripapillary (See Figure 11.18.1).

Figure 11.18.1: Polypoidal choroidal vasculopathy.

Other

Bilateral subretinal and/or sub-RPE blood, VH, circinate subretinal exudates, subretinal fibrosis (disciform scar), SRF, atypical CNV, and multiple serous PEDs.

Risk Factors

More common in females, individuals of African or Asian descent, and in patients with HTN. Can occur at a younger age compared to neovascular AMD, but usually without significant drusen or GA.

• Neovascular AMD: See 11.17, Neovascular or Exudative (Wet) Age-Related Macular Degeneration.

• Retinal arterial macroaneurysm (RAM): See 11.19, Retinal Arterial Macroaneurysm.

• Peripheral exudative hemorrhagic chorioretinopathy (PEHCR [peripheral CNV]): Occurs in the peripheral retina and presents with SRF, subretinal blood, exudation, RPE detachment, and/or subretinal fibrosis.

• CSCR: See 11.15, Central Serous Chorioretinopathy

1. Slit-lamp biomicroscopy with a handheld lens to detect signs of exudation.

2. ICGA is the gold standard for diagnosis. ICGA is used to confirm the presence of a branching network of vessels arising from the inner choroidal circulation with terminal aneurysmal dilations (popcorn lesions). Unlike occult CNV, the IPCV lesions do not stain late unless active leakage is present.

3. With IVFA, often a notch may be seen at the margin of a PED, suggesting the location of a polyp. This study is primarily performed to evaluate for other causes of CNV.

4. OCT is used to assess for ME, SRF, and PEDs and can detect polyps in some eyes.

Asymptomatic lesions may be observed and may resolve spontaneously. IPCV with exudation and/or hemorrhagic complications has been treated with anti-VEGF monotherapy with or without PDT. The EVEREST-II and PLANET studies demonstrated level I evidence that anti-VEGF monotherapy as well as combination therapy give excellent functional visual outcomes in patients presenting with symptomatic IPCV. Pars plana vitrectomy (PPV) can be considered for VH.

The prognosis of IPCV is generally better than for neovascular AMD. Symptomatic or macular IPCV is followed every 1 to 2 months with periodic OCT, IVFA, and ICGA as needed for disease progression. Consider treatment, or retreatment, if symptomatic, persistent, or new leakage occurs.