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Symptoms ⬇

Decreased central vision; may be sudden or gradual.

Signs ⬆ ⬇

Critical

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

11-18.1 Polypoidal choroidal vasculopathy OS.

Gervasio-ch011-image035

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 geographic atrophy.

Differential Diagnosis ⬆ ⬇

Reference(s) ⬆ ⬇

Cheung CMG, Lai TYY, Ruamviboonsuk P, et al. Polypoidal choroidal vasculopathy: definition, pathogenesis, diagnosis, and management. Ophthalmology. 2018;125(5):708-724.Koh A, Lai TYY, Takahashi K, et al. Efficacy and safety of ranibizumab with or without verteporfin photodynamic therapy for polypoidal choroidal vasculopathy: a randomized clinical trial. JAMA Ophthalmol. 2017;135:1206-1213.

Work Up ⬆ ⬇

Workup
  1. Slit lamp biomicroscopy with a 60-, 90-diopter, or fundus contact 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. IVFA is 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.

Treatment ⬆ ⬇

Asymptomatic lesions may be observed and may resolve spontaneously. IPCV with exudation and/or hemorrhagic complications has been treated with anti-VEGF monotherapy, PDT, or a combination. 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. Thermal laser photocoagulation, feeder vessel treatment, and pneumatic displacement of large submacular hemorrhage have also been used with varying success.

Follow Up ⬆

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.