Pachychoroid neovasculopathy has clinical properties that differ from conventional neovascular age-related macular degeneration

Abstract

To determine the clinical properties of pachychoroid neovasculopathy (PNV) that differ from conventional neovascular age-related macular degeneration (nAMD) and suggest that they are different clinical entities. To accomplish this, we reviewed the medical records of 100 consecutive patients diagnosed with nAMD. All of the patients were Japanese, and their mean age was 75.5 years. There were 72 men and 28 women. For the bilateral cases, only the right eye was analyzed. An eye was diagnosed with PNV when a macular neovascularization (MNV) was detected just above the dilated choroidal vessels. The Indocyanine green angiographic (ICGA) and en face optical coherence tomographic (OCT) images were used to assess the vertical symmetry of the medium and large choroidal vessels. The subfoveal choroidal thickness (SCT) was also measured manually in the OCT images. After reclassification, there were 29 (29%) patients with typical nAMD (25 with type 1 MNV, 4 with type 2 MNV), 43 (43%) with PNV, 21 (21%) with polypoidal choroidal vasculopathy, and 7 (7%) with retinal angiomatous proliferation. Of the 43 PNV, 17 (39.5%) had polypoidal lesions and 26 (60.5%) had no polypoidal lesions. The percentage of eyes with vertical asymmetry of the medium and large choroidal vessels was significantly greater in the 35 PNV (81.4%) than in the 16 non-PNV (28.1%; P < 0.01) cases. The mean SCT was significantly thicker in the PNV eyes than in the non-PNV eyes (298 ± 96 μm vs. 228 ± 82 μm; P < 0.01). The response of PNV to anti-vascular endothelial growth factor treatments was better than that of non-PNV eyes [higher dry macula rate after the loading period (90.9% vs. 59.1%), fewer total number of injections (11.0 ± 2.9 vs. 13.4 ± 3.2), and longer treatment intervals for the anti-VEGF therapy (8.4 ± 3.1 vs. 13.4 ± 3.2 weeks) at 2 years (all P < 0.01)]. These differences in the morphology and response to anti-VEGF treatments suggest that PNV is a separate clinical entity to conventional nAMD.

Figure 1

Conventional classification and re-classification of patients with MNV secondary to age-related macular degeneration and including PNV. PNV pachychoroid neovasculopathy, MNV macular neovascularization, PCV polypoidal choroidal vasculopathy, RAP retinal angiomatous proliferation

Figure 2

Findings in an 81-year-old man with PCV by conventional classification or with PNV after re-classification. En face OCT shows vertical asymmetry of the choroidal vessels with dilated choroidal vessels (pachyvessels) corresponding to the polypoidal lesions in the ICGA images. ICGA shows an abnormal network of vessels connecting superiorly from the central fovea with a polypoidal lesion at its apical tip (white arrow). Baseline OCT shows SRF with elevated RPE. The SCT is 385 µm. OCTA shows an abnormal network of vessels including those in the central fovea as MNV. OCT optical coherence tomography, ICGA indocyanine green angiography, SRF subretinal fluid, RPE retinal pigment epithelium, SCT subfoveal choroidal thickness, OCTA optical coherence tomography angiography, MNV macular neovascularization, PCV polypoidal choroidal vasculopathy.

Figure 3

Findings in a 70-year-old woman. A representative case of a type 1 MNV with conventional classification or a PNV with reclassification. En face OCT image shows vertical asymmetry of choroidal vessels, with dilated choroidal vessels (pachyvessels) around the fovea. ICGA shows no polypoidal lesion. Baseline OCT shows SRF with elevated RPE; SCT is 317 µm. OCTA shows MNV at the fovea. OCT optical coherence tomography, IA indocyanine green angiography, SRF subretinal fluid, RPE retinal pigment epithelium, SCT subfoveal choroidal thickness, OCTA optical coherence tomography angiography, MNV macular neovascularization, PNV pachychoroid neovasculopathy

Figure 4

Different ways to divide the MNV including PNV. Example 1. PNV, PCV, and type 1/type 2 MNV. Example 2. PNV is divided by the presence or absence of polypoidal lesions. Example 3. PNV, PCV, and type 1/type 2 MNV are divided into pachychoroid-driven and drusen-driven. MNV macular neovascularization, PNV pachychoroid neovasculopathy, PCV polypoidal choroidal vasculopathy.