Remodeling of macular vortex veins in pachychoroid neovasculopathy

Abstract

Superior and inferior macular vortex veins are divided by a horizontal watershed passing through the macula. We evaluated macular vortex vein remodeling in eyes with pachychoroid neovasculopathy (PNV). Thirty eyes of 30 patients with treatment-naïve PNV and 30 normal eyes of 30 age-, gender-, and refraction-matched subjects were studied. We assessed the features of macular vortex veins employing en face optical coherence tomography (OCT) and determined central choroidal thickness (CCT) using B-mode OCT. Of the 30 normal eyes, a horizontal watershed was identified in 24 eyes (80%), while venous anastomosis between the superior and inferior vortex veins was observed in 6 eyes (20%). Mean CCT was 233 μm. Of the 30 eyes with PNV, vortex veins were dilated in all 30 eyes with PNV. In 27 of the 30 PNV eyes (90%), the horizontal watershed had disappeared, and collateral veins had instead developed via anastomosis between the superior and inferior vortex veins, making this finding significantly more frequent than in normal eyes (P < 0.001). Mean CCT was 357 μm, significantly thicker than that of normal eyes (P < 0.001). Remodeling of choroidal drainage routes by venous anastomosis between superior and inferior vortex veins was common in eyes with PNV. This observation suggests longstanding congestion of the choroidal veins.

Figure 1

Images of a normal eye of a 56-year-old man. The refraction in the left eye was +0.50 diopters. (A) Color fundus photograph shows a normal fundus appearance. (B) En face optical coherence tomography (OCT) image (12 mm × 12 mm) showing vortex veins in the deep layer of the choroid. Superior and inferior vortex veins are symmetrical and there is a horizontal watershed zone (dashed line). (C) 12 mm horizontal B-mode OCT image through the fovea shows normal appearances of the retina and choroid. The central choroidal thickness is 194 µm.

Figure 2

Images of an eye with pachychoroid neovasculopathy in a 53-year-old man. The refraction in the right eye was +0.00 diopters. Best-corrected visual acuity in the right eye was −0.08 logarithm of the minimum angle of resolution unit. (A) Color fundus photograph shows a retinal pigment epithelium (RPE) abnormality at the fovea. A large choroidal vessel can be seen under the fovea. (B,C) 12 mm horizontal and vertical B-mode optical coherence tomography (OCT) images through the fovea show pachychoroid with dilated outer choroidal vessels (vortex veins). A shallow irregular RPE detachment accompanied by slight serous retinal detachment is observed at the fovea. The central choroidal thickness is 353 µm. (D) En face OCT image (12 mm × 12 mm) showing dilated vortex veins in the deep layer of the choroid. Superior vortex veins are dominant as compared to the inferior vortex veins. The horizontal watershed zone has disappeared, showing instead collateral veins due to anastomoses between the superior and inferior vortex veins. (E) OCT angiography (3 mm × 3 mm) shows network vessels of choroidal neovascularization (CNV) between the detached RPE and Bruch’s membrane. CNV was detected over the dilated vortex veins. (F,G) Fluorescein angiography (early and late phases) shows window defects and some oozing at the fovea. (H,I) Indocyanine green angiography (early and late phases) shows dilated choroidal vessels with hyperpermeability at the macular area and suspected CNV at the fovea.

Figure 3

Images of an eye with pachychoroid neovasculopathy in a 54-year-old man. The refraction in the left eye was −1.00 diopter. Best-corrected visual acuity in the left eye was 0.22 logarithm of the minimum angle of resolution unit. (A) Color fundus photograph shows a retinal pigment epithelium (RPE) abnormality at the macular area. (B,C) 12 mm horizontal and vertical B-mode OCT images through the fovea show pachychoroid with dilated outer choroidal vessels (vortex veins). Dilated vortex veins are notable between the papilla and subfovea in the horizontal B-mode OCT image. A shallow irregular RPE detachment accompanied by serous retinal detachment is observed at the fovea. The central choroidal thickness is 386 µm. (D) En face OCT image (12 mm × 12 mm) showing dilated vortex veins in the deep layer of the choroid. Superior and inferior vortex veins are symmetrical. The horizontal watershed zone has disappeared, with collateral veins due to anastomoses between the superior and inferior vortex veins instead being observed in the peripapillary area. (E) OCT angiography (3 mm × 3 mm) shows network vessels of choroidal neovascularization (CNV) between the detached RPE and Bruch’s membrane. CNV was detected over the dilated vortex veins. (F,G) Fluorescein angiography (early and late phases) shows window defects and some oozing at the macular area. (H,I) Indocyanine green angiography (early and late phases) shows suspected CNV at the fovea and choroidal vascular hyperpermeability around the fovea.

Figure 4

Images of an eye with pachychoroid neovasculopathy in a 68-year-old man. The refraction in the left eye was +1.50 diopters. Best-corrected visual acuity in the left eye was 0.10 logarithm of the minimum angle of resolution unit. (A) Color fundus photograph shows a retinal pigment epithelium (RPE) abnormality with pachydrusen around the macular area. Markedly dilated vortex veins are seen at the fovea. (B,C) 12 mm horizontal and vertical B-mode OCT images through the fovea show pachychoroid with dilated outer choroidal vessels (vortex veins). Dilated vortex veins are notable under the fovea. A shallow irregular RPE detachment accompanied by serous retinal detachment is observed at the fovea. The central choroidal thickness is 465 µm. (D) En face OCT image (12 mm × 12 mm) showing dilated vortex veins in the deep layer of the choroid. The horizontal watershed zone has disappeared, with anastomoses between the superior and inferior vortex veins instead being observed. The collateral veins at the macula show sinusoid like dilatation. (E) OCT angiography (3 mm × 3 mm) shows network vessels of choroidal neovascularization (CNV) between the detached RPE and Bruch’s membrane. CNV was detected over the dilated vortex veins. (F,G) Fluorescein angiography (early and late phases) shows window defects at the posterior pole of the fundus and some oozing in the macular area. (H,I) Indocyanine green angiography (early and late phases) shows suspected CNV at the fovea, choroidal vascular hyperpermeability and punctate hyperfluorescent spots corresponding to pachydrusen around the macular area.