In central serous chorioretinopathy (CSC), the macula is detached because of fluid leakage at the level of the retinal pigment epithelium. The fluid appears to originate from choroidal vascular hyperpermeability, but the etiology for the fluid is controversial. The choroidal vascular findings as elucidated by recent optical coherence tomography (OCT) and wide-field indocyanine green (ICG) angiographic evaluation show eyes with CSC have many of the same venous patterns that are found in eyes following occlusion of the vortex veins or carotid cavernous sinus fistulas (CCSF). The eyes show delayed choroidal filling, dilated veins, intervortex venous anastomoses, and choroidal vascular hyperpermeability. While patients with occlusion of the vortex veins or CCSF have extraocular abnormalities accounting for the venous outflow problems, eyes with CSC appear to have venous outflow abnormalities as an intrinsic phenomenon. Control of venous outflow from the eye involves a Starling resistor effect, which appears to be abnormal in CSC. Similar choroidal vascular abnormalities have been found in peripapillary pachychoroid syndrome. However, peripapillary pachychoroid syndrome has intervortex venous anastomoses located in the peripapillary region while in CSC these are seen to be located in the macular region. Spaceflight associated neuro-ocular syndrome appears to share many of the pathophysiologic problems of abnormal venous outflow from the choroid along with a host of associated abnormalities. These diseases vary according to their underlying etiologies but are linked by the venous decompensation in the choroid that leads to significant vision loss. Choroidal venous overload provides a unifying concept and theory for an improved understanding of the pathophysiology and classification of a group of diseases to a greater extent than previous proposals.

Purpose: Pachychoroid spectrum diseases are regarded as being different manifestations of a common pathogenic process. We suggest that pachychoroid diseases are consequences of chronic vortex vein stasis. Methods: We describe how we came to this conclusion based on our own recent reports as well as a search of the related literature. Results: Central serous chorioretinopathy (CSC) is the first stage of pachychoroid spectrum diseases. CSC is caused by congestion of choroidal veins, which are branches of the vortex veins. The venous outflow tract of the choroid is divided into four quadrants, based on horizontal and vertical watershed zones, with one or two vortex veins in each quadrant being independently responsible for venous outflow. In acute CSC, vortex vein stasis frequently causes asymmetric dilatation of the vortex veins in the horizontal watershed. The area of geographic filling delay in the choriocapillaris coincides with the area of this asymmetrically dilated vortex veins. With chronic stasis of the vortex veins, venous anastomosis occurs in the watershed zone as a means of compensating for the stasis, and the choriocapillaris becomes occluded in the area of filling delay. The anastomotic vessels dilate, becoming often hyperpermeable, and are then recognizable as pachyvessels. With the development of choriocapillaris ischemia, choroidal neovascularization (CNV) occurs at the site of pachyvessels. This is termed pachychoroid neovasculopathy (PNV). Polypoidal choroidal vasculopathy is regarded as a variant of PNV. Conclusions: Intervortex venous anastomosis is among the key factors underlying the development of pachychoroid diseases. Remodeling of the venous drainage route though the anastomosis across the watershed zones is apparently a common response to chronic vortex vein stasis.

To investigate the correlation between geographic filling delays in the choriocapillaris using indocyanine green angiography (ICGA) images and dilated vortex veins in central serous chorioretinopathy (CSC). Observational case series. Thirty-two eyes of 32 patients, 21 with acute and 11 with chronic CSC. Digital ICGA and fluorescein angiography (FA), with videoangiography, and swept-source optical coherence tomography (SS-OCT) for B-scan and en-face choroidal imaging were performed. Overlapping of the filling delay areas in the choriocapillaris in the early-phase ICGA images and the region of dilated vortex veins in the en-face images were analyzed. The consistency of both areas was graded as follows. Grade 3: filling delay area is entirely involved in the dilated vortex vein region. Grade 2: 50% or more of filling delay area overlaps with the dilated vortex vein region. Grade 1: less than 50% of filling delay area overlaps with the dilated vortex vein region. Grade 0: no tendency for overlapping of two areas. We evaluated the asymmetry of upper and lower vortex veins in en-face images of the Haller layer. Using the binarization method, we quantified the luminal and stromal areas of the choroid. The ratios of the Haller layer area and luminal areas in the Haller layer to total choroidal area were examined. The consistency of overlapping of the two areas was grade 2.62 ± 0.49 in acute CSC and grade 1.55 ± 0.78 in chronic CSC (p = 0.0005). Asymmetry of upper and lower vortex veins was seen in 17 of 22 eyes (81%) with acute CSC and 6 of 11 eyes (54.5%) with chronic CSC (p = 0.114). Central choroidal thickness was 411 ± 79 μm in acute CSC and 326 ± 64 μm in chronic CSC (p = 0.004). In the posterior fundus with a 4500 μm diameter, the ratio of the Haller layer area to total choroidal area was 63.7 ± 8.6% in acute CSC and 57.1 ± 7.9% in chronic CSC (p = 0.047). The ratio of the luminal area in the Haller layer area to total choroidal area was 46.9 ± 7.6% in acute CSC and 40.0 ± 6.9% in chronic CSC (p = 0.014) Filling delay areas in the choriocapillaris and dilated vortex vein regions showed marked overlapping in acute CSC. Increased choroidal thickness was attributed to dilated vortex veins. These findings suggest that the blood flow into the choriocapillaris is delayed as a result of congestion of the dominant vortex veins that supply this geographic area. CSC may be a disease characterized by vortex vein congestion that develops in eyes with asymmetric vortex veins.

Swept source optical coherence tomography (SS-OCT) was introduced in clinical practice in 2012. Because of its deeper penetration and faster acquisition time, SS-OCT has the ability to visualize choroid, vitreous, and retinal structures behind dense preretinal hemorrhages. Swept source optical coherence tomography has positively influenced and hugely contributed to the research of the vitreous body. It is the first ophthalmic diagnostic technology to demonstrate the entire structure of the posterior precortical vitreous pocket (PPVP) in vivo. The roles of the PPVP in physiological posterior vitreous detachment and vitreoretinal interface disorders have now been elucidated. The presence of a connecting channel between the PPVP and Cloquet's canal suggests that the aqueous humor drains into the premacular space. Deeper penetration of SS-OCT has made it possible to view the choroid. It also has an important role in central serous chorioretinopathy and uveitis. We have also been able to treat Harada disease by monitoring the choroidal thickness by SS-OCT.