Vortex vein varix (VVV) is a benign dilation of the vortex vein ampullae located at the equator of the eye. On fundus examination, it appears as a unilateral or bilateral saccular or teardrop-shaped elevated structure along the oblique meridians of the equator.
The color is brownish to gray, and the size is typically 1 to 3 times the disc diameter. However, larger variations have been reported, and larger ones tend to occur more frequently in the supratemporal quadrant.
Physiological vortex vein ampullae are present in about 44% of individuals. On the other hand, the prevalence of dilated vortex vein varices is not accurately recorded due to their rarity and incidental discovery.
They are more easily found in myopic eyes or lightly pigmented eyes. They may also be discovered incidentally in patients being followed up for symptoms of posterior vitreous detachment (PVD). They are not thought to be directly related to floaters or photopsia.
Vortex vein varix itself is a benign dilation and not a malignant lesion. However, clinically, differentiation from malignant diseases such as choroidal melanoma or metastatic choroidal tumors is of utmost importance, and detailed examination may be necessary for a definitive diagnosis. For details, see the section on “Diagnosis and Examination Methods.”
Vortex vein varices usually do not cause specific symptoms.
They appear as characteristic elevated lesions along the equator and oblique meridians of the fundus.
Morphological Features
Color: Brownish to gray, sometimes appearing blue or purple.
Shape: Single or multiple elevations, sac-like or teardrop-shaped.
Size: Typically 1 to 3 times the optic disc diameter. Large ones are more common in the superotemporal quadrant.
Location: Along the equator and oblique meridians (unilateral or bilateral).
Dynamic Findings
Disappearance with pressure: The lesion shrinks or disappears with digital pressure or three-mirror lens compression. This is the most important diagnostic finding.
Change with gaze: The appearance of the lesion may change with changes in gaze direction.
Pulsatility: In some cases, pulsatile vortex vein blood flow may be observed.
It may be accompanied by changes in the retinal pigment epithelium (pigment epithelial detachment or atrophic changes), which can cause visual symptoms.
In myopic eyes or eyes with light pigmentation, diagnosis is easier based on clinical findings. If reduction or disappearance is confirmed by dynamic fundus examination using digital pressure or a three-mirror lens, it provides diagnostic evidence. If uncertain, additional tests such as OCT, ICG angiography, or ultrasound can confirm the diagnosis.
The exact etiology of vortex vein aneurysm is unknown. Several mechanisms have been proposed:
The classification of vortex veins (types I–IV) is shown below. Type IV (complete type with ampulla) may be more common in patients with vortex vein aneurysm, but further research is needed for confirmation.
| Type | Characteristics |
|---|---|
| Type I | No vortex vein. Tributaries pass directly through the sclera |
| Type II | Incomplete type. Some tributaries pass through the sclera in parallel |
| Type III | Complete type. All tributaries join before passing through the sclera |
| Type IV | Complete type with ampulla. All tributaries form an ampulla before passing through the sclera |
The following factors may be involved:
Diagnosis is made by combining clinical evaluation and imaging. A detailed history (including ocular trauma, surgery, and systemic diseases) is essential. Lesions are more easily detected in myopic eyes or lightly pigmented eyes.
The characteristics and usefulness of each examination method are described below.
Differentiation from the following diseases is important, with priority given to excluding malignant conditions.
This is the most important differential diagnosis. Dynamic fundus examination (disappearance with finger pressure or three-mirror lens compression) and B-mode ultrasound (flattening with external pressure) are the first steps. ICG angiography confirms the diagnosis of vortex vein varix by demonstrating dye pooling in the ampulla of the vortex vein. Note that on ultrasound, melanoma shows characteristic acoustic attenuation (internal echoes), while vortex vein varix deforms and disappears with compression.
Specific treatment for vortex vein varix itself is usually unnecessary. The main management is regular follow-up.
Complications are generally rare, and spontaneous resolution has been reported.
Venous blood from the choroid is collected by the vortex veins in each of the four quadrants of the fundus (superonasal, inferonasal, superotemporal, inferotemporal) and exits the eye through the sclera. Hayreh first demonstrated that the choroidal venous system is divided into four independent regions, with no anastomoses between separate vortex vein systems in healthy eyes1). However, later studies observed preferential drainage to the superotemporal or inferotemporal quadrant in more than half of healthy individuals1). The number of vortex vein ampullae in healthy eyes can be up to eight1).
The number of vortex vein ampullae observed on fundus images may exceed the number of vortex veins confirmed histologically before they pass through the sclera. This suggests that some ampullae may merge within the sclera before exiting the eye1).
Obstruction of vortex vein outflow leads to choroidal congestion, resulting in the following sequence of changes.
In experiments involving vortex vein ligation in monkey eyes, it was shown that vortex vein ligation induces choroidal vascular remodeling without exudative retinal detachment1). In this experimental model, findings characteristic of pachychoroid disease were observed, including vortex vein dilation, delayed filling of the choriocapillaris, formation of anastomoses between vortex veins, choroidal thickening, and pulsatile vortex vein flow1).
If vortex vein congestion persists, anastomoses may form to relieve pressure into adjacent quadrant vortex vein systems1). When congestion saturates all quadrants, generalized venous stasis and chronic increased vascular permeability persist, potentially establishing a vicious cycle of choriocapillaris ischemia → retinal pigment epithelium damage → outer retinal atrophy1).
In recent years, the pathophysiological relationship between vortex vein congestion and the pachychoroid disease spectrum has attracted attention.
A review by Cheung et al. (Eye 2025) discusses that congestion of the vortex vein ampulla may contribute to the development of the pachychoroid disease spectrum (including central serous chorioretinopathy, polypoidal choroidal vasculopathy, and pachychoroid neovasculopathy) 1).
Pang CE et al. 1) demonstrated congestion of the vortex vein ampulla in CSC eyes using ultra-widefield ICGA, and proposed that outflow congestion may be involved in the pathogenesis of the pachychoroid disease spectrum. UWF imaging allows evaluation of the vortex vein anatomy from the posterior pole to the ampulla, and it has been shown that pachyvessels communicate beyond quadrants in the periphery.
In pachychoroid disease eyes, vortex vein anastomoses, asymmetry of choroidal drainage distribution, and focal narrowing/dilation have been reported to be observed in over 90% of normal eyes 1).
According to Sen et al., the characteristic “pachyvessels” (dilated outer choroidal vessels) of pachychoroid disease may be formed by venous remodeling of the vortex vein system. In eyes with CSC, PCV, PNV, etc., vortex vein anastomoses have been observed in 90% of CSC, 95% of PNV, and 98% of PCV 2). These anastomotic channels may function as new drainage routes to reduce choroidal congestion 2).
Vortex vein stasis leads to dilation of the Haller layer and thinning of the inner choriocapillaris and Sattler layer. Thinning of the choriocapillaris can lead to ischemia, which may contribute to the formation of neovascular complexes seen in PNV and PCV 2).
In recent years, it has become clear that congestion and dilation of the vortex vein system are involved in the pathophysiology of the pachychoroid disease spectrum. Although vortex vein varix itself does not directly cause pachychoroid disease, outflow obstruction of the vortex vein system is thought to predispose to the development of CSC, PCV, etc., through choroidal congestion and vascular remodeling.
Ultra-widefield (UWF) ICGA has recently become widespread as an important tool for evaluating the entire vortex vein anatomy from the posterior pole to the ampulla 1).
In a review by Cheung et al. (Eye 2025) 1), it was shown that UWF ICGA imaging can comprehensively evaluate dilation, congestion, and anastomosis patterns of the vortex veins. The study reported examples of dilated and congested vortex veins in the inferotemporal quadrant, as well as cases showing extensive vortex vein dilation and congestion in all four quadrants.
En face SS-OCT (swept-source) and OCT angiography (OCTA) are also being explored for evaluating the vortex vein system, but visualization with OCTA can be difficult due to low flow velocity 1).
Research is investigating whether individual differences in vortex vein arrangement in healthy eyes (e.g., hypoplastic vortex vein drainage in one quadrant) may contribute to the susceptibility to choroidal congestion in certain individuals 1).
Since asymmetry of choroidal drainage and localized narrowing/dilation were observed in over 90% of eyes with pachychoroid disease, evaluation of vortex vein anatomy may be useful for future disease risk assessment 1).
Many aspects of the formation mechanism, natural course, and relationship with pachychoroid disease of vortex vein varices remain unclear, and the following issues remain.
Cheung CMG, Teo KYC, Spaide RF, et al. Pachychoroid disease: review and update. Eye (Lond). 2025;39:819-834. doi:10.1038/s41433-024-03253-4. PMID:39095470; PMCID:PMC11933466.
Sen S, Bhavesh MT, Dhar S, Gupta R. Polypoidal choroidal vasculopathy: a review. Clin Ophthalmol. 2023;17:53-75.
