Choroidal Vascular Anomalies: Posterior Vortex Veins, Choroidal Macrovessels, Choroidal Macroaneurysms, and Choroidal Vessel Varix
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Choroidal vascular anomalies, such as posterior vortex veins, choroidal macrovessels, choroidal macroaneurysms, choroidal vessel varices, and pachyvessels are increasingly recognized in ophthalmic practice due to advancements in imaging technologies. These anomalies, once overlooked or misdiagnosed, have gained attention as potential contributors to various ocular pathologies, including central serous chorioretinopathy (CSCR), polypoidal choroidal vasculopathy (PCV), choroidal neovascularization (CNV), macroaneurysms, and hemorrhage, all of which can significantly impact vision. These vascular anomalies may mimic other serious conditions such as choroidal tumors, melanoma, metastatic lesions, and choroidal granulomas, leading to unnecessary and extensive diagnostic workups if not correctly identified. It is crucial for ophthalmologists to be familiar with these anomalies to avoid misdiagnosis and ensure proper management. Although typically benign and not requiring regular follow-up unless complications arise, patient education is essential to ensure awareness of potential risks. Secondary complications such as choroidal neovascularization (CNV), hemorrhage, or exudation can impact vision and should be promptly evaluated if they develop. En face Optical Coherence Tomography (OCT) is a valuable tool for visualizing choroidal vessels and is readily available in commercial OCT devices, providing detailed imaging that can aid in the diagnosis and monitoring of these conditions. If uncertainty remains, Indocyanine Green Angiography (ICG) can be employed to further investigate the vascular anomalies.
Normal Choroidal Vasculature: Arterial Supply, Venous Drainage, and Submacular Organization
The choroid is a highly vascular layer of the eye, primarily nourished by the posterior ciliary arteries (PCAs), which branch into short and long divisions. Short posterior ciliary arteries (SPCAs), particularly the paraoptic and distal branches, enter the globe near the optic nerve and fan out to supply the posterior choroid and outer retina. The long PCAs (medial and lateral) traverse anteriorly to supply the anterior uvea and a peripheral choroidal sector. The choroidal vascular architecture is segmental and end-arterial in nature, meaning there is minimal collateral flow between territories, rendering watershed zones between segments especially vulnerable to ischemia.
Choriocapillaris circulation, which underlies the retinal pigment epithelium (RPE), is organized in a mosaic of lobules, each independently supplied by a terminal arteriole and drained at the periphery. These lobules are densely packed in the macula and progressively sparser in the periphery. Venous outflow occurs through four vortex veins that drain each quadrant of the choroid, exiting the globe obliquely in the equatorial zone. The segmental nature of both the arterial and venous systems reinforces the existence of anatomical watershed zones, where perfusion is weakest. These watershed zones frequently meet in the macular region, correlating with clinical susceptibility to choroidal ischemia in diseases like age-related macular degeneration.
The submacular choroid is uniquely structured, as multiple SPCAs enter the sclera in the macular area and emit recurrent centripetal branches to supply the central choroid. This convergence of end-arterial segments creates a physiologic vulnerability due to overlapping watershed zones. Though thicker than surrounding regions, the submacular choroid does not benefit from redundant supply but rather reflects the density of vascular entry points.[1] This anatomical vulnerability is clinically evident in conditions like geographic atrophy (GA), where progressive loss of the retinal pigment epithelium and photoreceptors unmask the underlying choroidal vasculature. In such cases, prominent submacular vessels—often representing short posterior ciliary artery (SPCA) entry sites—become visible. An excellent example is shown in the figure, where fundus photography and OCT imaging over a five-year period demonstrate the evolution of GA in an elderly patient, including visualization of submacular SPCAs and structural changes in the retina and choroid.
Posterior Vortex Veins or Macular Vortex Veins or Choriovaginal Veins
Disease Entity
Disease
Posterior vortex veins, macular vortex veins, and choriovaginal veins are anatomical variants of the choroidal venous system. These veins are responsible for draining the choroid, which supplies blood to the outer retina. Posterior vortex veins are mainly around the optic disc or just temporal to the optic disc, macular vortex veins and choriovaginal veins are less common variants that drain near or within the macular region. The presence of macular vortex veins is particularly significant in conditions like high myopia and pachychoroid spectrum diseases, where they may contribute to pathological changes in the retina.[3][4][5]
Etiology
The etiology of posterior and macular vortex veins involves developmental variations in the venous drainage system of the choroid. Macular vortex veins may develop due to altered choroidal outflow, often associated with high myopia or other structural abnormalities of the eye. These veins can also be linked to changes in scleral thickness and structure, particularly in highly myopic eyes.[6][7] Choriovaginal veins connect the choroidal and retinal venous systems. These veins pass from the choroid through the sclera near the optic disc margin, ultimately draining into the venous plexus within the pial sheath of the optic nerve. This establishes a connection between the choroidal circulation and the retinal venous system via the pial branches of the central retinal vein.[8]
Risk Factors
Risk factors for the development of macular vortex veins include:
- High Myopia: The elongation of the eyeball in high myopia can lead to changes in choroidal blood flow, increasing the likelihood of vortex vein anomalies.[9]
- Pachychoroid Spectrum Diseases: These conditions are characterized by a thickened choroid and may be associated with abnormal vortex veins.[10]
- Age: Although less clearly defined, age-related changes in the choroid may contribute to the complications associated with vortex vein anomalies like CNV.[11]
- Systemic and ocular diseases: While choriovaginal vein is generally not associated with systemic abnormalities, it has been reported in conditions such as trisomy 13 with congenital glaucoma.[8]
General Pathology
Posterior and macular vortex veins exhibit abnormal dilation or positioning relative to typical choroidal drainage patterns. In macular vortex veins, the drainage of choroidal blood near or within the macula can lead to significant clinical consequences, including an increased risk of choroidal neovascularization (CNV).[12]
Pathophysiology
The pathophysiology of macular vortex veins involves altered venous drainage leading to increased venous pressure and congestion within the choroid. This can result in various pathological changes, including pachyvessel formation, choroidal thickening, and, in some cases, CNV. The abnormal location of these veins near the macula may directly impact central vision.[13][3]
Primary Prevention
Primary prevention of posterior or macular vortex veins is not well established due to their congenital and developmental nature. However, managing underlying conditions like high myopia and patients education regarding complicatios like CNV symptoms may help early distinguishing of complications associated with these anomalies.[2]
Diagnosis
History
A detailed patient history focusing on visual symptoms, particularly those related to central vision, and any history of high myopia or other relevant ocular conditions is crucial. Understanding the patient's history of retinal conditions or previous surgeries can also provide valuable diagnostic clues.[14]
Physical Examination
A comprehensive ophthalmic examination, including dilated fundus examination, is essential. The examination may reveal abnormal venous structures, particularly in the macular area in cases of macular vortex veins.[15]
Signs
Signs of macular vortex veins may include:
- Presence of large, dilated choroidal vessels near the macula
- Associated retinal pigment epithelial changes
- Potential subretinal fluid or hemorrhage if CNV is present[7]
Symptoms
Patients may present with symptoms such as:
- Blurred or distorted central vision
- Scotomas in the central field
- Photopsia (flashes of light)[3]
Clinical Diagnosis
Clinical diagnosis involves identifying the characteristic features of vortex veins using multimodal imaging techniques. The abnormal location and dilation of the veins near the macula can be visualized through advanced imaging.[9]
Diagnostic Procedures
Key diagnostic procedures include:
- Optical Coherence Tomography (OCT): Provides detailed images of the retina and choroid, revealing structural changes associated with macular vortex veins.[15]
- Indocyanine Green Angiography (ICGA): Useful for visualizing the choroidal vasculature and confirming the presence of abnormal veins. [10]
- Fluorescein Angiography (FA): May be used to assess the presence of CNV and other related retinal pathologies.[13]
Laboratory Tests
Laboratory tests are generally not necessary for diagnosing posterior or macular vortex veins unless systemic vascular conditions are suspected.[3]
Differential Diagnosis
Differential diagnoses include:
- Choroidal neovascularization from other causes
- Central serous chorioretinopathy (CSC)
- Polypoidal choroidal vasculopathy (PCV)
- Choroidal melanoma[9]
- Sclerochoroidal calcification
Management
General Treatment
Treatment is primarily directed towards managing complications, such as CNV, rather than the vortex veins themselves.[14]
Medical Therapy
Anti-VEGF therapy is the mainstay treatment for CNV associated with macular vortex veins. This therapy helps reduce neovascularization and preserve vision.
Medical Follow-Up
Patients with macular vortex veins do not require follow-up, but if CNV is present or if there are significant retinal changes. Regular follow-up recommended to monitor cases with suspected for complications or other diseases .[12]
Surgery
Intervention is not typically required for posterior or macular vortex veins themselves but may be necessary to address CNV or retinal detachment associated with pathologic myopia[2]
Surgical Follow-Up
Post-surgical follow-up is essential to ensure proper healing and monitor for recurrence or new complications.[7][10]
Complications
- Complications can include:
- Development of CNV leading to vision loss
- Retinal detachment in severe cases of associated pathologic myopia
- Subretinal hemorrhage[11]
Prognosis
The prognosis depends on the presence and management of complications like CNV. With appropriate treatment, many patients can maintain stable vision, though some may experience progressive vision loss.[15]
Choroidal Macrovessels
Disease Entity
Choroidal macrovessel (CM) is a rare, anomalous vascular lesion of the choroid characterized by an abnormally dilated, tortuous vessel extending through the choroidal layers. It is often diagnosed incidentally on multimodal imaging but can sometimes cause visual symptoms due to secondary effects on the retina and retinal pigment epithelium (RPE) [16].
Disease
CM is a non-progressive vascular anomaly that is typically asymptomatic but can be associated with subretinal fluid, ellipsoid zone disruption, and pigmentary changes, leading to visual disturbances in some cases [17].
Etiology
The pathogenesis of CM remains unclear. Several hypotheses have been proposed:
A congenital anomaly due to aberrant choroidal vessel development. An acquired vascular remodeling response secondary to choroidal ischemia. A variant of an aberrant posterior ciliary artery that fails to follow normal vascular pathways [17].
Risk Factors
- Age: More commonly diagnosed in middle-aged to elderly patients, though rare cases have been reported in younger individuals, including a 12-year-old patient [16].
- Sex: Slight female predominance.
- Systemic Conditions: Some cases have been associated with hypertension, diabetes mellitus, hypothyroidism, and hyperlipidemia, but no definitive causal relationship has been established [16].
General Pathology
Histopathologic data is scarce, but imaging studies suggest that CM is a hyporeflective, vascular lesion occupying the entire thickness of the choroid. It is often associated with elevation of the overlying retina and posterior displacement of the sclerochoroidal junction [17].
Pathophysiology
The functional impact of CM is largely dependent on its location and secondary effects:
Compression of the choriocapillaris may lead to localized ischemia. Subretinal fluid accumulation may occur, possibly due to choroidal venous congestion or mechanical disruption of the RPE pump [16].
Primary Prevention
There are no known preventive measures for CM. However, regular ophthalmic examinations are advised, especially in individuals with systemic vascular risk factors [17].
Diagnosis
History
Typically asymptomatic and found incidentally.
- In symptomatic cases, patients may report:
- Metamorphopsia
- Decreased central vision (if involving the fovea)
- Scotomas [16].
Physical Examination
- Fundus examination:
- Orange-red, serpiginous lesion extending from the macula to the periphery.
- Retinal pigment epithelium (RPE) alterations may be present.
Signs
- A visible choroidal vessel coursing through the macular region.
- Retinal elevation and posterior shadowing on imaging.
- Possible subretinal fluid [17].
Clinical Diagnosis
Multimodal imaging is critical to differentiate CM from choroidal tumors, inflammatory diseases, and parasitic infections [17].
Diagnostic Procedures
- Optical Coherence Tomography (OCT):
- Enhanced Depth Imaging (EDI-OCT):
- Hyporeflective lesion occupying the choroid.
- Posterior shadowing with retinal elevation.
- Possible ellipsoid zone disruption [16].
- Enhanced Depth Imaging (EDI-OCT):
- Fluorescein Angiography (FA):
- May show early focal hyperfluorescence but no leakage, differentiating it from inflammatory and neoplastic conditions [16].
- Indocyanine Green Angiography (ICGA):
- Shows early hyperfluorescence with a serpiginous vessel-like shape.
- Absence of late-phase leakage distinguishes it from choroidal tumors [17].
Laboratory
No specific laboratory markers exist for CM, but systemic evaluation may be warranted in patients with associated conditions.
Tests
Ocular B-scan ultrasonography may show a nodular hypoechogenic lesion. OCT angiography (OCTA) can confirm the vascular nature of the lesion [17].
Differential Diagnosis
- Choroidal hemangioma (distinguished by dome-shaped appearance on OCT).
- Diffuse unilateral subacute neuroretinitis (parasitic infections causing track-like lesions).
- Congenital retinal macrovessels (located in the retina rather than the choroid).
- Vortex vein ampullae (venous origin, unlike CM which appears arterial) [16].
Management
General Treatment
No intervention is required for asymptomatic cases. Observation is advised; however, no standardized guidelines regarding follow-up are available. In uncertain cases, follow-up imaging may be considered to monitor for potential secondary complications. [17].
Medical Therapy
No established pharmacologic treatment for CM. In cases with subretinal fluid, off-label use of anti-VEGF therapy may be considered [16].
Medical Follow-Up
No guideline available. OCT scans maybe considered to assess for changes in lesion size and secondary complications. [17].
Surgery
No surgical intervention is indicated for CM itself. If secondary complications arise (e.g., macular edema, CNV), intervention is guided by the specific pathology [16].
Complications
- Subretinal fluid accumulation.
- Retinal pigment epithelial atrophy.
- Choroidal neovascularization (rare cases).
- Progressive vision loss in rare cases [17].
Prognosis
Generally stable over time with a favorable visual prognosis if the macula is not significantly affected. Rare cases may experience progressive visual decline due to secondary complications [16].
Choroidal Macroaneurysms
Choroidal macroaneurysms are localized dilations of choroidal vessels, typically involving arteries. They can lead to visual impairment due to exudation, hemorrhage, or both, affecting the overlying retinal tissue.[18]
Disease Entity
Disease
Choroidal macroaneurysms are localized dilations of choroidal vessels, typically involving arteries rather than veins. These aneurysms can lead to visual impairment due to exudation, hemorrhage, or a combination of both, affecting the overlying retinal tissue.[18]
Etiology
The etiology of choroidal macroaneurysms is not entirely understood but is believed to involve degenerative changes in the arterial walls, possibly exacerbated by systemic vascular conditions such as hypertension or arteriosclerosis.[19]
Risk Factors
Risk factors for choroidal macroaneurysms include:
- Systemic hypertension
- Advanced age
- Arteriosclerosis[18]
Pathophysiology
Choroidal macroaneurysms involve the weakening and dilation of choroidal arteries, often associated with anomalous short posterior ciliary arteries. This can lead to blood leakage, retinal detachment, hemorrhage, or macular edema.[18]
Diagnosis
Diagnostic Procedures
Key diagnostic procedures include:
- Optical Coherence Tomography (OCT): Provides cross-sectional images of the retina, revealing the extent of fluid accumulation and structural changes.
- Fluorescein Angiography (FA): Highlights the choroidal circulation and can identify areas of leakage or aneurysmal dilation.
- Indocyanine Green Angiography (ICGA): Used to visualize the choroidal vessels more effectively, identifying the aneurysmal dilation.[20]
Differential Diagnosis
Differential diagnoses include:
- Polypoidal Choroidal Vasculopathy (PCV)
- Retinal Artery Macroaneurysm
- Central Serous Chorioretinopathy (CSC)[18]
Management
General Treatment
Treatment approaches may include:
- Observation for asymptomatic or stable cases
- Anti-VEGF therapy (effectiveness may vary)
- Laser photocoagulation
- Photodynamic therapy (may be ineffective in some cases)
A case report highlighted that direct laser photocoagulation was successful when anti-VEGF and photodynamic therapies were ineffective.[18]
Prognosis
Prognosis varies depending on the severity, location, and complications. Some patients maintain stable vision with appropriate management, while others may experience progressive vision loss.[21]
Choroidal Varix
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Disease Entity
Choroidal varix refers to the abnormal dilation of a vortex vein ampulla, which is often asymptomatic but can be mistaken for more serious choroidal or retinal conditions due to its appearance. This condition typically presents as a dark, elevated lesion on the choroid and may affect the overlying retinal pigment epithelium (RPE). The varices are usually benign but can cause significant clinical concern due to their resemblance to malignant lesions, such as choroidal melanomas.[18]
Disease
Choroidal varices are typically characterized by the dilation of vortex vein ampullae, which can present as isolated or multiple lesions. These varices can appear brownish-red to gray and are usually located in the mid-peripheral choroid or equatorial region. Although they are often detected incidentally, their appearance may lead to confusion with more serious conditions, requiring careful diagnosis.[22]
Etiology
The exact etiology of choroidal varices is not well understood. However, it is believed that increased ocular venous pressure or mechanical factors such as gaze-evoked kinking of the episcleral portion of a vortex vein may contribute to their development. Other potential contributing factors include narrowing of the scleral emissary canal and conditions that elevate venous pressure, such as Valsalva maneuvers or prone positioning.[22]
Diagnosis
History
A thorough patient history is crucial, particularly focusing on any previous ocular surgeries, trauma, or symptoms that might suggest increased venous pressure. Patients are usually asymptomatic, and varices are often discovered incidentally during routine examinations.[22]
Physical Examination
Physical examination, including funduscopy, often reveals a dark, elevated choroidal lesion. The varix may change in appearance depending on the gaze or applied pressure, which can help differentiate it from other conditions. Digital pressure to the globe during ophthalmoscopic examination can confirm the diagnosis.[22]
Diagnostic Procedures
Key diagnostic procedures include:
- Optical Coherence Tomography (OCT): Reveals a hyporeflective cavity corresponding to the dilated choroidal vessels.
- Indocyanine Green Angiography (ICGA): Demonstrates early hyperfluorescence and dye pooling within the varix.
- Fundus Autofluorescence: Shows hypofluorescent rings surrounding the lesion.
- B-scan ultrasonography: Reveals gaze-dependent changes, unlike melanoma.
- Ultra-widefield contact lens: A novel technique for applying ocular pressure, resulting in resolution of the varix.[22]
Differential Diagnosis
Differential diagnoses include:
- Choroidal melanoma
- Choroidal hemangioma
- Subretinal hemorrhage
- Metastatic choroidal lesions
- Choroidal nevus[22]
Management
General Treatment
Choroidal varices are generally benign and do not require treatment unless they cause significant symptoms or are mistaken for malignant lesions. Management typically involves monitoring with periodic imaging to ensure stability.[22]
Prognosis
The prognosis for patients with choroidal varices is generally excellent, as these lesions are benign and often remain stable over time. In some cases, the varix may resolve spontaneously, as noted in rare instances.[23]
Pachyvessels
Disease Entity
Pachyvessels are abnormally dilated choroidal vessels found within the Haller's layer of the choroid, commonly associated with pachychoroid spectrum diseases. These vessels are linked to various ocular conditions, including central serous chorioretinopathy (CSC), pachychoroid neovasculopathy (PNV), and polypoidal choroidal vasculopathy (PCV). The presence of pachyvessels is typically indicative of underlying choroidal thickening and altered choroidal hemodynamics.[24]
Role in Disease
Pachyvessels play a central role in the pathophysiology of pachychoroid diseases. These enlarged vessels contribute to choroidal thickening, which can compress the overlying choriocapillaris and cause ischemia. This ischemia may lead to retinal pigment epithelium (RPE) changes, serous retinal detachment, and the development of choroidal neovascularization (CNV).[25]
Etiology
The etiology of pachyvessels is not entirely understood, but they are believed to arise from chronic venous congestion within the choroid [26]. This congestion could be due to systemic factors like hypertension or localized factors such as thickened sclera, which restricts normal venous outflow.[27]
Risk Factors
Risk factors for the development of pachyvessels include:
- Central Serous Chorioretinopathy (CSC): A condition frequently associated with pachyvessels due to choroidal thickening.[28]
- Hypertension: Systemic hypertension is a significant risk factor due to its potential to increase choroidal venous pressure.[25]
- Age: Aging can lead to structural changes in the choroid, contributing to the development of pachyvessels.[27]
- Hyperopia: Some studies suggest a potential association between hyperopia and pachychoroid spectrum diseases, possibly due to the relative crowding of ocular structures.[29]
While not definitively established as direct risk factors, the following conditions have been explored in relation to choroidal changes:
- Myopia: High myopia has been associated with various structural changes in the posterior segment of the eye, including alterations in choroidal thickness. However, its direct role in the development of pachyvessels specifically is still under investigation.[30]
- Nanophthalmos: Nanophthalmos, characterized by a small eye with a thick sclera, may predispose individuals to choroidal effusion and altered choroidal hemodynamics, potentially contributing to the development of pachyvessels. However, further research is needed to clarify this association.[31]
General Pathology
Pachyvessels are characterized by their significant dilation and thickening within the Haller's layer of the choroid. These vessels can cause compression of the overlying choriocapillaris, leading to reduced perfusion and ischemia in the outer retina. This can result in RPE dysfunction, serous retinal detachment, and choroidal neovascularization.[24][25]
Pathophysiology
The presence of pachyvessels leads to a cascade of pathological events. The dilated vessels compress the choriocapillaris, causing reduced blood flow and ischemia in the outer retina. This ischemia can lead to RPE dysfunction, serous retinal detachment, and the potential development of CNV. Additionally, choroidal hyperpermeability often accompanies pachyvessels, exacerbating retinal pathology.[24][28]
Primary Prevention
Primary prevention of pachyvessels focuses on managing systemic risk factors, particularly hypertension. Regular monitoring of individuals with a history of CSC or other pachychoroid spectrum diseases may help prevent complications associated with pachyvessels.
Diagnosis
History
Patients may report symptoms such as blurred vision or metamorphopsia, often linked to underlying conditions like CSC. A history of hypertension or other vascular diseases is also significant in the context of pachyvessels. Refractive error (hyperopia, myopia) and any history of ocular developmental anomalies (e.g., nanophthalmos) should also be noted.
Physical Examination
Ophthalmic examination typically reveals signs of serous retinal detachment or RPE changes, particularly in the macula. Imaging is crucial for the identification of pachyvessels. A comprehensive refraction should be performed. Axial length measurement may be useful in certain cases.
Signs
- Dilated and thickened choroidal vessels visible on imaging.
- Compression of the choriocapillaris leading to ischemic changes in the retina.[24]
Symptoms
- Blurred vision
- Metamorphopsia
- Scotomas in cases where CNV develops.
Clinical Diagnosis
The diagnosis of pachyvessels is confirmed through multimodal imaging, where their characteristic dilation and thickening are visible.
Diagnostic Procedures
- Optical Coherence Tomography (OCT): Visualizes the thickened choroid and pachyvessels.[27]
En Face OCT is a vital imaging modality in the detection and management of pachyvessels and related lesions in pachychoroid spectrum diseases. This advanced technique provides a broad, detailed view of the choroid, allowing for the precise mapping of pachyvessels, which appear as large hyporeflective spaces within the Haller’s layer. Enhanced Depth Imaging optical coherence tomography (EDI-OCT) or Swept Source OCT (SS-OCT) is particularly effective in identifying areas of choroidal thickening, congestion, and interactions at choroidal watershed zones, which are critical for understanding disease pathophysiology. It also correlates structural abnormalities with functional changes, such as choroidal hyperpermeability, aiding in the comprehensive assessment of conditions like central serous chorioretinopathy (CSC) and pachychoroid neovasculopathy (PNV). Overall, OCT enhances the early detection, accurate diagnosis, and targeted management of these conditions, surpassing traditional imaging techniques in its detailed and expansive visualization capabilities.
- Indocyanine Green Angiography (ICGA): Highlights hyperpermeable areas and dilated vessels.[28]
- Optical Coherence Tomography Angiography (OCT-A): Assesses blood flow and identifies CNV.[25]
Laboratory Tests
Laboratory tests are not typically necessary for diagnosing pachyvessels unless there is a need to assess systemic conditions.
Differential Diagnosis
Differential diagnoses include:
- Polypoidal choroidal vasculopathy (PCV)
- Central serous chorioretinopathy (CSC)
- Choroidal neovascularization (CNV) from other causes[27]
Management
General Treatment
Management primarily involves treating associated complications like CNV. Regular monitoring and imaging are crucial to identify and address any progression or complications related to pachyvessels.[25]
Medical Therapy
- Anti-VEGF Therapy: Used to manage CNV associated with pachyvessels, aiming to reduce neovascularization and preserve vision.[28]
Medical Follow-Up
Patients with pachyvesselsdo not require follow-up, but if CNV is present or CSCR if there are significant retinal changes. Regular follow-up recommended to monitor cases with suspected for complications or other diseases .[24]
Surgery
Surgical intervention is not typically required for pachyvessels themselves.[27]
Surgical Follow-Up
Post interventional follow-up is crucial to ensure proper healing and monitor for recurrence or new complications.[25][28]
Complications
Complications related to pachyvessels include:
- Development of CNV leading to vision loss.
- Serous retinal detachment in severe cases.
- Subretinal hemorrhage.[24]
Prognosis
The prognosis for patients with pachyvessels depends on the presence and management of complications like CNV. With appropriate treatment, many patients can maintain stable vision, though some may experience progressive vision loss.[25]
Choroidal Synphlebia
Disease Entity
Choroidal Synphlebia (CS) is defined as a confluent choroidal venous structure at least 750 𝜇m in diameter in its smallest lateral or vertical dimension, representing fusion or coalescence of major veins with loss of normal medium-sized vessels (Sattler’s layer) overlying it.[32]
Associated Diseases
A case series of 19 eyes of 16 patients described central serous chorioretinopathy (CSC) as the most common associated disease (84% of eyes), followed by neovascular age-related macular degeneration (nAMD) (2 cases) and myopia (1 case).
General Pathology
There are two main morphologic patterns described:
- Broad confluence of large veins resulting in a lobulated venous lake
- Caput medusae with smaller tributaries converging radially into a dilated central channel.
Both subtypes occur near or at presumed watershed zones for choroidal venous drainage, where large choroidal vessels are not expected.
Pathophysiology
- Developmental venous anomaly hypothesis
During normal embryogenesis, the primitive vascular plexus undergoes progressive partitioning of vascular lumens, establishing the hierarchical choroidal architecture: choriocapillaris, Sattler's layer, and Haller's layer. In CS, a failure of this partitioning is hypothesised to result in the persistence of anomalous confluent venous channels within the outer choroid. So, CS may represent the choroidal equivalent of developmental venous anomalies (DVAs) of the brain, where transmedullary veins fail to regress.[33]
- Acquired venous remodeling hypothesis
An alternative hypothesis implicates somatic mutations in two key regulators of vascular signalling. TIE2, a receptor tyrosine kinase expressed on endothelial cells, promotes vessel stabilisation when activated by Angiopoietin-1; its dysregulation destabilises vessel walls and predisposes to abnormal remodelling. PIK3CA encodes the catalytic subunit of PI3K, and gain-of-function mutations lead to constitutive activation of the PI3K/AKT/mTOR axis, promoting endothelial proliferation and pathological venous dilation. Since TIE2 signals through PI3K/AKT, dysregulation of either gene may converge on the same downstream effectors. The somatic nature of these mutations accounts for the focal distribution of CS lesions, mirroring the mechanism described in venous malformations elsewhere.[34][35]
- Hemodynamic implications
Normally, resistance distributed across arterioles, choriocapillaris, and collecting venules prevents excessive pressure transmission to the capillary bed. In CS, replacement of Sattler's layer vessels by large venous lakes eliminates key resistance elements, creating a low-resistance pathway between the choriocapillaris and the outer choroidal veins. The resulting venous hypertension at the capillary level increases transudation across Bruch's membrane, disrupting the outer blood-retinal barrier and producing the multifocal choroidal hyperpermeability characteristic of CSC.
- Relationship with CSC and the pachychoroid disease spectrum
Pachychoroid disease is characterised by dilated outer choroidal vessels, inner choroidal layer attenuation, and focal Bruch's membrane disruption. CSC is a manifestation presenting with subretinal fluid accumulation secondary to RPE dysfunction and choroidal hyperpermeability, with progressive RPE atrophy in chronic cases. CS may represent a structural extreme of this spectrum, where venous remodelling produces discrete anatomical lakes rather than diffuse dilation. [36]
Clinical Importance
Although CS itself is asymptomatic and requires no direct treatment, its identification carries important clinical implications. Its association with refractory CSC suggests that CS may represent choroidal hyperpermeability independently of other triggers, contributing to treatment resistance in a subgroup of patients. CS may serve as an imaging marker of pathological choroidal venous remodeling, with its presence indicating a degree of structural disruption of the normal layered choroidal architecture. Finally, CS may reflect chronic venous overload of the microvasculature, being a potential marker of hemodynamic stress Recognising CS on multimodal imaging may help identify patients at higher risk of recurrent or chronic disease and guide therapeutic decision-making.
Diagnosis
Physical Examination
Fundus examination: featureless or with partial visualization of the choroidal venous circulation.
Diagnostic Procedures
Swept-Source Optical Coherence Tomography (OCT):[32]
- Confluent vascular structure measuring at least 750 µm in its smallest lateral or vertical dimension.
- The vessels occupy nearly the entire thickness of the choroid, leading to a lack of visible vessels in the overlying Sattler’s layer and the inner choroid.
- Lesions are frequently located in the macula, often near presumed choroidal watershed zone.
Indocyanine Green Angiography (ICGA):[32]
- Hypercyanescence lesion, where individual veins are not visible.
- Vessels merge into a single lobulated structure with indistinct internal boundaries.
Differential Diagnosis
Pachyvessels associated with CSC
Vortex vein ampullae
Choroidal vascular bulbosities (“sausaging”)
Polypoidal choroidal vasculopathy
Choroidal hemangioma
Dilated Haller vessels without venous confluence
Typical neovascular AMD without pachychoroid features
Management
Medical Teraphy
No established pharmacologic treatment for CS itself. Treatment is indicated for the underlying etiology. However, the hemodynamic alterations imposed by CS may persist independently of standard interventions, potentially explaining the refractory course in some CSC patients, so CS should be considered as a possible substrate underlying treatment resistance. One reported case of a patient refractory to multiple photodynamic therapy sessions who responded to faricimab should be mentioned: by blocking ANG-2, faricimab restores TIE2-mediated vessel stabilisation, directly implicating the ANG/TIE2 axis in CS pathogenesis and suggesting a potential therapeutic role for this pathway in refractory cases.[34]
Follow-Up
Recognition of CS may explain atypical or treatment-resistant CSC and neovascular presentations. Follow-up imaging may be considered to monitor changes in lesion size and secondary complications, such as microvascular damage, local ischemia, chronic subretinal fluid with RPE dysfunction, serous retinal detachment and macular neovascularization.
Prognosis
Prognosis varies depending on the severity, location and complications of the underlying etiology. CS is currently interpreted more as a marker of chronic choroidal stress than as an independent driver of poor vision. In the available series, the venovenous anastomotic structures are structurally stable and the prognosis is largely dictated by the underlying disease rather than by the synphlebia itself. In clinical practice, it should be thought of as evidence of long-standing venous congestion and advanced choroidal remodeling.
Future Perspectives
Artificial intelligence (AI) and deep learning algorithms may allow for objective classification of CS subtypes and longitudinal monitoring of disease progression, currently limited by the subjective nature of imaging interpretation. OCTA analysis may also help to better delineate the extent of choriocapillaris attenuation overlying venous lakes and clarify the relationship between structural vascular changes and functional perfusion deficits. Deep-learning algorithms are becoming increasingly capable of automating choroidal thickness and choroidal vascularity index (CVI, which is emerging as a robust biomarker across several diseases) from SS-OCT and vessel density and flow voids from OCTA. Applying these tools to CS, AI may be able to detect and quantify venovenous anastomoses and pachyvessels, generate “venous congestion maps” and feed these data into risk-stratification models that, ultimately, may be used as a medical decision support software in real world settings. Also, the identification of somatic mutations in TIE2 and PIK3CA in similar vascular anomalies raises the possibility of future molecular profiling of CS and targeted therapy in refractory cases.[37][38][39]
References
- ↑ Hayreh, S. S. (2010). Physiological anatomy of the choroidal vasculature. In A. Levin et al. (Eds.), *Encyclopedia of the Eye* (Vol. 3, pp. 414–430). Elsevier.
- ↑ 2.0 2.1 2.2 Nitta K, Akiyama H (July 02, 2024) Different Vortex Vein Anomalies Observed in a Single Case: Macular Vortex Vein in One Eye and Varix of Vortex Vein Ampulla in the Other Eye. Cureus 16(7): e63668. doi:10.7759/cureus.63668
- ↑ 3.0 3.1 3.2 3.3 Schouten IM, Palkar AH, Bhende M. Macular vortex vein with choroidal neovascularisation in pathologic myopia. Indian J Ophthalmol. 2019;67(10):1717-1718.
- ↑ Sharma A, Parachuri N, Kumar N, et al. Vortex vein anastomosis and pachychoroid—an evolving understanding. Eye. 2021;35(6):1545-1547.
- ↑ Guiqin He, Xiongze Zhang, Xuenan Zhuang, Yunkao Zeng, Yuhong Gan, Yongyue Su, Miaoling Li, Yuying Ji, Lan Mi, Xuelin Chen, Feng Wen; A Novel Exploration of the Choroidal Vortex Vein System: Incidence and Characteristics of Posterior Vortex Veins in Healthy Eyes. Invest. Ophthalmol. Vis. Sci. 2024;65(2):21. https://doi.org/10.1167/iovs.65.2.21.
- ↑ Ohno-Matsui K, Morishima N, Ito M, et al. Posterior routes of choroidal blood outflow in high myopia. Retina. 1996;16(5):419-425.
- ↑ 7.0 7.1 7.2 Patel G, He G, Zhang X, Wen F. Subfoveal focal choroidal excavation with macular vortex vein. Ophthalmology Retina. 2023;17(6):41-43.
- ↑ 8.0 8.1 https://entokey.com/vascular-anomalies-of-the-fundus/
- ↑ 9.0 9.1 9.2 Matsumoto H, Hoshino J, Arai Y, et al. Vortex vein anastomosis at the watershed in pachychoroid spectrum diseases. Ophthalmol Retina. 2020;4(9):938-945.
- ↑ 10.0 10.1 10.2 Hiroe T, Kishi S. Dilatation of asymmetric vortex vein in central serous chorioretinopathy. Ophthalmol Retina. 2018;2(2):152-161
- ↑ 11.0 11.1 Matsumoto H, Kishi S, Mukai R, Akiyama H. Remodeling of macular vortex veins in pachychoroid neovasculopathy. Sci Rep. 2019;9:14689.
- ↑ 12.0 12.1 Spaide RF. Choroidal blood flow: review and potential explanation for the choroidal venous anatomy including the vortex vein system. Retina. 2020;40(9):1851-1864.
- ↑ 13.0 13.1 Milani P, Mazzola M, Bergamini F. Suprachoroidal hemorrhage and vortex vein varix: a potential association. Eur J Ophthalmol. 2020;30(5):943-946.
- ↑ 14.0 14.1 Weidmayer SL, Demirci H. The spontaneous resolution of a vortex vein varix: case report. BMC Ophthalmol. 2021;21(1):101.
- ↑ 15.0 15.1 15.2 Spaide RF, Ledesma-Gil G, Cheung CMG. Intervortex venous anastomosis in pachychoroid-related disorders. Retina. 2020;40(9):1851-1864.
- ↑ 16.00 16.01 16.02 16.03 16.04 16.05 16.06 16.07 16.08 16.09 16.10 Gallo B, de Silva SR, Mahroo OA, et al. Choroidal macrovessels: multimodal imaging findings and review of the literature. Br J Ophthalmol. 2022;106(5):568-575. https://doi.org/10.1136/bjophthalmol-2020-318095
- ↑ 17.00 17.01 17.02 17.03 17.04 17.05 17.06 17.07 17.08 17.09 17.10 Mopuru R, Liu TYA, Arevalo JF. Choroidal Macrovessel Diagnosed on Multimodal Imaging, including Swept-Source Optical Coherence Tomography Angiography. Case Rep Ophthalmol. 2022;13(2):215-219. https://doi.org/10.1159/000521895
- ↑ 18.0 18.1 18.2 18.3 18.4 18.5 18.6 Kinoshita T, Mori J, Imaizumi H. Visual impairment associated with choroidal macroaneurysm in a patient with presumed anomalous short posterior ciliary artery. Am J Ophthalmol Case Rep. 2022 Nov 20;28:101755. doi: 10.1016/j.ajoc.2022.101755. PMID: 36439652; PMCID: PMC9694064.
- ↑ Pichi F, Morara M, Veronese C, et al. Multimodal Imaging in Choroidal Macroaneurysms. Retina. 2013;33(7):1568-1577. doi:10.1097/IAE.0b013e318285c9a4
- ↑ Shields CL, Salazar PF, Mashayekhi A, Shields JA. Peripheral exudative hemorrhagic chorioretinopathy simulating choroidal melanoma in 173 eyes. Ophthalmology. 2009;116(3):529-535. doi:10.1016/j.ophtha.2008.10.017
- ↑ Kishi S, Teramoto T, Tanaka T, et al. Visualization of Choroidal Vessels in Polypoidal Choroidal Vasculopathy by Indocyanine Green Angiography. Jpn J Ophthalmol. 1994;38(1):47-52.
- ↑ 22.0 22.1 22.2 22.3 22.4 22.5 22.6 Veronese C, Staurenghi G, Pellegrini M, et al. Multimodal Imaging in Vortex Vein Varices. Retin Cases Brief Rep. 2017;11(Suppl 1):163-165. doi:10.1097/ICB.0000000000000575
- ↑ Veronese C, Staurenghi G, Pellegrini M, et al. Multimodal Imaging in Vortex Vein Varices. Retin Cases Brief Rep. 2017;11(Suppl 1):163-165. doi:10.1097/ICB.0000000000000575
- ↑ 24.0 24.1 24.2 24.3 24.4 24.5 Gal-Or O, Dansingani KK, Sebrow D, et al. Inner Choroidal Flow Signal Attenuation in Pachychoroid Disease. Retina. 2018;38(10):1984-1992.
- ↑ 25.0 25.1 25.2 25.3 25.4 25.5 25.6 Wang T, Chan W, Tsai SH, Chen LJ. Clinical features of pachyvessels associated with polypoidal choroidal vasculopathy in chronic central serous chorioretinopathy. Sci Rep. 2021;11:13867.
- ↑ [Anthony E, Jayakumar J, Baskaran P. Spontaneous remodeling of abnormal choroidal vasculature. Ophthalmology Retina. 2025;9(3):234–236. doi:10.1016/j.oret.2025.01.003]
- ↑ 27.0 27.1 27.2 27.3 27.4 [Hilely A, Au A, Lee WK, et al. Pachyvitelliform Maculopathy: An Optical Coherence Tomography Analysis of a Novel Entity. Br J Ophthalmol. 2022;106(7):882-889.]
- ↑ 28.0 28.1 28.2 28.3 28.4 Govetto A, Sarraf D, Scialdone A. "Hide and Seek" Neurosensory Retinal Detachments in Peripapillary Pachychoroid Syndrome Associated with Pulmonary Arterial Hypertension. Retin Cases Brief Rep. 2021;16(1):118-125.
- ↑ Yamazaki T, Koizumi H, Yamagami C, et al. Enhanced depth imaging optical coherence tomography of the choroid in central serous chorioretinopathy. Am J Ophthalmol. 2012;153(4):716-724.
- ↑ [ Kim M, Lee DY, Lee WK, et al. Choroidal thickness in myopic eyes with and without myopic maculopathy. Ophthalmology. 2013;120(8):1652-1658.]
- ↑ Ryan, S.J., Sadda, S.R., Hinton, D.R., Schachat, A.P., Wilkinson, C.P., & Wiedemann, P. (Eds.). Retina (6th ed.). Elsevier.
- ↑ 32.0 32.1 32.2 Spaide RF. Pilot study of choroidal synphlebia. Retina. 2026;46(3):410-416. doi:10.1097/IAE.0000000000004755.
- ↑ Ma L, Hoz SS, Grossberg JA, Lang MJ, Gross BA. Developmental venous anomalies. Neurosurg Clin N Am. 2024;35(3):355–361. doi:10.1016/j.nec.2024.02.007.
- ↑ 34.0 34.1 Du Z, Liu JL, You YH, et al. Genetic landscape of common venous malformations in the head and neck. J Vasc Surg Venous Lymphat Disord. 2021;9(4):1007–1016.e7. doi:10.1016/j.jvsv.2020.11.016.
- ↑ Limaye N, Kangas J, Mendola A, et al. Somatic activating PIK3CA mutations cause venous malformation. Am J Hum Genet. 2015;97(6):914–921. doi:10.1016/j.ajhg.2015.11.011.
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Spaide RF, Gemmy Cheung CM, Matsumoto H, et al. Venous overload choroidopathy: a hypothetical framework for central serous chorioretinopathy and allied disorders. Prog Retin Eye Res. 2022;86:100973. doi:10.1016/j.preteyeres.2021.100973.
- ↑ Yoon J, Han J, Ko J, et al. Classifying central serous chorioretinopathy subtypes with a deep neural network using optical coherence tomography images: a cross-sectional study. Sci Rep. 2022;12:422. doi:10.1038/s41598-021-04424-z.
- ↑ López-Varela E, de Moura J, Novo J, et al. Fully automatic segmentation and monitoring of choriocapillaris flow voids in OCTA images. Comput Med Imaging Graph. 2023;104:102172. doi:10.1016/j.compmedimag.2022.102172
- ↑ Sterba M, Pokorna P, Faberova R, et al. Targeted treatment of severe vascular malformations harboring PIK3CA and TEK mutations with alpelisib is highly effective with limited toxicity. Sci Rep. 2023;13:10499. doi:10.1038/s41598-023-37468-4.
