Narrative review of the current management of ectopic varices: an update

Abstract

Background and Objective

Ectopic varices are a rare complication of portal hypertension that occur outside the esophagogastric region and represent a potentially life-threatening source of gastrointestinal bleeding. Treatments are frequently guided by institutional experience rather than standardized treatment algorithms due to their rarity and variable anatomy. This narrative review summarizes the epidemiology, clinical presentation, diagnostic evaluation, and management strategies.

Methods

A literature review was performed using PubMed and Google Scholar. Given that much of the available evidence consists of case reports, case series, and retrospective studies, no time restrictions were applied to the search. The search strategy broadly included studies relevant to ectopic varices, and no language restrictions were applied.

Key Content and Findings

Ectopic varices account for small proportion of variceal hemorrhage but are associated with significant morbidity and mortality. Clinical presentation varies according to anatomic location. Cross-sectional imaging, endoscopy and angiography play important roles in diagnosis and treatment planning. In the absence of bleeding, nonselective beta-blockers are the primary medical therapy to reduce portal pressure and prevent hemorrhage. For suspected or confirmed bleeding, prompt hemodynamic stabilization is essential, and splanchnic vasoactive agents such as octreotide or terlipressin should be initiated without delay even prior to confirmatory testing. Systemic vasopressors are reserved for patients in shock. Endoscopic band ligation and sclerotherapy are first-line treatment options after initiating vasopressor therapy, although their use may be limited by anatomical accessibility. If endoscopic therapy is unsuccessful or not feasible, interventional radiology (IR) techniques such as transjugular intrahepatic portosystemic shunt (TIPS), antegrade transvenous obliteration (ATO), retrograde transvenous obliteration (RTO), and recanalization of chronically occluded visceral veins are pursued based on patient anatomy and portal hemodynamics. Surgical interventions are reserved for refractory cases.

Conclusions

Management of ectopic varices requires an individualized, multidisciplinary approach that incorporates medical, endoscopic, IR, and surgical therapies.

Introduction

Portal hypertension can lead to complications such as esophageal varices, hepatic encephalopathy, and hepatorenal syndrome (1). Esophageal varices occur in about 50% of cirrhotic patients (2), while ectopic varices, defined as dilated portosystemic collaterals outside the gastroesophageal region (3), account for about 5% of variceal bleeds but carry up to 40% mortality in a study from the United Kingdom (4). Compared with the mortality rate of gastroesophageal varices, which has decreased from 23.6% to 18.3% in the recent decade, there are fewer data on the mortality improvements in ectopic variceal bleeding (5). Unlike esophageal varices, which bleed at a hepatic venous pressure gradient (HVPG) >12 mmHg, ectopic varices may bleed at lower pressures, necessitating different management approaches (4,6). In recent years, advances in endoscopic and interventional radiology (IR) techniques have expanded available treatment options, but the optimal approach remains unclear and often depends on local expertise. Given the rarity of ectopic varices and the evolving role of multidisciplinary management strategies, a contemporary review synthesizing available evidence and comparing current therapeutic approaches in warranted. This review summarizes the etiology, presentation, diagnosis, and treatment of ectopic varices. We present this article in accordance with the Narrative Review reporting checklist (available at https://cdt.amegroups.com/article/view/10.21037/cdt-2026-1-0083/rc).

Methods

A narrative review of literature was performed using PubMed and Google Scholar to identify studies related to ectopic varices, including epidemiology, clinical presentation, diagnosis, and management strategies. No date restrictions were applied to the search because much of the available evidence consists of case reports, case series, retrospective studies, and institutional experience. The search strategy broadly included terms related to ectopic varices and their management, including endoscopic, IR, and surgical therapies. No language restrictions were applied. Relevant publications from non-PubMed indexed sources, including university and institutional journals, were also considered when they provided clinically relevant data regarding ectopic varices. Reference lists of selected articles were also reviewed to identify additional relevant studies. Table 1 summarizes the search strategy.

Table 1. Outlines the search strategy.

Items	Specification
Date of search	Date of initial search is August 2024
Databases and other sources searched	PubMed and Google Scholar were searched. Reference lists of selected articles were also reviewed. Relevant non-PubMed indexed publications were considered when clinically applicable
Search terms used	Search terms included “ectopic varices”, “duodenal varices”, “rectal varices”, “jejunal varices”, “ileal varices”, “stomal varices”, “portal hypertension”, “variceal bleeding”, “TIPS”, “BRTO”, “RTO”, “ATO”, “embolization”, “endoscopic therapy”. MeSH and filters not applied
Timeframe	No restrictions
Inclusion and exclusion criteria	Included all relevant articles. Excluded articles without clinical relevance to ectopic varices
Selection process	Studies were selected by the authors based on clinical relevance to the epidemiology, diagnosis, and management of ectopic varices. Articles were reviewed collaboratively

Etiology of ectopic varices

Ectopic varices develop when resistance to portal blood flow through the liver exceeds that of collateral pathways (7). The leading cause is cirrhosis, most often alcohol-related, followed by noncirrhotic portal hypertension from portal vein thrombosis (8). Other causes include surgery, vascular compression or thrombosis, congenital anomalies, and prior variceal therapy (9,10).

Types and bleeding prevalence of ectopic varices

Studies show rectal varices are the most common ectopic varices. A nationwide Japanese survey of 173 cases, conducted using a structured questionnaire collecting clinical, endoscopic, and treatment data, found 77 rectal and 57 duodenal varices, with smaller numbers in the jejunum, ileum, and colon (11).

Bleeding risk varies by location. In 169 bleeding cases, 26% were stomal, 17% duodenal, and 8% rectal (12). A separate review of 161 cases found stomal varices most frequent (27% of cases), followed by jejunal or ileal (18%), duodenal (17%), and colonic (15%), with less frequent sites including peritoneal, vaginal, and biliary tract varices (13). Together, these studies suggest stomal varices carry the highest risk of bleeding. Table 2 summarizes the frequency and bleeding risk of the various locations.

Table 2. Outlines the comparison between ectopic varices locations.

Location	Relative frequency	Bleeding risk/contribution	Key clinical notes
Rectal	Most common	Low bleeding risk (~0.5–3.6%); ~8% of bleeding cases	Often incidental; associated with severity of portal hypertension; typically presents with hematochezia; frequently detected on endoscopy
Duodenal	Second most common	High bleeding risk; accounts for ~17–32.9% of ectopic varices; mortality up to 40%	Bleeding often massive and life-threatening; commonly associated with extrahepatic portal hypertension or venous thrombosis
Jejunoileal	Less common	Moderate bleeding risk; ~18% of cases; active bleeding relatively uncommon (~5.5%)	Often presents with obscure gastrointestinal bleeding; diagnosis may require capsule or balloon enteroscopy
Stomal (peristomal)	Variable frequency	Highest bleeding risk; ~26–27% of bleeding cases	Occurs in patients with prior bowel surgery; bleeding is often recurrent but associated with relatively low mortality (~3–4%)
Colonic (non-rectal)	Less common	Moderate bleeding risk; ~15% of cases	May present with lower gastrointestinal bleeding; often associated with portal hypertension or prior surgical history
Peritoneal/retroperitoneal	Rare	Variable bleeding risk; overall uncommon	Often difficult to diagnose; typically identified on cross-sectional imaging
Biliary	Rare	Rare but potentially severe bleeding	May present as hemobilia; diagnosis often requires imaging and endoscopic evaluation
Genitourinary (bladder, uterine, ovarian)	Rare	Rare but clinically significant bleeding	Often associated with prior surgery, malignancy, or venous obstruction

Rectal varices

Rectal varices form via collaterals between the superior rectal veins (portal) and middle/inferior rectal veins (systemic), typically appearing >4 cm above the rectal verge (14,15). They are common in patients with esophageal varices (up to 95% in patients with rectal varices) and may develop after endoscopic therapy due to collateralization (11,16-18) (Figure 1). Incidence rises with portal hypertension and correlates with disease severity (16,19).

Figure 1.

55-year-old woman with cirrhosis of the liver secondary to Hepatitis C presented with rectal bleeding. The portal venogram in the frontal view obtained with catheter in the main portal vein demonstrates prominent left gastric vein with gastroesophageal varices (yellow color) and prominent inferior mesenteric vein with (red arrow) (A) secondary to hepatofugal flow in the main portal vein. The black arrow points to the small diameter of the splenic vein. A later-phase portal venogram in frontal projection (B,C) shows a prominent inferior mesenteric vein (arrow) with multiple rectal varices (stars) due to retrograde flow.

Although rectal varices frequently occur, bleeding is uncommon (0.5–3.6%), usually presenting as hematochezia when the collaterals rupture (16). Endoscopy shows bluish submucosal swellings. Endoscopic ultrasound (EUS) improves detection (85% vs. 45%) and can assess bleeding risk using Doppler flow (18).

Duodenal varices

Duodenal varices are most often found in the first and second parts of the duodenum, with inflow from the superior mesenteric or pancreaticoduodenal veins and outflow to the inferior vena cava (IVC) (14,20). They can also be submucosal porto-porto or portomesenteric collaterals secondary to portal or central mesenteric venous occlusion secondary to thrombosis or external compression from a tumor. They account for around 17–32.9% of all ectopic varices, but bleeding is often massive and fatal, with mortality up to 40% from initial bleeding (21). They are more common in extrahepatic portal hypertension, in patients with prior abdominal surgery, and in those with splenic or portal vein thrombosis. Patients typically present with massive lower gastrointestinal bleeding and diagnosis relies on endoscopy and Doppler ultrasound (US) revealing portal venous and portomesenteric confluence thrombosis (22,23). Cross sectional imaging, specifically, computed tomography angiography (CTA) in arterial and portal venous phase, is critical in delineating the anatomy to identify afferent and efferent veins, extent of portal and mesenteric venous occlusion, and other potential varices.

Jejunoileal varices

Jejunoileal varices form through collaterals of the mesenteric and retroperitoneal venous systems, with risk factors including cirrhosis and prior abdominal surgery (14,24). Patients often present with hematochezia though active bleeding is uncommon (5.5% in one series) (25).

Capsule endoscopy has helped visualize the small bowel for diagnosis. One study found that its diagnostic yield for identifying small bowel bleed was 63% (29 of 46 patients) (26). Small bowel varices were detected in 4 of these 46 patients (8.7%). However, it is limited by nonstandardized interpretation and the risk of missing small or inactive lesions (27). Other modalities for diagnosis include multidetector computed tomography (MDCT), CTA, single and double balloon enteroscopy, Tc-99m red blood cell (RBC) scintigraphy (28-31).

Stomal varices

Stomas provide relief after major bowel surgery, but stomal varices are a potential complication caused by diversion of blood flow between the portal and systemic circulation (32,33). Reported prevalence is around 5%, though the true rate is uncertain since diagnosis often occurs only during active bleeding (33). Among 117 patients with liver disease who underwent colectomy, 27% of individuals with stomas developed bleeding from stoma varices within 5 years (34).

Most cases can be managed with manual compression, and mortality is relatively low (3–4%) (35,36). Stomal varices are often identified during bleeding episodes or incidentally on imaging. A case series noted bluish skin discoloration, engorged submucosal veins, and a raspberry-like stoma appearance in active cases (37). Confirmation methods included Doppler US, magnetic resonance angiography (MRA), CTA, and tagged RBC scan. MDCT reconstructions also aid in vascular assessment (38).

Management of ectopic variceal bleeding

Comparison of the treatment modalities is depicted in Table 3.

Table 3. Outlines the comparison between treatment modalities.

Feature	Endoscopic therapy	TIPS	ATO	RTO	Surgery
Primary mechanism	Direct ligation or injection (e.g., n-BCA) of varices	Decompresses portal system via portosystemic shunt	Embolizes afferent (inflow) veins	Embolizes efferent (outflow) vein and variceal complex	Surgical ligation, resection, or shunt creation
Hemodynamic effect	No global decompression	Decreases portal pressure globally	No global decompression	No global decompression; may increase portal pressure	Variable (decompression if shunt performed)
Best clinical use	First-line for accessible varices	Diffuse portal hypertension; need for global decompression	Complex or multiple inflow vessels; poor access for RTO	Well-defined portosystemic shunt with accessible outflow	Refractory cases when other therapies fail
Anatomical requirement	Endoscopic accessibility	Patent hepatic and portal veins	Portal venous access (transhepatic, transplenic, or via TIPS)	Accessible systemic venous outflow (e.g., gonadal or iliac vein)	Operable candidate; anatomy amenable to surgery
Effectiveness for ectopic varices	Effective for initial hemostasis; limited by access	Variable; often requires adjunctive embolization	Effective for targeted inflow control	Effective for localized varices with defined outflow	Effective but high morbidity
Risk of rebleeding	Variable; higher in small bowel and duodenal varices	Moderate if used alone	Moderate (especially if incomplete embolization)	Generally low in selected patients	Variable depending on procedure
Key limitations	Limited by accessibility and operator experience	Persistent collateral flow; risk of hepatic encephalopathy	Technically challenging; recurrence if feeders are missed	Limited by anatomy; may worsen portal hypertension	Invasive; high perioperative risk
Complications	Embolization (n-BCA), ulceration, rebleeding	Hepatic encephalopathy, shunt dysfunction	Non-target embolization, access site bleeding	Increased portal pressure, ascites, new varices	Bleeding, infection, hepatic decompensation
Role in combination therapy	Often followed by IR if unsuccessful	Frequently combined with ATO or RTO	Often used with TIPS	Can be combined with TIPS or used alone	Typically last-line; rarely combined
When preferred	When varices are accessible and patient is stable	When global portal decompression is needed	When inflow vessels are complex or multiple	When a single, accessible outflow vein is present	When all other therapies fail
Effect on portal hypertension complications	Neutral	Improves (decompression)	Neutral	May worsen	Variable

ATO, antegrade transvenous obliteration; IR, interventional radiology; n-BCA, N-butyl cyanoacrylate; RTO, retrograde transvenous obliteration; TIPS, transjugular intrahepatic portosystemic shunt.

Initial stabilization

Initial management of ectopic variceal bleeding includes clinical assessment, labs, stabilization, and specialist consultation. Hemodynamic management with fluids or blood products should be initiated as needed. Antibiotic prophylaxis has been shown to improve outcomes, with meta-analysis showing a 9.1% survival benefit in cirrhotic patients (39).

Vasoactive therapy should be initiated as soon as variceal bleeding is suspected, even prior to confirmatory diagnostic testing. Splanchnic vasoconstrictors such as octreotide are typically preferred due to their favorable safety profile and may be administered as monotherapy or in combination with midodrine, while terlipressin may be particularly beneficial in patients with hepatorenal syndrome (40). Nonselective beta-blockers also play a central role in primary medical management.

Diagnostic evaluation

American College of Gastroenterology recommends upper endoscopy within 24 hours of presentation of an upper gastrointestinal bleeding (41). From an IR perspective, a CTA in the arterial and portal venous phase provides critical information for planning endovascular management of varices. It is essential for localizing varices, mapping portosystemic collaterals, and identifying underlying causes such as portal or splenic vein thrombosis. Endoscopy provides direct visualization and therapy but may be limited by anatomy. Therefore, imaging should be performed early in patient care, and angiography must be tailored to each patient’s suspected ectopic varices based on prior imaging and endoscopic findings.

First-line therapy: endoscopic management

Variceal band ligation is widely used for esophageal varices, with success rate of 81.6% in one study of 603 patients from hospitals in Tunisia (42). More recently, band ligation has been applied to rectal, duodenal, and jejunal varices (43-45), but complications have been reported, including development of large collateral vessels causing severe hemorrhage (17). Rebleeding rates vary by site, with 15.8% of duodenal varices recurring in one study of 19 patients (46).

In terms of sclerotherapy for ectopic varices, various types of injections have been used to achieve hemostasis. N-butyl cyanoacrylate (n-BCA), a rapidly polymerizing adhesive agent when in contact with blood, is the most used sclerosant agent for endoscopic therapy for ectopic varices because it causes less tissue damage than the other agents (21,47). n-BCA is typically mixed with lipiodol in various ratios to obtain the preferred polymerization time. For duodenal varices, n-BCA is injected endoscopically in ≤1 mL boluses through a sclerotherapy needle to limit embolic risk, while rectal varices are treated similarly but usually with undiluted n-BCA for the lower embolic risk and less need for radiographic confirmation. The exact technique and mixture used is heavily operator dependent. In addition, argon plasma coagulation, which delivers ionized argon gas through an endoscopic probe to achieve superficial vessel coagulation, and coils have been shown to be effective for the treatment of ectopic varices (48-50). In many cases, combination therapy is used, as combination therapy has been shown to be more effective than monotherapy. For example, a combination of a n-BCA and coils, done under EUS had higher success rates and lower rebleeding rates than either technique alone (51,52). When using the combination, the coil acts as a scaffold to stabilize n-BCA therefore minimizing non-target embolization and enhances the durability of the occlusion.

Endoscopic Doppler US detects blood flow in suspect varices, confirms hemostasis after treatment, and reduces rebleeding risk by ensuring complete obliteration. A Doppler probe on the endoscope is placed against the mucosa to detect flow before and after therapy. In combined n-BCA and coil sclerotherapy, EUS guides needle access into the varix, coils are deployed to form a thrombogenic scaffold, and 0.5–2 mL of n-BCA is injected before immediate needle withdrawal; Doppler then verifies obliteration. The technique applies similarly across ectopic variceal sites and is operator dependent. In a study of patients with gastric varices who required conventional endoscopic n-BCA injections, where the operator injects n-BCA to the presumed vessel visualized on endoscopy, vs. those who received n-BCA injections through EUS, the patients who underwent EUS had lower rebleeding rates at >48 hours (18.5%) than those who received injections without US on endoscopy (44.7%) (53). In addition, there was tendency toward improved survival with EUS. Common varix locations it is used are duodenal, rectal, jejunoileal, stomal, and biliary.

n-BCA can rarely cause stroke, pulmonary embolism, and portal and splenic vein thrombosis in the setting of large injections, high-flow varices, or portosystemic shunts (54-56). Risk is minimized with limited volume and undiluted n-BCA injections.

Following initial hemostasis of ectopic variceal bleeding, management includes repeat sclerotherapy every 2–4 weeks until obliteration, long-term surveillance, and nonselective beta-blockers if not contraindicated (57).

Second-line therapy: IR management

Following failure or temporary hemodynamic management with endoscopic therapy, IR plays a central role. IR techniques such as transvenous occlusion, transjugular intrahepatic portosystemic shunt (TIPS) placement, and recanalization of occluded visceral veins may be necessary to achieve hemostasis. When endoscopy is not feasible or fails, these image-guided interventions provide definitive therapy, and a multidisciplinary team approach integrating hepatology, gastroenterology, IR, and surgery is considered standard of care to individualize management and optimize outcomes.

TIPS placement

TIPS placement reduces portal pressure by creating a low-resistance channel between the portal and hepatic veins. According to recent practice guidance from the American Association for the Study of Liver Diseases (AASLD), TIPS is a well-established and defined treatment strategy in the management of acute esophageal and gastric variceal hemorrhage, particularly in cases of massive or refractory bleeding, where early or preemptive TIPS has been shown to improve outcomes (58).

In contrast, the role of TIPS in ectopic variceal hemorrhage is more limited and less predictable. Unlike gastroesophageal varices, ectopic varices may bleed at lower portosystemic pressure gradients. As a result, decompression of the portal system with TIPS may not sufficiently reduce flow through collateral pathways, allowing persistent downhill flow through the ectopic varices and ongoing bleeding despite technically successful shunt placement (57). This hemodynamic limitation likely contributes to the higher rates of rebleeding observed when TIPS is used as monotherapy in ectopic varices.

Based on the practice guidance from the AASLD, TIPS is contraindicated in patients with congestive heart failure [stage C/D or ejection fraction (EF) <50%], severe pulmonary hypertension with mean pulmonary artery pressure (mPAP) >45 mmHg, severe uncontrolled hepatic encephalopathy, and uncontrolled sepsis. Model for end-stage liver disease (MELD) score is used for predictor of success. In addition, patients with portal vein thrombus, biliary dilation, hepatic tumors, and polycystic livers may pose a technical challenge. Therefore, patient history and technical experience should be considered when selecting patients for TIPS placement.

TIPS is often most effective when combined with adjunctive embolization techniques, such as antegrade transvenous occlusion (ATO) or retrograde transvenous occlusion (RTO), to achieve definitive hemostasis. Combination therapy allows for both global portal decompression and targeted obliteration of the bleeding variceal complex.

Isolated embolization with ATO or RTO may be pursued as an alternative to TIPS, particularly when TIPS is contraindicated or unlikely to adequately control bleeding based on anatomical or hemodynamic factors. However, because these approaches do not decompress the portal system, they may increase overall portal pressure and potentially exacerbate other complications of portal hypertension. Therefore, selection of TIPS, combination therapy, or isolated embolization should be individualized and determined on a case-by-case basis within a multidisciplinary framework.

ATO

ATO of the varices is frequently used to control active bleeding. It can be used for ectopic varices located in the duodenum, jejunum, ileum, colon, rectum, stoma, bladder, and gallbladder. This technique, which involves embolization of the afferent veins and the varices, is often used when there are multiple small portosystemic shunts or a large portosystemic shunt that cannot be occluded through the retrograde systemic venous access, and when TIPS placement is not likely to decompress the ectopic varices adequately to reduce or mitigate the bleeding risk. An antegrade approach to the ectopic varix allows the clinician to map the extent of the varices and define the number and size of efferent veins. Selecting the access route requires computed tomography (CT) or magnetic resonance (MR) venography to define portal anatomy, identify afferent and efferent veins, and evaluate technical feasibility. Transhepatic access is generally preferred when intrahepatic portal branches are patent and provide a direct route. This route is often used for bile duct, peritoneal, and pelvic varices. Trans-splenic access is favored when the portal vein is occluded, the liver is inaccessible, or if the splenic vein is the main feeder. It is a common access site for peristomal, colonic, or pelvic varices. If a TIPS is present or planned, it offers an optimal route for catheterization of the portal system and embolization of varices at any location (Figure 2). Ultimately, a multidisciplinary review of imaging by hepatology, gastroenterology, IR, and surgery is essential to select the safest and most direct route.

Figure 2.

Contrast-enhanced (A) axial and (B) coronal CT images showing parastomal varices (arrow) in a 60-year-old man with A1AT deficiency, emphysema, cirrhosis, and diverticulitis after colectomy with colostomy, complicated by recurrent parastomal variceal bleeding. (C) Percutaneous transhepatic portal venous access was obtained and an angled angiographic catheter was advanced through the inferior mesenteric vein into the afferent vein of the varix. Microcatheter advanced to this base catheter reveals afferent vein reaching up to the parastomal varix (yellow arrow). (D) Subsequently, after manually percutaneously compressing the efferent veins, embolization was performed with 3 cc of sclerosant (a 1:1:2 mixture of lipiodol, 3% sodium tetradecyl sulfate, and air) followed by two 6-mm detachable microcoils. Postembolization venogram reveal complete occlusion of the afferent vein and nonvisualization of the parastomal varices (yellow arrow). Portosystemic gradient was 8 mmHg at the end of the procedure, therefore TIPS was not performed. A1AT, alpha-1 antitrypsin; CT, computed tomography; TIPS, transjugular intrahepatic portosystemic shunt.

The embolic agents used for ATO include liquid agents such as n-BCA, ethylene vinyl alcohol copolymer, ethanolamine oleate, and sodium tetradecyl sulfate. These agents are typically used in conjunction with a proximal occluding balloon catheter or detachable coils/vascular plugs in the dominant afferent vein to reduce the risk of nontarget embolization in the systemic or visceral veins by reducing or stabilizing flow through the varices (59-61). Using a balloon catheter or coils/plugs also prevents reflux of the liquid agent into the portal and visceral venous system. Liquid embolic agents penetrate deep into the varices, causing thrombosis and occlusion of the varices with durable control of bleeding. In cases where the flow is high, a low polymerization time is preferred, less lipiodol (n-BCA: lipiodol ratio of 1:2 or 1:3) is recommended. When there are multiple afferent and efferent veins and the risk of nontarget liquid embolization cannot be mitigated, either coil or plug embolization should be performed before n-BCA embolization. However, due to the lack of distal penetration with these methods, the efficacy and durability of these techniques are relatively low (59-61).

Many case reports have demonstrated successful outcomes using ATO to manage life-threatening hemorrhage due to ectopic varices (62-65). This technique is particularly valuable when therapies such as TIPS placement are contraindicated or in emergencies with hemodynamic instability (62,65). Note, however, that a retrospective study in 14 patients who underwent percutaneous ATO demonstrated evidence of recurrent bleeding in 8 patients over a period ranging from 23 days to 27 months after the intervention (64). Rebleeding occurs primarily due to partial or incomplete obliteration of all afferent veins and complex collateral circulation. Repeat embolization involves re-accessing the portal system, ideally via the same route, and selectively embolizing persistent or newly recruited vessels with coils, plugs, or n-BCA.

RTO

RTO involves blocking the efferent veins of the portosystemic shunt and embolizing the varices. It can be used to treat peristomal, bile duct, peritoneal, uterine, and ovarian varices. The technique is similar for the treatment of each. The efferent vein is occluded to prevent the escape of sclerosant or embolic material into the systemic circulation with temporary balloon inflation or the use of vascular plugs and/or coils (Figure 3). Subsequently, varices can be obliterated using liquid embolic agents such as n-BCA, ethylene vinyl alcohol copolymer, ethanolamine oleate, and sodium tetradecyl sulfate. The primary difference in treating varices at different sites is the draining vein accessed: peristomal varices via the iliac vein and uterine or ovarian varices via the gonadal vein using the femoral or jugular approach. RTO allows for more effective eradication of ectopic varices by obliterating vascular structures from lower-pressure areas (efferent vein) to higher-pressure areas (afferent vein), leading to low rebleeding rates (66). RTO was originally used to embolize gastric varices. However, for ectopic varices, the anatomy of the shunt is more intricate and may be difficult to appreciate on cross-sectional imaging. Therefore, the use of RTO is relatively limited in the management of ectopic varices.

Figure 3.

Contrast enhanced CT scan of the abdomen in axial plane of a 45-year-old patient with massive upper gastrointestinal bleeding (A) reveals chronic pancreatitis (red star) with complete splenic vein occlusion and large perisplenic collateral vein (gastroepiploic) (arrows) leading up to the duodenal varices (yellow star). Portal venogram (B) revealed hepatopetal flow with non-visualization of the duodenal varix as expected. Percutaneous splenic venous access and selective gastroepiploic venogram (C) (red arrow) reveals it leading into the duodenal varices (star). Antegrade venogram revealed potential sites of communication in the portal venous system in the main and left portal vein. Subsequently, retrograde access into the duodenal varix (red star—image D) through left portal vein (red arrow—image D) and main portal vein (red arrow—image E) was obtained. (F) Outflow from the varix (arrows) were first occluded using Amplatz vascular plug to prevent nontarget embolization of liquid emboli into the portal venous system. (G) Immediately after that n-BCA embolization and coil embolization of the varix through the afferent vein was performed with complete obliteration of the varix. Portosystemic gradient at the end of the procedure was 6 mmHg. CT, computed tomography; n-BCA, N-butyl cyanoacrylate.

Balloon-assisted RTO uses temporary balloon occlusion of the efferent vein to allow liquid embolic treatment of the varices but permanent occlusion with plugs or coils is now more common. In this technique, embolization is performed through a catheter positioned beyond the occluded efferent vein. When multiple small efferent veins exist, Gelfoam is used first to block them and prevent systemic leakage of the liquid embolic agent (59).

It should be noted that the rebleeding rate with BRTO may be as high as 17% to 31% (20). Patients with rebleeding after BRTO are typically treated with repeat BRTO, ATO or TIPS, but require a multidisciplinary approach with hepatology, gastroenterology and IR for assessment. Complications associated with BRTO include hypotension, hypertension, pulmonary embolism, renal failure, chills, and fever due to sclerosant escape from incomplete occlusion, toxic effects of sclerosing agents, and acute hemodynamic changes from shunt closure (67).

Selection of IR technique

Selection of the optimal IR approach should be individualized based on portal hemodynamics, variceal anatomy, and patient-specific factors. In general, TIPS is preferred for global portal decompression, whereas ATO and RTO are better suited for targeted treatment of specific variceal complexes. Comparison of TIPS, ATO, and RTO are summarized in Table 3.

Recanalization of chronically occluded visceral veins

Ectopic varices can be caused by chronically occluded visceral veins. In such cases, recanalization of the veins followed by balloon venoplasty and/or stent placement may provide decompression within the varices to mitigate the bleeding risk. Frequently, in addition to recanalization, embolization of the varices is also performed often after recanalization for persistent bleeding (Figure 4). Other decompressive techniques such as proximal splenic artery embolization and percutaneous mesocaval shunt placement have been reported (20,68). These techniques are reserved for cases where standard endovascular embolization or decompression is not feasible. Proximal splenic artery embolization, using coils or an Amplatz Vascular Plug in the mid-splenic artery, reduces splenic perfusion and lowers porto-mesenteric flow and pressure. Beyond treating ectopic variceal bleeding, it is also used post-liver transplant to control ascites by decreasing portal pressures (69).

Figure 4.

Coronal reformatted images of a contrast enhanced CT scan of a 35-year-old female with upper gastrointestinal bleeding demonstrate (A) presence of duodenal varix (star) and (B,C) chronic short segment occlusion of the at least two tributaries of the SMV (arrows). Superior mesenteric venogram in frontal projection (D) demonstrates large duodenal varix (star) with afferent vein (arrow) from one of the tributaries of SMV. Venogram obtained at a later phase (E) demonstrates duodenal varix (star) with efferent vein (jejunal and posterior SMVs) (arrow) draining into the main portal vein (double arrows). (F) Superior mesenteric venogram after recanalization and stent placement in the occluded tributaries (arrows) and coil embolization of the duodenal varices (star) with collapse of afferent and efferent veins. CT, computed tomography; SMV, superior mesenteric vein.

Percutaneous mesocaval shunt creation involves transjugular access to the IVC and percutaneous transsplenic access to the porto-mesenteric veins. Snares are aligned under fluoroscopy, and a needle is advanced through both to establish a tract, followed by placement of a stent graft connecting the IVC and superior mesenteric vein (SMV). Like TIPS, this shunt decompresses the porto-mesenteric system and ectopic varices by diverting flow into the lower pressure IVC.

Rescue therapy

For cases in which endoscopic and IR methods fail, surgical treatment may be the last resort. Some of the available techniques include oversewing of the varix through an incision, circumferential stapled anoplasty, duodenal devascularization, and stapling with or without mesocaval shunt creation when TIPS placement is technically not feasible (70-73). Surgical mesocaval shunt placement involves midline laparotomy and creating an anastomosis between the SMV and the IVC using a synthetic graft, unlike the percutaneous mesocaval shunt insertion mentioned in the previous paragraph.

Discussion

The reviewed literature demonstrates that ectopic varices can occur throughout the gastrointestinal tract and are associated with significant morbidity and mortality, particularly in cases of acute hemorrhage. Key findings from the literature emphasize the importance of prompt hemodynamic management, vasoactive therapy, endoscopic evaluation, and multidisciplinary management (Figure 5). Advances in IR techniques including TIPS, ATO, RTO, and embolization have expanded therapeutic options and improved management of anatomically challenging lesions.

Figure 5.

Algorithm for the treatment of ectopic varices beings with medical stabilization. Endoscopic therapy with or without Doppler ultrasound is first line. If unsuccessful, IR is engaged for endovascular management. Surgery is last line if IR treatment is unsuccessful. Long term management includes surveillance with repeat endoscopy and medications such as nonselective beta-blockers. ATO, angiographic transjugular obliteration; CTA, computed tomography angiography; EUS, endoscopic ultrasound; IR, interventional radiology; MR, magnetic resonance; n-BCA, N-butyl-2-cyanoacrylate; RTO, retrograde transvenous obliteration; TIPS, transjugular intrahepatic portosystemic shunt.

However, most studies are retrospective, involve small patient populations, and lack standardized treatment protocols or long-term follow up. Significant heterogeneity exists in patient selection, treatment strategies, institutional protocols, definitions of treatment success, and reported outcomes. Many recommendations are derived from institutional experience rather than prospective comparative data or randomized controlled trials.

Future research should focus on multicenter prospective studies evaluating comparative outcomes between IR techniques. Additional investigation is needed to better define optimal patient selection criteria and the role of combination therapies. Development of standardized treatment algorithms and consensus guidelines may further improve management and outcomes in patients with ectopic variceal bleeding.

Conclusions

Ectopic varices are a rare but potentially life-threatening complication of portal hypertension that requires a structured, multidisciplinary approach to management. Effective care begins with prompt hemodynamic stabilization, early initiation of vasoactive therapy and antibiotics, and timely diagnostic evaluation with endoscopy and cross-sectional imaging. Endoscopic therapy remains first-line for accessible varices, although its efficacy is often limited by anatomical considerations. IR plays a central role in definitive management, with TIPS providing global portal decompression and ATO and RTO offering targeted embolization strategies based on variceal anatomy and hemodynamics. Surgical intervention is reserved for refractory cases. Given the heterogeneity of ectopic varices and the complexity of available therapies, close collaboration among gastroenterology, hepatology, IR, and surgery is essential to individualize treatment and optimize outcomes.

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