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Gastrointest Interv 2012; 1:3–10
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Gastrointestinal Intervention
journal homepage: www.gi-intervention.org
Review Article
Diagnosis and management of ectopic varices
Nabeel M. Akhter, Ziv J. Haskal*
a b s t r a c t
Ectopic varices are large portosystemic collaterals in locations other than the gastroesophageal region. They account for up to 5% of all variceal bleeding;
however, hemorrhage can be massive with mortality reaching up to 40%. Given their sporadic nature, literature is limited to case reports, small case series and
reviews, without guidelines on management. As the source of bleeding can be obscure, the physician managing such a patient needs to establish diagnosis
early. Multislice computed tomography with contrast and reformatted images is a rapid and validated modality in establishing diagnosis. Further
management is dictated by location, underlying cause of ectopic varices and available expertise. Therapeutic options may include double balloon enteroscopy,
transcatheter embolization or sclerotherapy, with or without portosystemic decompression, i.e., transjugular intrahepatic portosystemic shunts. In this
article we review the prevalence, etiopathogenesis, anatomy, presentation, and diagnosis of ectopic varices with emphasis on recent advances in
Copyright Ó 2012, Society of Gastrointestinal Intervention. Published by Elsevier. All rights reserved.
Keywords: Balloon-occluded retrograde transvenous obliteration, Ectopic varices, Portal hypertension, Percutaneous embolization,
Transjugular intrahepatic portosystemic shunt
The term “ectopic varices” has historically been used to describe
abnormally dilated veins associated with gastrointestinal mucosa
with propensity for gastrointestinal hemorrhage, and it has also been
loosely used for portosystemic collaterals in the abdominal wall and
retroperitoneum. Ectopic varices may be best defined as large pressurized portosystemic venous collaterals occurring anywhere in the
abdomen except in the gastroesophageal region.1,2
Ectopic varices account for up to 5% of all variceal bleeding.3
They may be present in duodenum, jejunum, ileum, colon, anorectum, peristomal, biliary, peritoneal, retroperitoneal, umbilical,
urinary bladder, uterine, ovaries, and other locations.
In a review of 169 cases of bleeding ectopic varices, 26% bled from
peristomal varices,17% from duodenal,17% from jejunal and ileal, 14%
from colonic, 9% from peritoneal, 8% rectal, and a few from infrequent
sites such as the ovary and vagina.1 A study of 37 patients with liver
cirrhosis who underwent capsule endoscopy, 8.1% were found to
have small bowel varices.4 One 1968 angiographic study of patients
with portal hypertension demonstrated an unusually high rate of
paraduodenal varices, in 46 out of 106 (40%) patients; this likely
described all regional venous collaterals, and not solely submucosal
duodenal varices.5 Anorectal varices have been reported in approximately 44% of patients with cirrhosis,6 although only a fraction
become symptomatic. Thus, true incidences are likely subject to
reporting bias and the modality used for diagnosis and only a fraction
of diagnosed ectopic varices are likely to become symptomatic.
Etiology and pathogenesis
Ectopic varices represent natural portosystemic shunts
secondary to portal hypertension. These collaterals occur where the
portal venous system is in juxtaposition to the systemic venous
system. Under normal circumstances the resistance within these
collaterals is high, while it is low in the portal venous system. With
the development of intrahepatic portal hypertension, these shunts
act to divert flow from the increased intrahepatic vascular resistance. Surgical literature and cadaveric studies have shown portosystemic communications via (a) gastroesophageal plexus to
azygous-coronary system, (b) hemorrhoidal plexus, (c) recanalized
umbilical vein, and (d) pancreatoduodenal venous arcade to inferior
vena cava through retroperitoneal veins of Retzius. Most reported
cases of hemorrhage from ectopic varices in the West are associated
with intrahepatic portal hypertension in contrast to extrahepatic
portal hypertension, because of low prevalence of the latter. Prior
abdominal surgery predisposes patients with portal hypertension to
develop varices in unusual locations, like urinary bladder, ovaries
University of Maryland School of Medicine, Baltimore, MD, United States
Received 14 July 2012; Revised 14 July 2012; Accepted 31 July 2012
* Corresponding author. Division of Vascular and Interventional Radiology, University of Maryland School of Medicine, 22 South Greene Street, Baltimore, MD 21201, United
E-mail address: [email protected] (Z.J. Haskal).
2213-1795/$ – see front matter Copyright Ó 2012, Society of Gastrointestinal Intervention. Published by Elsevier. All rights reserved.
Gastrointestinal Intervention 2012 1(1), 3–10
and bare area of the liver due to adhesions.1,2,7 Finally, ectopic varices
can develop in the absence of portal hypertension due to congenital
anomalous portosystemic anastomoses,8 abnormal vessel structures,9 arteriovenous fistulae,10 rare familial conditions,11 or related
to thromboses.12
Wall tension in all varices is a recognized risk factor for rupture.
Per LaPlace’s law it is proportional to transmural pressure across
the vessel wall and the radius of the vessel. Hence, portal pressure
and vessel size are determinants of ectopic variceal hemorrhage.13
In our and others’ experience ectopic varices, like gastric varices
may bleed at a portosystemic gradient of less than 12 mmHg.14,15
emphasizing the need for different transcatheter treatments then
those used for bleeding esophageal varices.
Presentation and diagnosis
Ectopic varices present in a multitude of ways. For example,
duodenal varices may manifest with mild or massive hematemesis
or lower gastrointestinal bleeding.16 Intestinal varices distal to
duodenum, usually present with hematochezia, melena, or intraperitoneal bleeding.17–19 Urinary bladder varices may present with
hematuria,20 and biliary varices with biliary obstruction,21 or rarely
hemobilia and gastrointestinal bleeding.22 Ruptured varices at the
right diaphragm can cause hemothoraces and dyspnea.23 Abdominal wall varices (e.g., umbilical) can rupture externally or internally, whereas those located around the falciform ligament,
diaphragm, splenic ligament or in the rectovesical region may
rupture into the peritoneal cavity, leading to occult, potentially fatal
intraperitoneal hemorrhage.19,23,24
Brisk upper gastrointestinal bleeding and hematochezia should
be first evaluated with emergency upper gastrointestinal endoscopy. If this fails to define an acute source of hemorrhage, intravenous contrast enhanced computed tomography (CT) may be
preferable to colonoscopy (in the unprepped acute setting). Thus,
presence of ectopic varices should be strongly considered in
patients with known liver disease or stigmata of portal hypertension, particularly when both upper and lower endoscopies fail to
identify a source of bleeding.2 Elective capsule endoscopy has been
successful in detecting jejunal and small bowel varices,4,25 although
its role in acute bleeding is small. Similarly, double balloon
enteroscopy or push enteroscopy has the potential to visualize the
greater small bowel and allow intervention.3,26 Endoscopic ultrasound has been reported for the hemodynamic assessment of
rectal27 and biliary varices.28
Color Doppler ultrasound has shown its value in the diagnosis of
umbilical (Fig. 1A and D), duodenal,29 rectal,30 gall bladder,21 and
choledochal varices.31 However, intravenous contrast enhanced
multislice CT (Fig. 2A and B), should be considered the primary
modality for diagnosis of ectopic varices, given the rapid and
ubiquitous availability. CT, CT angiography, and CT enteroclysis have
all been used for successful diagnosis of duodenal32,33 and colonic
varices.34 While technetium TC-99m red blood cell scintigraphy has
Fig. 1. A 53-year-old female with history of alcoholic cirrhosis transferred from outside the hospital with external bleeding around the umbilicus from cutaneous varices. (A)
Preprocedure color Doppler ultrasound in periumbilical region shows flow in varices (white arrow); (B) digital subtraction image DSA from the transjugular intrahepatic portosystemic shunt (TIPS) procedure during the initial portogram shows the left portal vein (white arrow), with retrograde flow in the recanalized umbilical vein (black arrows), leading
to large periumbilical varices (lowest black arrow). Initial portosystemic gradient: 21 mmHg; (C) portogram after TIPS and embolization of umbilical vein varix with liquid embolic
agent (Onyx, ev3 Endovascular, Inc. Plymouth, MN. USA), shows antegrade flow without retrograde filling of umbilical vein. Post TIPS portosystemic gradient: 15 mmHg; (D) postTIPS and embolization ultrasound of periumbilical region shows thrombus in the periumbilical varices (white arrows).
Nabeel M. Akhter and Ziv J. Haskal / Diagnosis and management of ectopic varices
Fig. 2. A 54-year-old male with history of alcoholic cirrhosis presented with massive lower gastrointestinal bleeding, and was hypotensive with systolic blood pressure of 50 mmHg.
(A) Axial image from contrast-enhanced computed tomography (CT) shows a large superior mesenteric vein (white arrow head), cecal and ascending colon varices (white arrow);
(B) coronal reformatted image from contrast enhanced CT shows cecal and ascending colon varices (white arrow), large right pleural effusion representing hepatic hydrothorax
(asterisk); (C) DSA from the transjugular intrahepatic portosystemic shunt (TIPS) procedure with catheter in peripheral superior mesenteric vein shows retrograde flow in cecal and
ascending colon varices (black arrows) with portosystemic shunt through retroperitoneal veins draining into inferior vena cava (white arrow). Initial portosystemic gradient:
12 mmHg; (D) image during embolization of cecal varices with multiple coils and liquid embolic agent (Onyx), balloon inflated (black arrow) in peripheral superior mesenteric vein
to promote thrombosis; (E) DSA after TIPS showing antegrade flow in portal vein and non filling of cecal varices. Post TIPS portosystemic gradient: 7 mmHg. After the procedure
patient became hemodynamically stable and was extubated 2 days later, with no more episodes of hematochezia.
been used,35 its modern role in this setting is appropriately
magnetic resonance (MR) angiography36 can equally outline
unusual portosystemic collaterals, but has little role in the acute
setting. Of note, barium studies of the colon or small bowel may
misdiagnose ectopic varices as polyps or tumors,11 emphasizing the
importance of tomographic imaging and clinical suspicion.
Specific anatomic locations
Duodenal varices
Cirrhosis is the most commonly associated cause of duodenal
varices, accounting for 30% of the cases,37 but causes vary and
include portal venous thrombosis and obstruction of the splenic
vein and inferior vena cava.38,39 Duodenal varices have also
developed after band ligation of esophageal varices, presumably as
alternate sites of spontaneous portosystemic shunting.40 These
varices are formed by the collateral veins originating from the
portal vein trunk or superior mesenteric vein draining into the
inferior vena cava through retroperitoneal veins of Retzius.41
Duodenal variceal rupture can lead to severe hemorrhage, with
mortality as high as 40% from initial bleeding.42
Jejunal, ileal, colonic, and anorectal varices
Small bowel varices are commonly seen in patients with intrahepatic portal hypertension who have previously undergone
abdominal surgery. Adhesions bring the parietal surface of the
viscera in contact with the abdominal wall, and portal hypertension
results in formation of varices below the intestinal mucosa.7 A triad
of portal hypertension, hematochezia without hematemesis and
prior abdominal surgery characterize hemorrhage from small
intestinal varices.43 These varices most commonly flow into
systemic circulation through the gonadal veins, and less commonly
through branches of the internal iliac veins.
Rectal varices are distinguished from hemorrhoids by their
typical presence above the dentate line. The differentiation of
hemorrhoids and anorectal varices is an important one. Anal varices collapse with digital pressure, whereas hemorrhoids do not; of
note, the prevalence of hemorrhoids in patients with liver disease is
not higher than in the general population.6 These varices represent
a portosystemic collateral pathway between the superior rectal
veins of the inferior mesenteric system to the middle and inferior
rectal veins of the iliac system (Fig. 3). They can be present with
mild or uncontrolled hematochezia.44 The most common colonic
sites are the cecum (Fig. 2C) and rectum,45 although isolated
colonic varices, in the authors experience, rank among the rarest.
Gastrointestinal Intervention 2012 1(1), 3–10
Stomal varices
Ectopic stomal varices refer to abnormally dilated veins that have
developed in the stomal mucosa, often recognized by a purplish hue
around the stoma.2 They are common in patients with intrahepatic
portal hypertension secondary to primary sclerosing cholangitis,7
and are frequently seen in patients with ileostomies after proctocolectomy for inflammatory bowel disease associated with primary
sclerosing cholangitis (Fig. 4A).46 The mechanism of stomal variceal
hemorrhage is related to local trauma and variceal erosion.18 The
overall morbidity is high, given the propensity for recurrent
bleeding requiring multiple blood transfusions but mortality is low,
between 3% and 4%,47 because of the ability to institute local
measures such as pressure dressing, epinephrine soaked gauze, gel
foam packing, and suture ligation. These therapies are however, not
effective in preventing recurrent bleeding.48 Importantly, stomal
varices can be missed by endoscopy, emphasizing the need for
clinical suspicion and CT imaging in occult cases.
Biliary tract varices
Fig. 3. A 49-year-old female with history of primary biliary cirrhosis presented with
upper gastrointestinal hemorrhage. Repeated endoscopy and banding was unable to
stop hemorrhage. Balloon tamponade was done with an orogastric tube. DSA during
the transjugular intrahepatic portosystemic shunt (TIPS) procedure with the catheter
in the inferior mesenteric vein (black arrows) shows retrograde flow, with filling of
hemorrhoidal plexus and anorectal varices (white arrow). Initial portosystemic
gradient: 18 mmHg and post TIPS gradient: 6 mmHg. Post TIPS there was no retrograde
flow in inferior mesenteric vein and anorectal varices. The upper gastrointestinal
hemorrhage ceased, and the balloon tamponade tube was deflated at end of procedure.
The patient was discharged from hospital after 5 days.
In extrahepatic portal vein thrombosis, gall bladder and common
bile duct varices can occur in as many as 30% of patients.21 They
maybe an incidental finding or present with biliary obstruction
secondary to mass effect on extrahepatic bile ducts.31 The appearance of biliary tree, by direct endoscopic retrograde cholangiopancreatography may mimic that of primary sclerosing
cholangitis, and is termed as pseudosclerosing cholangitis.21 Rarely,
biliary varices can lead to hemobilia or even catastrophic
Vesical varices and other unusual sites
The urinary bladder wall is an unusual collateral route for
venous splanchnic blood flow and these varices may develop after
abdominal surgery secondary to adhesions.7 Bleeding from vesical
varices is a rare event in patients with portal hypertension and an
Fig. 4. Middle-aged male with uncontrolled bleeding from a recent ileostomy, with no source detected on endoscopy. (A) DSA during the transjugular intrahepatic portosystemic
shunt (TIPS) procedure with the catheter in the peripheral superior mesenteric vein shows stomal varices (white arrow), a faint outline of the ileostomy bag is seen (black arrows);
(B) post coil embolization (white arrows) of stomal varices, with the catheter in the superior mesenteric vein (black arrows), shows non-filling of stomal varices. The TIPS resulted in
cessation of further bleeding from the stoma.
Nabeel M. Akhter and Ziv J. Haskal / Diagnosis and management of ectopic varices
unusual cause of hematuria20; however, due to intraperitoneal
hemorrhage, this can be catastrophic.19
Other more unusual sites include right diaphragm,23 splenorenal ligament,24 splenoparametrial,49 adnexal,50 vaginal,51 umbilical
(Fig. 1),52 and cutaneous varices53 leading to unusual manifestations like pleural effusion, dyspnea, hematuria, and percutaneous
There are no randomized trials or set guidelines that dictate the
management of bleeding ectopic varices. The management
depends upon location of hemorrhage, presentation, local physician expertise, and the underlying causes of portal hypertension. A
multidisciplinary team approach is in order, with input from
intensivists, gastroenterologists, interventional radiologists, and
surgeons. The medical management of variceal hemorrhage is
discussed in other texts and we will confine our discussion to
interventional management.
Endoscopic interventional procedures
Many ectopic varices are within reach of standard endoscopy,7
and, with the advent of double balloon or push enteroscopy, the
reach has extended even further.26 The options include endoscopic
band ligation (EBL) and endoscopic injection sclerotherapy (EIS)
with different agents including N-butyl-2-cyanoacrylate (NBCA),
thrombin or other sclerosants. EBL was found to be superior to EIS
for the treatment of esophageal varices, on the basis of lower
rebleeding rate, mortality, complications, speed of application, and
the need for fewer endoscopic treatments; however, the main
disadvantage remains the observed higher rate of variceal recurrence in comparison to sclerotherapy. Sclerotherapy on the other
hand may lead to deep ulceration, which can lead to rebleeding,
stricture formation, or perforation.54,55 Combination of two in the
form of endoscopic scleroligation (ESL), has shown to be superior
with decreased recurrence rates.56 No set protocol exists for the use
of above mentioned modalities for the management of ectopic
varices and the choice is often situation and expertise dependent.
Endoscopic band ligation and sclerotherapy have been
successfully used solo or in combination with each other and other
interventional radiological modalities in controlling hemorrhage
from duodenal,57,58 jejunal,26 colonic,59 anorectal,60 and stomal
Interventional radiological procedures
Multiple image-guided approaches to management of ectopic
varices have been extensively reported. They include direct
percutaneous access to varices, antegrade and retrograde sclerotherapy and/or embolotherapy, and portosystemic shunts.
Transjugular and transhepatic as well as retrograde transjugular or
transfemoral venous approaches have been proven useful.
Transjugular intrahepatic portosystemic shunts (TIPS)
Many publications have emphasized the role of TIPS in the
management of bleeding ectopic varices in cirrhotics caused by
intrahepatic portal hypertension.14,42,62 TIPS have proven more
effective in preventing recurrent esophageal variceal rebleeding
than endoscopic therapy.63,64 It has been shown that a >50%
reduction in pressure gradient protected patients from rebleeding
and even a reduction between 25% and 50% was also effective,
with a probability of rebleeding of only 7% and less risk of
encephalopathy and liver failure.65 The portosystemic gradient
thresholds for adequate ectopic varix decompression are not those
oft-quoted for esophageal varices (12 mmHg). As ectopic varices
decompress through varied pathways other then the coronary–
azygous system, procedural endpoints must be individualized. In
the authors’ experience, endoscopically verified selective decompression of esophageal, but not gastric varices can occur at
12 mmHgdthe same phenomenon holds for ectopic varices,
emphasizing the need for routine definitive embolization or
sclerotherapy in cases where TIPS are created. While TIPS provide
a primary intervention or salvage modality in duodenal,42 small
bowel,66 colonic (Fig. 2D and E),66 anorectal (Fig. 3),67 stomal (Fig. 4A
and B),68 urinary,69 umbilical (Fig. 1B and C), and cutaneous70 variceal
hemorrhage, the need for concomitant embolization must be
emphasized. In bleeding ectopic varices, TIPS with embolization was
superior with only two out of seven patients presenting with
rebleeding in comparison to five out of 12 patients that had TIPS only
without initial embolization.62 The decision to use a TIPS-approach
depends upon local expertise, and the severity of underlying liver
disease and comorbidities (e.g., Model of End Stage Liver Disease
(MELD) score, encephalopathy, ischemic liver disease). Finally, shunts
can be reduced or even reversed should portal decompression prove
Balloon occluded retrograde transvenous obliteration (B-RTO)
As opposed to TIPS, which is a shunt creation procedure, BRTO is a shunt occlusion procedure. Further, it uses a retrograde
rather than antegrade approach. This technique has been
employed for control of gastric variceal bleeding, with success
rates reaching 89%,71 without detriment in hepatic function or in
hepatic encephalopathy. However, since increased portal pressure is not addressed by this procedure, there is a risk of
increased pressure and bleeding from varices that have not been
sclerosed and are at other sites, or development of ascites.
Aggravation of esophageal varices is recognized after B-RTO of
gastric varices.72 To forego or salvage bleeding from varices after BRTO, different procedures have been utilized, including TIPS,73 percutaneous obliteration,74 and endoscopic interventions.57 While largely
used for primary prophylaxis of gastric varices in the East, increasing
case reports have described its successful role in treatment of varices
within the duodenum,57,75 small bowel,76 colon,77 stoma,78 and
Percutaneous embolization
Percutaneous embolization has been widely reported using
a transhepatic approach. While, successful embolization rates
reach 80%, up to 65% rebleeding rate within 5 months have been
described.80 The earlier use of this approach largely predates the
routine role of TIPS for other indications. The transhepatic route
may provide more rapid access to portal system for some lessexperienced operators; however, its added risks include transhepatic tract bleeding, recurrent hemorrhage due to lack of secondary
portal decompression, and added contraindications in ascites
patients. Certainly embolization is important for durable control of
varices.81 In cases where decompression is contraindicated, a transjugular approach (without shunt creation) can still provide direct
access to the varices. In some cases, portal hypertension leading to
varices may be wholly due to extrahepatic obstruction, e.g., after
Whipple or similar surgeries. Correction of portal vein stenoses or
thromboses with stents may provide adequate decompression and
restore hepatopetal flow.82 Finally, more recent case reports have
described direct, ultrasound guided abdominal wall,52 or stomal
variceal,83 puncture, and sclerotherapy or coil embolization. Percutaneous embolization by itself or in association with other interventional modalities has been reported for management of hemorrhage
from ectopic varices in duodenum,84 small bowel,25,82 anorectum,85
stoma,47,81,83 mesentery,86 umbilicus,52 and urinary bladder.87
Gastrointestinal Intervention 2012 1(1), 3–10
Surgical interventions
The role of surgery in the modern management of ectopic varices
has naturally lessened, as the operative morbidity and mortality in
bleeding patients with liver disease is high. Surgery may be considered a salvage option in select patients when endoscopic and interventional radiological procedures have failed. Although distal
splenorenal shunts and small diameter portocaval shunts carry less
risk of encephalopathy and hepatic failure,88 than mesocaval or
portocaval shunts, they are rarely performed in acute settings.
Devascularization procedures may have a small role, may not require
long segment resections and can be done in patients with portal
venous thrombosis, but they still require great expertise and carry
high morbidity and mortality rates.89 Other unusual shunts include
double selective shunting for esophagogastric and rectal varices and
utilization of large collateral vessels for nonconventional portosystemic shunt creation in children with portal venous thrombosis.90,91
Surgery has been used for dearterialization of bleeding duodenal
varices,92 enterectomy for bleeding ileal varices,93 resection and
anastomosis for colorectal varices,94 and stapled anoplasty for anorectal varices.95
No randomized studies exist to guide the best management of
ectopic varices; the bulk of knowledge comes from case reports and
small retrospective case series. It is clear that many approaches have
value in each specific case. Regardless of the approach, it is important to emphasize that complete obliteration and embolization of
the target varix (as with BRTO) is preferable for preventing recurrent
symptoms over focal embolization of feeding veins, which can allow
new collateral channels to develop.56,62,96,97 The need for portal
decompression should be approached on a case-by-case basis,
taking into account liver function, the presence of encephalopathy,
In conclusion, physicians need a high clinical index of suspicion to
make the diagnosis of ectopic varices, and be especially vigilant in
patients with liver disease, as bleeding ectopic varices may be the first
manifestation of underlying portal hypertension. Given the varied
locations and complex manifestations, a multimodality approach is
needed, with input from gastroenterologists, interventional radiologists and surgeons. Early diagnosis is imperative, and if endoscopy
fails to reveal a source, contrast enhanced CT is strongly recommended. Interventional radiological procedures using TIPS and BRTO approaches may offer definitive therapies in combination with
endoscopic approaches.
Conflict of interest
Dr Haskal has disclosed research support from, and scientific
advisory role with WL Gore and Associates, and royalties from Cook
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