Ectopic Varices Research Articles

Vesical Varices: An Unusual Cause of Hematuria

AbstractPortal hypertension is a common complication of cirrhosis and may lead to the formation of venous collateral channels. Usually, varices due to portal hypertension develop in the lower esophagus, stomach, rectum, or other parts of the digestive tract. Extraintestinal ectopic varices are rare. Here, we present a patient with ectopic urinary bladder varices, who presented with hematuria and symptomatic anemia. The bleeding was managed with minimally invasive endovascular approach.

Endoscopic ultrasound-guided vascular interventions: A review (with videos).

Endoscopic ultrasound (EUS) has evolved from a diagnostic to an interventional modality, allowing precise vascular access and therapy. EUS-guided vascular access of the portal vein has received increasing attention in recent years as a diagnostic and therapeutic tool. EUS-guided portal pressure gradient directly measures the hepatic vein portal pressure gradient and is crucial for understanding of liver function and prognostication of liver disease. EUS facilitates the sampling of portal venous blood to obtain circulating tumor cells (CTCs) in pancreatobiliary malignancies. This technique aids in the diagnosis and staging of cancers. EUS-guided interventions have a substantial potential for diagnosing portal vein tumor thrombus (PVTT) in patients with hepatocellular carcinoma. EUS-guided coil and glue embolization have higher efficacy for the treatment of gastric varices than direct endoscopic glue. Pseudoaneurysm (PsA), a rare vascular complication of acute and chronic pancreatitis, is typically managed with interventional radiology (IR)-guided embolization and surgery. EUS is increasingly used in specialized centers for non-variceal gastrointestinal bleeding, particularly for pseudoaneurysm-related bleeding. There is limited data on EUS-guided intervention for bleeding ectopic varices, rectal varices and Dieulafoy lesions, but it is becoming more widely accepted. In this extensive review, we evaluated both current and potential future applications of EUS-guided vascular interventions, including EUS-guided gastric variceal bleed therapy, rectal and ectopic varices, pseudoaneurysmal bleeding, splenic artery embolization, portal pressure gradient measurement, portal vein sampling for CTCs, fine needle aspiration of PVTT, intrahepatic portosystemic shunt placement, liver tumor ablation and EUS-guided cardiac intervention.

Transjugular Intrahepatic Portosystemic Shunt (TIPS) for Treatment of Bleeding from Cardiofundal and Ectopic Varices in Cirrhosis

Acute variceal bleeding in cirrhosis represents a critical clinical event that significantly impacts patient prognosis, with mortality rates increasing further after a second episode. This underscores the need for immediate intervention and optimal prophylaxis. The creation of a transjugular intrahepatic portosystemic shunt (TIPS) has been proven to be highly effective for managing esophageal variceal bleeding. However, the use of TIPS for managing cardiofundal gastric varices and ectopic varices remains debated due to their unique vascular anatomy and the limited data available. These varices, although less prevalent than esophageal varices, are complex and heterogeneous vascular shunts between the splanchnic venous system and the systemic veins. Indeed, while endoscopic therapy with tissue adhesives is widely endorsed for achieving hemostasis in active hemorrhage, there is no consensus regarding the optimal approach for secondary prophylaxis. Recent research emphasizes the efficacy of endovascular techniques over endoscopic treatments, such as TIPS and endovascular variceal embolization techniques. This review examines the use of TIPS in managing acute variceal bleeding in patients with cirrhosis, focusing specifically on cardiofundal gastric varices and ectopic varices, discussing optimal patient care based on the latest evidence, aiming to improve outcomes for this challenging subset of patients.

Never say never: a novel approach to tackle a bleeding ectopic varix

Never say never: a novel approach to tackle a bleeding ectopic varix

Embolization of Bleeding Ileal Varices post Adnexal Biopsy in Liver Cirrhosis without Gastroesophageal Varices

AbstractBleeding ileal varices are rare in liver cirrhosis, where the gastroesophageal varices frequently accompany ectopic varices. Small bowel varices usually develop in patients with a history of abdominal surgery. We report a case of bleeding ileal varices without accompanying gastroesophageal varices, managed by percutaneous embolization, in a case of liver cirrhosis with no history of laparotomy. Computed tomography–guided percutaneous adnexal biopsy had been undertaken a year before the onset of bleeding.

Interventional Management of Variceal Bleeding: Techniques and Emerging Concepts

AbstractDespite technical advancements and disease understanding, variceal bleeding remains the leading cause of mortality in patients with cirrhosis. Endoscopic therapies are the main cornerstone of therapy in variceal bleeding. Interventional radiology (IR) plays a significant role in managing variceal bleeding, especially in cases where endoscopic therapies are not feasible or failed. Cross-sectional imaging is often critical to identify relevant anatomy before IR therapies. Transjugular intrahepatic portosystemic shunt (TIPS) is indicated as salvage therapy in patients with refractory variceal bleeding. Retrograde transvenous variceal embolization (RTO) procedures provide superior bleeding control in patients with gastric and ectopic varices, however, without increasing the risk of hepatic encephalopathy and liver function deterioration. Antegrade transvenous obliteration is a viable alternative when RTO is not feasible. Left-sided portal hypertension is a distinct entity resulting from splenic vein stenosis or occlusion, and variceal bleeding in left-sided portal hypertension does not respond to TIPS, requiring variceal embolization, partial splenic embolization, or splenic vein recanalization. Occasionally, endovascular splenorenal or mesorenal shunt and splenic vein occlusion with distal splenorenal diversion are performed to control variceal bleeding. This article entails the basic concepts and procedural aspects of various interventional radiological procedures performed in patients with variceal bleeding.

EUS-Guided Vascular Interventions: Recent Advances.

Endoscopic ultrasound (EUS)-guided vascular interventions were first reported in 2000 in a study that evaluated the utility of EUS in sclerotherapy of esophageal varices. Currently, gastric variceal therapy and portosystemic pressure gradient (PPG) measurements are the most widely utilized applications. Ectopic variceal obliteration, splenic artery embolization, aneurysm/pseudoaneurysm treatment, portal venous sampling, and portosystemic shunt creation using EUS are some of the other emerging interventions. Since the release of the American Gastroenterological Association (AGA)'s commentary in 2023, which primarily endorses EUS-guided gastric variceal therapy and EUS-PPG measurement, several new studies have been published supporting the use of EUS for various vascular conditions. In this review, we present the recent advances in this field, critically appraising new studies and trials.

Interventional Radiology for Bleeding Ectopic Varices: Individualized Approach Based on Vascular Anatomy.

Ectopic varices are rare but potentially life-threatening conditions usually resulting from a combination of global portal hypertension and local occlusive components. As imaging, innovative devices, and interventional radiologic techniques evolve and are more widely adopted, interventional radiology is becoming essential in the management of ectopic varices. The interventional radiologist starts by diagnosing the underlying causes of portal hypertension and evaluating the afferent and efferent veins of ectopic varices with CT. If decompensated portal hypertension is causing ectopic varices, placement of a transjugular intrahepatic portosystemic shunt is considered the first-line treatment, although this treatment alone may not be effective in managing ectopic variceal bleeding because it may not sufficiently resolve focal mesenteric venous obstruction causing ectopic varices. Therefore, additional variceal embolization should be considered after placement of a transjugular intrahepatic portosystemic shunt. Retrograde transvenous obliteration can serve as a definitive treatment when the efferent vein connected to the systemic vein is accessible. Antegrade transvenous obliteration is a vital component of interventional radiologic management of ectopic varices because ectopic varices often exhibit complex anatomy and commonly lack catheterizable portosystemic shunts. Superficial veins of the portal venous system such as recanalized umbilical veins may provide safe access for antegrade transvenous obliteration. Given the absence of consensus and guidelines, a multidisciplinary team approach is essential for the individualized management of ectopic varices. Interventional radiologists must be knowledgeable about the anatomy and hemodynamic characteristics of ectopic varices based on CT images and be prepared to consider appropriate options for each specific situation. ©RSNA, 2024 Supplemental material is available for this article.

Ectopic Variceal Bleed of Duodenum Successfully Treated with TIPS and 2 Years Follow-Up

Ectopic Variceal Bleed of Duodenum Successfully Treated with TIPS and 2 Years Follow-Up

Embolization with or without portal vein stenting for bleeding ectopic jejunal varices in hepatopetal portal collaterals due to extrahepatic portal vein occlusion or stenosis after hepatobiliary and pancreatic surgery

PurposeTo evaluate the efficacy and safety of embolization with or without portal vein stenting for bleeding ectopic jejunal varices in the hepatopetal portal collateral due to extrahepatic portal vein occlusion or stenosis after hepatobiliary and pancreatic surgery.Materials and methodsThis study included consecutive patients who underwent embolization for bleeding ectopic jejunal varices in the hepatopetal collateral due to extrahepatic portal vein occlusion or stenosis after hepatobiliary and pancreatic surgery between September 2012 and December 2020. The safety, technical and clinical success rates (no re-bleeding within 1 month) and re-bleeding-free survival after the first therapy and overall survival were assessed.ResultsFourteen sessions in 11 patients were included. Four patients (7 sessions) underwent variceal embolization only, and the remaining seven patients (7 sessions) underwent portal vein stenting and variceal embolization. Technical success was achieved in all 14 sessions (100%). Clinical success was achieved in 13 of 14 sessions (92.9%). No treatment-related serious complications including liver failure were observed. One-year and 2-year re-bleeding-free survival rate after the first endovascular therapy in all 11 patients was 90.9 and 60.6%, respectively. Two patients who experienced re-bleeding had repeat embolization treatment. There was no significant difference in re-bleeding-free survival after endovascular therapy between the combination with stenting and embolization group and the embolization-only group (p = 0.13).ConclusionEmbolization with or without portal vein stenting of bleeding ectopic jejunal varices in the hepatopetal portal collateral due to extrahepatic portal vein occlusion or stenosis after hepatobiliary and pancreatic surgery can be considered a safe, effective, and repeatable therapy for long-term hemostasis of uncontrollable bleeding.

Beta-blockers or Placebo for Primary Prophylaxis (BOPPP) of oesophageal varices: study protocol for a randomised controlled trial

BackgroundLiver disease is within the top five causes of premature death in adults. Deaths caused by complications of cirrhosis continue to rise, whilst deaths related to other non-liver disease areas are declining. Portal hypertension is the primary sequelae of cirrhosis and is associated with the development of variceal haemorrhage, ascites, hepatic encephalopathy and infection, collectively termed hepatic decompensation, which leads to hospitalisation and mortality. It remains uncertain whether administering a non-selective beta-blocker (NSBB), specifically carvedilol, at an earlier stage, i.e. when oesophageal varices are small, can prevent VH and reduce all-cause decompensation (ACD).Methods/designThe BOPPP trial is a pragmatic, multicentre, placebo-controlled, triple-blinded, randomised controlled trial (RCT) in England, Scotland, Wales and Northern Ireland. Patients aged 18 years or older with cirrhosis and small oesophageal varices that have never bled will be recruited, subject to exclusion criteria. The trial aims to enrol 740 patients across 55 hospitals in the UK. Patients are allocated randomly on a 1:1 ratio to receive either carvedilol 6.25 mg (a NSBB) or a matched placebo, once or twice daily, for 36 months, to attain adequate power to determine the effectiveness of carvedilol in preventing or reducing ACD.The primary outcome is the time to first decompensating event. It is a composite primary outcome made up of variceal haemorrhage (VH, new or worsening ascites, new or worsening hepatic encephalopathy (HE), spontaneous bacterial peritonitis (SBP), hepatorenal syndrome, an increase in Child–Pugh grade by 1 grade or MELD score by 5 points, and liver-related mortality. Secondary outcomes include progression to medium or large oesophageal varices, development of gastric, duodenal, or ectopic varices, participant quality of life, healthcare costs and transplant-free survival.DiscussionThe BOPPP trial aims to investigate the clinical and cost-effectiveness of carvedilol in patients with cirrhosis and small oesophageal varices to determine whether this non-selective beta-blocker can prevent or reduce hepatic decompensation. There is clinical equipoise on whether intervening in cirrhosis, at an earlier stage of portal hypertension, with NSBB therapy is beneficial. Should the trial yield a positive result, we anticipate that the administration and use of carvedilol will become widespread with pathways developed to standardise the administration of the medication in primary care.Ethics and disseminationThe trial has been approved by the National Health Service (NHS) Research Ethics Committee (REC) (reference number: 19/YH/0015). The results of the trial will be submitted for publication in a peer-reviewed scientific journal. Participants will be informed of the results via the BOPPP website (www.boppp-trial.org) and partners in the British Liver Trust (BLT) organisation.Trial registrationEUDRACT reference number: 2018–002509-78.ISRCTN reference number: ISRCTN10324656. Registered on April 24 2019.

New sights in ectopic varices in portal hypertension.

Ectopic varices and associated bleeding, although rare, pose a significant risk to patients with portal hypertension, carrying a relatively high mortality rate. These varices can occur in various anatomical regions, excluding the gastroesophageal region, which is typically associated with portal vein drainage. The limited data available in the literature, derived mostly from case reports and series, make the diagnosis and treatment of ectopic variceal bleeding particularly challenging. Furthermore, it is crucial to recognize that ectopic varices in different sites can exhibit variations in key decision-making factors such as aetiology and vascular anatomy, severity and bleeding risk and hepatic reserve. These factors significantly influence treatment strategies and underscore the importance of adopting individualized management approaches. Therefore, the objective of this review is to provide a comprehensive overview of the fundamental knowledge surrounding ectopic varices and to propose site-oriented, stepwise diagnosis and treatment algorithms for this complex clinical issue. A multidisciplinary treatment approach is strongly recommended in managing ectopic varices. In addition, to enhance clinical reference, we have included typical case reports of ectopic varices in various sites in our review, while being mindful of potential publication bias.

Ectopic biliary varices as a cause of portal biliopathy and mechanical jaundice syndrome

The authors consider ectopic biliary varices as a possible cause of portal biliopathy in extrahepatic portal hypertension. The main diagnostic methods including ultrasound CT, MRI, endoscopic ultrasonography, cholangioscopy, difficulties of differential diagnosis and clinical manifestations of portal biliopathy are presented. Various treatment options including portosystemic shunting and endoscopic biliary decompression are discussed. The authors emphasize the role of differentiated treatment depending on bile duct lesions. The study not only reviews this problem, but also highlights the issues requiring further analysis for selection of optimal diagnostic and treatment methods.

Bleeding Ectopic Varices at Hepaticojejunostomy site after Portal Vein Reconstruction managed by Emergency Proximal Splenorenal Shunt – A case report

Bleeding Ectopic Varices at Hepaticojejunostomy site after Portal Vein Reconstruction managed by Emergency Proximal Splenorenal Shunt – A case report

Ectopic varices: anatomical features for surgeons and interventional radiologists

Ectopic varices are defined as dilated portosystemic collateral veins located in unusual sites, other than the gastroesophageal region. They develop secondary to portal hypertension, surgical procedures, anomalies in venous outflow, or abdominal vascular thrombosis and may be familial. Ectopic varices represent a clinical challenge because they are difficult to localize. Missing or misinterpreting these lesions can have serious consequences, and treatment options are unclear. Ectopic varices may be detected during panendoscopy, enteroscopy, endoscopic ultrasound, wireless capsule endoscopy, diagnostic angiography, multislice helical computed tomography, magnetic resonance angiography, color Doppler flow imaging, laparotomy, laparoscopy and occasionally during autopsy. They can be an important cause of bleeding and hepatic encephalopathy, so radiologists must effectively identify them to assist in making therapeutic decisions. Knowledge of the anatomy and course of these unusual portosystemic collaterals is also important for interventional radiologists and surgeons as it helps avoid inadvertent vascular damage during invasive procedures. In this article, we explore the parts of the gastrointestinal tract and organs that may be involved in ectopic varices. Literature search was conducted in the MedLine database on the PubMed platform.

Combination of transjugular intrahepatic portosystemic shunt and antegrade through-the-TIPS coil embolization for bleeding mixed-type ectopic ileal varices

A 61-year-old man with alcoholic cirrhosis and a history of severe cholecystitis leading to secondary thrombosis of the recanalized paraumbilical vein was admitted to our hospital for recurrent gastrointestinal bleeding and severe anemia. Capsule endoscopy and CT angiography detected profuse bleeding in the proximal ileum from ectopic ileal varices. Hepatic venous-portal gradient (HVPG) measurement was consistent with severe portal hypertension. Persistent bleeding despite transjugular intrahepatic portosystemic shunt (TIPS) placement required a combined approach with antegrade through-the-TIPS coil embolization of the ileal varices.

An Audit of Extrahepatic Portal Vein Obstruction: Experience from Tertiary Referral Center.

Extrahepatic portal vein obstruction (EHPVO) is a common cause of portal hypertension in India. (1) To evaluate the clinical presentation and the natural history of EHPVO; (2) to describe the risk factors, rebleeding rates and development of portal biliopathy on follow-up; and requirement of surgery in EHPVO at a tertiary care center. Data from 318 consecutive patients with EHPVO from June 2012 to October 2020 were analyzed. All patients underwent liver biochemistry, ultrasonography (USG) abdomen, upper gastrointestinal (GI) endoscopy, and viral serology. Color Doppler, computed tomography (CT) abdomen and magnetic resonance cholangiopancreatography (MRCP) were done as indicated. Mean age of presentation was 15.08 years [standard deviation (SD) 12.74; 6 months-60 years; 210 males)]. The presenting features were upper GI bleed (n = 227) (age at first bleed 11 years; 4 months-56 years), left hypochondrium pain or lump (n = 67), and only lower GI bleed (n = 1). Incidentally detected EHPVO on USG was seen in 10.69% (n = 34) patients. Postbleed ascites were seen in 10.69% (n = 34) patients. Six patients had symptomatic portal biliopathy and 14 had hypersplenism. Around 14.77% (n = 47) of patients had a history of being delivered at home, while 3.45% (n = 11) had a history of umbilical sepsis. During follow-up, 35.3% (n = 82) of patients had rebled. On imaging, associated splenic vein (SV) collaterals and superior mesenteric vein (SMV) collaterals were seen in 35.84% (n = 114) and 11.01% (n = 35) patients, respectively. Gallbladder varices were seen in 44.3% (n = 106), while gallstones in 5.66% (n = 18). On endoscopy, 87.42% (n = 278) patients had esophageal varices, 18.86% (n = 60) had isolated fundic varices, and three had ectopic varices. Only two patients had rectal varices and colopathy. Emergency devascularization was required in 3.45% (n = 14) patients for the failure of variceal bleed control, 1.88% (n = 7) underwent splenectomy, and four patients had proximal splenorenal shunt (PSRS) surgery. Extrahepatic portal hypertension (EHPVO) is an important cause of portal hypertension (PHT) in our country. The majority of them present with GI bleed; postbleed ascites were seen only in ~10%. Rebleed occurs in one-third of cases. Gallbladder varices were common; portal biliopathy occurred in 10% and were usually asymptomatic.

Portosystemic Shunt Surgery for Extrahepatic Portal Venous Obstruction Beyond Endoscopic Variceal Eradication: Two Decades of Pediatric Surgical Experience

This exclusively surgical series on pediatric extrahepatic portal venous obstruction (EHPVO) defines surgical indications beyond endoscopic eradication of esophageal varices (EEEV), the selection of an appropriate surgical procedure, and the long-term post-surgical outcome. EHPVO management protocol at the reporting institute has been endotherapy untilesophageal variceal eradication and surgery for select adverse sequelae manifesting after EEEV. One hundred and thirty-nine EHPVO cases underwent surgery for the following indications in combination: i) massive splenomegaly with severe hypersplenism (n=132, 95%); ii) growth retardation (GR, n=95, 68%); iii) isolated gastric (IGV) and ectopic varices (n=49, 35%); iv) Portal cavernoma cholangiopathy (PCC) (n=07, 5%). A portosystemic shunt (PSS) was performed in 119 (86%) cases. Types of PSS performed were as follows: central end-to-side splenorenal shunt with splenectomy (n=104); side-to-side splenorenal shunt (n=4); mesocaval shunt (n=1); inferior mesenteric vein (IMV) to left renal vein shunt (n=2); IMV to inferior vena cava shunt (n=3); H-graft interposition splenorenal shunt (n=1); spleno-adrenal shunt (n=3); makeshift shunt (n=1). Esophagogastric devascularization (n=20, 14%) was opted for only for non-shuntable anatomy. At a median follow-up (FU) of 41 (range: 6-228) months, PSS block was detected in 13 (11%) cases, with recurrent variceal bleeding in 4 cases. PCC-related cholestasis regressed in 5 of 7 cases. Issues of splenomegaly were resolved, and growth z-scores improved significantly. Endotherapy for secondary prophylaxis untilEEEV has resulted in a shift in surgical indications for EHPVO. Beyond EEEV, surgery was indicated predominantly for non-variceal sequelae, namely massive splenomegaly with severe hypersplenism, GR, and PCC. Varices warranted surgery infrequently butmore often from sites less amenable to endotherapy, i.e., IGV and ectopic varices. The selection of PSS was tailored to anatomy and surgical indications. On long-term FU post surgery, PSS block was detected in 13% of patients. PCC-related cholestasis regressed in 71%, and issues of splenomegaly resolved with significantly improved growth Z scores.

Massive Hemoperitoneum after Paracentesis in a Patient with Budd-Chiari Syndrome: Treated with a TIPS after Negative Arteriogram.

Paracenteses are considered safe procedures; however, in patients with portal hypertension, the rapid shifts in intraabdominal pressure can prompt hemorrhage from an ectopic varix. Little literature exists on the appropriate management in this clinical setting. Here, we describe a patient with portal hypertension secondary to Budd-Chiari syndrome, presenting with massive hemoperitoneum following paracentesis. Angiography was performed, without revealing an arterial source of bleeding. Subsequently, transjugular intrahepatic portosystemic shunt placement was performed via a recanalized middle hepatic vein, reducing the patient's portosystemic gradient from 15 to 6 mm Hg. This patient developed no further signs or symptoms of bleeding and remained hemodynamically stable until discharge. Follow-up imaging confirmed patency of her shunt and resolution of her ascites, without the need for future paracentesis. This case highlights that in the absence of arterial extravasation, the possibility of ectopic variceal hemorrhage should be considered, which can be successfully treated with portosystemic shunt creation.

Incidental Detection of Ectopic Ovary Varices by 99mTc-Red Blood Cell Bleeding Scintigraphy in a Patient With Cirrhosis.

A 72-year-old woman with cirrhosis underwent a 99mTc-labeled red blood cell (RBC) bleeding scan because of recurrent gastrointestinal bleeding. The images showed abnormal 99mTc-RBC accumulation in a mass in the region of the left ovary. Contrast-enhanced CT showed expansion of left ovary vein from the left renal vein, which twisted into an enhanced round mass in the left ovary area. Finally, a diagnosis of ectopic ovary varices was made, which is secondary to cirrhosis with portal hypertension. Ovary varices should be included as differential diagnoses of 99mTc-RBC bleeding scintigraphy.