Portal hypertension that presents with ectopic small bowel variceal bleeding is unusual and poses challenges in both localization and management.¹ Endoscopic variceal ligation procedures are effective for managing esophageal variceal bleeding; however, they are less effective for small bowel variceal bleeding. In cases of ileal variceal bleeding, interventional procedures such as a transjugular intrahepatic portosystemic shunt (TIPS), with or without locoregional embolization, are effective and offer a viable non-surgical option.²

A 56-year-old woman with cirrhosis due to non-alcoholic steatohepatitis (Model of End Stage Liver Disease score: 14) presented to the emergency department with a 2-day history of hematochezia, hypotension (systolic arterial pressure of 70 mmHg), and anemia (hemoglobin of 6 g/dL). An urgent upper gastrointestinal (GI) endoscopy showed grade 2 esophageal varices without signs of recent or active bleeding, while ileocolonoscopy revealed fresh blood in the distal ileum. After stabilizing the patient, multiphase computed tomography (CT) of the abdomen was performed, which indicated features of chronic liver disease. Ectopic ileal varices in the right lower abdomen were identified (Fig. 1A), with afferent varices communicating with the superior mesenteric veins (Fig. 1B) and an efferent vein draining into the inferior vena cava via gonadal/ovarian veins (Fig. 1C). Based on the endoscopic and CT findings, ectopic ileal varices were determined to be the likely source of bleeding. Confirmatory capsule endoscopy was not performed due to the patient’s hemodynamic instability. The case was discussed at a multidisciplinary team meeting, and it was decided to perform an emergency TIPS procedure and antegrade embolization of the bleeding ileal varices through the created TIPS tract.

Figure 1. (A) Coronal computed tomography (CT) of the abdomen (portovenous phase), showing an ileal varix projecting into the ileal loop in the right lower quadrant (arrow). (B) Curved reformatted CT of the abdomen, showing the afferent of the varix (arrow) originating from the superior mesenteric vein. (C) Curved reformatted contrast-enhanced CT of the abdomen, showing the efferent of the varix draining into the inferior vena cava (arrow) via the gonadal veins.

TIPS was performed via the right jugular venous approach. The middle hepatic vein was cannulated instead of the right hepatic vein, as the latter displayed a reduced caliber and posterior angulation due to atrophy of the right posterior hepatic lobe, complicating its cannulation. Subsequently, the left portal vein was punctured (Fig. 2). Balloon dilation of the tract was followed by the placement of two stents: a 10 mm × 100 mm uncovered stent (E-Luminexx^TM; BD) and a 10 mm × 80 mm stent graft (Fluency^TM stent graft; BD). These were positioned cranially from the hepatic vein-inferior vena cava junction and caudally into the main portal vein (Fig. 3A). The portal pressure decreased from 34 mmHg prior to TIPS to 10 mmHg. A microcatheter was navigated through the TIPS tract to an ectopic ileal varix via the superior mesenteric vein (Fig. 3B), and coil embolization of the ileal varices was performed using a single detachable coil (Interlock-18 Fibered IDC Occlusion System; Boston Scientific Corp.). This was followed by proximal N-butyl cyanoacrylate glue embolization (glue with Lipiodol mixture; 1:3) (Fig. 3C). The patient showed gradual improvement in hemodynamic status over the next 24 hours with resolution of melena. At the 2-month follow-up, the patient was doing well with resolution of ascites, no further episodes of melena, or any significant episodes of hepatic encephalopathy.

Figure 2. (A) Fluoroscopic angiographic image showing cannulation of the middle hepatic vein (arrow). (B) Image showing puncture of the left portal vein (arrow). (C) Image showing the passage of the wire (arrow) from the hepatic vein into the portal vein.

Figure 3. (A) Digital subtraction angiographic image showing a patent transjugular intrahepatic portosystemic shunt stent (arrow) cranially extending into the suprahepatic inferior vena cava and caudally into the portal vein. (B) Digital subtraction angiographic image showing opacification of the ileal varix (arrow) with afferents from the superior mesenteric vein. (C) Image showing (arrow) coil and glue cast within the occluded ileal varix.

The study was approved by the ethical committee of institutional review board (2024/005/EXP/002) and waiver for informed consent was granted.

Ectopic varices are dilated portosystemic collateral veins located at unusual sites other than the gastroesophageal region and account for approximately 1% to 5% of all variceal bleeds.¹ These varices pose a diagnostic challenge due to their difficult localization through routine upper and lower GI endoscopy. Various diagnostic tools are available, including capsule endoscopy, double balloon enteroscopy, Technetium-99m red blood cell scintigraphy, CT angiography, and CT enteroclysis.² Ileal varices are particularly complex and are often associated with a history of abdominal surgery or postoperative adhesions. Typically, they drain into the systemic circulation through the gonadal vein, as observed in our case.³

The management of ectopic variceal bleeding is often challenging due to their location. Endoscopic banding or ligation is recommended for rectal varices or small bowel varices via push enteroscopy, although rebleeding due to collateralization remains a potential issue.⁴ Emergency portocaval shunt surgery or local bowel resection also carries high morbidity and mortality rates in patients with cirrhosis. Percutaneous transhepatic obliteration of small intestinal varices has been described but is associated with risks of intraperitoneal hemorrhage and is technically challenging in the presence of ascites. Retrograde obliteration via systemic veins, such as the gonadal vein, as observed in our case, is another treatment option; however, the marked tortuosity of these veins can sometimes hinder access to the ileal varix.⁵

The role of TIPS in managing bleeding ectopic varices in patients with cirrhosis is well-established and effective in preventing recurrent esophageal variceal rebleeding. Ectopic varices decompress through pathways other than the coronary-azygous system. Various studies have shown that TIPS can serve as a primary intervention or a salvage modality in managing duodenal, small bowel, colonic, rectal, and stomal variceal hemorrhage. However, the importance of concomitant ectopic embolization must be emphasized. In a study by Vangeli et al⁶ on bleeding ectopic varices, TIPS combined with variceal embolization was found to be superior and had a lower rebleeding rate compared to when TIPS was performed alone.

TIPS combined with local embolization is an effective therapy for bleeding ectopic ileal varices.⁶ TIPS targets a reduction in portal pressure (below 12 mmHg), which helps prevent rebleeding through collateralization and also serves as a conduit for local embolization. Using TIPS in conjunction with embolization yields better long-term outcomes compared to using either modality alone. Typically, these ectopic varices have blood flow directed away from the portal vein, and isolated TIPS may not effectively resolve the focal mesenteric venous obstruction that causes ectopic varices.

TIPS is considered the first-line treatment for patients with decompensated portal hypertension experiencing ectopic variceal bleeding. However, it may not adequately address focal mesenteric venous obstruction that causes ectopic varices. In such cases, locoregional variceal embolization should be considered as an alternative treatment option. Various interventional radiological techniques have been described for these scenarios.⁷ Retrograde transvenous obliteration is effective when an accessible efferent or afferent vein is present. Kim et al⁸ described the use of balloon occlusion techniques for retrograde embolization of duodenal varices. Other options include antegrade transvenous obliteration of ectopic varices through the TIPS tract, as demonstrated in our case, and occasionally via recanalized umbilical veins.

In conclusion, in patients with portal hypertension who experience variceal bleeding and have negative esophago-gastro endoscopy results, the possibility of ectopic variceal bleeding should be considered. A multidisciplinary approach is essential for managing these bleeding ectopic varices. Thorough anatomical knowledge and an understanding of hemodynamic characteristics help in the choice of the optimal treatment option.

None.

The data that support the findings of this study are available from the corresponding author upon reasonable request.

No potential conflict of interest relevant to this article was reported.