Refractory ileocolic variceal bleeding in hemorrhagic shock: Endoscopy-negative stigmata, failed TIPS, and successful control with targeted embolization of an ectopic varix

Abstract

Ectopic varices are portosystemic collaterals located outside the gastroesophageal region and represent a rare cause of variceal bleeding. They often pose diagnostic and therapeutic challenges due to their atypical locations and limited accessibility by conventional endoscopy. We report the case of a 68-year-old woman with a history of liver transplantation complicated by cirrhosis who presented with recurrent hematochezia and hemorrhagic shock. Endoscopic evaluation failed to identify the bleeding source. Computed tomography angiography (CTA) revealed varices in the distal superior mesenteric vein (SMV) territory involving the terminal ileum. The patient underwent transjugular intrahepatic portosystemic shunt (TIPS) placement, but bleeding persisted. Subsequent coil embolization of the ileocolic varix successfully controlled the hemorrhage. This case underscores the importance of recognizing ectopic varices as a rare source of gastrointestinal bleeding and highlights that TIPS alone may be insufficient, with adjunctive embolization often required for definitive management.

Introduction

Liver cirrhosis is prevalent in the United States with rates up to 2.6% of the population [1]. The clinical presentation may vary widely depending on the stage of disease. Compensated liver cirrhosis may present with signs of fatigue, malaise, abdominal discomfort, pruritus, and muscle cramps while decompensated disease may present with ascites, variceal hemorrhage, and hepatic encephalopathy [2]. Of these complications, variceal imposes the highest risk of mortality with an estimated rate of 20% in the 6 weeks following an acute bleeding event [3]. The most common locations for varices to develop are in the distal esophagus followed by the gastric cardia. Ectopic varices arise outside of the gastroesophageal region and account for less than 10% of total varices and less than 5% of variceal hemorrhage among patients with liver cirrhosis [4]. Our case report is of a 68-year-old woman with a history of liver transplantation complicated by cirrhosis and bleeding ileocolic varix.

Case report

A 68-year-old woman presented to the emergency department after noticing multiple episodes of blood in her stool. She had a known history of liver transplantation 16 years prior, complicated by autoimmune hepatitis and cirrhosis. Her vitals were concerning for tachycardia, tachypnea, and hypotension. Initial labs were significant for a hemoglobin of 6.4 g/dL (11.6-15 g/dL) and creatinine of 1.6 mg/dL (0.5-1.1 mg/dL). Immediate management included transfusion of 3 units of packed red blood cells and infusion of 2L IV fluids. Upon admission to the intensive care unit, this patient underwent an esophagogastroduodenoscopy (EGD) and colonoscopy. Both of these studies failed to identify a bleeding source although the patient continued to experience hematochezia. A computed tomography angiography (CTA) of the abdomen and pelvis showed a tortuous, dilated vessel coming off of the distal SMV (Fig. 1) consistent with ileocolic varix. This location was not amenable to endoscopic intervention, and with clinical evidence suggestive of a bleeding varix, an emergent transjugular intrahepatic shunt (TIPS) procedure was performed. A postoperative CTA was performed the following day with evidence of a patent portosystemic shunt but without decompression of the varix (Fig. 2). The patient’s hemoglobin continued to downtrend and she continued to have hematochezia, necessitating further intervention. The patient was brought back to the angiography suite where the nondecompressed varix was visualized (Fig. 3). Coil embolization of the varix was performed and postembolization angiography demonstrated cessation of flow (Fig. 4). Over the following days this patient’s hemoglobin had stabilized and she had no recurrent episodes of hematochezia.

Fig. 1.

CTA demonstrating an ileocolic varix (arrow) from the distal SMV.

Fig. 2.

(A) Post-TIPS CTA demonstrating a patent portosystemic shunt (arrow) and (B) nondecompressed ileocolic varix (arrow).

Fig. 3.

Post-TIPS digital subtraction angiography (DSA) demonstrating nondecompressed ileocolic varix (arrow).

Fig. 4.

Postembolization DSA demonstrating coil takedown (arrow) of the ileocolic varix.

Discussion

Ectopic variceal bleeding is an uncommon but life-threatening complication of portal hypertension, accounting for approximately 2%-5% of gastrointestinal bleeds in this patient population [5]. Ectopic varices are portosystemic collateral vessels that develop outside the gastroesophageal region, most frequently in the small bowel, colon, and rectum, but may occur in atypical sites such as the biliary tree or urinary bladder. Their variable locations and limited accessibility make diagnosis and treatment particularly challenging.

Initial diagnostic evaluation typically begins with EGD or colonoscopy if the suspected site is endoscopically accessible, allowing for both diagnosis and possible therapeutic intervention. When endoscopy is inconclusive or the bleeding source is suspected to lie elsewhere, contrast-enhanced CTA or magnetic resonance angiography is the preferred next step [6]. These imaging modalities can delineate vascular anatomy and identify bleeding sources not amenable to direct visualization.

For variceal hemorrhage refractory to medical and endoscopic management, TIPS placement is the standard next intervention, aiming to reduce portal pressures and decompress varices [7]. Guidelines recommend achieving a post-TIPS hepatic venous pressure gradient (HVPG) of <12 mmHg or at least a 50% reduction from baseline [6]. However, given the rarity of ectopic varices, supporting evidence is limited to small retrospective series, and it remains unclear whether these HVPG thresholds are equally predictive of rebleeding risk in ectopic sites.

Several studies demonstrate that TIPS alone may be insufficient for durable hemostasis in ectopic variceal bleeding, necessitating adjunctive targeted embolization [[8], [9], [10], [11]]. While a variety of embolic agents have been reported—such as coils, liquid embolics, or vascular plugs - there is no consensus regarding the optimal embolization technique for ectopic sites.

Our case illustrates the diagnostic and therapeutic complexity of ectopic ileal variceal hemorrhage, emphasizing the need for a multidisciplinary approach involving gastroenterology, interventional radiology, and potentially surgery. Management should proceed in a stepwise fashion: initial endoscopic intervention if feasible, followed by TIPS with or without embolization, and finally surgical takedown when less invasive measures fail.

In summary, ectopic ileal variceal bleeding presents significant diagnostic and therapeutic challenges due to its rarity, variable anatomy, and frequent inaccessibility to endoscopic treatment. While TIPS remains the cornerstone of therapy for portal hypertension-related bleeding, our case adds to the growing body of evidence that TIPS alone may be insufficient in certain ectopic locations. Simultaneous targeted embolization of the culprit varices at the time of TIPS offers a promising strategy to reduce rebleeding risk. Given the paucity of high-quality data, future prospective studies are warranted to evaluate the efficacy, safety, and optimal patient selection for this combined approach.

Patient consent

Written informed consent was obtained from the patient for publication of this case report and any accompanying images.