The majority of varices and variceal bleeding associated with portal hypertension arise in the stomach and esophagus. Varices that arise outside of the gastroesophageal region are considered ectopic varices. Ectopic varices are a rare manifestation of portal hypertension and account for only 5% of variceal bleeding.1–4 In a review of 169 cases of bleeding ectopic varices, the prevalence is 26% from peristomal, 17% from duodenal, 17% from jejunal or ileal, 14% from colonic, 9% from peritoneal, 8% from rectal, and from infrequent sites such as ovary and vagina.1,4 Duodenal varices are usually a result of portal hypertension from liver cirrhosis, and duodenal variceal bleeding carries significant mortality, reported as high as 40%.1–3 Treatment of ectopic varices includes endoscopic therapies (band ligation, injection sclerotherapy) and endovascular therapies (transjugular intrahepatic portosystemic shunt [TIPS], balloon-occluded antegrade transvenous obliteration [BATO], and balloon-occluded retrograde transvenous obliteration [BRTO]).1–3,5–16 Surgery is reserved for refractory cases. This case report will highlight the clinical course of a patient with bleeding duodenal varices secondary to alcoholic cirrhosis and our endovascular management with TIPS and subsequent transcatheter variceal embolization.

A 53-year-old male who had been treated for alcoholic cirrhosis admitted to University of North Carolina Hospital for acute hematemesis and hematochezia. He had previously undergone esophagogastroduodenoscopy (EGD) guided band ligation of lower esophageal varices several years prior. Past medical history and surgical history are otherwise unremarkable. Social history includes current alcohol use. The patient was admitted to medical intensive care unit (MICU) with hypotension to the 70/40 mmHg and evidence of active bleeding on a physical exam. His initial laboratory results were hemoglobin 10.9 g/dL, platelets 100 × 109/L, international normalized ratio 1.59, total bilirubin 3.0 mg/dL, creatinine 0.66 mg/dL, and sodium 138 mmol/L. The model for end-stage liver disease (MELD) score was 16. The patient was given blood products, started on standard medical therapy for variceal bleeding, and then prepared for EGD. At EGD, there was evidence of a bleeding duodenal varix, which was band ligated, and the patient was transferred back to the MICU in stable condition. At that point, our interventional radiology department was consulted for possible TIPS placement or BATO of duodenal varix. In the interim, computed tomography angiogram of the abdomen was obtained, which confirmed the presence of a large duodenal varix (Fig. 1). We contemplated that the patient would benefit from BATO via a transhepatic approach for the treatment of duodenal varix rather than TIPS given a relatively high MELD score of 16 and the patient’s ongoing alcohol use.17 Prior to this elective BATO, the patient had decreasing hemoglobin and new hematochezia, necessitating emergency TIPS (Fig. 2). The TIPS procedure was performed under general anesthesia, and a 10 mm × 7 cm Viatorr covered stent (Gore Medical, Flagstaff, AZ, USA) was placed. The stent was dilated with an 8 mm × 4 cm Conquest balloon (Bard Peripheral Vascular, Tempe, AZ, USA). The portosystemic pressure gradient reduced from 15 mmHg pre-TIPS to 5 mmHg post-TIPS. Additionally, there was a significant reduction in the filling of the coronary and duodenal varices on post-TIPS digital subtraction angiogram (DSA) when compared with pre-TIPS DSA. The decision at that time was to end the procedure. The patient was transferred back to the MICU and was doing well, without signs of bleeding. However, the next day the patient began to have decreased hemoglobin and new hematochezia. The patient was then brought back to interventional radiology for transcatheter embolization of the duodenal and coronary varices via the TIPS under moderate sedation (Fig. 3). This transcatheter variceal embolization procedure with endovascular plug devices (MVP Micro Vascular Plug [Reverse Medical, Irvine, CA, USA]; Amplatzer Vascular Plug 4 [Abbott, Plymouth, MN, USA]) and Nester micro-coils (Cook Medical, Bloomington, IN, USA) was successful. The patient was discharged five days later without any evidence of bleeding or hepatic encephalopathy. The patient did not have any recurrent bleeding or encephalopathy, and liver function tests were within normal limits in two months follow-up clinic visit. His follow-up EGD findings were normal except grade I lower esophageal varices.

Figure 1. (A) Axial computed tomography (CT) angiography image showing submucosal varix (arrow) in the right lateral wall of the second portion of the duodenum. (B) Coronal CT angiography image showing submucosal varix (arrow) in the right lateral wall of the second portion of the duodenum. (C) Coronal CT angiography image showing the draining vein of the duodenal varix into the inferior vena cava (arrowhead).

Figure 2. (A) Digital subtraction portal venogram before transjugular intrahepatic portosystemic shunt (TIPS) stent placement demonstrates duodenal varix (arrow) originating from the superior mesenteric vein and draining into the retroperitoneal veins and then inferior vena cava (not shown). There is a noted coronary varix (arrowhead). (B) Portal venogram after TIPS stent placement demonstrates substantially decreased contrast filling of duodenal varix (arrow), and the coronary varix is not seen but its orifice (arrowhead).

Figure 3. (A) Digital subtraction venogram during transcatheter embolization of varices via transjugular intrahepatic portosystemic shunt demonstrates duodenal varix (arrow) and its draining vein (retroperitoneal vein of Retzius) and ultimately draining into the inferior vena cava (not shown). (B) Digital subtraction venogram demonstrates coronary varix (arrow). (C) Post-embolization superior mesenteric venogram demonstrates embolized duodenal varix with Nester coils (arrows) and MVP Plug (obscured by overlapped coils) as well as coronary varix with Amplatzer 4 Plug (arrowhead).

While bleeding from ectopic varices (5%) is much less common than those from the gastroesophageal region (95%), the consequences from ectopic variceal bleeding can be catastrophic with a mortality rate seen as high as 40%.1–3 The afferent veins of duodenal varices originate from the portal vein or superior mesenteric vein, and the varices drain into the inferior vena cava through retroperitoneal veins of Retzius.1,2 Duodenal varices can be successfully treated with endoscopic (band ligation, injection sclerotherapy) or endovascular approaches (BATO, BRTO or TIPS).1–3,5–16 BATO and BRTO are typically performed in the non-emergency setting. As in this case, BATO was the chosen therapy after optimal medical management was initiated and endoscopic treatment completed. However, acute interval bleeding necessitated emergency TIPS instead of the planned BATO. TIPS has been described as a treatment for bleeding ectopic varices caused by portal hypertension, particularly with greater than 50% reduction in pressure gradient.1,14 In our case, there was a pressure gradient drop from 15 mmHg pre-TIPS to 5 mmHg post-TIPS and reduced filling of the coronary and duodenal varices on DSA. Due to the successful TIPS and angiographic findings, the decision was made not to embolize the duodenal varices. However, the patient had recurrent bleeding next day, and transcatheter variceal embolization through the TIPS was performed successfully. The decision algorithm in these cases is exceptionally challenging given the lack of randomized trials, as most of the data come from small retrospective studies and case reports.1 This case, however, does highlight the possible utility and benefit of variceal embolization at the time of TIPS, rather than relying on the TIPS alone. Although there is no consensus, the necessity of combined variceal embolization in addition to TIPS has been emphasized in several reports.1,10,14 It may be required routine definitive embolization even with successful TIPS for the treatment of ectopic variceal bleeding because 20%–25% ectopic variceal bleeds occur in the presence of adequate decompression of portosystemic pressure gradient less than 12 mmHg.1,10

In conclusion, we present a rare case of duodenal variceal bleeding, which was successfully treated with TIPS and subsequent transcatheter embolization. Even with significant decompression of portal hypertension by TIPS, it was necessitated to embolize duodenal varix due to recurrent bleeding within 24 hours. Although there are no large series reported, initial definitive embolization with TIPS can be a more effective and safer method of duodenal variceal bleeding rather than TIPS alone.

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