Chapter 32: Management of Duodenal Varices Duodenal varices (DVs) are an uncommon manifestation of portal hypertension or mesenteric/portal vein thrombosis, representing approximately 17% of ectopic varices.1,2 Pathologically they represent dilated mesoportal varicosities and portosystemic collaterals in the duodenal wall.3 Despite their infrequency, they may be potentially life threatening as they carry a fourfold risk of bleeding compared with esophageal varices and a mortality rate approaching 40% secondary to unrelenting hemorrhage.3–6 The most commonly encountered varices are esophageal and gastric, which are amenable to medical and endoscopic interventions.4–12 Due to the anatomic location and hemodynamics, however, the medical and endoscopic management of DVs is limited.1,3,4,6,13 In addition to surgical resection, endovascular management with decompressive transjugular intrahepatic portosystemic shunt (TIPS)14,22,23 or transvenous obliteration remains a potential treatment option. In the limited data in the literature, the rebleed rate after TIPS is approximately 21% to 37% and approximately 13% following transvenous obliteration.2,29 The chapter discusses specifically duodenal varices (DVs). However, with the exception of anatomical location specific to DVs, the general classification and management approach applies to all ectopic mesenteric varices, whether in the small bowel or large bowel. DVs are considered “true veins” when compared with esophageal varices. DVs, however, have thinner walls and larger diameters, resulting in greater wall tension and increased rates of bleeding.3 DVs are portoportal or portosystemic retroperitoneal collateral vessels ( Fig. 32.1 Classification of ectopic varices. (a,b) Baseline anatomy images to help interpret the images of the classification system. The image demonstrates a representative portal or mesenteric vein branch (Port Circ) on the right and a representative systemic vein branch (Syst Circ) on the left of a cross-section through a bowel loop, which is representative of the gastrointestinal tract (GIT). Typical portal venous (splanchnic veins) branches would include the portal vein proper, the mesenteric vein, and the splenic vein. Typical systemic veins include the inferior vena cava, the gonadal veins, renal veins, and retroperitoneal and paravertebral veins. Varices are seen in the wall of the bowel. (a) Ectopic varices are supplied and drained by portal collaterals (hollow black arrows) and drained (efferent collateral) by a portosystemic collateral (solid black arrow). (b) Ectopic varices are supplied and drained by portal collaterals (hollow black arrows) and not drained by a portosystemic collateral. The efferent collateral drainage is portal and not systemic. In both (a) and (b), there is no portal venous occlusion. (Reprinted with permission from Saad WE, Lippert A, Saad NE. Ectopic varices: Anatomical classification, hemodynamic classification, and hemodynamic-based management. Tech Vasc Interv Radiol 2013;16(2):158–175.) Table 32.1 Typical Locations for Duodenal Varices with Various Locoregional Pathologies
Introduction
Anatomy and Pathophysiology and Hemodynamic Classification
Fig. 32.1).3 The portal venous supply of DVs includes pancreaticoduodenal veins, cystic branches from the superior mesenteric veins, gastroduodenal veins, and pyloric veins.3,7 The systemic venous drainage of DVs is commonly via the gonadal veins, particularly the right gonadal vein, and the capsular renal veins, all of which drain into the inferior vena cava.3,7 The left gonadal vein may be involved in DVs and is typically associated with DVs in the third and fourth portions of the duodenum. In rare instances, DVs may drain directly into the inferior vena cava or right renal vein.3
Etiologies | Location |
Generalized or global portal hypertension of all etiologies | D1a and D2b |
Status after gastrectomy | D2 |
Status after balloon-occluded retrograde transvenous obliteration of gastric varices | D1 and D2 |
Mesenteric carcinoid | D3c−D4d>D2 |
Chronic pancreatitis | D1 + D2>D3 |
Splenoportal thrombosis | D1 + D2>D3 |
Mesoportal thrombosis | D2 + D3 |
Focal mesenteric occlusion | D3 or D4 |
a D1: first part of the duodenum (duodenal bulb)
b D2: second part of duodenum (duodenal sweep or descending part)
c D3: third part of the duodenum (transverse retroperitoneal part)
d D4: ascending part to the ligament of Treitz.
Reprinted with permission from Saad WE, Lippert A, Saad NE. Ectopic varices: Anatomical classification, hemodynamic classification, and hemodynamic-based management. Tech Vasc Interv Radiol 2013; 16(2):158–175.
Location of DVs is due to locoregional pathology including splanchnic vein (splenic, splenoportal, mesenteric, mesoportal, or portal) stenosis, thrombosis, or occlusion, adhesions and scarring from prior surgeries, or inflammatory processes (
Table 32.1). The most common site for DVs is in the duodenal sweep in the first portion (D1: Duodenal bulb), the second portion (D2), and the proximal portion of the third portion of the duodenum (D3). DVs in the distal duodenum (D3 and D4) are usually associated with splenic vein thrombosis with or without portal vein thrombosis.
There are two classifications for DVs (as in all ectopic varices): the nonocclusive, or oncotic type (secondary to portal hypertension), and the occlusive type (due to mesenteric or portal vein thrombosis) (
Table 32.2; 
Fig. 32.2).3
Endoscopy and Imaging
Standard imaging evaluation for DVs has not been established. Endoscopy, however, is the initial modality of choice to diagnose and localize the ectopic DVs.5 Contrast-enhanced computed tomography (CT) and magnetic resonance imaging (MRI) are important for the evaluation and management of DVs as these modalities determine if portal hypertension is present and identify portal vein and mesenteric thrombosis. Secondary signs of portal hypertension include splenomegaly, ascites, hepatic hydrothorax, and development of portosystemic collaterals.3 CT and MRI may also identify DVs that were not appreciated on endoscopy. The use of ultrasound is limited to evaluating the patency of the intrahepatic and extrahepatic portal vein, and the distal splenic and mesenteric veins at their confluence. Ultrasound may also determine the direction of mesoportal blood flow, specifically whether flow is hepatofugal (away from the liver), hepatopetal (toward the liver), or fluctuating.3
In the setting of splanchnic vein thrombosis (thrombosis of the portal vein, mesenteric vein, and/or splenic vein), it is important to identify if the DVs occur along the “spleno-portal axis” versus “meso-portal varices” or both axes. This is important to help the management approach of duodenal varices.
Management of Duodenal Varices
Medical management of ectopic DVs includes supportive care with fluid resuscitation and, if necessary, administration of octreotide and systemic vasopressin.1 Endoscopic-guided management includes banding, sclerotherapy, or injection of thrombin, N-butyl cyanoacrylate, or histoacryl.15 Sclerosants typically include ethanolamine oleate, sodium tetradecyl sulfate, and polidocanol.9,21 These endoscopic options are suitable for short-term hemostasis, but long-term hemostasis is difficult to achieve by endoscopic means alone. Bleeding after endoscopic management of DVs is common.
Surgical management of DVs includes resection of varices, duodenectomy, and suture ligation. Surgical portosystemic shunts may also be created, but carry an increased morbidity when compared with the percutaneous approaches including TIPS creation.4,6,17
Endovascular management included TIPS decompression with or without sclerosis16 for nonocclusive (Type-a) DVs and recanalization with or without sclerosis for occlusive (Type-b) DVs (
Fig. 32.3). In the setting of splanchnic thrombosis along the spleno-portal axis, partial splenic artery embolization is another option. On the other hand, recanalization is needed if the thrombosis is along the meso-portal axis.
