Although gastroesophageal varices occur commonly in patients with portal hypertension, varices located at other sites are unusual. Bleeding from these ectopic varices is an uncommon complication of portal hypertension, and the mortality rate in such cases is high. This problem should be suspected when neither upper nor lower gastrointestinal endoscopy reveals a bleeding site in a patient with portal hypertension and gastrointestinal hemorrhage. In most of these cases, the varices are in the small or large intestine; however, they may also be found in the biliary system, vagina, bladder and peritoneum. The majority of patients with ectopic varices exhibit portal hypertension associated with either cirrhosis or portal vein thrombosis (1-3).
Hemorrhage from ectopic varices, including those in the duodenum, is often massive and life-threatening. Therefore, it is imperative that this condition be diagnosed promptly and treated with early intervention. Duodenal varices resulting from retroperitoneal porto-systemic shunts are caused by increased hepatofugal blood flow through the cystic branch of the superior mesenteric vein, the superior and inferior pancreaticoduodenal veins, and the gastroduodenal and pyloric veins (4). Patients with Budd–Chiari syndrome exhibit obstruction of hepatic vein outflow at any level, from the small hepatic veins to the junction between the inferior vena cava and the right atrium (5). If obstruction continues, portal hypertension and cirrhosis eventually develop. Formation of venous collaterals is an important compensatory mechanism in these cases.
The treatment possibilities for Budd-Chiari syndrome include attempts to reperfuse the obstructed hepatic veins with thrombolytic therapy, percutaneous angioplasty or surgery. Liver transplantation is considered a major form of restoration of hepatic outflow. For patients with short-segment stenosis of the hepatic veins, percutaneous angioplasty is the treatment of choice. Most authors have documented immediate relief of obstruction with this method, and recent reports on the use of wall stents have noted long-term patency rates of 80% to 90% (6, 7). Percutaneous angioplasty with stent placement may be used in select patients to defer and perhaps avoid shunt surgery or liver transplantation. We opted to insert wall stents in our case; however, though our patient’s hepatic vein outflow improved, his gastrointestinal bleeding problems continued. The presence of a collateral vessel connecting the superior mesenteric vein with the left renal vein permitted our treatment of this collateral with coil replacement. We expect that our patient’s portal pressure will decrease in time with improvement of chronic changes in liver.
The therapeutic alternatives for bleeding varices include sclerotherapy of the varices, ligation of varices, transjugular intrahepatic portosystemic stent-shunt, portosystemic shunting, resection of a segment of bleeding site, balloon-occluded retrograde transvenous obliteration, beta-blocker therapy, and vasopressin infusion via the superior mesenteric artery (8-11). However, because ectopic varices are uncommon, none of these treatment modalities has been investigated in large series of patients, and it is not clear which method or methods are superior. Thus, the management of ectopic varices depends on the experience of the physician, and must be planned on a case-by-case basis. In our patient, we used band ligation to treat a bleeding duodenal varix that we detected on extended UGI endoscopy. This did not prevent more bleeding from recurring, but it stabilized the patient’s condition long enough for us to implant stents. Although stent placement improved the hepatic vein outflow, coil embolization was also needed to stop the variceal bleeding.
This report describes a patient who presented with upper gastrointestinal bleeding, which is a common sign of ectopic varices. However, this case is of particular interest because his duodenal varices, a rare cause of hemorrhage, were beyond the reach of a standard UGI endoscope. We recommend that any patient with portal hypertension who presents with gastrointestinal bleeding of unknown origin should be very thoroughly investigated for ectopic varices. Although variceal band ligation may be the treatment of choice according to the physician’s level of experience, coil embolization seems to be more effective in selected cases.