Review Article - Ectopic varices in portal hypertension

Varices most commonly occur and bleed in the gastro-esophageal region but ectopic varices can arise at extra-gastro-esophageal locations in the gastrointestinal tract like duodenum, jejunum, ileum, colon, rectum, biliary tree and at the site of a surgical ostomy. These varices pose diagnostic as well as therapeutic challenges during endoscopic procedures. Ectopic varices can also occur outside the gastrointestinal tract, giving rise to diagnostic difficulties on imaging and unusual hemorrhage. Although ectopic varices bleed less commonly than gastro-esophageal varices, they are difficult to diagnose and problematic to treat. The absence of stigmata of recent esophageal or gastric variceal bleeding and certain clues in the patient′s history and clinical presentation should raise the clinician′s suspicion of an extra-gastro-esophageal site of variceal bleeding. Patients with extrahepatic causes of portal hypertension, cirrhotic patients with a prior history of gastrointestinal surgery and patients who present with profound bleeding but without hematemesis should in particular be evaluated further if an obvious site of gastro-esophageal variceal bleeding is not observed at UGI endoscopy. Accurate preoperative diagnosis is often difficult in patients bleeding from ectopic varices from extra-gastro-esophageal sites, rebleeding is certain even in those patients who respond to medical treatment, and the optimal surgical decision making protocol has not yet been evolved due to the low prevalence of these ectopic varices. Nevertheless, an appreciation and awareness of these unusual causes of gastro-intestinal bleeding, allied with prompt and appropriate diagnosis can lead to successful endoscopic, radiological or surgical management of ectopic variceal bleeding.

Keywords
Cirrhosis, ectopic varices, gastrointestinal bleeding, portal hypertension