Narrative review of the current management of ectopic varices: an update

Ectopic varices are a rare complication of portal hypertension that occur outside the esophagogastric region and represent a potentially life-threatening source of gastrointestinal bleeding. Treatments are frequently guided by institutional experience rather than standardized treatment algorithms due to their rarity and variable anatomy. This narrative review summarizes the epidemiology, clinical presentation, diagnostic evaluation, and management strategies.

A literature review was performed using PubMed and Google Scholar. Given that much of the available evidence consists of case reports, case series, and retrospective studies, no time restrictions were applied to the search. The search strategy broadly included studies relevant to ectopic varices, and no language restrictions were applied.

Ectopic varices account for small proportion of variceal hemorrhage but are associated with significant morbidity and mortality. Clinical presentation varies according to anatomic location. Cross-sectional imaging, endoscopy and angiography play important roles in diagnosis and treatment planning. In the absence of bleeding, nonselective beta-blockers are the primary medical therapy to reduce portal pressure and prevent hemorrhage. For suspected or confirmed bleeding, prompt hemodynamic stabilization is essential, and splanchnic vasoactive agents such as octreotide or terlipressin should be initiated without delay even prior to confirmatory testing. Systemic vasopressors are reserved for patients in shock. Endoscopic band ligation and sclerotherapy are first-line treatment options after initiating vasopressor therapy, although their use may be limited by anatomical accessibility. If endoscopic therapy is unsuccessful or not feasible, interventional radiology (IR) techniques such as transjugular intrahepatic portosystemic shunt (TIPS), antegrade transvenous obliteration (ATO), retrograde transvenous obliteration (RTO), and recanalization of chronically occluded visceral veins are pursued based on patient anatomy and portal hemodynamics. Surgical interventions are reserved for refractory cases.

Management of ectopic varices requires an individualized, multidisciplinary approach that incorporates medical, endoscopic, IR, and surgical therapies.