Diagnosis and management of ectopic varices

Ectopic varices are large portosystemic collaterals in locations other than the gastroesophageal region. They account for up to 5% of all variceal bleeding; however, hemorrhage can be massive with mortality reaching up to 40%. Given their sporadic nature, literature is limited to case reports, small case series and reviews, without guidelines on management. As the source of bleeding can be obscure, the physician managing such a patient needs to establish diagnosis early. Multislice computed tomography with contrast and reformatted images is a rapid and validated modality in establishing diagnosis. Further management is dictated by location, underlying cause of ectopic varices and available expertise. Therapeutic options may include double balloon enteroscopy, transcatheter embolization or sclerotherapy, with or without portosystemic decompression, i.e., transjugular intrahepatic portosystemic shunts. In this article we review the prevalence, etiopathogenesis, anatomy, presentation, and diagnosis of ectopic varices with emphasis on recent advances in management.