Ectopic varices: anatomical classification, hemodynamic classification, and hemodynamic-based management.

Ectopic varices are dilated splanchnic (mesoportal) veins/varicosities and/or are dilated portosystemic collaterals that can occur along the entire gastrointestinal tract outside the common pathologic variceal sites. Ectopic varices are complex and highly variable entities that are not fully understood. Ectopic varices represent 2%-5% of a gastrointestinal tract variceal bleeding. However, ectopic varices have a 4-fold increased risk of bleeding when compared with esophageal varices and can have a mortality rate as high as 40%. All treatment strategies and techniques have been utilized in managing these potentially mortal varices and have shown poor outcomes. The debate of whether to manage these varices by decompression with a transjugular portosystemic shunt, or other portosystemic shunts, vs transvenous obliteration is unresolved. The rebleed rates after transjugular portosystemic shunt decompression are 20%-40%. The rebleed rates after transvenous obliteration and the mortality rate at 3-6 months are 30%-40% and 50%-60%, respectively. Hemodynamically from an etiology standpoint, there are 2 types: occlusive (type-b) and nonocclusive (oncotic or type-a). Hemodynamically from a vascular-shunting standpoint, there could be a component of portoportal or portosystemic shunting or both with varying dominance. This is the basis of the new classification system described herein. Management strategies (decompression vs sclerosis) are discussed. The ideal management strategy is a treatment that leads to prompt hemostasis but also addresses the etiology or hemodynamics of the ectopic varices. It is the hope that with better understanding, description, and categorization of ectopic varices comes a more systematic approach to this rare but menacing problem.