Variceal bleeding due to extrahepatic portal hypertension may be first manifested at any age. Its cause is unknown although omphalitis, peritonitis, exchange transfusions via the umbilical vein, or distant sepsis have occurred during the neonatal period in a few patients. Whatever the cause, the primary site of the venous block is always in the portal vein. The splenic and superior mesenteric veins may or may not be occluded.
This communication deals with three patients who had an unusual form of extrahepatic portal hypertension. All had chronic alcoholism, but none had cirrhosis. All had recurrent pancreatitis with distal or segmental occlusion of the splenic vein, but with widely patent superior mesenteric and portal veins. All had portal hypertension and had bled from esophagogastric varices. Splenoportal venography was essentially identical in all and demonstrated the extensive collateral circulation which circumvented the splenic vein obstruction, but which was insufficient to adequately decompress the
Turrill FL, Mikkelsen WP. "Sinistral" (Left-Sided) Extrahepatic Portal Hypertension. Arch Surg. 1969;99(3):365-368. doi:10.1001/archsurg.1969.01340150073014