Left-sided of “sinistral” portal hypertension is also called Limited or Segmental portal hypertension, which is caused by various causes (mainly pancreatic diseases). It is a rare form of extrahepatic portal hypertension (about 5%) caused by compression and obstruction of the splenic vein, resulting in increased and more than normal pressure in the spleno-gastic compartment and splenic area, without any abnormality in the liver or the main portal vein. It is more common in males, with a male to female ratio of approximately 2:1 and a mean age of 44 (1 to 70) years. Although this disease is rare, it can cause fatal variceal bleeding, so it should attract the attention of clinical workers. 1, etiology 1, 1 pancreatic disease for the main cause of the disease, pancreatitis accounted for about 56% to 65%, pancreatic pseudocysts about 12% to 75%, pancreatic tumors about 9% to 18%. 1,2 Systemic or local factors (1) certain medical sources, such as umbilical vein cannulation, splenectomy, Warran’s shunt, gastrectomy, pancreatic transplantation; (2) retroperitoneal diseases, such as retroperitoneal fibrosis, Hodgkin’s disease, liposarcoma, peripancreatic lymphoma; (3) others, such as splenic artery aneurysm or splenic A-V fistula, gastric ulcer, abdominal or splenic portal tuberculosis, myeloproliferative disease, and aggressive gastric cancer. 1,3 Idiopathic is a minority. 2. Pathology The splenic vein in normal state is about 0,5cm in diameter and 12cm in length, located at the posterior superior margin of the pancreas and below the splenic artery, and travels from the splenic hilum and tail of the pancreas to the neck of the pancreas, and collects the pancreatic veins, converges the inferior mesenteric vein, and finally merges into the portal vein at an angle of 90°. Pancreatic or retroperitoneal diseases are prone to peripancreatic tissue inflammatory edema, fibrous tissue hyperplasia, splenic vein spasm, compression, stenosis, intimal damage, blood flow stagnation and thrombosis. As a result, the splenic vein becomes partially or completely occluded and obstructed, the drainage of splenic blood is blocked, and venous and intrapleural pressures increase and exceed normal, thus creating the syndrome. Subsequently, collateral venous vessels are established. The splenic blood must converge into the portal vein through the Hepatoportal or Portoportal pathway: (1) The blood flows into the stomach through the short gastric vein and then converges into the portal vein through the coronary vein (or left gastric vein) and the right gastric vein, resulting in dilated and tortuous veins in the gastric body, fundus and cardia. If the coronary vein (about 17%) converges into the splenic vein, the coronary vein will be blocked due to the embolism of the splenic vein, and the splenic blood must enter the esophageal plexus through the short gastric vein, and finally flow into the odd vein system, so the esophageal varices appear. If the short gastric vein fails to effectively drain splenic blood, esophageal or gastroesophageal varices are also likely to occur; (2) blood flows into superior mesenteric vein and portal vein through left gastroesophageal vein, omental vein branch and right gastroesophageal vein, thus the gastroesophageal vein is dilated and tortuous. After splenic vein obstruction, the occurrence of gastric varices is about 49,5%, gastroesophageal varices 46% and esophageal varices 1%. In addition, splenic blood and venous blood flow can also be diverted via off-hepatic (Hepatofugal) or portal (Portasystemic) pathways so that the pressure in the splenogastric region is reduced: (1) through the left gastroretinal vein, the left colonic vein retinal branch flows into the inferior mesenteric vein and then into the portal vein. As a result, the colonic vein varices and bleeds; (2) through the phrenic vein and intercostal vein into the vena cava system, but it is less common. Sometimes, pancreatic disease can cause obstruction of splenic vein, superior mesenteric vein and portal vein, and the abdominal visceral venous pressure is increased, and varices and bleeding of duodenal and jejunal veins appear. 3, clinical manifestations Mainly: (1) Gastrointestinal bleeding (about 45%-73%), Moossa (1985) collected 51 cases of isolated splenic vein obstruction and gastrointestinal bleeding, 25 cases (49%) were gastric varices, 4 cases (8%) were esophageal varices, 22 cases (43%) were gastroesophageal varices. Patients had anemia, repeated vomiting of blood, black stools and bloody stools, and a few ruptured colonic venous tumors could discharge large amounts of fresh blood stools; (2) splenomegaly (about 32%-60%) and hypersplenism (about 34%); (3) recurrent abdominal pain (about 26%); (4) normal liver function and no abnormalities in various types of hepatitis markers. 4, imaging 4, 1 X-ray barium angiography Positive rate is about 50%. Thickening, distortion, filling defect or deformation of gastric mucosal folds can be seen from the gastric greater curvature to the cardia, but it should be distinguished from gastric tumor. 4,2 Endoscopy The detection rate is about 0-40%. Gastroscopic observation of gastric varices is more difficult and unreliable. If light blue or purple-blue grape-like “mucosa” or red sign is seen, varicose veins can be diagnosed. However, they need to be distinguished from normal gastric mucosal folds, gastritis, gastric tumors, or vasodilated disease. 4,3 B-US and Doppler show normal liver and portal vein, pancreatic disease and blood flow, but splenic vein is more difficult to show. 4.4 CT enhancement film shows pancreatic disease and collateral circulation. 4,5 Visceral angiography has a 100% confirmation rate. Visceral angiography of the celiac artery and superior mesenteric artery can show the site and degree of obstruction of portal branches (splenic veins), splenomegaly, varicose veins of the stomach or gastroesophagus, and dilated veins of the gastric omentum, short gastric veins, and coronary veins. Due to the development of arterial portal angiography, percutaneous splenic hilar angiography is now less commonly used. Percutaneous transhepatic portal angiography is the most direct portal angiography. 5.Diagnosis Based on the patient’s history of primary disease or history of medically induced injury, recurrent bleeding from the gastrointestinal tract, splenomegaly, hypersplenism, normal liver function and liver biopsy, and imaging (angiography) data, comprehensive analysis, the diagnosis can be clearly made preoperatively. Combined with the surgical findings: (1) normal liver; (2) splenomegaly; (3) pancreatic or retroperitoneal lesions; (4) significant dilatation and tortuosity of the short gastric vein, coronary vein and gastroretinal vein with elevated pressure, while the portal vein pressure was normal; (5) no varices in the mesenteric vein system of the small intestine. Accordingly, the diagnosis of this disease was established. However, it needs to be differentiated from intrahepatic type (cirrhosis) or other extrahepatic portal hypertension. In case of gastrointestinal bleeding, it should be distinguished from gastric ulcer and gastritis. 6. Treatment 6.1 Splenectomy is the procedure of choice for the treatment of this disease, with an efficacy of 92% and little postoperative rebleeding. The aim is to reduce collateral blood flow, lower venous pressure in the splenogastric region and prevent variceal bleeding. Depending on the situation, additional procedures such as caudal or total resection of the pancreatic body, drainage of the pancreatic cyst or subtotal resection of the stomach can be performed intraoperatively. If the local inflammatory adhesions are significant, the operation is difficult and bleeding is high, then retrograde subperitoneal splenectomy can be done after controlling the blood flow of the spleen. 6.2 Acute hemostasis In case of fatal variceal bleeding: (1) endoscopic ligation of variceal vein or injection of sclerosing agent (the rate of rebleeding is higher); (2) incision of gastric antrum and suture of variceal vessels; (3) gastric vascular dissection. 6.3 Splenic artery embolization In acute high-risk cases not suitable for surgery, transcatheter splenic artery embolization can be performed to reduce splenic blood flow and stop bleeding from gastroesophageal varices, but the efficacy is not very precise and there is a possibility of complicating splenic abscess. 6.4 Transhepatic portal vein placement In a few cases of gastrointestinal hemorrhage, transhepatic portal vein placement can be used to achieve hemostasis.