“Patients with extrahepatic portal vein thrombosis may present from infancy through adulthood with variceal bleeding. Physiologically, such patients differ from patient s with cirrhosis and variceal bleeding in that they have a normal liver and maintain good portal perfusion through hepatopedal collaterals.”
“Therapeutic options range from noninterventive, through ablative procedures, to shunt operations. The goal should be definitive control of bleeding and return to a normal lifestyle. Distal splenorenal shunt offers the best option if technically feasible, but if no shuntable veins are patent, ablative procedures and sclerotherapy may be required. A noninterventive, noninvestigational approach is inappropriate in patients who can be offered definitive
therapy. Splenectomy for hypersplenism should not be done in these patients.” (Galloway)

“The key points of acute variceal bleeding management are: volume infusion, pharmacological and endoscopic control of hemorrhage and infection prophylaxis.
During active bleeding, the volume resuscitation should be undertaken promptly with the goal of restore blood pressure and perfusion, but it might be done carefully to avoid overload volume which could increase portal vein pressure and subsequently the risk of rebleeding by EGV. Actually, the use of saline solution and blood transfusion have been administrated during acute bleeding looking for hemodynamic stability keeping systolic arterial pressure
around 90-100 mmHg, cardiac frequency <100 beats/min and hemoglobin level in 7-9 g/dl (hematocrit in 21-27%)2,19. EGV=Esophagogastric varices; BL=band ligation; TIPS=transjugular intrahepatic portosystemic shunt FIGURE 1 – Flowchart of management of acute variceal hemorrhage from Liver Surgery Unit of University of São Paulo Medical School The better results to control acute bleeding with more than 90% of succeed interventions are obtained combining endoscopy therapy and drugs that decrease the splanchnic blood flow as somatostatin, octeotride and terlipressin9,13. The pharmacological treatment has efficacy in the control of acute bleeding and to avoid rebleeding, it should to be use in variceal bleeding suspicion, even before of endoscopy approach. The only drug associated with decrease of mortality was terlipressin (decreasing the risk in 34%), thus, it is the best choice to treat EGV bleeding23. Terlipressin is a synthetic analog of vasopressin with less collateral effects and longer half-life than it. The initial dose is 2 mg followed by 1-2 mg every 4 h (adjusted by weight : <50 Kg 1 mg; 50-70 Kg 1.5mg; >70 kg 2 mg) during 2-5
days. The endoscopy is mandatory and should be done as soon as possible in EGV bleeding, just after hemodynamic management, in the first 12 h after patient admission. The
BL is the preferable modality since it has effective bleeding control in 86-92% of cases. When BL was compared to sclerotherapy, it presented lower risk of rebleeding, lower
frequency of adverse effects, lower number of sessions to obliterated EGV and better overall survival25. Therefore, BL should be treatment of choice, but sclerotherapy is also
acceptable when BL is not possible.” (Coelho)

Coelho FF, et al . Management of variceal hemorrhage: current concepts. Arq Bras Cir Dig. 2014 Apr-Jun;27(2):138-44.