Rex Shunt as a Treatment of Type II Abernethy Malformation with Severe Dysplasia of Portal Vein

Background: Ligation of abnormal portosystemic shunt has been used to treat type II Abernethy malformation, but it may not be suitable for all patients. In this study, Rex shunt was carried out to manage type II Abernethy malformation with portal venous dysplasia. The outcomes are evaluated retrospectively. Methods: Between June 2014 and January 2015, 2 boys (age: 4.8 and 12.8 years, respectively) with type II Abernethy malformation underwent Rex shunt with ligation of inferior mesenteric vein (IMV). The portal venous pressures were measured intraoperatively to decide the extent of inferior mesenteric venous ligation. An ileal vein (6 mm in diameter) and a venae sigmoideae (7 mm in diameter) were interposed between the left portal vein and superior mesenteric vein, respectively. To minimize postoperative portal hypertension, the IMV was partially ligated. Five months later, the IMV was completely ligated in a second operation because of persistent bloody stool and anemia (hemoglobin < 100 g/L). Results: The duration of the operations was 180 and 240 min. The extrahepatic portal pressure increased after bypass, but the portal pressure was less than 24 cm H2O in both patients. The fecal blood loss and frequency of hemafecia decreased after surgery. The bypass vein was patent, and the portal blood flow was shown increased on postoperative ultrasound and computed tomography. There was no hypersplenism and esophageal gastric varices. Conclusions: The surgical management of Abernethy type II malformation should be individualized. Rex shunt with ligation of portosystemic shunt is feasible and effective in patients with severe dysplasia of portal vein.