Evaluation of Rex Shunt on Cavernous Transformation of the Portal Vein in Children

Children with cavernous transformation of the portal vein (CTPV) develop severe complications from prehepatic portal hypertension, such as recurrent variceal bleeding and thrombocytopenia. In this study, we reported the results of 30 children with symptomatic CTPV that were treated by a Rex shunt. The effectiveness of this surgical approach was evaluated.

A retrospective review was performed of 30 children aged between 3 and 18 years with CTPV, who underwent a Rex shunt between 2008 and 2015. All children were evaluated based on symptoms, complete blood count, portal system color-flow Doppler ultrasound or computed tomography angiography portography and gastroscopy for gastroesophageal varices pre- and postoperatively. Children were also evaluated during follow-up. Intraoperative evaluations included liver biopsy, portography and portal pressure.

Twenty-one patients demonstrated intermittent bleeding from gastroesophageal varices, 3 patients showed hypersplenism with varying degrees of leucopenia, anemia and thrombocytopenia, and in 6 patients both bleeding and hypersplenism were observed. Rex was successful in 28 patients (93.3%). The portal pressure immediately decreased significantly after placing of the shunt (P < 0.01). During the clinical follow-up period within 2–82 months, transaminase levels were maintained in the normal range. Blood flow velocity and diameter of the left portal vein significantly increased after surgery (P < 0.01). In addition, leukocyte and platelet counts increased postoperatively and anemia improved significantly (P < 0.01). Gastroscopy results indicated that the degree of gastroesophageal varices significantly alleviated postoperatively within 3 months and 1 year (P < 0.01). In 2 patients who demonstrated nodular cirrhosis and chronic active hepatitis, success of the Rex shunt was not achieved after operation. We found that for Rex effectiveness hepatic pathology and patient age were major determinants.

Rex shunt is an effective approach for the treatment of children suffering from CTPV at an early stage that do not show additional liver lesions.