Surgical ligation of a portosystemic shunt for the treatment of type II Abernethy malformation in 12 children.

OBJECTIVE

This study aimed to investigate the feasibility and effectiveness of surgical ligation in the treatment of a congenital extrahepatic portosystemic shunt (Abernethy malformation) in children.

METHODS

Twelve children (aged 10 days to 13.3 years; six boys and six girls) with Abernethy malformation were admitted to the Capital Institute of Pediatrics (Beijing, China) from May 2014 to November 2019 owing to hyperammonemia. Among them, nine suffered from hematochezia, two had elevated liver transaminase levels or jaundice, and one had hypoxemia. Their surgical procedures and outcomes were retrospectively analyzed. Portal pressure measurement and angiography of the portal vein were performed before and after the occlusion of the portosystemic shunt during the surgery. Six patients underwent a single-stage ligation of the portosystemic shunt, five underwent a two-stage ligation of the portosystemic shunt, and one was treated with a partial ligation of the portosystemic shunt.

RESULTS

Each of the operations was successfully completed with an operative time ranging from 60 to 240 minutes and with an intraoperative blood loss ranging from 50 to 200 mL. Three children had a blood transfusion. The postoperative portal pressure was significantly higher than that before the surgery in all children. The patients were followed for 5 to 70 months. The symptoms of hematochezia were relieved in nine patients after the surgery. The levels of blood bilirubin and transaminase returned to normal after the surgery in two patients. The oxygen saturation returned to normal in one patient. The level of blood ammonia was normal after the surgery in all patients.

CONCLUSIONS

The surgical ligation of the portosystemic shunt was an effective method to treat a type II Abernethy malformation. According to the portal pressure after the occlusion of the portosystemic shunt, a single or staged procedure was selected.