Laparoscopic ligation of portosystemic shunt for the treatment of congenital intrahepatic portosystemic shunt in one newborn infant

Pediatr Surg Int. 2020 Dec;36(12):1501-1506. doi: 10.1007/s00383-020-04753-6. Epub 2020 Oct 6.


Background: Congenital portosystemic shunt (CPSS) is a rare congenital portal malformation. 35.5% of CPSS is intrahepatic. Certain intrahepatic CPSS cases can be spontaneously closed within 2 years after birth. However, the intrahepatic CPSS with clinical symptoms or large shunt requires immediate treatment. In the present study, we used a laparoscopic ligation of portosystemic shunt to treat one newborn case.

Materials and methods: An abnormal shunt between the portal and the hepatic vein was initially detected by a prenatal ultrasound in one newborn infant. The postnatal ultrasound and CT indicated a connection between the left portal and hepatic vein and a venous cyst at the anterior edge of the left liver. Owing to the hyperammonemia and the large shunt from the portal into the hepatic vein, the infant was treated with a laparoscopic ligation of the shunt. During the operation, the left portal vein and its branches were dissected and the anterior branch of the left portal vein was clamped using a Hem-o-lok. The venous cyst was sutured and ligated using a prolene suture.

Results: The surgery was completed successfully. The duration of the operation time was 60 min. The postoperative hospital stay was 3 days. The preoperative portal angiography indicated an intrahepatic shunt between the left portal and the left hepatic vein and a venous cyst. Postoperative portal angiography indicated the disappearance of the abnormal shunt and the venous cyst. The portal pressure was increased from the preoperative 6 cmH2O to the postoperative 12 cmH2O. The duration of the follow-up was 22 months. Following surgery, the level of serum ammonia returned to normal. The postoperative CT demonstrated that the abnormal shunt had disappeared. No symptoms of portal hypertension were noted following surgery, such as splenomegaly, hypothrombocytopenia, and upper gastrointestinal bleeding.

Conclusion: The laparoscopic ligation of portosystemic shunt is suitable for the treatment of the intrahepatic CPSS in the newborn patient.

Keywords: Children; Congenital portosystemic shunt; Infant; Laparoscopic surgery; Ligation.

Publication types

  • Case Reports

MeSH terms

  • Hepatic Veins / abnormalities*
  • Hepatic Veins / surgery*
  • Humans
  • Infant, Newborn
  • Laparoscopy / methods*
  • Ligation
  • Male
  • Portal Vein / abnormalities*
  • Portal Vein / surgery*
  • Treatment Outcome
  • Vascular Malformations / surgery*