Laparoscopic ligation of a congenital extrahepatic portosystemic shunt for children with hyperammonemia: a single-institution experience.

PURPOSE: A congenital extrahepatic portosystemic shunt (CEPS) associated with hyperammonemia requires occlusion of the shunt vessels. We evaluated the effectiveness and safety of laparoscopic ligation of a CEPS in children with hyperammonemia.

METHODS: The subjects of this retrospective study were seven children with hyperammonemia who underwent laparoscopic ligation of a CEPS. Their median age was 5.2 years (range 1-16 years). Before the laparoscopic procedure, a catheter was inserted through the femoral vein and placed in the portal vein via the shunt vessel. The shunt vessel was dissected and taped laparoscopically. After measuring the portal vein pressure under temporal occlusion, the shunt vessels were ligated.

RESULTS: The types of shunts according to location were patent ductus venosus (n = 2), splenorenal shunt (n = 2), gastrorenal shunt (n = 2), and superior mesenteric vein-inferior vena cava shunt (n = 1). Laparoscopic ligation of the shunt vessel was completed uneventfully in all patients. The median portal vein pressure was 19 mmHg after ligation. The median preoperative blood ammonia level was 94 µg/dL (range 71-259 µg/dL), which decreased after ligation in all patients. There was no incidence of postoperative liver failure.

CONCLUSION: Laparoscopic ligation of a CEPS is safe and effective for children with hyperammonemia.