Abstract
Purpose: A congenital portosystemic shunt (CPSS) causes hyperammonemia, hepatopulmonary syndrome, and encephalopathy, as it drains the portal blood flow into the systemic circulation. In symptomatic cases, occlusion of the shunt vessel is effective. In this study, we aimed to review the feasibility and usefulness of surgical management in cases of CPSS.
Methods: We treated 5 patients by surgery between January 2000 and December 2012. We retrospectively reviewed their perioperative courses. The appropriate portal vein pressure (PVP) to be maintained after shunt ligation is controversial. Our indications for one-stage ligation of the shunt vessel include three criteria: a postligation PVP of <25 mmHg, a PVP gap of <5 mmHg, and no signs of intestinal congestion.
Results: The patients’ ages ranged from 1 year and 7 months to 8 years. There were 4 male patients and 1 female patient. CPSS was detected in 3 patients while investigating hypergalactosemia, in 1 patient while investigating the absence of the portal vein, and in 1 patient while investigating cyanosis and liver function disorder. Preoperative hyperammonemia was found in all the patients. The ammonia levels ranged from 71–134 mg/dl (mean: 94 mg/dl). One patient showed pulmonary hypertension. All the patients showed extrahepatic CPSS. The intraoperative PVPs after ligation ranged from 13–30 mmHg (mean: 20.8 mmHg). Four patients underwent one-stage laparoscopic ligation and 1 patient, in which the PVP was 30 mmHg, underwent banding. Their perioperative courses were uneventful.
Conclusions: In our patients, we performed the ligation of CPSS effectively and safely on the basis of our criteria.
