A Case of Shunt Vessel Closure for Symptomatic Congenital Portosystemic Shunt with Simultaneous Pancreatoduodenectomy.

Background: Congenital portosystemic shunt (CPSS) is a condition in which portal blood flow bypasses the liver and directly enters the systemic circulation. CPSS is often diagnosed during childhood, but it can also be incidentally diagnosed in adulthood during imaging for other conditions. Reports of surgical treatment of CPSS in adult patients are rare.

Methods: A 60-year-old woman was referred to our department for further evaluation of a dilated pancreatic duct and diagnosed with ampullary carcinoma. She had a tendency toward somnolence and had taken sodium valproate for multiple seizures for 10 years. Serum ammonia level was elevated and contrast-enhanced computed tomography revealed a shunt vessel between P7 of the intrahepatic portal vein and the right hepatic vein (RHV). She was diagnosed with CPSS. Angiography showed moderate development of the intrahepatic portal system and an acceptable portal pressure increase during shunt clamping, which allows the shunt to be resected. Imaging also revealed multiple hepatic nodules with irregular shapes which was considered as focal nodular hyperplasia. Pancreatoduodenectomy (PD) with shunt closure and hepatic mass biopsy was performed. The shunt was located between P7 and RHV. The shunt was clamped at first. PD was performed while the shunt was clamped. After removing specimen, the portal pressure was 9 mmHg: this was within the acceptable range to resect the shunt. No evidence of intestinal congestion was observed, therefore the shunt vessel was closed using an automatic suturing device. On the first postoperative day, the serum ammonia level normalized. Six months after-surgery, she remained under outpatient clinic observation with no cancer recurrence. The preoperative tendency toward somnolence significantly improved.

Conclusions: We report a case of symptomatic CPSS coexisting with duodenal ampullary carcinoma. The shunt closure with simultaneous PD was feasible in this case. CPSS is recommended to treat even in adult cases because it is potentially symptomatic.