Successful Surgical Ligation Under Intraoperative Portal Vein Pressure Monitoring of a Large Portosystemic Shunt Presenting as an Intrapulmonary Shunt: Report of a Case

Abstract

We report a rare case of patent ductus venosus (PDV) with collapsed intrahepatic portal branches and an intrapulmonary shunt. Excellent improvement of the intrahepatic portal vein flow was achieved by ligating the large ductus venosus under intraoperative portal vein pressure (PVP) monitoring. A 3-year-old boy being followed up for hypergalactosemia at a local hospital was found to have mild lip cyanosis, exertional dyspnea, clubbed fingers, and mild liver dysfunction with high levels of transaminase and ammonia. Cardiac catheterization indicated an intrapulmonary shunt with a ratio of 33%. Abdominal ultrasonography and computed tomography showed remarkable communication between the portal vein and the inferior vena cava. We performed laparotomy and successfully ligated the PDV under PVP monitoring. The PVP did not increase until the catheter was removed 7 days postoperatively. The patient's liver function test results returned to normal within 2 weeks. His serum galactose level was 0 mg/dl after drinking milk, and his SpO2 in room air and exertional dyspnea also improved. He was discharged 18 days after his operation, without any complications. We propose that ligation of a PDV under PVP monitoring could be a treatment of choice, bearing in mind that PDV is associated with collapsed intrahepatic portal branches.

Successful treatment of pulmonary hypertension secondary to congenital extrahepatic portocaval shunts (Abernethy type 2) by living donor liver transplantation after surgical shunt ligation

“…In this case, although the surgical ligation of CEPS was initially performed successfully (with no postoperative portal hypertension) [3], the patient developed a large intrahepatic systemic shunt and pulmonary hypertension 4 years after the initial operation.…”

“…As intraoperative liver biopsy showed only mild fatty liver (macrovesicular steatosis: 15%) without fibrosis, surgical ligation of the portosystemic shunt was performed successfully on March 24, 2003, with intraoperative portal venous pressure monitoring. The patient was discharged on postoperative day (POD) 22 without any postoperative complications (including portal hypertension) [3].…”

“…As Abernethy type 2 CEPS is very rare, the therapeutic selection is still controversial-either shunt ligation or liver transplantation [2]. We previously reported the successful surgical ligation of a large portosystemic shunt in a 3-year-old boy with Abernethy type 2 CEPS using an intrapulmonary shunt [3]. Four years after surgical ligation, the same patient showed gradual signs of relapse of multiple intrahepatic systemic shunts and pulmonary hypertension.…”

The Role of Liver Transplantation for Congenital Extrahepatic Portosystemic Shunt

“…There were 30 cases of LT for CEPS reported in the English medical literature published or being in press by the end of 2011, including our own previously reported cases (19,28,29,31,32). Congenital absence of the portal vein and Abernethy malformation were also used to retrieve the cases as a suitable synonym for CEPS.…”

Congenital absence of the portal vein: translated version

“…If the presence of IPVS can be confirmed from the radiological or pathological findings, shunt closure for type II Abernathy malformation is indicated . Regarding the indication for two‐stage shunt closure, in order to predict portal hypertension after shunt closure, the portal venous pressure during balloon occlusion may be measured and evaluated at the time of portography under balloon occlusion . It has been reported that it is difficult to close the shunt vessels if the portal venous pressure is 25–30 mmHg or more after balloon occlusion and that it can be treated by two‐stage shunt closure (closed 3–10 months after the first treatment) even if it is 32 mmHg or more .…”