An efficacy of surgical treatment for congenital extrahepatic portosystemic shunt: Report of two cases

Abstract

[Background] In patients with a congenital extrahepatic portosystemic shunt (CEPS), surgical treatments are indicated for those patients who are refractory to medical treatment. We herein report our experience with 2 patients who successfully underwent ligation of the shunt vessels for symptomatic CEPS. [Case 1] An 8-year-old male patient who diagnosed with ductus venosus and multiple liver tumors during a school examination was observed to have enlarged tumors, and ligation of the ductus venosus was therefore performed. Liver tumors were found to be reduced at the time of the 1.5-year of follow-up examination. [Case 2] A 1.3-year-old female patient who at birth was found to have a shunt vessel, in which mesenteric venous blood drained directly into the suprarenal inferior vena cava, was observed to have progressive hepatopulomonary syndrome, and ligation of the shunt vessel was therefore performed. The patient's hepatopulomonary syndrome considerably improved at the time of the 0.8- year of follow-up examination. [Conclusion] Surgical treatments for patients with symptomatic CEPS should be performed without delay because of radical treatment for various complications. Pre-operative assessment of intrahepatic portal vein and measurement of portal vein pressure are essential for choosing the optimal surgical treatment. © 2010 The Japan Society of Hepatology.