A CPSS Patient Previously Diagnosed With Congenital Absence of Portal Vein Who Underwent Shunt Ligation

Abstract

We encountered a 14-year-old girl with congenital portal vein deficiency. At 1 month of age, she was diagnosed as having hypergalactosemia, congenital absence of the portal vein, pulmonary hypertension, atrial septal defect, and polysplenia. We suggested liver transplantation because of severe pulmonary hypertension, but the result of lung biopsy showed that it was incompatible owing to pulmonary arteriole hypoplasia. Her subsequent symptoms were relatively stable. At the age of 14, intrahepatic portal vein with hypoplasia was confirmed, and surgical ligation of the vascular shunt was indicated by angiography. The portal vein pressure was not increased at intraoperative measurement, and the small intestine had no congestion findings, so we completely ligated the shunt vessel. After the operation, the shunt rate and pulmonary hypertension were improved, and the patient had no major complications until one year after the operation. Even if the patient is diagnosed as having congenital portal vein deficiency, there are cases in which shunt ligation can be safely performed as in this patient. Therefore, in symptomatic cases, repeated evaluations, such as those by angiography, are important and necessary.