Three Cases of Congenital Portosystemic Shunt With Different Treatment Approaches Selected

Abstract

Congenital portosystemic shunt (CPSS) can cause hepatopulmonary syndrome and encephalopathy, among other complications. Interruption of the shunt blood flow is the definitive treatment and can prevent such complications. However, there is no standardized treatment approach for CPSS because of the varied pathophysiological states that can manifest. We treated three cases of CPSS with three different approaches. A one-year-old girl was treated for shunt vessel transection by laparoscopic liver resection. A 10-month-old boy with congenital heart disease underwent shunt vessel banding by laparotomy. A 17-year-old boy underwent transcatheter embolization. CPSS may cause severe complications, such as progressive pulmonary hypertension. Therefore, even when no clinical manifestations are apparent, CPSS should be treated as early as possible. If hypoplasia of the intrahepatic portal system (IHPS) is mild or moderate and the portal venous pressure (PVP) under the occlusion test is <25 mmHg, shunt closure can be performed in a single step. If hypoplasia of IHPS is severe or the PVP is ≥25 mmHg, the patient is a candidate for 2-step shunt closure. Endovascular closure is the first option for treatment, whereas surgical closure should be considered second. We should carefully select our approach on the basis of the preoperative evaluation of the anatomy of the shunt vessel and IHPS.