Outcomes of Congenital Portosystemic Shunt Ligation

Abstract

The surgical strategy for congenital portosystemic shunts depends on the anatomical type. We evaluated the effectiveness of the ligation of congenital portosystemic shunt (CPSS) type II for children and reviewed the literature on this rare entity. We conducted a retrospective chart review of four patients of CPSS type II treated by ligation of the shunt vessel between January 2013 and December 2017. There were two male patients and two female patients. The patients’ ages at diagnosis ranged from one to six months. Congenital heart disease was found in all the patients. Three patients underwent one-stage ligation of the shunt vessel, and one patient underwent two-stage ligation owing to high pressure of the portal vein. After the operation, blood ammonia levels decreased to normal in all the patients, and no early complication was detected. However, one patient showed pulmonary hypertension six months after surgery, and additional medical treatment was needed. In our series of patients, we effectively performed the ligation of CPSS type II. However, appropriate timing of ligation is important to minimize complications, such as pulmonary hypertension. Further study is needed to clarify the most appropriate timing of repair for CPSS and to improve the prognosis of CPSS patients.