BackgroundEndoscopic injection sclerotherapy (EIS) is a life-saving procedure for pediatric patients with bleeding gastric varices (GV) associated with advanced liver cirrhosis and severe portal hypertension. Because of the lack of an endoscopic banding ligation device for pediatric patients, EIS is usually performed for bleeding esophageal varices (EV) in infants with congenital biliary atresia.Case presentationWe present a case of a 15-month-old female infant with type I biliary atresia with jaundice (total serum bilirubin, 22.2 mg/dL), hypoalbuminemia (serum albumin level, 2.58 g/dL), coagulopathy (prothrombin time > 20 s compared with that of a normal control), ascites, splenomegaly, portal hypertension (portal vein velocity, 3.9–5.6 cm/sec of hepatopetal flow), and repeated bleeding of the varices after receiving three doses of intravascularly administered Histoacryl 1 ampoule mixed with Lipiodol UF 8 mL in the EV. Prominent GV and EV were occluded by EIS. The sclerosing agent was also present in the main portal vein, splenic mesenteric junction, and splenic vein, causing an engorged inferior mesenteric vein. The patient underwent total hepatectomy and living donor liver transplantation (LDLT) by left lateral segment graft (segments 2, 3, and 4 of the middle hepatic vein trunk) and left portal vein graft to the recipient inferior mesenteric vein anastomosis. Portal vein stent placement via segment 4 of the portal vein stump was performed from the inferior mesenteric vein to the umbilical portion of the left portal vein. The patient is still alive and doing well after the LDLT.ConclusionsEIS is a life-saving procedure in cases involving bleeding EV complicated by gastric, main portal vein, splenic mesenteric junction, and splenic vein occlusions; hence, it should be kept in mind as a treatment for EV complications in pediatric patients.