Intrahepatic biliary cyst after a Kasai procedure

A 30-day-old female infant presented with a cystic lesion at the porta hepatis. The cyst was first detected on a prenatal ultrasound at 16 weeks of gestation and diagnosed as congenital biliary dilatation. The size of the cyst did not change over the course of gestation and the infant's liver function was stable after birth, although she had a persisting yellowish stool. Surgical cholangiography was performed at 30 days, and a complete obstruction of the hepatic hilum was identified as a type IIId cyst. However, cholangitis recurred at 10 months post-surgery, with marked dilation of the right and left hepatic ducts. Right cyst-intestinal anastomosis and left cyst-gastric anastomosis were performed. Cyst recurrence is sometimes a complication in patients with type III biliary atresia who undergo the Kasai procedure. The mechanisms of cyst formation are still unknown, and there is no consensus regarding the treatment. Our case suggests that cysto-enterstomy is a feasible treatment for recurrent cysts.