Internal surgical drainage for bile lakes following hepatoportoenterostomy for biliary atresia

Bile lakes may be present at time of diagnosis or develop later following hepatoportoenterostomy (HPE) in patients with biliary atresia (BA). We report a patient with recurrent cholangitis following HPE who developed multiple large biliary cystic lesions. Percutaneous drainage resulted in resolution of jaundice and cholangitis but capping of drains resulted in recurrence of symptoms. Therefore, internal drainage of the bile lakes was accomplished using cystjejunostomy to the roux limb of the previous portoenterostomy. At 18 months of follow-up, the patient has remained jaundice free with only one recurrent cholangitis episode which occurred after stopping her antibiotic prophylaxis. Although this patient will likely require liver transplantation in the next few years, this case suggests that cystjejunostomy may be a viable surgical option to improve bile flow and prevent cholangitis in patients who develop bile lakes following HPE. Keywords: Bile lake, Biliary atresia, Cystjejunostomy