[Bile lake post Kasai hepatoportoenterostomy in biliary atresia].

In this case report we describe a boy with extrahepatic biliary atresia who underwent a Kasai hepatoportoenterostomy at six weeks of age. Beginning several weeks post-op, he had recurrent cholangitis inadequately controlled by various antibiotic prophylaxis regimens. Imaging revealed the development of several bile lakes in the liver hilum. Due to the recurrent nature of his cholangitis, and some evidence of acutely impaired biliary drainage, he underwent a refashioning of his portoenterostomy with resultant improved drainage. However, shortly thereafter, the patient developed ongoing fever and anemia. Culture of the bile lake aspirate grew multiresistant Klebsiella and a 6 week course of parenteral meropenume controlled his fever and his anemia improved. Following treatment cessation his fever and anemia returned. A biliary drain was inserted into his larger bile lake and following another course of parenteral antibiotics he has remained free of clinically detected cholangitis despite ongoing contamination of drained bile fluid. The development of bile lakes after Kasai hepatoportoenterostomy is not an uncommon finding. This have been associated with worse prognosis including increased incidence of cholangitis. Often, conservative treatment with prophylactic antibiotics suffices, however, in rare cases, more aggressive intervention may be considered including percutaneous bile drainage or surgical management. The benefit of these management strategies must be balanced with the potential gain regarding quality of life and delaying transplant, on an individual basis.