Laparoscopic partial external biliary diversion procedure in progressive familial intrahepatic cholestasis: a new approach.

摘要

BACKGROUND: Progressive familial intrahepatic cholestasis results in fibrosis, cirrhosis, and liver insufficiency if untreated. Medical therapy often fails and partial external biliary diversion has been recommended to prevent early liver transplantation. We present a new technique of performing a laparoscopic partial external biliary diversion and report our experience in a first series of infants. METHODS: From October to November 2004, four consecutive patients with progressive familial intrahepatic cholestasis underwent the laparoscopic partial biliary diversion procedure. A three-trocar technique was used. A proximal jejunal conduit was constructed after exteriorization of the small bowel via the infraumbilical trocar incision. After repositioning of the bowel, an isoperistaltic cholecystojejunostomy was carried out laparoscopically. The distal jejunal conduit was placed as a stoma at the right abdominal trocar site. RESULTS: There were no intraoperative events. The mean duration of the operation was 156.5 min. The postoperative course was uneventful in all patients with full enteral feedings on day 2. The laboratory and clinical signs of cholestasis were reduced up to a mean follow-up of 2 months (range, 1.5-2.5). CONCLUSION: The laparoscopic partial biliary diversion procedure is feasible with all the benefits of minimally invasive surgery. Long-term results remain to be evaluated.