• Mei Diao, Long Li, Jin-Shan Zhang, Mao Ye, and Wei Cheng.
• *Department of Pediatric Surgery, Capital Institute of Pediatrics, Beijing, P. R. China †Department of Paediatric Surgery, Monash Children's, Monash Medical Center, Southern Health, Department of Paediatrics and Department of Surgery, Southern Medical School, Faculty of Medicine, Nursing and Health Sciences, Monash University, Clayton, Victoria, Australia.
• Ann. Surg.. 2013 Dec 1;258(6):1028-33.

ObjectiveConventionally, liver transplantation, ileoileal bypass, and partial external or internal biliary diversion are used in the treatment of progressive familial intrahepatic cholestasis (PFIC). However, postoperative recurrence, chronic diarrhea, and permanent stoma are the major concerns. We present a novel approach of laparoscopic cholecystocolostomy with antireflux Y-loop for the management of children with PFIC.MethodsBetween August 2003 and April 2011, 20 children with PFIC (median age: 1.47 years; range: 10.8 months to 5.11 years) successfully underwent laparoscopic cholecystocolostomies for bile diversions. Gallbladder was incised longitudinally for cholecystocolostomy. Transverse colon was divided proximal to splenic flexure. End-to-side anastomosis was established between distal transverse colon and mid-descending colon. The mobilized splenic flexure and proximal descending colon, that is, the stem of the Y-loop, was anastomosed to the gallbladder.ResultsThe mean operative time was 2.02 ± 0.18 hours (range: 2-2.5 hours). The mean postoperative hospital stay was 8 days (range: 5-10 days). Average time for full resumption of diet was 3 days (range: 2-4 days). Average Y-loop length was 17.65 cm (range: 15-20 cm). The median follow-up period was 54 months (range: 12-104 months). All patients were jaundice free after 7 to 20 days and pruritus subsided in 3 to 14 days. Liver function parameters significantly improved postoperatively. Success rate (normalization of serum bile acids at postoperative 12 months) was 85%. No mortality or morbidities associated with diarrhea, cholangitis, or intrahepatic reflux were observed.ConclusionsThe novel approach of laparoscopic cholecystocolostomy offers a safe and effective treatment option for PFIC in children with good success rates and minimal morbidity.

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