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Cholecystocolonic aananstomosis for treatment of progressive familial intrahepatic cholestasis: a primary study

Objective To describe the initial experience with a novel approach,cholecystocolonic Y-anastomosis partial bile shunt for the surgical treatment of progressive familial intrahepatic cholestasis (PFIC,Byler's disease).Methods Between August 2003 and December 2010,eight patients with PFIC (7 male and 1 female),aged 12 to 69 months old (mean age,31.5 months old),underwent cholecystocolonic Y-anastomosis for treatment of incontrollable pruritus and jaundice.They all had hyperbilirubinemia (serum total bilirubin concentration 51.5-185.5 μmol/L) and high serum bile acid (serum total bile acid concentration 251.8-528 μmol/L) ; one patient only showed itching and high serum bile acid (serum total bile acid concentration 376.5 μmol/L),and normal serum bilirubin (6.2 μmol/L).The surgical procedure involved the creation of a Y-type transverse colonic conduit,anastomosed proximally to the gallbalder to keep the continuity with the descending colon.The ascending colon was anastomosed to the descending colon.Results Eight patients showed normal extrahepatic bile duct diameter and morphology via intraoperative angiography during the surgery.Among them,two were completed by open surgery,and six were finished by laparoscopic surgery.The average length of the colonic conduits was 20.6 cm (ranged from 14 to 25.5 cm).Postoperatively,all eight children had relief from their jaundice within 7 to 20 days and from pruritus within 3 days to 2 weeks.During 3 to 88 months follow-up period,the liver functional parameters (AST and ALT) kept normal and no diarrhea and cholangitis occurred.Conclusions Cholecystocolonic ananstomosis for PFIC treatment is safe and effective for the surgical treatment of PFIC,to relieve the jaundice and pruritus and promote the liver funcation. 
 
Key words: 
Cholestasis;  intrahepatic; Anastomosis, Roux-en-Y