Laparoscopic cholecystocolostomy: a novel surgical approach for the treatment of progressive familial intrahepatic cholestasis.

OBJECTIVE

Conventionally, 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.

METHODS

Between 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.

RESULTS

The 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.

CONCLUSIONS

The novel approach of laparoscopic cholecystocolostomy offers a safe and effective treatment option for PFIC in children with good success rates and minimal morbidity.