Laparoscopic button cholecystostomy for progressive familial intrahepatic cholestasis in two children

Eur J Pediatr Surg. 2014 Oct;24(5):433-6. doi: 10.1055/s-0033-1360457. Epub 2013 Dec 10.

Abstract

Background: Untreated, progressive familial intrahepatic cholestasis (PFIC) results in fibrosis, cirrhosis, and liver failure. It has been shown that partial external biliary diversion (PEBD) may prevent from liver transplantation in patients without cirrhosis. The aim of this study is to present a new laparoscopic technique using a button instead of a bowel conduit for PEBD.

Patients and methods: Two boys with PFIC (patient 1, 17 months; patient 2, 12 years) underwent laparoscopic button cholecystostomy using a 3-trocar technique by insertion of a 14 French MIC KEY button (Kimberly-Clark Worldwide, Inc, Draper, Utah, United States) at the gallbladder fundus secured with two absorbable purse-string sutures. Beside the suitability of the procedure, end points included course of serum bile acids, total bilirubin, liver enzymes, and pruritus at a follow-up of 6 months.

Results: No complications related to the operation occurred. Relieve of pruritus was achieved in both the children, due to adequate bile drainage during a follow-up period of 6 months. In patient 2, a 10-mm gallstone was removed simultaneously. In patient 1, serum bile acids decreased from 12.3 to 6.6 µmol/L and in patient 2, serum bile acids decreased from 106.3 to 2.9 µmol/L. Total bilirubin, aspartate amino transferase, alanine amino transferase, and gamma-glutamyltransferase are kept in normal ranges during follow-up. Patient's and parent's acceptance with the button was excellent.

Conclusion: Laparoscopic button cholecystostomy is a simple, safe, and sufficient technique for PEBD in patients with PFIC. It achieves an adequate bile flow with consecutive relief of pruritus and avoids an enteric anastomosis.

MeSH terms

  • Bile
  • Bile Acids and Salts / blood
  • Child
  • Cholecystostomy / methods*
  • Cholestasis, Intrahepatic / blood
  • Cholestasis, Intrahepatic / complications
  • Cholestasis, Intrahepatic / surgery*
  • Drainage
  • Gallstones / surgery
  • Humans
  • Infant
  • Laparoscopy / methods*
  • Male
  • Pruritus / etiology

Substances

  • Bile Acids and Salts

Supplementary concepts

  • Cholestasis, progressive familial intrahepatic 1