Skip to main content
Log in

Laparoscopic partial external biliary diversion procedure in progressive familial intrahepatic cholestasis

A new approach

  • Published:
Surgical Endoscopy And Other Interventional Techniques Aims and scope Submit manuscript

Abstract

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Cohran VC, Heubi JE (2003) Treatment of pediatric cholestatic liver disease. Curr Treat Options Gastroenterol 6: 403–415

    PubMed  Google Scholar 

  2. Emond JC, Whitington PF (1995) Selective surgical management of progressive familial intrahepatic cholestasis (Byler’s disease). J Pediatr Surg 30: 1635–1641

    Article  PubMed  CAS  Google Scholar 

  3. Hollands CM, Rivera-Pedrogo FJ, Gonzalez-Vallina R, Loret-de-Mola O, Nahmad M, Burnweit CA (1998) Ileal exclusion for Byler’s disease: an alternative surgical approach with promising early results for pruritus. J Pediatr Surg 33: 220–224

    Article  PubMed  CAS  Google Scholar 

  4. Ismail H, Kalicinski P, Markiewicz M, Jankowska I, Pawlowska J, Kluge P, Eliadou E, Kaminski A, Szymczak M, Drewniak T, et al. (1999) Treatment of progressive familial intrahepatic cholestasis: liver transplantation or partial external biliary diversion. Pediatr Transplant 3: 219–224

    Article  PubMed  CAS  Google Scholar 

  5. Jacquemin E, Hermans D, Myara A, Habes D, Debray D, Hadchouel M, Sokal EM, Bernard O (1997) Ursodeoxycholic acid therapy in pediatric patients with progressive familial intrahepatic cholestasis. Hepatology 25: 519–523

    Article  PubMed  CAS  Google Scholar 

  6. Jansen PL, Muller MM (1998) Progressive familial intrahepatic cholestasis types 1, 2, and 3. Gut 42: 766–767

    Article  PubMed  CAS  Google Scholar 

  7. Kaliciński PJ, Ismail H, Jankowska I, Kamiński A, Pawłowska J, Drewniak T, Markiewicz M, Szymczak M (2003) Surgical treatment of progressive familial intrahepatic cholestasis: comparison of partial external biliary diversion and ileal bypass. Eur J Pediatr Surg 13: 307–311

    PubMed  Google Scholar 

  8. Kurbegov AC, Setchell KD, Haas JE, Mierau GW, Narkewicz M, Bancroft JD, Karrer F, Sokol RJ (2003) Biliary diversion for progressive familial intrahepatic cholestasis: improved liver morphology and bile acid profile. Gastroenterology 125: 1227–1234

    Article  PubMed  Google Scholar 

  9. Melter M, Rodeck B, Kardorff R, Hoyer PF, Petersen C, Ballauff A, Brodehl J (2000) Progressive familial intrahepatic cholestasis: partial biliary diversion normalizes serum lipids and improves growth in noncirrhotic patients. Am J Gastroenterol 95: 3522–3528

    Article  PubMed  CAS  Google Scholar 

  10. Soubrane O, Gauthier F, DeVictor D, Bernard O, Valayer J, Houssin D, Chapnis Y (1990) Orthotopic liver transplantation for Byler’s disease. Transplantation 50: 804–806

    PubMed  CAS  Google Scholar 

  11. Ure BM, Nustede R, Becker H (2005) Laparoscopic resection of congenital choledochal cyst, hepaticojejunostomy and external made Roux-en-Y anastomosis with subsequent adhesive small bowel obstruction. J Pediatr Surg (in press)

  12. Ure BM, Schier F, Schmidt AI, Nustede R, Petersen C, Jesch NK (2005) Laparoscopic resection of congenital choledochal cyst, choledochojejunostomy and extraabdominal Roux-en-Y anastomosis. Surg Endosc (in press)

  13. Whitington PF, Whitington GL (1988) Partial external diversion of bile for the treatment of intractable pruritus associated with intrahepatic cholestasis. Gastroenterology 95: 130–136

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to M. L. Metzelder.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Metzelder, M.L., Bottländer, M., Melter, M. et al. Laparoscopic partial external biliary diversion procedure in progressive familial intrahepatic cholestasis. Surg Endosc 19, 1641–1643 (2005). https://doi.org/10.1007/s00464-005-0035-7

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00464-005-0035-7

Keywords

Navigation