Skip to main content
SpringerLink
Log in

Intrahepatische Gallengangsteine und Gallengangmissbildungen

Intrahepatic choleductolithiasis and bile duct malformations

  • Schwerpunkt
  • Published:
Der Gastroenterologe Aims and scope

Zusammenfassung

Bei rezidivierenden Cholangitiden sollten intrahepatische Gallengangsteine differenzialdiagnostisch berücksichtigt werden. Ein intrahepatisches Gallengangsteinleiden ist mit einem deutlich erhöhten Risiko für die Entwicklung eines cholangiozellulären Karzinoms vergesellschaftet. Mit Ultraschall, CT und MRT allein können Cholangiokarzinome bei Patienten mit intrahepatischen Gallengangsteinen häufig nur schwer von einer peribiliären Entzündung unterschieden werden. Primäres Therapieziel bei intrahepatischen Gallengangsteinen ist die Elimination von Cholangitisschüben, die Beseitigung von Galleabflussstörungen und die Vermeidung der Entwicklung einer sekundär biliären Zirrhose und eines Cholangiokarzinoms. Abhängig von Ausdehnung und Befallsmuster kommen interventionell-endoskopische oder chirurgische Verfahren zum Einsatz, adjuvante medikamentöse Ansätze zur Rezidivprophylaxe befinden sich in der Entwicklung. Bei Gallengangmissbildungen (z. B. bilobäre Caroli-Erkrankung, Caroli-Syndrom) sollte frühzeitig eine Lebertransplantation angestrebt werden.

Abstract

In cases of recurrent cholangitis, hepatolithiasis should be considered in the differential diagnosis. Intrahepatic bile duct stones are associated with a significantly elevated risk for the development of cholangiocarcinoma. In cases of hepatolithiasis a differential diagnosis between cholangiocarcinoma and peribiliary inflammation may be difficult by ultrasound, CT or MRI imaging alone. The primary therapeutic goal in patients with hepatolithiasis is the elimination of cholangitis and bile stasis and avoidance of the development of secondary biliary cirrhosis or cholangiocarcinoma. Dependent on localization and extent of hepatolithiasis, interventional endoscopic as well as surgical therapeutic strategies may be chosen. Adjuvant pharmacologic prevention of recurrence of bile duct stones is under development. In cases of bile duct malformations, such as bilobar Caroli’s disease or Caroli’s syndrome, liver transplantation should be considered at an early stage. An interdisciplinary approach is of vital importance.

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

Access this article

Log in via an institution

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

Instant access to the full article PDF.

Institutional subscriptions

Abb. 1
Abb. 2
Abb. 3

Literatur

  1. Ammori BJ, Jenkins BL, Lim PC et al. (2002) Surgical strategy for cystic diseases of the liver in a western hepatobiliary center. World J Surg 26: 462–469

    Article  PubMed  Google Scholar 

  2. Balsells J, Margarit C, Murio E et al. (1993) Adenocarcinoma in Caroli’s disease treated by liver transplantation. HPB Surg 7: 81–86 (discussion 86–87)

    Article  PubMed  CAS  Google Scholar 

  3. Chijiiwa K, Ichimiya H, Kuroki S et al. (1993) Late development of cholangiocarcinoma after the treatment of hepatolithiasis. Surg Gynecol Obstet 177: 279–282

    PubMed  CAS  Google Scholar 

  4. Desmet VJ (1992) Congenital diseases of intrahepatic bile ducts: variations on the theme „ductal plate malformation“. Hepatology 16: 1069–1083

    Article  PubMed  CAS  Google Scholar 

  5. Holzinger F, Z’Graggen K, Büchler MW (1999) Mechanisms of biliary carcinogenesis: a pathogenetic multi-stage cascade towards cholangiocarcinoma. Ann Oncol (Suppl 4) 10: 122–126

    Google Scholar 

  6. Huang MH, Chen CH, Yang JC et al. (2003) Long-term outcome of percutaneous transhepatic cholangioscopic lithotomy for hepatolithiasis. Am J Gastroenterol 98: 2589–2590

    Google Scholar 

  7. Jan YY, M.F. Chen MF, Wang CS et al. (1996) Surgical treatment of hepatolithiasis: long-term results. Surgery 120: 509–514

    Article  PubMed  CAS  Google Scholar 

  8. Kasper HU, Stippel DL, Töx U et al. (2006) Primäres Cholangiokarzinom auf dem Boden einer Caroli-Erkrankung: Fallbericht und Literaturübersicht. Pathologe 27: 300–304

    Article  PubMed  Google Scholar 

  9. Kim TK, Kim BS, Kim JH et al. (2002) Diagnosis of intrahepatic stones: superiority of MR cholangiopancreatography over endoscopic retrograde cholangiopancreatography. AJR Am J Roentgenol 179: 429–434

    PubMed  Google Scholar 

  10. Lee SK, Seo DW, Myung SJ et al. (2001) Percutaneous transhepatic cholangioscopic treatment for hepatolithiasis: an evaluation of long-term results and risk factors for recurrence, Gastrointest Endosc 53: 318–323

    Google Scholar 

  11. Lindström CG (1977) Frequency of gallstone disease in a well-defined Swedish population. A prospective necropsy study in Malmö. Scand J Gastroenterol 12: 341–346

    Article  PubMed  Google Scholar 

  12. Maki T (1966) Pathogenesis of calcium bilirubinate gallstone: role of E. coli, beta-glucuronidase and coagulation by inorganic ions, polyelectrolytes and agitation. Ann Surg 164: 90–100

    Article  PubMed  CAS  Google Scholar 

  13. Matsushiro T, Suzuki N, Sato T, Maki T (1977) Effects of diet on glucaric acid concentration in bile and the formation of calcium bilirubinate gallstones. Gastroenterology 72(Pt 1):630–633

    PubMed  CAS  Google Scholar 

  14. Mori T, Sugiyama M, Atomi Y (2006) Gallstone disease: Management of intrahepatic stones. Best Pract Res Clin Gastroenterol 20: 1117–1137

    Article  PubMed  Google Scholar 

  15. Ohta T, Nakagawa T, Yoshimitsu Y et al. (1993) The role of 16,16-dimethyl prostaglandin E2 on the intrahepatic biliary branches in dogs. Hepatology 17: 1062–1065

    PubMed  CAS  Google Scholar 

  16. Parada LA, Hallen M, Hagerstrand I et al. (1999) Clonal chromosomal abnormalities in congenital bile duct dilatation (Caroli’s disease). Gut 45: 780–782

    Article  PubMed  CAS  Google Scholar 

  17. Park MS, Yu JS, Kim KW et al. (2001) Recurrent pyogenic cholangitis: comparison between MR cholangiography and direct cholangiography. Radiology 220: 677–682

    Article  PubMed  CAS  Google Scholar 

  18. Ros E, Navarro S, Bru C et al. (1993) Ursodeoxycholic acid treatment of primary hepatolithiasis in Caroli’s syndrome. Lancet 342: 404–406

    Article  PubMed  CAS  Google Scholar 

  19. Shoda J, He BF, Tanaka N et al. (1995) Primary dual defect of cholesterol and bile acid metabolism in liver of patients with intrahepatic calculi. Gastroenterology 108: 1534–1546

    Article  PubMed  CAS  Google Scholar 

  20. Shoda J, Kano M, Asano T et al. (1999) Secretory low molecular weight phospholipase A2 and their specific receptor in bile ducts of patients with intrahepatic calculi-factors of pathophysiological significance for chronic proliferative cholangitis. Hepatology 29: 1026–1036

    Article  PubMed  CAS  Google Scholar 

  21. Shoda J, Oda K, Suzuki H et al. (2001) Etiological significance of Metabolic defects of cholesterol, phospholipid, and bile acid in the liver of patients with intrahepatic calculi. Hepatology 33: 1194–1205

    Article  PubMed  CAS  Google Scholar 

  22. Shoda J, Tanaka N, Osuga T (2003) Hepatolithiasis – epidemiology and pathogenesis update. Front Biosci 8: e398–409

    Article  PubMed  CAS  Google Scholar 

  23. Smith AJ, Vree JML de, Ottenhoff R et al. (1998) Hepatocyte-specific expression of the human MDR3 P-glycoprotein gene restores the biliary phosphatidylcholine excretion absent in mdr2 (-/-). Hepatology 28: 530–536

    Article  PubMed  CAS  Google Scholar 

  24. Su CH, Shyr YM, Lui WY, P’Eng FK (1997) Hepatolithiasis associated with cholangiocarcinoma. Br J Surg 84: 969–973

    Article  PubMed  CAS  Google Scholar 

  25. Tanimura H, Ishimoto K, Uchiyama K (1998) Epidemiological status of hepatolithiasis. J Biliary Tract Pancreas 19: 1015–1020

    Google Scholar 

  26. Taylor AC, Palmer KR (1998) Caroli’s disease. Eur J Gastroenterol Hepatol 10: 105–108

    Article  PubMed  CAS  Google Scholar 

  27. Tazuma S (2006) Gallstone disease: Epidemiology, pathogenesis, and classification of biliary stones (common bile duct and intrahepatic). Best Pract Res Clin Gastroenterol 20: 1075–1083

    Article  PubMed  Google Scholar 

  28. Tomida S, Abei M, Yamaguchi T et al. (1999) Long-term ursodeoxycholic acid therapy is associated with reduced risk of biliary pain and acute cholecystitis in patients with gallbladder stones: a Cohort analysis. Hepatology 30: 6–13

    Article  PubMed  CAS  Google Scholar 

  29. Valls C, Guma A, Puig I et al. (2000) Intrahepatic peripheral cholangiocarcinoma: CT evaluation. Abdom Imaging 25: 490–496

    Article  PubMed  CAS  Google Scholar 

  30. Yeh CN, Jan YY, Chen MF (2006) Hepatectomy for peripheral cholangiocarcinoma in elderly patients. Ann Surg Oncol 13: 1553–1559

    Article  PubMed  Google Scholar 

Download references

Interessenkonflikt

Der korrespondierende Autor gibt an, dass kein Interessenkonflikt besteht.

Author information

Authors and Affiliations

  1. Klinik für Allgemein-, Viszeral- und Transplantationschirurgie, Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625, Hannover, Deutschland

    H. Schrem, H. Bektas & J. Klempnauer

  2. Klinik für Gastroenterologie, Hepatologie und Endokrinologie, Medizinische Hochschule Hannover, Hannover, Deutschland

    J. Wedemeyer & M.P. Manns

Authors
  1. H. Schrem
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. J. Wedemeyer
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. H. Bektas
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. M.P. Manns
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. J. Klempnauer
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to H. Schrem.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Schrem, H., Wedemeyer, J., Bektas, H. et al. Intrahepatische Gallengangsteine und Gallengangmissbildungen. Gastroenterologe 3, 14–21 (2008). https://doi.org/10.1007/s11377-007-0139-9

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11377-007-0139-9

Schlüsselwörter

Keywords

Search

Navigation