Skip to main content

Advertisement

SpringerLink
Log in

Portal cavernoma cholangiopathy: diagnosis, imaging, and intervention

  • Published:
Abdominal Radiology Aims and scope Submit manuscript

Abstract

The term portal cavernoma cholangiopathy refers to the biliary tract abnormalities that accompany extrahepatic portal vein obstruction (EHPVO) and subsequent cavernous transformation of the portal vein. EHPVO is a primary vascular disorder of the portal vein in children and adults manifested by longstanding thrombosis of the main portal vein. Nearly all patients with EHPVO have manifestations of portal cavernoma cholangiopathy, such as extrinsic indentation on the bile duct and mild bile duct narrowing, but the majority are asymptomatic. However, progressive portal cavernoma cholangiopathy may lead to severe complications, including secondary biliary cirrhosis. A spectrum of changes is seen radiologically in the setting of portal cavernoma cholangiopathy, including extrinsic indentation of the bile ducts, bile duct stricturing, bile duct wall thickening, angulation and displacement of the extrahepatic bile duct, cholelithiasis, choledocholithiasis, and hepatolithiasis. Radiologists must be aware of this disorder in order to provide appropriate imaging evaluation and interpretation, to facilitate appropriate treatment and to distinguish this entity from its potential radiologic mimics.

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

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8
Fig. 9
Fig. 10

Similar content being viewed by others

References

  1. Condat B, Vilgrain V, Asselah T, et al. (2003) Portal cavernoma-associated cholangiopathy: a clinical and MR cholangiography coupled with MR portography imaging study. Hepatology 37(6):1302–1308. doi:10.1053/jhep.2003.50232

    Article  PubMed  Google Scholar 

  2. Dhiman RK, Saraswat VA, Valla DC, et al. (2014) Portal cavernoma cholangiopathy: consensus statement of a working party of the Indian national association for study of the liver. J Clin Exp Hepatol 4(Suppl 1):S2–S14. doi:10.1016/j.jceh.2014.02.003

    Article  PubMed  PubMed Central  Google Scholar 

  3. Besa C, Cruz JP, Huete A, Cruz F (2012) Portal biliopathy: a multitechnique imaging approach. Abdom Imaging 37(1):83–90. doi:10.1007/s00261-011-9765-2

    Article  PubMed  Google Scholar 

  4. Chandra R, Kapoor D, Tharakan A, Chaudhary A, Sarin SK (2001) Portal biliopathy. J Gastroenterol Hepatol 16(10):1086–1092

    Article  CAS  PubMed  Google Scholar 

  5. Sarin SK, Agarwal SR (2002) Extrahepatic portal vein obstruction. Semin Liver Dis 22(1):43–58. doi:10.1055/s-2002-23206

    Article  PubMed  Google Scholar 

  6. Arora A, Sarin SK (2015) Multimodality imaging of primary extrahepatic portal vein obstruction (EHPVO): what every radiologist should know. Br J Radiol 88(1052):20150008. doi:10.1259/bjr.20150008

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  7. Khanna R, Sarin SK (2014) Non-cirrhotic portal hypertension—diagnosis and management. J Hepatol 60(2):421–441. doi:10.1016/j.jhep.2013.08.013

    Article  PubMed  Google Scholar 

  8. Walser EM, Runyan BR, Heckman MG, et al. (2011) Extrahepatic portal biliopathy: proposed etiology on the basis of anatomic and clinical features. Radiology 258(1):146–153. doi:10.1148/radiol.10090923

    Article  PubMed  Google Scholar 

  9. Chawla YK, Bodh V (2015) Portal vein thrombosis. J Clin Exp Hepatol 5(1):22–40. doi:10.1016/j.jceh.2014.12.008

    Article  PubMed  PubMed Central  Google Scholar 

  10. Dhiman RK, Puri P, Chawla Y, et al. (1999) Biliary changes in extrahepatic portal venous obstruction: compression by collaterals or ischemic? Gastrointest Endosc 50(5):646–652

    Article  CAS  PubMed  Google Scholar 

  11. Dilawari JB, Chawla YK (1992) Pseudosclerosing cholangitis in extrahepatic portal venous obstruction. Gut 33(2):272–276

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  12. Khuroo MS, Yattoo GN, Zargar SA, et al. (1993) Biliary abnormalities associated with extrahepatic portal venous obstruction. Hepatology 17(5):807–813

    Article  CAS  PubMed  Google Scholar 

  13. Sezgin O, Oguz D, Altintas E, Saritas U, Sahin B (2003) Endoscopic management of biliary obstruction caused by cavernous transformation of the portal vein. Gastrointest Endosc 58(4):602–608

    Article  PubMed  Google Scholar 

  14. Dhiman RK, Behera A, Chawla YK, Dilawari JB, Suri S (2007) Portal hypertensive biliopathy. Gut 56(7):1001–1008. doi:10.1136/gut.2006.103606

    Article  PubMed  Google Scholar 

  15. Agarwal AK, Sharma D, Singh S, Agarwal S, Girish SP (2011) Portal biliopathy: a study of 39 surgically treated patients. HPB (Oxford) 13(1):33–39. doi:10.1111/j.1477-2574.2010.00232.x

    Article  Google Scholar 

  16. Chattopadhyay S, Govindasamy M, Singla P, et al. (2012) Portal biliopathy in patients with non-cirrhotic portal hypertension: does the type of surgery affect outcome? HPB (Oxford) 14(7):441–447. doi:10.1111/j.1477-2574.2012.00473.x

    Article  Google Scholar 

  17. Oo YH, Olliff S, Haydon G, Thorburn D (2009) Symptomatic portal biliopathy: a single centre experience from the UK. Eur J Gastroenterol Hepatol 21(2):206–213. doi:10.1097/MEG.0b013e3283060ee8

    Article  PubMed  Google Scholar 

  18. Khare R, Sikora SS, Srikanth G, et al. (2005) Extrahepatic portal venous obstruction and obstructive jaundice: approach to management. J Gastroenterol Hepatol 20(1):56–61. doi:10.1111/j.1440-1746.2004.03528.x

    Article  PubMed  Google Scholar 

  19. Kumar A, Sharma P, Arora A (2015) Review article: portal vein obstruction–epidemiology, pathogenesis, natural history, prognosis and treatment. Aliment Pharmacol Ther 41(3):276–292. doi:10.1111/apt.13019

    Article  CAS  PubMed  Google Scholar 

  20. Valla DC (2008) Thrombosis and anticoagulation in liver disease. Hepatology 47(4):1384–1393. doi:10.1002/hep.22192

    Article  CAS  PubMed  Google Scholar 

  21. Rajekar H, Vasishta RK, Chawla YK, Dhiman RK (2011) Noncirrhotic portal hypertension. J Clin Exp Hepatol 1(2):94–108. doi:10.1016/S0973-6883(11)60128-X

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  22. Uflacker R (2003) Applications of percutaneous mechanical thrombectomy in transjugular intrahepatic portosystemic shunt and portal vein thrombosis. Tech Vasc Interv Radiol 6(1):59–69. doi:10.1053/tvir.2003.36433

    Article  PubMed  Google Scholar 

  23. Saint JH (1961) The epicholedochal venous plexus and its importance as a means of identifying the common duct during operations on the extrahepatic biliary tract. Br J Surg 48:489–498

    Article  CAS  PubMed  Google Scholar 

  24. Kalra N, Shankar S, Khandelwal N (2014) Imaging of portal cavernoma cholangiopathy. J Clin Exp Hepatol 4(Suppl 1):S44–S52. doi:10.1016/j.jceh.2013.07.004

    Article  PubMed  Google Scholar 

  25. He ZP, Fan LJ (2002) Diagnosis and treatment of portal biliopathy. Hepatobiliary Pancreat Dis Int 1(4):581–586

    PubMed  Google Scholar 

  26. Lohr JM, Kuchenreuter S, Grebmeier H, Hahn EG, Fleig WE (1993) Compression of the common bile duct due to portal-vein thrombosis in polycythemia vera. Hepatology 17(4):586–592

    Article  CAS  PubMed  Google Scholar 

  27. Wani ZA, Bhat RA, Bhadoria AS, Maiwall R (2015) Extrahepatic portal vein obstruction and portal vein thrombosis in special situations: need for a new classification. Saudi J Gastroenterol 21(3):129–138. doi:10.4103/1319-3767.157550

    Article  PubMed  PubMed Central  Google Scholar 

  28. Llop E, de Juan C, Seijo S, et al. (2011) Portal cholangiopathy: radiological classification and natural history. Gut 60(6):853–860. doi:10.1136/gut.2010.230201

    Article  PubMed  Google Scholar 

  29. Marin D, Galluzzo A, Plessier A, et al. (2011) Focal nodular hyperplasia-like lesions in patients with cavernous transformation of the portal vein: prevalence, MR findings and natural history. Eur Radiol 21(10):2074–2082. doi:10.1007/s00330-011-2161-z

    Article  PubMed  Google Scholar 

  30. Shin SM, Kim S, Lee JW, et al. (2007) Biliary abnormalities associated with portal biliopathy: evaluation on MR cholangiography. AJR Am J Roentgenol 188(4):W341–W347. doi:10.2214/AJR.05.1649

    Article  PubMed  Google Scholar 

  31. Aguirre DA, Farhadi FA, Rattansingh A, Jhaveri KS (2012) Portal biliopathy: imaging manifestations on multidetector computed tomography and magnetic resonance imaging. Clin Imaging 36(2):126–134. doi:10.1016/j.clinimag.2011.07.001

    Article  PubMed  Google Scholar 

  32. Varma V, Behera A, Kaman L, Chattopadhyay S, Nundy S (2014) Surgical management of portal cavernoma cholangiopathy. J Clin Exp Hepatol 4(Suppl 1):S77–S84. doi:10.1016/j.jceh.2013.07.005

    Article  PubMed  Google Scholar 

  33. Menias CO, Surabhi VR, Prasad SR, et al. (2008) Mimics of cholangiocarcinoma: spectrum of disease. Radiographics 28(4):1115–1129. doi:10.1148/rg.284075148

    Article  PubMed  Google Scholar 

  34. Perlemuter G, Bejanin H, Fritsch J, et al. (1996) Biliary obstruction caused by portal cavernoma: a study of 8 cases. J Hepatol 25(1):58–63

    Article  CAS  PubMed  Google Scholar 

  35. Chawla Y, Duseja A, Dhiman RK (2009) Review article: the modern management of portal vein thrombosis. Aliment Pharmacol Ther 30(9):881–894. doi:10.1111/j.1365-2036.2009.04116.x

    Article  CAS  PubMed  Google Scholar 

  36. Thervet L, Faulques B, Pissas A, et al. (1993) Endoscopic management of obstructive jaundice due to portal cavernoma. Endoscopy 25(6):423–425. doi:10.1055/s-2007-1009120

    Article  CAS  PubMed  Google Scholar 

  37. Garcia-Pagan JC, Hernandez-Guerra M, Bosch J (2008) Extrahepatic portal vein thrombosis. Semin Liver Dis 28(3):282–292. doi:10.1055/s-0028-1085096

    Article  PubMed  Google Scholar 

  38. Hymes JL, Haicken BN, Schein CJ (1977) Varices of the common bile duct as a surgical hazard. Am Surg 43(10):686–688

    CAS  PubMed  Google Scholar 

  39. Mork H, Weber P, Schmidt H, Goerig RM, Scheurlen M (1998) Cavernous transformation of the portal vein associated with common bile duct strictures: report of two cases. Gastrointest Endosc 47(1):79–83

    Article  CAS  PubMed  Google Scholar 

  40. Chaudhary A, Dhar P, Sarin SK, et al. (1998) Bile duct obstruction due to portal biliopathy in extrahepatic portal hypertension: surgical management. Br J Surg 85(3):326–329. doi:10.1046/j.1365-2168.1998.00591.x

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Radiology, Virginia Commonwealth University Medical Center, 1250 East Marshall Street, PO Box Number 980615, Richmond, VA, 23298, USA

    Lauren N. Moomjian & Sarah G. Winks

Authors
  1. Lauren N. Moomjian
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Sarah G. Winks
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Lauren N. Moomjian.

Ethics declarations

Funding

No funding was received for this study.

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

This article does not contain any studies with human participants or animals performed by any of the authors.

Informed consent

Statement of informed consent was not applicable since the manuscript does not contain any patient data.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Moomjian, L.N., Winks, S.G. Portal cavernoma cholangiopathy: diagnosis, imaging, and intervention. Abdom Radiol 42, 57–68 (2017). https://doi.org/10.1007/s00261-016-0957-7

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00261-016-0957-7

Keywords

Search

Navigation