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Current Hepatology Reports

, Volume 17, Issue 4, pp 316–323 | Cite as

Portal Cavernoma Cholangiopathy

  • Dominique-Charles Valla
Portal Hypertension (J Gonzalez-Abraldes and E Tsochatzis, Section Editors)
  • 13 Downloads
Part of the following topical collections:
  1. Topical Collection on Portal Hypertension

Abstract

Purpose of Review

Portal cavernoma cholangiopathy is a rare condition, a proper recognition of which is critical. Solid data on this condition are scarce. This review aims at updating current knowledge on its definition, manifestations, diagnosis, and management.

Recent Findings

A consensus has been reached to prefer the denomination portal cavernoma cholangiopathy for the bile duct irregularities associated with portal cavernoma. Such irregularities are characterized by stenosis with or without dilatation and are mostly related to the impinging on bile duct lumen by portoportal collaterals. While bile duct irregularities are found in over 80% of patients with portal cavernoma, clinical manifestations, and complications (cholecystitis and biliary stones, but rarely cholestasis) occur in only 5–35% of them. Diagnosis can be and should be based on findings at magnetic resonance cholangiography and portography. Differential diagnosis includes primary and secondary sclerosing cholangitis, and cholangiocarcinoma. Asymptomatic patients may be managed expectantly. Endoscopic procedures are to be used in the first line for complications. Combined endoscopic and surgical approaches including portosystemic shunting can be considered in refractory or recurrent cases.

Summary

Portal cavernoma cholangiopathy mostly is a morphologic entity which is present in a majority of patients with portal cavernoma. The minority of patients with acute biliary complications should be treated as conservatively as possible. Late consequences of chronic cholestasis appear to be rare.

Keywords

Portal cholangiopathy Portal biliopathy Portal cavernoma Portal vein thrombosis Biliary pain Cholestasis 

Notes

Compliance with Ethical Standards

Conflicts of Interest

Dominique-Charles Valla declares no potential conflict of interest.

Human and Animal Rights and Informed Consent

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

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. 1.
    Chawla Y, Agrawal S. Portal cavernoma cholangiopathy – history, definition and nomenclature. J Clin Exp Hepatol. 2014;4:S15–7.CrossRefGoogle Scholar
  2. 2.
    •• Dhiman RK, Saraswat VA, Valla DC, Chawla Y, Behera A, Varma V, et al. Portal cavernoma cholangiopathy: consensus statement of a working party of the Indian National Association for the study of the liver. J Clin Exp Hepatol. 2014;4:S2–S14 A landmark paper for the definition, pathogenesis, manifestations, diagnosis, and treatment of portal cavernoma cholangiopathy.CrossRefGoogle Scholar
  3. 3.
    Bhatia V. Endoscopic retrograde cholangiography in portal cavernoma cholangiopathy – results from different studies and proposal for uniform terminology. J Clin Exp Hepatol. 2014;4:S37–43.CrossRefGoogle Scholar
  4. 4.
    Kalra N, Shankar S, Khandelwal N. Imaging of portal cavernoma cholangiopathy. J Clin Exp Hepatol. 2014;4:S44–52.CrossRefGoogle Scholar
  5. 5.
    Chandra R, Kapoor D, Tharakan A, Chaudhary A, Sarin SK. Portal biliopathy. J Gastroenterol Hepatol. 2001;16:1086–92.CrossRefGoogle Scholar
  6. 6.
    •• Llop E, de Juan C, Seijo S, Garcia-Criado A, Abraldes JG, Bosch J, et al. Portal cholangiopathy: radiological classification and natural history. Gut. 2011;60:853–60 A longitudinal study dissecting the natural history of the initial course of portal cavernoma cholangiopathy and its relationship with bile duct dilatation.CrossRefGoogle Scholar
  7. 7.
    • Puri P. Portal cavernoma cholangiopathy: Is it compression by collaterals or ischemic injury to bile ducts during portal vein thrombosis ? J Clin Exp Hepatol. 2014;4:S27–33 An excellent overview of the hypotheses on pathogenesis.CrossRefGoogle Scholar
  8. 8.
    • Rameshbabu CS, Sharma M. Biliary tract anatomy and its relationship with venous drainage. J Clin Exp Hepatol. 2014;4:S18–26 An excellent overview on the anatomy of the biliary system and its portal venous drainage.CrossRefGoogle Scholar
  9. 9.
    Sharma M, Rameshbabu CS. Portal cavernoma cholangiopathy: an endoscopic ultrasound based approach. J Clin Exp Hepatol. 2014;4:S53–61.CrossRefGoogle Scholar
  10. 10.
    Takagi T, Irisawa A, Shibukawa G, Hikichi T, Obara K, Ohira H. Intraductal ultrasonographic anatomy of biliary varices in patients with portal hypertension. Endosc Ultrasound. 2015;4:44–51.CrossRefGoogle Scholar
  11. 11.
    • Rai GP, Nijhawan S, Madhu MP, Sharma SS, Pokharna R. Comparative evaluation of magnetic resonance cholangiopancreatography/magnetic resonance splenoportography and endoscopic ultrasound in the diagnosis of portal cavernoma cholangiopathy. Indian J Gastroenterol. 2015;34:442–7 A study showing the increased sensitivity of endoscopic ultrasound to detect dilated portal collaterals within the wall of the bile ducts.CrossRefGoogle Scholar
  12. 12.
    Oh HC, Akisik F, Sherman S. Single-operator cholangioscopy for the diagnosis of portal cavernoma cholangiopathy. Clin Gastroenterol Hepatol. 2017;15:e57–8.CrossRefGoogle Scholar
  13. 13.
    Dhiman RK, Puri P, Chawla Y, Minz M, Bapuraj JR, Gupta S, et al. Biliary changes in extrahepatic portal vein obstruction: compression by collaterals or ischemic. Gastrointest Endosc. 1999;50:646–52.CrossRefGoogle Scholar
  14. 14.
    •• Condat B, Vilgrain V, Asselah T, O’Toole D, Rufat P, Zappa M, et al. Portal cavernoma-associated cholangiopathy: a clinical and MR cholangiography coupled with MR portography imaging study. Hepatology. 2003;37:1302–8 The study showing, by confronting MR cholangiography and MR portography, that bile duct irregularities at cholangiography are related to impinging on the bile duct lumen by the portoportal collaterals.CrossRefGoogle Scholar
  15. 15.
    • Vibert E, Azoulay D, Aloia T, Pascal G, Veilhan LA, Adam R, et al. Therapeutic strategies in symptomatic portal biliopathy. Ann Surg. 2007;246(1):97–104 A landmark retrospective survey on surgical therapy.CrossRefGoogle Scholar
  16. 16.
    • Agarwal AK, Sharma D, Sing S, Agarwal S, Girish SP. Portal biliopathy: a study of 39 surgically treated patients. HPB. 2011;13:33–9 Another landmark retrospective survey on surgical therapy.CrossRefGoogle Scholar
  17. 17.
    Dhiman RK, Singh P, Duseja A, et al. Pathogenesis of portal hypertensive biliopathy (PHB) is it compression by collaterals or ischemia? (Abstract). J Gastroenterol Hepatol. 2006;21:A506.Google Scholar
  18. 18.
    Huston DG, Pereira R, Zeppa R, Levy J, Schiff ER, Fink P. The fate of esophageal varices following selective distal splenorenal shunt. Ann Surg. 1976;183:496–501.CrossRefGoogle Scholar
  19. 19.
    Slieker JC, Farid WRR, van Eijck CHJ, Lange JF, van Bommel J, Metselaar HJ, et al. Significant contribution of the portal vein to blood flow through the common bile duct. Ann Surg. 2012;255:523–7.CrossRefGoogle Scholar
  20. 20.
    • Chiu B, Superina R. Extrahepatic portal vein thrombosis is associated with an increased incidence of cholelithisasis. J Pediatr Surg. 2004;39:1059–61 The first and best documented study on an increased prevalence of choletlithiasis in patients with portal cavernoma cholangiopathy.CrossRefGoogle Scholar
  21. 21.
    Harmanci A, Bayraktar Y. How can portal vein cavernous transformation cause chronic incomplete biliary obstruction? World J Gastroenterol. 2012;18:3375–8.CrossRefGoogle Scholar
  22. 22.
    • Jabeen S, Robani I, Choh NA, Ashraf O, Shaheen F, Gojwari T, et al. Spectrum of biliary abnormalities in portal cavernoma cholangiopathy (PCC) secondary to idiopathic extrahepatic portal vein obstruction (EHVPO) – a prospective magnetic resonance cholangiopancreatography (MRCP) based study. Br J Radiol. 2016;89:2016036 A comprehensive MR study on biliary anomalies in patients with portal cavernoma.CrossRefGoogle Scholar
  23. 23.
    Eto T. Gallstone formation in dogs after selective occlusion of the portal vein branches. Jpn J Surg. 1988;18:268–75.CrossRefGoogle Scholar
  24. 24.
    • Shukla A, Gupte A, Kavir V, Dhore P, Bhatia S. Long term outcomes of patients with significant biliary obstruction due to portal cavernoma cholangiopathy and extrahepatic portal vein obstruction (EHVPO) with no shuntable veins. J Clin Exp Hepatol. 2017;7:328–33 One of the rare papers with prolonged follow-up of patients with portal cholangiopathy treated in a conservative manner because no shunt surgery was deemed feasible. CrossRefGoogle Scholar
  25. 25.
    Adachi Y, Kamisako T, Yamamoto T. The effects of temporary occlusion of the superior mesenteric vein or splenic vein on biliary bilirubin and bile excretion in rats. J Lab Clin Med. 1991;118:261–8.PubMedGoogle Scholar
  26. 26.
    Duseja A. Portal cavernoma cholangiopathy – clinical characteristics. J Clin Exp Hepatol. 2014;4:S34–6.PubMedGoogle Scholar
  27. 27.
    •• Franceschet I, Zanetto A, Ferrarese A, Burra P, Senzolo M. Therapeutic approaches for portal biliopathy: a systematic review. World J Gastroenterol. 2016;22:9909–20 The only systematic review on therapy for portal cavernoma cholangiopathy.CrossRefGoogle Scholar
  28. 28.
    Khare R, Sikora SS, Srikanth G, Choudhuri G, Saraswat VA, Kumar A, et al. Extrahepatic portal venous obstruction and obstructive jaundice: approach to management. J Gastroenterol Hepatol. 2005;20:56–61.CrossRefGoogle Scholar
  29. 29.
    Sharma BC, Singh RP, Narasimhan KL, Rao KLN, Mitra SK, Dilawari JB. Effect of shunt surgery on spleen size, portal pressure and oesophageal varices in patients with non-cirrhotic portal hypertension. J Gastroenterol Hepatol. 1997;12:582–4.CrossRefGoogle Scholar
  30. 30.
    Deltenre P, Valla DC. Ischemic cholangiopathy. J Hepatol. 2006;44:806–17.CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  1. 1.DHU UNITY, Service d’hépatologieHôpital Beaujon, APHP, Clichy-la-GarenneClichyFrance
  2. 2.CRI, UMR 1149Université Paris-Diderot and InsermParisFrance

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