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01-07-2018 | Review and Perspectives

Vascular liver diseases on the clinical side: definitions and diagnosis, new concepts

Authors: Dominique-Charles Valla, Dominique Cazals-Hatem

Published in: Virchows Archiv | Issue 1/2018

Abstract

The components of the hepatic vascular system (hepatic arteries, portal and hepatic veins, sinusoids, and lymphatics) can be damaged by various types of injury. Each of the resulting conditions is rare, which has limited knowledge and awareness. In the last two decades, international collaborations have allowed to reach critical masses of data, which has driven significant progresses in understanding and management of vascular disorders of the liver. The present paper discusses definitions, denominations, and diagnosis of such vascular disorders with the exception of those affecting hepatic arteries. Evolving pathogenic or pathophysiologic views relevant to the clinical aspects are also overviewed.

EASL Clinical Practice Guidelines (2016) Vascular diseases of the liver. J Hepatol 64(1):179–202CrossRef

DeLeve LD, Valla DC, Garcia-Tsao G (2009) Vascular disorders of the liver. Hepatology 49(5):1729–1764CrossRefPubMed

de Franchis R (2015) Expanding consensus in portal hypertension: report of the Baveno VI Consensus Workshop: stratifying risk and individualizing care for portal hypertension. J Hepatol 63(3):743–752CrossRefPubMed

Faraoun SA, Boudjella Mel A, Debzi N, Benidir N, Afredj N, Guerrache Y et al (2016) Budd-Chiari syndrome: an update on imaging features. Clin Imaging 40(4):637–646CrossRefPubMed

Seijo S, Plessier A, Hoekstra J, Dell'era A, Mandair D, Rifai K et al (2013) Good long-term outcome of Budd-Chiari syndrome with a step-wise management. Hepatology 57(5):1962–1968CrossRefPubMed

Poisson J, Plessier A, Kiladjian JJ, Turon F, Cassinat B, Andreoli A, de Raucourt E, Goria O, Zekrini K, Bureau C, Lorre F, Cervantes F, Colomer D, Durand F, Garcia-Pagan JC, Casadevall N, Valla DC, Rautou PE, Marzac C, French national network for vascular liver diseases (2017) Selective testing for calreticulin gene mutations in patients with splanchnic vein thrombosis: a prospective cohort study. J Hepatol 67(3):501–507CrossRefPubMed

Qi X, Wu F, Ren W, He C, Yin Z, Niu J et al (2013) Thrombotic risk factors in Chinese Budd-Chiari syndrome patients. An observational study with a systematic review of the literature. Thromb Haemost 109(5):878–884CrossRefPubMed

Shrestha SM, Okuda K, Uchida T, Maharjan KG, Shrestha S, Joshi BL et al (1996) Endemicity and clinical picture of liver disease due to obstruction of the hepatic portion of the inferior vena cava in Nepal. J Gastroenterol Hepatol 11(2):170–179CrossRefPubMed

Valla DC (2009) Primary Budd-Chiari syndrome. J Hepatol 50(1):195–203CrossRefPubMed

10.

Simonetto DA, Yang HY, Yin M, de Assuncao TM, Kwon JH, Hilscher M, Pan S, Yang L, Bi Y, Beyder A, Cao S, Simari RD, Ehman R, Kamath PS, Shah VH (2015) Chronic passive venous congestion drives hepatic fibrogenesis via sinusoidal thrombosis and mechanical forces. Hepatology 61(2):648–659CrossRefPubMedPubMedCentral

11.

Cazals-Hatem D, Vilgrain V, Genin P, Denninger MH, Durand F, Belghiti J, Valla D, Degott C (2003) Arterial and portal circulation and parenchymal changes in Budd-Chiari syndrome: a study in 17 explanted livers. Hepatology 37(3):510–519CrossRefPubMed

12.

Hadengue A, Poliquin M, Vilgrain V, Belghiti J, Degott C, Erlinger S, Benhamou JP (1994) The changing scene of hepatic vein thrombosis: recognition of asymptomatic cases. Gastroenterology 106(4):1042–1047CrossRefPubMed

13.

Sempoux C, Paradis V, Komuta M, Wee A, Calderaro J, Balabaud C, Quaglia A, Bioulac-Sage P (2015) Hepatocellular nodules expressing markers of hepatocellular adenomas in Budd-Chiari syndrome and other rare hepatic vascular disorders. J Hepatol 63(5):1173–1180CrossRefPubMed

14.

Moucari R, Rautou PE, Cazals-Hatem D, Geara A, Bureau C, Consigny Y, Francoz C, Denninger MH, Vilgrain V, Belghiti J, Durand F, Valla D, Plessier A (2008) Hepatocellular carcinoma in Budd-Chiari syndrome: characteristics and risk factors. Gut 57(6):828–835CrossRefPubMed

15.

Ki M, Choi HY, Kim KA, Kim BH, Jang ES, Jeong SH (2016) Incidence, prevalence and complications of Budd-Chiari syndrome in South Korea: a nationwide, population-based study. Liver Int 36(7):1067–1073CrossRefPubMed

16.

Chait Y, Condat B, Cazals-Hatem D, Rufat P, Atmani S, Chaoui D, Guilmin F, Kiladjian JJ, Plessier A, Denninger MH, Casadevall N, Valla D, Brière JB (2005) Relevance of the criteria commonly used to diagnose myeloproliferative disorder in patients with splanchnic vein thrombosis. Br J Haematol 129(4):553–560CrossRefPubMed

17.

Berzigotti A, Garcia-Criado A, Darnell A, Garcia-Pagan JC (2014) Imaging in clinical decision-making for portal vein thrombosis. Nat Rev Gastroenterol Hepatol 11(5):308–316CrossRefPubMed

18.

Plessier A, Darwish-Murad S, Hernandez-Guerra M, Consigny Y, Fabris F, Trebicka J, Heller J, Morard I, Lasser L, Langlet P, Denninger MH, Vidaud D, Condat B, Hadengue A, Primignani M, Garcia-Pagan JC, Janssen HLA, Valla D, European Network for Vascular Disorders of the Liver (EN-Vie) (2010) Acute portal vein thrombosis unrelated to cirrhosis: a prospective multicenter follow-up study. Hepatology 51(1):210–218CrossRefPubMed

19.

Valla DC, Condat B (2000) Portal vein thrombosis in adults: pathophysiology, pathogenesis and management. J Hepatol 32(5):865–871CrossRefPubMed

20.

Elkrief L, Corcos O, Bruno O, Larroque B, Rautou PE, Zekrini K, Bretagnol F, Joly F, Francoz C, Bondjemah V, Cazals-Hatem D, Boudaoud L, de Raucourt E, Panis Y, Goria O, Hillaire S, Valla D, Plessier A (2014) Type 2 diabetes mellitus as a risk factor for intestinal resection in patients with superior mesenteric vein thrombosis. Liver Int 34(9):1314–1321CrossRefPubMed

21.

Turnes J, Garcia-Pagan JC, Gonzalez M, Aracil C, Calleja JL, Ripoll C et al (2008) Portal hypertension-related complications after acute portal vein thrombosis: impact of early anticoagulation. Clin Gastroenterol Hepatol 6(12):1412–1417CrossRefPubMed

22.

Vilgrain V, Condat B, Bureau C, Hakime A, Plessier A, Cazals-Hatem D et al (2006) Atrophy-hypertrophy complex in patients with cavernous transformation of the portal vein: CT evaluation. Radiology 241(1):149–155CrossRefPubMed

23.

Lautz TB, Keys LA, Melvin JC, Ito J, Superina RA (2013) Advantages of the meso-Rex bypass compared with portosystemic shunts in the management of extrahepatic portal vein obstruction in children. J Am Coll Surg 216(1):83–89CrossRefPubMed

24.

Sharma P, Sharma BC, Puri V, Sarin SK (2009) Natural history of minimal hepatic encephalopathy in patients with extrahepatic portal vein obstruction. Am J Gastroenterol 104(4):885–890CrossRefPubMed

25.

Dhiman RK, Saraswat VA, Valla DC, Chawla Y, Behera A, Varma V, Agarwal S, Duseja A, Puri P, Kalra N, Rameshbabu CS, Bhatia V, Sharma M, Kumar M, Gupta S, Taneja S, Kaman L, Zargar SA, Nundy S, Singh SP, Acharya SK, Dilawari JB (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–S14CrossRefPubMedPubMedCentral

26.

Rosenqvist K, Eriksson LG, Rorsman F, Sangfelt P, Nyman R (2016) Endovascular treatment of acute and chronic portal vein thrombosis in patients with cirrhotic and non-cirrhotic liver. Acta Radiol 57(5):572–579CrossRefPubMed

27.

Smalberg JH, Spaander MV, Jie KS, Pattynama PM, van Buuren HR, van den Berg B, Janssen HL, Leebeek FW (2008) Risks and benefits of transcatheter thrombolytic therapy in patients with splanchnic venous thrombosis. Thromb Haemost 100(6):1084–1088CrossRefPubMed

28.

Klinger C, Riecken B, Schmidt A, De Gottardi A, Meier B, Bosch J et al (2017) Transjugular local thrombolysis with/without TIPS in patients with acute non-cirrhotic, non-malignant portal vein thrombosis. Dig Liver Dis 49(12):1345–1352CrossRefPubMed

29.

Condat B, Pessione F, Hillaire S, Denninger MH, Guillin MC, Poliquin M, Hadengue A, Erlinger S, Valla D (2001) Current outcome of portal vein thrombosis in adults: risk and benefit of anticoagulant therapy. Gastroenterology 120(2):490–497CrossRefPubMed

30.

Francoz C, Valla D, Durand F (2012) Portal vein thrombosis, cirrhosis, and liver transplantation. J Hepatol 57(1):203–212CrossRefPubMed

31.

Nery F, Chevret S, Condat B, de Raucourt E, Boudaoud L, Rautou PE, Plessier A, Roulot D, Chaffaut C, Bourcier V, Trinchet JC, Valla DC, on behalf of Groupe d'Etude et de Traitement du Carcinome Hépatocellulaire (2015) Causes and consequences of portal vein thrombosis in 1,243 patients with cirrhosis: results of a longitudinal study. Hepatology 61(2):660–667CrossRefPubMed

32.

Tripodi A, Anstee QM, Sogaard KK, Primignani M, Valla DC (2011) Hypercoagulability in cirrhosis: causes and consequences. J Thromb Haemost 9(9):1713–1723CrossRefPubMed

33.

Zocco MA, Di Stasio E, De Cristofaro R, Novi M, Ainora ME, Ponziani F et al (2009) Thrombotic risk factors in patients with liver cirrhosis: correlation with MELD scoring system and portal vein thrombosis development. J Hepatol 51(4):682–689CrossRefPubMed

34.

Luca A, Caruso S, Milazzo M, Marrone G, Mamone G, Crino F et al (2012) Natural course of extrahepatic nonmalignant partial portal vein thrombosis in patients with cirrhosis. Radiology 265(1):124–132CrossRefPubMed

35.

Maruyama H, Okugawa H, Takahashi M, Yokosuka O (2013) De novo portal vein thrombosis in virus-related cirrhosis: predictive factors and long-term outcomes. Am J Gastroenterol 108(4):568–574CrossRefPubMed

36.

Berry K, Taylor J, Liou IW, Ioannou GN (2015) Portal vein thrombosis is not associated with increased mortality among patients with cirrhosis. Clin Gastroenterol Hepatol 13(3):585–593CrossRefPubMed

37.

Doenecke A, Tsui TY, Zuelke C, Scherer MN, Schnitzbauer AA, Schlitt HJ, Obed A (2010) Pre-existent portal vein thrombosis in liver transplantation: influence of pre-operative disease severity. Clin Transpl 24(1):48–55CrossRef

38.

Englesbe MJ, Schaubel DE, Cai S, Guidinger MK, Merion RM (2010) Portal vein thrombosis and liver transplant survival benefit. Liver Transpl 16(8):999–1005CrossRefPubMedPubMedCentral

39.

Qi X, Dai J, Yang M, Ren W, Jia J, Guo X (2015) Association between portal vein thrombosis and survival in non-liver-transplant patients with liver cirrhosis: a systematic review of the literature. Gastroenterol Res Pract 2015:480842PubMedPubMedCentral

40.

Stine JG, Shah PM, Cornella SL, Rudnick SR, Ghabril MS, Stukenborg GJ, Northup PG (2015) Portal vein thrombosis, mortality and hepatic decompensation in patients with cirrhosis: a meta-analysis. World J Hepatol 7(27):2774–2780CrossRefPubMedPubMedCentral

41.

Ghabril M, Agarwal S, Lacerda M, Chalasani N, Kwo P, Tector AJ (2016) Portal vein thrombosis is a risk factor for poor early outcomes after liver transplantation: analysis of risk factors and outcomes for portal vein thrombosis in waitlisted patients. Transplantation 100(1):126–133CrossRefPubMed

42.

Hibi T, Nishida S, Levi DM, Selvaggi G, Tekin A, Fan J, Ruiz P, Tzakis AG (2014) When and why portal vein thrombosis matters in liver transplantation: a critical audit of 174 cases. Ann Surg 259(4):760–766CrossRefPubMed

43.

Loffredo L, Pastori D, Farcomeni A, Violi F (2017) Effects of anticoagulants in patients with cirrhosis and portal vein thrombosis: a systematic review and meta-analysis. Gastroenterology 153(2):480–487 e1CrossRefPubMed

44.

Villa E, Camma C, Marietta M, Luongo M, Critelli R, Colopi S et al (2012) Enoxaparin prevents portal vein thrombosis and liver decompensation in patients with advanced cirrhosis. Gastroenterology 143(5):1253–60 e1–4CrossRefPubMed

45.

Wanless IR, Wong F, Blendis LM, Greig P, Heathcote EJ, Levy G (1995) Hepatic and portal vein thrombosis in cirrhosis: possible role in development of parenchymal extinction and portal hypertension. Hepatology 21(5):1238–1247PubMed

46.

Luo X, Wang Z, Tsauo J, Zhou B, Zhang H, Li X (2015) Advanced cirrhosis combined with portal vein thrombosis: a randomized trial of TIPS versus endoscopic band ligation plus propranolol for the prevention of recurrent esophageal variceal bleeding. Radiology 276(1):286–293CrossRefPubMed

47.

Luca A, Miraglia R, Caruso S, Milazzo M, Sapere C, Maruzzelli L, Vizzini G, Tuzzolino F, Gridelli B, Bosch J (2011) Short- and long-term effects of the transjugular intrahepatic portosystemic shunt on portal vein thrombosis in patients with cirrhosis. Gut 60(6):846–852CrossRefPubMed

48.

Schouten JN, Garcia-Pagan JC, Valla DC, Janssen HL (2011) Idiopathic noncirrhotic portal hypertension. Hepatology 54(3):1071–1081CrossRefPubMed

49.

Mikkelsen WP, Edmondson HA, Peters RL, Redeker AG, Reynolds TB (1965) Extra- and intrahepatic portal hypertension without cirrhosis (hepatoportal sclerosis). Ann Surg 162(4):602–620CrossRefPubMedPubMedCentral

50.

Khanna R, Sarin SK (2014) Non-cirrhotic portal hypertension—diagnosis and management. J Hepatol 60(2):421–441CrossRefPubMed

51.

Cazals-Hatem D, Hillaire S, Rudler M, Plessier A, Paradis V, Condat B, Francoz C, Denninger MH, Durand F, Bedossa P, Valla DC (2011) Obliterative portal venopathy: portal hypertension is not always present at diagnosis. J Hepatol 54(3):455–461CrossRefPubMed

52.

Guido M, Sarcognato S, Sonzogni A, Luca MG, Senzolo M, Fagiuoli S et al (2016) Obliterative portal venopathy without portal hypertension: an underestimated condition. Liver Int 36(3):454–460CrossRefPubMed

53.

Verheij J, Schouten JN, Komuta M, Nevens F, Hansen BE, Janssen HL et al (2013) Histological features in western patients with idiopathic non-cirrhotic portal hypertension. Histopathology 62(7):1083–1091CrossRefPubMed

54.

Seijo S, Reverter E, Miquel R, Berzigotti A, Abraldes JG, Bosch J, García-Pagán JC (2012) Role of hepatic vein catheterisation and transient elastography in the diagnosis of idiopathic portal hypertension. Dig Liver Dis 44(10):855–860CrossRefPubMed

55.

Schouten JN, Nevens F, Hansen B, Laleman W, van den Born M, Komuta M et al (2012) Idiopathic noncirrhotic portal hypertension is associated with poor survival: results of a long-term cohort study. Aliment Pharmacol Ther 35(12):1424–1433CrossRefPubMed

56.

Siramolpiwat S, Seijo S, Miquel R, Berzigotti A, Garcia-Criado A, Darnell A, Turon F, Hernandez-Gea V, Bosch J, Garcia-Pagán JC (2014) Idiopathic portal hypertension: natural history and long-term outcome. Hepatology 59(6):2276–2285CrossRefPubMed

57.

Hillaire S, Bonte E, Denninger MH, Casadevall N, Cadranel JF, Lebrec D, Valla D, Degott C (2002) Idiopathic non-cirrhotic intrahepatic portal hypertension in the West: a re-evaluation in 28 patients. Gut 51(2):275–280CrossRefPubMedPubMedCentral

58.

Matsutani S, Maruyama H, Akiike T, Kobayashi S, Yoshizumi H, Okugawa H, Fukuzawa T, Kimura K, Saisho H (2005) Study of portal vein thrombosis in patients with idiopathic portal hypertension in Japan. Liver Int 25(5):978–983CrossRefPubMed

59.

Valla DC, Cazals-Hatem D (2016) Sinusoidal obstruction syndrome. Clin Res Hepatol Gastroenterol 40(4):378–385CrossRefPubMed

60.

Harb R, Xie G, Lutzko C, Guo Y, Wang X, Hill CK, Kanel GC, DeLeve LD (2009) Bone marrow progenitor cells repair rat hepatic sinusoidal endothelial cells after liver injury. Gastroenterology 137(2):704–712CrossRefPubMedPubMedCentral

61.

Rubbia-Brandt L, Lauwers GY, Wang H, Majno PE, Tanabe K, Zhu AX et al (2010) Sinusoidal obstruction syndrome and nodular regenerative hyperplasia are frequent oxaliplatin-associated liver lesions and partially prevented by bevacizumab in patients with hepatic colorectal metastasis. Histopathology 56(4):430–439CrossRefPubMed

62.

Saadoun D, Cazals-Hatem D, Denninger MH, Boudaoud L, Pham BN, Mallet V, Condat B, Brière J, Valla D (2004) Association of idiopathic hepatic sinusoidal dilatation with the immunological features of the antiphospholipid syndrome. Gut 53(10):1516–1519CrossRefPubMedPubMedCentral

63.

Marzano C, Cazals-Hatem D, Rautou PE, Valla DC (2015) The significance of nonobstructive sinusoidal dilatation of the liver: impaired portal perfusion or inflammatory reaction syndrome. Hepatology 62(3):956–963CrossRefPubMed

64.

Ronot M, Kerbaol A, Rautou PE, Brancatelli G, Bedossa P, Cazals-Hatem D, Valla DC, Vilgrain V (2016) Acute extrahepatic infectious or inflammatory diseases are a cause of transient mosaic pattern on CT and MR imaging related to sinusoidal dilatation of the liver. Eur Radiol 26(9):3094–3101CrossRefPubMed

65.

Bernard O, Franchi-Abella S, Branchereau S, Pariente D, Gauthier F, Jacquemin E (2012) Congenital portosystemic shunts in children: recognition, evaluation, and management. Semin Liver Dis 32(4):273–287CrossRefPubMed

66.

Blanc T, Guerin F, Franchi-Abella S, Jacquemin E, Pariente D, Soubrane O, Branchereau S, Gauthier F (2014) Congenital portosystemic shunts in children: a new anatomical classification correlated with surgical strategy. Ann Surg 260(1):188–198CrossRefPubMed

Title: Vascular liver diseases on the clinical side: definitions and diagnosis, new concepts
Authors: Dominique-Charles Valla
Dominique Cazals-Hatem
Publication date: 01-07-2018
Publisher: Springer Berlin Heidelberg
Published in: Virchows Archiv / Issue 1/2018
Print ISSN: 0945-6317
Electronic ISSN: 1432-2307
DOI: https://doi.org/10.1007/s00428-018-2331-3

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