DOI QR코드

DOI QR Code

Porto-Sinusoidal Vascular Disease: A Concise Updated Summary of Epidemiology, Pathophysiology, Imaging, Clinical Features, and Treatments

  • Su Jin Jin (Department of Gastroenterology, Liver Center, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Won-Mook Choi (Department of Gastroenterology, Liver Center, Asan Medical Center, University of Ulsan College of Medicine)
  • Received : 2022.09.07
  • Accepted : 2022.10.26
  • Published : 2023.01.01

Abstract

Keywords

Acknowledgement

We thank Prof. So Yeon Kim (Asan Medical Center, South Korea) for sharing the representative images of the patients.

References

  1. Okudaira M, Ohbu M, Okuda K. Idiopathic portal hypertension and its pathology. Semin Liver Dis 2002;22:59-72  https://doi.org/10.1055/s-2002-23207
  2. Lubitz JM, Scott DW Jr. Banti's syndrome with multiple aneurysms and thromboses of the splenic blood vessels. Ann Intern Med 1947;26:594-599  https://doi.org/10.7326/0003-4819-26-4-594
  3. Lee H, Rehman AU, Fiel MI. Idiopathic noncirrhotic portal hypertension: an appraisal. J Pathol Transl Med 2016;50:17-25  https://doi.org/10.4132/jptm.2015.09.23
  4. Schouten JN, Garcia-Pagan JC, Valla DC, Janssen HL. Idiopathic noncirrhotic portal hypertension. Hepatology 2011;54:1071-1081  https://doi.org/10.1002/hep.24422
  5. European Association for the Study of the Liver. EASL clinical practice guidelines: vascular diseases of the liver. J Hepatol 2016;64:179-202  https://doi.org/10.1016/j.jhep.2015.07.040
  6. De Gottardi A, Rautou PE, Schouten J, Rubbia-Brandt L, Leebeek F, Trebicka J, et al. Porto-sinusoidal vascular disease: proposal and description of a novel entity. Lancet Gastroenterol Hepatol 2019;4:399-411  https://doi.org/10.1016/S2468-1253(19)30047-0
  7. Aggarwal S, Fiel MI, Schiano TD. Obliterative portal venopathy: a clinical and histopathological review. Dig Dis Sci 2013;58:2767-2776  https://doi.org/10.1007/s10620-013-2736-4
  8. Boyer JL, Sen Gupta KP, Biswas SK, Pal NC, Basu Mallick KC, Iber FL, et al. Idiopathic portal hypertension. Comparison with the portal hypertension of cirrhosis and extrahepatic portal vein obstruction. Ann Intern Med 1967;66:41-68  https://doi.org/10.7326/0003-4819-66-1-41
  9. Sama SK, Bhargava S, Nath NG, Talwar JR, Nayak NC, Tandon BN, et al. Noncirrhotic portal fibrosis. Am J Med 1971;51:160-169  https://doi.org/10.1016/0002-9343(71)90234-8
  10. Bhargava DK, Dasarathy S, Sundaram KR, Ahuja RK. Efficacy of endoscopic sclerotherapy on long-term management of oesophageal varices: a comparative study of results in patients with cirrhosis of the liver, non-cirrhotic portal fibrosis (NCPF) and extrahepatic portal venous obstruction (EHO). J Gastroenterol Hepatol 1991;6:471-475  https://doi.org/10.1111/j.1440-1746.1991.tb00890.x
  11. Dhiman RK, Chawla Y, Vasishta RK, Kakkar N, Dilawari JB, Trehan MS, et al. Non-cirrhotic portal fibrosis (idiopathic portal hypertension): experience with 151 patients and a review of the literature. J Gastroenterol Hepatol 2002;17:6-16  https://doi.org/10.1046/j.1440-1746.2002.02596.x
  12. Hernandez-Gea V, Baiges A, Turon F, Garcia-Pagan JC. Idiopathic portal hypertension. Hepatology 2018;68:2413-2423  https://doi.org/10.1002/hep.30132
  13. Barge S, Grando V, Nault JC, Broudin C, Beaugrand M, Ganne-Carrie N, et al. Prevalence and clinical significance of nodular regenerative hyperplasia in liver biopsies. Liver Int 2016;36:1059-1066  https://doi.org/10.1111/liv.12974
  14. Eapen CE, Nightingale P, Hubscher SG, Lane PJ, Plant T, Velissaris D, et al. Non-cirrhotic intrahepatic portal hypertension: associated gut diseases and prognostic factors. Dig Dis Sci 2011;56:227-235  https://doi.org/10.1007/s10620-010-1278-2
  15. Hillaire S, Bonte E, Denninger MH, Casadevall N, Cadranel JF, Lebrec D, et al. Idiopathic non-cirrhotic intrahepatic portal hypertension in the West: a re-evaluation in 28 patients. Gut 2002;51:275-280  https://doi.org/10.1136/gut.51.2.275
  16. Wanless IR. Micronodular transformation (nodular regenerative hyperplasia) of the liver: a report of 64 cases among 2,500 autopsies and a new classification of benign hepatocellular nodules. Hepatology 1990;11:787-797  https://doi.org/10.1002/hep.1840110512
  17. Cazals-Hatem D, Hillaire S, Rudler M, Plessier A, Paradis V, Condat B, et al. Obliterative portal venopathy: portal hypertension is not always present at diagnosis. J Hepatol 2011;54:455-461  https://doi.org/10.1016/j.jhep.2010.07.038
  18. Siramolpiwat S, Seijo S, Miquel R, Berzigotti A, Garcia-Criado A, Darnell A, et al. Idiopathic portal hypertension: natural history and long-term outcome. Hepatology 2014;59:2276-2285  https://doi.org/10.1002/hep.26904
  19. Schouten JN, Nevens F, Hansen B, Laleman W, van den Born M, Komuta M, et al. Idiopathic noncirrhotic portal hypertension is associated with poor survival: results of a long-term cohort study. Aliment Pharmacol Ther 2012;35:1424-1433  https://doi.org/10.1111/j.1365-2036.2012.05112.x
  20. Franchi-Abella S, Fabre M, Mselati E, De Marsillac ME, Bayari M, Pariente D, et al. Obliterative portal venopathy: a study of 48 children. J Pediatr 2014;165:190-193.e2  https://doi.org/10.1016/j.jpeds.2014.03.025
  21. Kang JH, Kim DH, Kim SY, Kang HJ, Lee JB, Kim KW, et al. Porto-sinusoidal vascular disease with portal hypertension versus liver cirrhosis: differences in imaging features on CT and hepatobiliary contrast-enhanced MRI. Abdom Radiol (NY) 2021;46:1891-1903  https://doi.org/10.1007/s00261-020-02831-w
  22. Ohnishi K, Saito M, Sato S, Terabayashi H, Iida S, Nomura F, et al. Portal hemodynamics in idiopathic portal hypertension (Banti's syndrome). Comparison with chronic persistent hepatitis and normal subjects. Gastroenterology 1987;92:751-758  https://doi.org/10.1016/0016-5085(87)90028-X
  23. Schouten JN, Van der Ende ME, Koeter T, Rossing HH, Komuta M, Verheij J, et al. Risk factors and outcome of HIV-associated idiopathic noncirrhotic portal hypertension. Aliment Pharmacol Ther 2012;36:875-885  https://doi.org/10.1111/apt.12049
  24. Valainathan SR, Sartoris R, Elkrief L, Magaz M, Betancourt F, Pellegrino S, et al. Contrast-enhanced CT and liver surface nodularity for the diagnosis of porto-sinusoidal vascular disorder: a case-control study. Hepatology 2022;76:418-428  https://doi.org/10.1002/hep.32367
  25. Glatard AS, Hillaire S, d'Assignies G, Cazals-Hatem D, Plessier A, Valla DC, et al. Obliterative portal venopathy: findings at CT imaging. Radiology 2012;263:741-750  https://doi.org/10.1148/radiol.12111785
  26. Arora A, Sarin SK. Multimodality imaging of obliterative portal venopathy: what every radiologist should know. Br J Radiol 2015;88:20140653 
  27. Seijo S, Reverter E, Miquel R, Berzigotti A, Abraldes JG, Bosch J, et al. Role of hepatic vein catheterisation and transient elastography in the diagnosis of idiopathic portal hypertension. Dig Liver Dis 2012;44:855-860  https://doi.org/10.1016/j.dld.2012.05.005
  28. Sharma P, Agarwal R, Dhawan S, Bansal N, Singla V, Kumar A, et al. Transient elastography (fibroscan) in patients with non-cirrhotic portal fibrosis. J Clin Exp Hepatol 2017;7:230-234  https://doi.org/10.1016/j.jceh.2017.03.002
  29. Furuichi Y, Moriyasu F, Taira J, Sugimoto K, Sano T, Ichimura S, et al. Noninvasive diagnostic method for idiopathic portal hypertension based on measurements of liver and spleen stiffness by ARFI elastography. J Gastroenterol 2013;48:1061-1068  https://doi.org/10.1007/s00535-012-0703-z
  30. Okuda K, Kono K, Ohnishi K, Kimura K, Omata M, Koen H, et al. Clinical study of eighty-six cases of idiopathic portal hypertension and comparison with cirrhosis with splenomegaly. Gastroenterology 1984;86:600-610  https://doi.org/10.1016/S0016-5085(84)80106-7
  31. Futagawa S, Fukazawa M, Musha H, Isomatsu T, Koyama K, Ito T, et al. Hepatic venography in noncirrhotic idiopathic portal hypertension. Comparison with cirrhosis of the liver. Radiology 1981;141:303-309  https://doi.org/10.1148/radiology.141.2.7291551
  32. Woran K, Semmler G, Jachs M, Simbrunner B, Bauer DJM, Binter T, et al. Clinical course of porto-sinusoidal vascular disease is distinct from idiopathic noncirrhotic portal hypertension. Clin Gastroenterol Hepatol 2022;20:e251-e266  https://doi.org/10.1016/j.cgh.2020.11.039
  33. Guido M, Sarcognato S, Sonzogni A, Luca MG, Senzolo M, Fagiuoli S, et al. Obliterative portal venopathy without portal hypertension: an underestimated condition. Liver Int 2016;36:454-460  https://doi.org/10.1111/liv.12936
  34. de Franchis R, Baveno VI Faculty. Expanding consensus in portal hypertension: report of the Baveno VI Consensus Workshop: stratifying risk and individualizing care for portal hypertension. J Hepatol 2015;63:743-752  https://doi.org/10.1016/j.jhep.2015.05.022
  35. Bissonnette J, Garcia-Pagan JC, Albillos A, Turon F, Ferreira C, Tellez L, et al. Role of the transjugular intrahepatic portosystemic shunt in the management of severe complications of portal hypertension in idiopathic noncirrhotic portal hypertension. Hepatology 2016;64:224-231  https://doi.org/10.1002/hep.28547
  36. Hirota S, Ichikawa S, Matsumoto S, Motohara T, Fukuda T, Yoshikawa T. Interventional radiologic treatment for idiopathic portal hypertension. Cardiovasc Intervent Radiol 1999;22:311-314  https://doi.org/10.1007/s002709900394
  37. Matsutani S, Maruyama H, Akiike T, Kobayashi S, Yoshizumi H, Okugawa H, et al. Study of portal vein thrombosis in patients with idiopathic portal hypertension in Japan. Liver Int 2005;25:978-983  https://doi.org/10.1111/j.1478-3231.2005.01151.x
  38. Hollande C, Mallet V, Darbeda S, Vallet-Pichard A, Fontaine H, Verkarre V, et al. Impact of obliterative portal venopathy associated with human immunodeficiency virus. Medicine (Baltimore) 2016;95:e3081