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Noninvasive differentiation of porto-sinusoidal vascular disorder from cirrhosis: a systematic review

  • Hepatobiliary
  • Published:
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Abstract

Background

Porto-sinusoidal vascular disorder (PSVD) is one of the common causes of portal hypertension and has overlapping features with early cirrhosis. The differentiation of PSVD from cirrhosis requires a liver biopsy, which is invasive and has potential complications. This systematic review aimed at summarizing the current evidence on the performance of noninvasive modalities for differentiating PSVD from cirrhosis.

Methods

A comprehensive search of electronic databases of MEDLINE, Embase, and Scopus was conducted from 2000 to October 2022 for the studies comparing the elastographic and radiological features of PSVD and cirrhosis, using liver biopsy as the gold standard.

Results

A total of 12 studies were included in the systematic review. Transient elastography (TE) as a modality was studied in five studies, MR elastography (MRE) in two, contrast CT in two, Contrast CT and MRI in two, and ARFI in only one. Both TE and MRE showed a significantly lower liver stiffness measurement and a higher splenic stiffness measurement with a higher SSM/LSM ratio with PSVD, compared to cirrhosis. Among the radiological features, focal nodular hyperplasia-like lesions, portal vein abnormalities (intrahepatic and extrahepatic), and a larger spleen size favored a diagnosis of PSVD. In contrast, surface nodularity and atrophy of segment IV with a segment I hypertrophy favored a diagnosis of cirrhosis.

Conclusion

Elastography and cross-sectional imaging can help differentiate PSVD from early cirrhosis with good accuracy. Further studies are required to assess the diagnostic role of a combination of both modalities.

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References

  1. Gioia S, Nardelli S, Ridola L, Riggio O. Causes and Management of Non-cirrhotic Portal Hypertension. Curr Gastroenterol Rep 2020; 22:56.

    Article  PubMed  PubMed Central  Google Scholar 

  2. De Gottardi A, Rautou PE, Schouten J, Rubbia-Brandt L, Leebeek F, Trebicka J, et al; VALDIG group. Porto-sinusoidal vascular disease: proposal and description of a novel entity. Lancet Gastroenterol Hepatol 2019; 4:399-411.

    Article  PubMed  Google Scholar 

  3. Chougule A, Rastogi A, Maiwall R, Bihari C, Sood V, Sarin SK. Spectrum of histopathological changes in patients with non-cirrhotic portal fibrosis. Hepatol Int 2018;12:158-66.

    Article  PubMed  Google Scholar 

  4. Jain M, Venkataraman J, Varghese J, Vij M, Reddy MS, Rela M. Explant liver evaluation decodes the mystery of cryptogenic cirrhosis!. JGH Open 2020; 4:39-43.

    Article  PubMed  Google Scholar 

  5. Thomaides-Brears HB, Alkhouri N, Allende D, Harisinghani M, Noureddin M, Reau NS, et al. Incidence of Complications from Percutaneous Biopsy in Chronic Liver Disease: A Systematic Review and Meta-Analysis. Dig Dis Sci 2022; 67:3366-3394.

    Article  PubMed  Google Scholar 

  6. Intraobserver and interobserver variations in liver biopsy interpretation in patients with chronic hepatitis C. The French METAVIR Cooperative Study Group. Hepatology 1994; 20:15–20.

  7. Nicoară-Farcău O, Rusu I, Stefănescu H, Tanțău M, Badea RI, Procopeț B. Diagnostic challenges in non-cirrhotic portal hypertension-porto sinusoidal vascular disease. World J Gastroenterol 2020; 26:3000.

    Article  PubMed  PubMed Central  Google Scholar 

  8. Xiao G, Zhu S, Xiao X, Yan L, Yang J, Wu G. Comparison of laboratory tests, ultrasound, or magnetic resonance elastography to detect fibrosis in patients with nonalcoholic fatty liver disease: A meta-analysis. Hepatology 2017; 66:1486-1501.

    Article  CAS  PubMed  Google Scholar 

  9. Page MJ, McKenzie JE, Bossuyt PM, Boutron I, Hoffmann TC, Mulrow CD, et al. The PRISMA 2020 statement: an updated guideline for reporting systematic reviews. BMJ 2021; 372:n71.

    Article  PubMed  PubMed Central  Google Scholar 

  10. 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; 48:1061-8.

    Article  PubMed  Google Scholar 

  11. Parikh P, Ingle M, Sawant P. Role of Tissue Elastography in differentiating early cirrhosis from non-cirrhotic portal fibrosis. Hep Int 2014; 8:S388.

    Google Scholar 

  12. Sharma P, Agarwal R, Dhawan S, Bansal N, Singla V, Kumar A, Arora A. Transient elastography (Fibroscan) in patients with non-cirrhotic portal fibrosis. J Clin Exp Hepatol 2017; 7:230-4.

    Article  PubMed  PubMed Central  Google Scholar 

  13. Shukla A, Sawrav S, Udare A, Shah S, Jain A, Bhatia S. Role of Magnetic Resonance Elastography in Assessment of Liver Fibrosis in Patients With Non-cirrhotic Portal Fibrosis. J Clin Exp Hepatol 2017; 7:S99-100.

    Article  Google Scholar 

  14. Noufal BM, Venkateswaran AR, Solomon R, Shaikh MK, Malarvizhi M, Kini R. Transient elastography in non-cirrhotic portal fibrosis: A comparative study with child A cirrhosis in a tertiary care centre. Hep Int 2018; 12:S645.

    Google Scholar 

  15. Navin PJ, Gidener T, Allen AM, Yin M, Takahashi N, Torbenson MS, et al. The role of magnetic resonance elastography in the diagnosis of noncirrhotic portal hypertension. Clin Gastroenterol Hepatol 2020; 18:3051-3.

    Article  CAS  PubMed  Google Scholar 

  16. Zhao ZL, Wei Y, Wang TL, Peng LL, Li Y, Yu MA. Author Correction: Imaging and Pathological Features of Idiopathic Portal Hypertension and Differential Diagnosis from Liver Cirrhosis. Sci Rep 2020; 10:7586.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  17. Elkrief L, Lazareth M, Chevret S, Paradis V, Magaz M, Blaise L, et al. Liver stiffness by transient elastography to detect porto‐sinusoidal vascular liver disease with portal hypertension. Hepatology 2021; 74:364-78.

    Article  PubMed  Google Scholar 

  18. 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; 46:1891-903.

    Article  PubMed  Google Scholar 

  19. Ferreira-Silva J, Gaspar R, Liberal R, Cardoso H, Macedo G. Splenic-hepatic elastography index is useful in differentiating between porto-sinusoidal vascular disease and cirrhosis in patients with portal hypertension. Dig Liver Dis 2023; 55:75-80.

    Article  PubMed  Google Scholar 

  20. Lampichler K, Semmler G, Wöran K, Simbrunner B, Jachs M, Hartl L, et al. Imaging features facilitate diagnosis of porto-sinusoidal vascular disorder. Eur Radiol 2022; 33:1422–1432.

    Article  PubMed  PubMed Central  Google Scholar 

  21. 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.

    Article  CAS  PubMed  Google Scholar 

  22. Sarin SK, Kapoor D. Non‐cirrhotic portal fibrosis: current concepts and management. J Gastroenterol Hepatol 2002; 17:526-34.

    Article  CAS  PubMed  Google Scholar 

  23. Giri S, Agrawal D, Gopan A, Varghese J, Tripathy T. Diagnostic outcome and safety of plugged liver biopsy in high-risk patients: a systematic review and meta-analysis. Acta Radiol 2023 Jan 24:2841851231151513. doi: https://doi.org/10.1177/02841851231151513.

    Article  Google Scholar 

  24. Idilman IS, Li J, Yin M, Venkatesh SK. MR elastography of liver: current status and future perspectives. Abdom Radiol (NY) 2020; 45:3444-3462.

    Article  PubMed  Google Scholar 

  25. Vuppalanchi R, Mathur K, Pyko M, Samala N, Chalasani N. Liver stiffness measurements in patients with noncirrhotic portal hypertension—the devil is in the details. Hepatology 2018; 68:2438-40.

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Gastroenterology, Nizam’s Institute of Medical Sciences, 4th Floor, Specialty Block, Panjagutta, Hyderabad, Telangana, 500082, India

    Suprabhat Giri & Sumaswi Angadi

  2. Department of Gastroenterology, Seth GS Medical College and KEM Hospital, Mumbai, India

    Ankita Singh

  3. Institute of Liver and Gastrosciences, Apollo Multispecialty Hospital, Kolkata, India

    Akash Roy

  4. Department of Radiodiagnosis, All India Institute of Medical Sciences, Bhubaneswar, India

    Ranjan Kumar Patel & Taraprasad Tripathy

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  1. Suprabhat Giri
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  2. Ankita Singh
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  3. Akash Roy
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  4. Ranjan Kumar Patel
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  5. Taraprasad Tripathy
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  6. Sumaswi Angadi
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Contributions

SG,SA: Conceptualization, SG,AS,TT: Data curation, SG,AR,AS,TT: Formal analysis, Funding acquisition: N/A; SG,AS,SA: Investigation, SG,AR,SA: Methodology, SG,SA: Project administration, SG,AS,TT: Resources, SG: Software, SS, AR: Supervision, SG,AR,SA: Validation, SG: Visualization, SG,AS,SA: Roles/Writing—original draft, SG,AR,AS,RKP,TT,SA: Writing—review & editing:

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Correspondence to Sumaswi Angadi.

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Giri, S., Singh, A., Roy, A. et al. Noninvasive differentiation of porto-sinusoidal vascular disorder from cirrhosis: a systematic review. Abdom Radiol 48, 2340–2348 (2023). https://doi.org/10.1007/s00261-023-03927-9

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  • DOI: https://doi.org/10.1007/s00261-023-03927-9

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