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World J Gastroenterol. Jun 14, 2020; 26(22): 3000-3011
Published online Jun 14, 2020. doi: 10.3748/wjg.v26.i22.3000
Diagnostic challenges in non-cirrhotic portal hypertension - porto sinusoidal vascular disease
Oana Nicoară-Farcău, Ioana Rusu, Horia Stefănescu, Marcel Tanțău, Radu Ion Badea, Bogdan Procopeț
Oana Nicoară-Farcău, Ioana Rusu, Marcel Tanțău, Radu Ion Badea, Bogdan Procopeț, University of Medicine and Pharmacy “Iuliu Hatieganu”, Cluj-Napoca 400000, Romania
Oana Nicoară-Farcău, Horia Stefănescu, Marcel Tanțău, Bogdan Procopeț, Gastroenterology Department, Regional Institute of Gastroenterology and Hepatology “O. Fodor”, Cluj-Napoca 400000, Romania
Ioana Rusu, Pathology Department, Regional Institute of Gastroenterology and Hepatology “O. Fodor”, Cluj-Napoca 400000, Romania
Radu Ion Badea, Imagistic Department, Regional Institute of Gastroenterology and Hepatology “O. Fodor”, Cluj-Napoca 400000, Romania
Author contributions: Nicoară-Farcău O and Rusu I drafted the manuscript and prepared the figures and tables; Stefănescu H revised the first draft of the manuscript and provided input in the writing; Tanțău M and Badea RI revised the manuscript and provided input in the writing; Procopeț B coordonanted the writing of the manuscript, revised the manuscript and validated the final version of the manuscript.
Supported by UEFISCDI, Ministry of Education of Romania, No. PN-III-P1-1.1-PD-2016-0689.
Conflict-of-interest statement: Authors declare no potential conflicts of interest.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Bogdan Procopeț, MD, PhD, Doctor, Senior Lecturer, Staff Physician, Gastroenterology and Hepatology Department, 3rd Medical Clinic, University of Medicine and Pharmacy “Iuliu Hatieganu”, 19-21 Croitorilor Street, Cluj-Napoca 400162, Romania. bogdan.procopet@umfcluj.ro
Received: December 30, 2019
Peer-review started: December 30, 2019
First decision: February 16, 2020
Revised: March 31, 2020
Accepted: May 27, 2020
Article in press: May 27, 2020
Published online: June 14, 2020
Abstract

Non-cirrhotic portal hypertension consists of a group of diseases characterized by signs and complications of portal hypertension, which differ from cirrhosis through histological alterations, hemodynamic characterization and, clinical outcome. Because of the similarities in clinical presentation and imaging signs, frequently these patients, and particularly those with porto-sinusoidal vascular disease (PSVD), are misdiagnosed as having liver cirrhosis and thus raising difficulties in their diagnosis. The most challenging differentiation to be considered is between PSVD and cirrhosis and, although not pathognomonic, liver biopsy is still the standard of diagnosis. Although they still require extended validation before being broadly used, new non-invasive methods for the diagnosis of porto-sinusoidal vascular disease, like transient elastography, contrast-enhanced ultrasound or metabolomic profiling, have shown promising results. Another issue is the differentiation between PSVD and chronic extrahepatic portal vein obstruction, especially now when it is known that 40% of patients suffering from PSVD develop portal vein thrombosis. In this particular case, once the portal vein thrombosis occurred, the diagnosis of PSVD is impossible according to the current guidelines. Moreover, so far, the differentiation between PSVD and sinusoidal obstruction syndrome has not been clear so far in particular circumstances. In this review we highlighted the diagnostic challenges regarding the PSVD, as well as the current techniques used in the evaluation of these patients.

Keywords: Porto-sinusoidal vascular disease, Extrahepatic portal vein obstruction, Non-cirrhotic portal hypertension, Non-invasive diagnosis, Idiopathic portal hypertension, Cirrhosis

Core tip: Non-cirrhotic portal hypertension consists of a group of diseases characterized by signs and complications of portal hypertension. However, their diagnosis is sometimes difficult due to their similarities in clinical presentation, imaging signs and histological modifications despite advances in their understanding. Moreover, these disorders are sometimes misdiagnosed as cirrhosis. This review highlights their diagnostic challenges, especially with regard to porto sinusoidal vascular disease, extrahepatic portal vein obstruction, sinusoidal obstruction syndrome and cirrhosis and discusses the current available diagnostic tools.