Katharina Lampichler1, Georg Semmler2,3,4, Katharina Wöran5, Benedikt Simbrunner2,3,4,6,7,8, Mathias Jachs2,3,4, Lukas Hartl2,3,4, David Josef Maria Bauer2,3,4, Lorenz Balcar2,3,4, Lukas Burghart2,3,4, Michael Trauner2,4, Dietmar Tamandl1, Ahmed Ba-Ssalamah1, Mattias Mandorfer2,3,4, Thomas Reiberger9,10,11,12,13,14, Bernhard Scheiner2,3,4, Martina Scharitzer1. 1. Department of Biomedical Imaging and Image-Guided Therapy, Medical University of Vienna, Vienna, Austria. 2. Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Waehringer Guertel 18-20, 1090, Vienna, Austria. 3. Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria. 4. Rare Liver Disease (RALID) Center of the European Reference Network (ERN) RARE-LIVER, Medical University of Vienna, Vienna, Austria. 5. Clinical Institute of Pathology, Medical University of Vienna, Vienna, Austria. 6. Christian Doppler Laboratory for Portal Hypertension and Liver Fibrosis, Medical University of Vienna, Vienna, Austria. 7. Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria. 8. CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria. 9. Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Waehringer Guertel 18-20, 1090, Vienna, Austria. thomas.reiberger@meduniwien.ac.at. 10. Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria. thomas.reiberger@meduniwien.ac.at. 11. Rare Liver Disease (RALID) Center of the European Reference Network (ERN) RARE-LIVER, Medical University of Vienna, Vienna, Austria. thomas.reiberger@meduniwien.ac.at. 12. Christian Doppler Laboratory for Portal Hypertension and Liver Fibrosis, Medical University of Vienna, Vienna, Austria. thomas.reiberger@meduniwien.ac.at. 13. Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria. thomas.reiberger@meduniwien.ac.at. 14. CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria. thomas.reiberger@meduniwien.ac.at.
Abstract
OBJECTIVES: Porto-sinusoidal vascular disorder (PSVD) is a recently defined vascular liver disease. Since diagnosis remains challenging, we aimed to evaluate radiological features that are distinct between PSVD and cirrhosis. METHODS: Clinical, laboratory, and radiological parameters (CT/MRI) of patients with histologically-confirmed PSVD vs. cirrhosis vs. non-cirrhotic parenchymal liver disease were retrospectively evaluated. RESULTS: Sixty-three PSVD, 155 cirrhosis, and 41 non-cirrhotic patients were included. As compared to cirrhosis, PSVD patients were younger and had lower HVPG, liver stiffness, and MELD. Routine clinical and imaging findings indicative of portal hypertension were similarly common. Intrahepatic portal tract abnormalities (49% vs. 15%; p < 0.001), FNH-like lesions (30% vs. 1%; p < 0.001), and abnormal liver morphology defined as peripheral parenchymal atrophy and compensatory hypertrophy of central segments (32% vs. 7%; p < 0.001) were significantly more common in PSVD patients. Hypertrophy of segment I (70% vs. 84%; p = 0.019), atrophy of segment IV (24% vs. 47%; p = 0.001), and nodular liver surface (22% vs. 89%; p < 0.001) were more common in patients with cirrhosis. In patients with gadoxetic acid-enhanced MRI, we identified the distinct imaging feature of "periportal hyperintensity" in the hepatobiliary phase (HBP) in 42% of patients with PSVD (14/33) vs. 1% in cirrhosis (1/95) vs. 0% in non-cirrhotic controls (0/41); p < 0.001). CONCLUSIONS: Diagnosis of PSVD must be considered in younger patients presenting with clinical features of portal hypertension, portal tract abnormalities, and FNH-like lesions on CT/MRI. 'Periportal hyperintensity' in the HBP of gadoxetic acid-enhanced MRI was identified as a specific radiological feature of PSVD. KEY POINTS: • Cross-sectional imaging can provide essential information to identify patients with porto-sinusoidal vascular disorder (PSVD). • Intrahepatic portal tract abnormalities, FNH-like lesions, and abnormal liver morphology are common in PSVD patients. • Periportal hyperintensity on the hepatobiliary phase of gadoxetic acid-enhanced MRI seems to be specific for patients with PSVD.
OBJECTIVES: Porto-sinusoidal vascular disorder (PSVD) is a recently defined vascular liver disease. Since diagnosis remains challenging, we aimed to evaluate radiological features that are distinct between PSVD and cirrhosis. METHODS: Clinical, laboratory, and radiological parameters (CT/MRI) of patients with histologically-confirmed PSVD vs. cirrhosis vs. non-cirrhotic parenchymal liver disease were retrospectively evaluated. RESULTS: Sixty-three PSVD, 155 cirrhosis, and 41 non-cirrhotic patients were included. As compared to cirrhosis, PSVD patients were younger and had lower HVPG, liver stiffness, and MELD. Routine clinical and imaging findings indicative of portal hypertension were similarly common. Intrahepatic portal tract abnormalities (49% vs. 15%; p < 0.001), FNH-like lesions (30% vs. 1%; p < 0.001), and abnormal liver morphology defined as peripheral parenchymal atrophy and compensatory hypertrophy of central segments (32% vs. 7%; p < 0.001) were significantly more common in PSVD patients. Hypertrophy of segment I (70% vs. 84%; p = 0.019), atrophy of segment IV (24% vs. 47%; p = 0.001), and nodular liver surface (22% vs. 89%; p < 0.001) were more common in patients with cirrhosis. In patients with gadoxetic acid-enhanced MRI, we identified the distinct imaging feature of "periportal hyperintensity" in the hepatobiliary phase (HBP) in 42% of patients with PSVD (14/33) vs. 1% in cirrhosis (1/95) vs. 0% in non-cirrhotic controls (0/41); p < 0.001). CONCLUSIONS: Diagnosis of PSVD must be considered in younger patients presenting with clinical features of portal hypertension, portal tract abnormalities, and FNH-like lesions on CT/MRI. 'Periportal hyperintensity' in the HBP of gadoxetic acid-enhanced MRI was identified as a specific radiological feature of PSVD. KEY POINTS: • Cross-sectional imaging can provide essential information to identify patients with porto-sinusoidal vascular disorder (PSVD). • Intrahepatic portal tract abnormalities, FNH-like lesions, and abnormal liver morphology are common in PSVD patients. • Periportal hyperintensity on the hepatobiliary phase of gadoxetic acid-enhanced MRI seems to be specific for patients with PSVD.
Authors: Andrea De Gottardi; Pierre-Emmanuel Rautou; Jeoffrey Schouten; Laura Rubbia-Brandt; Frank Leebeek; Jonel Trebicka; Sarwa Darwish Murad; Valérie Vilgrain; Virginia Hernandez-Gea; Filipe Nery; Aurélie Plessier; Annalisa Berzigotti; Paulette Bioulac-Sage; Massimo Primignani; David Semela; Laure Elkrief; Pierre Bedossa; Dominique Valla; Juan Carlos Garcia-Pagan Journal: Lancet Gastroenterol Hepatol Date: 2019-05
Authors: Nina Bastati; Lucian Beer; Mattias Mandorfer; Sarah Poetter-Lang; Dietmar Tamandl; Yesim Bican; Michael Christoph Elmer; Henrik Einspieler; Georg Semmler; Benedikt Simbrunner; Michael Weber; Jacqueline C Hodge; Federica Vernuccio; Claude Sirlin; Thomas Reiberger; Ahmed Ba-Ssalamah Journal: Radiology Date: 2019-11-19 Impact factor: 11.105
Authors: Lucian Beer; Mattias Mandorfer; Nina Bastati; Sarah Poetter-Lang; Dietmar Tamandl; Dilyana Plamenova Stoyanova; Michael Christoph Elmer; Georg Semmler; Benedikt Simbrunner; Jacqueline C Hodge; Claude B Sirlin; Thomas Reiberger; Ahmed Ba-Ssalamah Journal: Eur Radiol Date: 2019-04-18 Impact factor: 5.315
Authors: Rafael Paternostro; Constanze Bardach; Benedikt S Hofer; Bernhard Scheiner; Philipp Schwabl; Ulrika Asenbaum; Ahmed Ba-Ssalamah; Martina Scharitzer; Theresa Bucscis; Benedikt Simbrunner; David Bauer; Michael Trauner; Mattias Mandorfer; Thomas Reiberger; Katharina Lampichler Journal: Liver Int Date: 2020-12-22 Impact factor: 5.828