Imaging features facilitate diagnosis of porto-sinusoidal vascular disorder

• DOI: 10.1007/s00330-022-09132-4
• Publication Date: 2022-09-27T10:02:51Z
• Authors: Katharina Lampichler; Georg Semmler; Katharina Wöran; Benedikt Simbrunner; Mathias Jachs; Lukas Hartl; David Josef Maria...; Lorenz Balcar; Lukas Burghart; Michael Trauner; Dietmar Tamandl; Ahmed Ba-Ssalamah; Mattias Mandorfer; Thomas Reiberger; Bernhard Scheiner; Martina Scharitzer
• Keywords: Gadolinium DTPA; Liver Cirrhosis; Liver Neoplasms; Contrast Media; Hepatobiliary-Pancreas; Magnetic Resonance Imaging; 3. Good health; 03 medical and health sciences; 0302 clinical medicine; Liver; Hypertension, Portal; Humans; Vascular Diseases; Retrospective Studies

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.