Imaging features facilitate diagnosis of porto-sinusoidal vascular disorder

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.

The value of periportal hyperintensity sign from gadobenate dimeglumine-enhanced hepatobiliary phase MRI for predicting clinical outcomes in patients with decompensated cirrhosis

“…The presence of periportal hyperintensity sign on HBP was recorded. Periportal hyperintensity sign was defined as a periportal ring or tramline enhancement around the intrahepatic portal veins present within several (more than one) hepatic segments [ 16 , 22 ].…”

Porto-Sinusoidal Vascular Disease in Congenital Erythropoietic Porphyria Needing Liver Transplantation

“…However, unlike cirrhosis, MELD scores may not be reliable to determine priority for LT in PSVD. 14,15 Importantly, LT does not address the underlying heme synthetic defect, necessitating allogenic HSCT for a definitive cure and to prevent disease recurrence. [16][17][18] While undergoing LT, caution should be exercised regarding the use of specific UV light filter blocks to avoid phototoxic injury in these patients.…”

Porto-sinusoidal vascular disease: a new definition of an old clinical entity

“…According to this scoring system, patients with PSVD had a median score of 2 points, while patients with cirrhosis had –1 point. The optimal cut-off was 1 point according to Youden’s index, with an AUROC of 0.913 [ 42 ].…”

“…The risk of PVT after 1, 5, and 10 years was 16%, 25%, and 48%, respectively [ 49 ]. The presence of focal nodular hyperplasia-like lesions was reported in 30% of patients with PSVD [ 42 ]. On the other hand, no study has analyzed the prevalence of PSVD in patients with FNH, as performing the liver biopsy and/or HVPG measurements in all patients with incidentally discovered FNH would probably not be justified.…”