Contrast-enhanced CT and liver surface nodularity for the diagnosis of porto-sinusoidal vascular disorder: A case-control study.

BACKGROUND AND AIMS: Porto-sinusoidal vascular disorder (PSVD) is a rare and commonly overlooked cause of portal hypertension. The interest of CT analysis, including quantification of liver surface nodularity (LSN) for PSVD diagnosis has not been established. This study aimed at assessing the performance of LSN and CT features for a PSVD diagnosis in patients with signs of portal hypertension. APPROACH AND RESULTS: This retrospective case-control study included a learning cohort consisting of 50 patients with histologically proven PSVD, according to VALDIG criteria, and 100 control patients with histologically proven cirrhosis, matched on ascites. All patients and controls had at least one sign of portal hypertension and CT available within 1 year of liver biopsy. Principal component analysis of CT features separated patients with PSVD from patients with cirrhosis. Patients with PSVD had lower median LSN than those with cirrhosis (2.4 vs. 3.1, p < 0.001). Multivariate analysis identified LSN < 2.5 and normal-sized or enlarged segment IV as independently associated with PSVD. Combination of these two features had a specificity of 90% for PSVD and a diagnostic accuracy of 84%. Even better results were obtained in an independent multicenter validation cohort including 53 patients with PSVD and 106 control patients with cirrhosis (specificity 94%, diagnostic accuracy 87%). CONCLUSIONS: This study that included a total of 103 patients with PSVD and 206 patients with cirrhosis demonstrates that LSN < 2.5 combined with normal-sized or enlarged segment IV strongly suggests PSVD in patients with signs of portal hypertension.

Liver Stiffness by Transient Elastography to Detect Porto-Sinusoidal Vascular Liver Disease With Portal Hypertension.

BACKGROUND AND AIMS: Porto-sinusoidal vascular liver disease (PSVD) is a rare cause of portal hypertension. PSVD is still often misdiagnosed as cirrhosis, emphasizing the need to improve PSVD diagnosis strategies. Data on liver stiffness measurement using transient elastography (TE-LSM) in PSVD are limited. The aim of this study was to evaluate the accuracy of TE-LSM to discriminate PSVD from cirrhosis in patients with signs of portal hypertension. APPROACH AND RESULTS: Retrospective multicenter study comparing TE-LSM in patients with PSVD, according to Vascular Liver Disease Interest Group criteria, with patients with compensated biopsy-proven cirrhosis associated with alcohol (n = 117), HCV infection (n = 110), or NAFLD (n = 46). All patients had at least one sign of portal hypertension among gastroesophageal varices, splenomegaly, portosystemic collaterals, history of ascites, or platelet count < 150 × 109 /L. The 77 patients with PSVD included in the test cohort had lower median TE-LSM (7.9 kPa) than the patients with alcohol-associated, HCV-related, and NAFLD-related cirrhosis (33.8, 18.2, and 33.6 kPa, respectively; P < 0.001). When compared with cirrhosis, a cutoff value of 10 kPa had a specificity of 97% for the diagnosis of PSVD with a 85% positive predictive value. A cutoff value of 20 kPa had a sensitivity of 94% for ruling out PSVD with a 97% negative predictive value. Of the patients, 94% were well-classified. Even better results were obtained in a validation cohort including 78 patients with PSVD. CONCLUSIONS: This study including a total of 155 patients with PSVD and 273 patients with cirrhosis demonstrates that TE-LSM < 10 kPa strongly suggests PSVD in patients with signs of portal hypertension. Conversely, when TE-LSM is >20 kPa, PSVD is highly unlikely.

CT-based liver surface nodularity for the detection of clinically significant portal hypertension: defining measurement quality criteria.

PURPOSE: To establish measurement quality criteria for the noninvasive assessment of clinically significant portal hypertension (CSPH) in patients with cirrhosis using CT-based liver surface nodularity (LSN) measurements. METHODS: Seventy-four consecutive patients with cirrhosis (mean 62 ± 13 years), including 30 with CSPH (41%), underwent CT and hepatic venous pressure gradient measurements. Three independent readers performed 15 LSN measurements/patient using dedicated software. LSN was computed based on the median and means of one to 15 measurements. Accuracy for diagnosing CSPH was assessed using receiver operating characteristic (ROC) curve analysis. Variability was assessed by the intra-class correlation coefficient (ICC) and the Bland-Altman plot (BA). Quality criteria were identified to maximize the accuracy of LSN and minimize variability. RESULTS: The area under the (AU) ROCs of mean and median LSN measurements based on one to 15 measurements ranged from 0.79 ± 0.05 to 0.91 ± 0.04 and 0.86 ± 0.04 to 0.91 ± 0.03, respectively, with no difference on pair-wise comparisons (all p > 0.05). AUROCs of LSN increased from one to eight and leveled off between eight and 15 measurements. Inter- and intra-reader variability decreased from one to 15 measurements, with only slight improvement after more than eight measurements. Intra- and inter-observer agreements were excellent with eight measurements (ICC = 0.90 [95%CI 0.84-0.94], and ICC = 0.93 [95%CI 0.89-0.95], respectively), and variability for intra-observer and inter-observer agreement was low (BA bias 4.2% (95% limits of agreement [LoA] [- 15.3; + 23.7%]) and 4.8% LoA [ - 17.5; + 27.1%], respectively). CONCLUSIONS: CT-based LSN measurement is highly reproducible and accurate. We suggest using at least 8 valid measurements to determine the mean LSN value for the detection of CSPH.