Porto-sinusoidal vascular disorder (PSVD) is one of the common causes of portal hypertension and has overlapping features with early cirrhosis. The differentiation of PSVD from cirrhosis requires a liver biopsy, which is invasive and has potential complications. This systematic review aimed at summarizing the current evidence on the performance of noninvasive modalities for differentiating PSVD from cirrhosis.
A comprehensive search of electronic databases of MEDLINE, Embase, and Scopus was conducted from 2000 to October 2022 for the studies comparing the elastographic and radiological features of PSVD and cirrhosis, using liver biopsy as the gold standard.
A total of 12 studies were included in the systematic review. Transient elastography (TE) as a modality was studied in five studies, MR elastography (MRE) in two, contrast CT in two, Contrast CT and MRI in two, and ARFI in only one. Both TE and MRE showed a significantly lower liver stiffness measurement and a higher splenic stiffness measurement with a higher SSM/LSM ratio with PSVD, compared to cirrhosis. Among the radiological features, focal nodular hyperplasia-like lesions, portal vein abnormalities (intrahepatic and extrahepatic), and a larger spleen size favored a diagnosis of PSVD. In contrast, surface nodularity and atrophy of segment IV with a segment I hypertrophy favored a diagnosis of cirrhosis.
Elastography and cross-sectional imaging can help differentiate PSVD from early cirrhosis with good accuracy. Further studies are required to assess the diagnostic role of a combination of both modalities.

BACKGROUND: Portal hypertension is secondary to either cirrhotic or non-cirrhotic causes, and complicating pregnancy poses a challenge to the treating team. A systematic review was performed to determine maternal and perinatal outcomes in women with portal hypertension. Outcomes were compared among those with cirrhotic (CPH) with non-cirrhotic portal hypertension (NCPH) as well as non-cirrhotic portal fibrosis (NCPF) with extra-hepatic portal vein obstruction (EHPVO).
METHODS: Medline and EMBASE databases were searched for studies reporting outcomes among pregnant women with portal hypertension. Reference lists from relevant papers and reviews were hand-searched for appropriate citations. Data were extracted to describe maternal complications, obstetric and neonatal outcomes. A random-effects model was used to derive pooled estimates of various outcomes, and final estimates were reported as percentages with a 95% confidence interval (CI). Cumulative, sequential and sensitivity analysis was studied to assess the temporal trends of outcomes over the period.
RESULTS: Information on 895 pregnancies among 581 patients with portal hypertension was included from 26 studies. Portal hypertension was diagnosed during pregnancy in 10% (95% CI 4-24%). There were 22 maternal deaths (0%, 95% CI 0-1%), mostly following complications from variceal bleeding or hepatic decompensation. Variceal bleeding complicated in 14% (95% CI 9-20%), and endoscopic interventions were performed in 12% (95% CI 8-17%) during pregnancy. Decompensation of liver function occurred in 7% (95% CI 3-12%). Thrombocytopenia was the most common complication (41%, 95% CI 23-60%). Miscarriages occurred in 14% (95% CI 8-20%), preterm birth in 27% (95% CI 19-37%), and low birth weights in 22% (95% CI 15-30%). Risk of postpartum hemorrhage was higher (RR 5.09, 95% CI 1.84-14.12), and variceal bleeding was lower (RR 0.51, 95% CI 0.30-0.86) among those with CPH compared to NCPH. Risk of various outcomes was comparable between NCPF and EHPVO.
CONCLUSIONS: One in ten pregnancies complicated with portal hypertension is diagnosed during pregnancy, and thrombocytopenia is the most common complication. Hepatic decompensation and variceal bleeding remain the most common cause of maternal deaths, with reduced rates of bleeding and its complications reported following the introduction of endoscopic procedures during pregnancy. CPH increases the risk of postpartum hemorrhage, whereas variceal bleeding is higher among NCPH.