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.

Rivaroxaban Prophylaxis in Noncirrhotic Portal Vein Thrombosis.

Rivaroxaban Prophylaxis in Noncirrhotic PVTThis trial assessed the effects of rivaroxaban on the risk of venous thromboembolism and portal hypertension-related bleeding in patients with noncirrhotic chronic portal vein thrombosis without major risk factors for thrombosis. Daily rivaroxaban use reduced the incidence of venous thromboembolism and did not increase major bleeding events.

Hepatobiliary MR contrast agents are useful to diagnose hepatocellular carcinoma in patients with Budd-Chiari syndrome.

BACKGROUND & AIMS: Hepatobiliary phase (HBP) images can discriminate between benign and malignant liver lesions, but it is unclear if this approach can be used in patients with Budd-Chiari syndrome (BCS). Thus, we aimed to assess the diagnostic utility of HBP images in patients with BCS. METHODS: This retrospective study included all patients admitted to our institution with a diagnosis of BCS and focal liver lesions on hepatobiliary contrast agent-enhanced MR imaging (HBCA-MRI) from 2000 to 2019. MR images were reviewed by 2 radiologists blinded to the diagnosis of the lesions. Patient and lesion characteristics were recorded, focusing on HBP imaging features. RESULTS: Twenty-six patients (mean 35 ± 11 years old [13-65]; 21 women [81%] 35 ± 12 years old [13-65]; 5 men [19%] 36 ± 10 years old [19-44]) with 99 benign liver lesions and 12 hepatocellular carcinomas (HCCs) were analyzed. Patients with HCC were significantly older than those with benign lesions (mean 50 ± 10 vs. 33 ± 9 years old, p = 0.003), with higher alpha-fetoprotein (AFP) levels (3/4 [75%] vs. 1/22 [5%] with AFP >15 ng/ml, p <0.001). Homogeneous hypointense signals were identified on HBP in 14 lesions, including 12/12 (100%) HCCs, and 2/99 (2%) benign lesions (p <0.001). Most benign liver lesions showed either peripheral (n = 52/99 [53%]) or homogeneous hyperintensity (n = 23/99 [23%]) on HBP. Lesions with signal hypointensity on HBP in patients with AFP serum levels >15 ng/ml were all HCCs. CONCLUSION: Most benign lesions showed homogeneous or peripheral hyperintensity on HBP images while all HCCs were homogeneously hypointense. HBP images are helpful to differentiate between benign lesions and HCCs and outperform other sequences. They should be systematically acquired for the characterization of focal lesions in patients with BCS. LAY SUMMARY: Hepatobiliary phase imaging is an approach that has recently been shown to discriminate between benign and malignant lesions in the liver. However, it was not known whether this imaging approach could be used effectively in patients with Budd-Chiari syndrome. Herein, we have shown that hepatobiliary phase imaging appears to be useful for differentiating between benign and malignant liver lesions in patients with Budd-Chiari syndrome.

Prevalence, features and predictive factors of liver nodules in Fontan surgery patients: The VALDIG Fonliver prospective cohort.

BACKGROUND & AIMS: Fontan surgery is used to treat a variety of congenital heart malformations, and may lead to advanced chronic liver disease in the long-term. This study examines the prevalence, characteristics and predictors of liver nodules in patients following Fontan surgery. METHODS: This was a prospective, cross-sectional, observational study conducted at 8 European centres. Consecutive patients who had undergone Fontan surgery underwent blood tests, abdominal ultrasonography (US), transient elastography (Fibroscan®), echocardiography, haemodynamic assessments, and abdominal MRI/CT scan. The primary outcome measure was liver nodules detected in the MRI/CT scan. Predictors of liver nodules were identified by multivariate logistic regression. RESULTS: One hundred and fifty-two patients were enrolled (mean age 27.3 years). The mean time elapsed from surgery to inclusion was 18.3 years. Liver nodule prevalences were 29.6% (95% CI 23-37%) on US and 47.7% (95% CI 39-56%) on MRI/CT. Nodules were usually hyperechoic (76.5%), round-shaped (>80%), hyperenhancing in the arterial phase (92%) and located in the liver periphery (75%). The sensitivity and specificity of US were 50% (95% CI 38-62%) and 85.3% (95% CI 75-92%), respectively. Inter-imaging test agreement was low (adjusted kappa: 0.34). In the multivariate analysis, time since surgery >10 years was the single independent predictor of liver nodules (odds ratio 4.18; p = 0.040). Hepatocellular carcinoma was histologically diagnosed in 2 of the 8 patients with hypervascular liver nodules displaying washout. CONCLUSION: While liver nodules are frequent in Fontan patients, they may go unnoticed in US. Liver nodules are usually hyperechoic, hypervascular and predominantly peripheral. This population is at risk of hepatocellular carcinoma, the diagnosis of which requires confirmatory biopsy. LAY SUMMARY: Fontan surgery is the standard of care for many patients with univentricular congenital cardiopathies. Recent advances have improved the survival of Fontan patients, and nowadays most of them reach adulthood. In this setting, Fontan-associated liver disease (FALD) is increasingly recognised, and has become a significant prognostic factor. Liver nodules are considered a component of FALD yet their prevalence, imaging features and predictors have hardly been evaluated. In this study, we observed that liver nodules are frequent, typically hyperechoic, hypervascular and predominantly peripheral in patients with FALD. This population is at risk of hepatocellular carcinoma, the diagnosis of which must be confirmed by biopsy.

Comparison of extracellular and hepatobiliary MR contrast agents for the diagnosis of small HCCs.

BACKGROUND & AIMS: The aim of this study was to use a head-to-head nodule comparison to compare the performance of extracellular contrast agent MRI (ECA-MRI) with that of hepatobiliary contrast agent MRI (HBA-MRI) for the non-invasive diagnosis of small hepatocellular carcinomas (HCCs). METHODS: Between August 2014 and October 2017, 171 patients with cirrhosis, each with 1 to 3 nodules measuring 1-3 cm, were enrolled across 8 centers. All patients underwent both an ECA-MRI and an HBA-MRI within a month. A non-invasive diagnosis of HCC was made when a nodule exhibited arterial phase hyper-enhancement (APHE) with washout at the portal venous phase (PVP) and/or delayed phase (DP) for ECA-MRI, or the PVP and/or HB phase (HBP) for HBA-MRI. The gold standard was defined by using a previously published composite algorithm. RESULTS: A total of 225 nodules, of which 153 were HCCs and 72 were not, were included. The sensitivites of both MRI techniques were similar. Specificity was 83.3% (95% CI 72.7-91.1) for ECA-MRI and 68.1% (95% CI 56.0-78.6) for HBA-MRI. In terms of HCC diagnosis on ECA-MRI, 138 nodules had APHE, 84 had washout at PVP, and 104 at DP; on HBA-MRI, 128 nodules had APHE, 71 had washout at PVP, and 99 at HBP. For nodules 2-3 cm in size, sensitivity and specificity were similar between the 2 approaches. For nodules 1-2 cm in size, specificity dropped to 66.1% (95% CI 52.2-78.2) for HBA-MRI vs. 85.7% (95% CI 73.8-93.6) for ECA-MRI. CONCLUSIONS: HBA-MRI specificity is lower than that of ECA-MRI for diagnosing small HCCs in patients with cirrhosis. These results raise the question of the proper use of HBA-MRI in algorithms for the non-invasive diagnosis of small HCCs. LAY SUMMARY: There are 2 magnetic resonance imaging (MRI)-based approaches available for the non-invasive diagnosis of hepatocellular carcinoma (HCC), using either extracellular or hepatobiliary contrast agents. The current results showed that the sensitivity of MRI with hepatobiliary contrast agents was similar to that with extracellular contrast agents, but the specificity was lower. Thus, hepatobiliary contrast agent-based MRI, although detailed in international guidelines, should be used with caution for the non-invasive diagnosis of HCC. CLINICAL TRIAL NUMBER: NCT00848952.

Low specificity of washout to diagnose hepatocellular carcinoma in nodules showing arterial hyperenhancement in patients with Budd-Chiari syndrome.

BACKGROUND & AIMS: It remains unclear whether the classic imaging criteria for the non-invasive diagnosis of hepatocellular carcinoma (HCC) can be applied to chronic vascular liver diseases, such as Budd-Chiari syndrome (BCS). Herein, we aimed to evaluate the diagnostic value of washout for the discrimination between benign and malignant lesions in patients with BCS. METHODS: This retrospective study included all patients admitted to our institution with a diagnosis of BCS and focal lesions on MRI from 2000 to 2016. MRI images were reviewed by 2 radiologists blinded to the nature of the lesions. Patient and lesion characteristics were recorded, with a focus on washout on portal venous and/or delayed phases. Lesions were compared using Chi-square, Fisher's, Student's t or Mann-Whitney U tests. RESULTS: A total of 49 patients (mean age 35 ±â€¯12 years; 34 women [69%] and 15 men [31%]) with 241 benign lesions and 12 HCC lesions were analyzed. Patients with HCC were significantly older (mean age 44 ±â€¯16 vs. 33 ±â€¯9 years, p = 0.005), with higher alpha-fetoprotein (AFP) levels (median 16 vs. 3 ng/ml, p = 0.007). Washout was depicted in 9/12 (75%) HCC, and 69/241 (29%) benign lesions (p <0.001). A total of 52/143 (36%) lesions ≥1 cm with arterial hyperenhancement showed washout (9 HCC and 43 benign lesions). In this subgroup, the specificity of washout for the diagnosis of HCC was 67%. Adding T1-w hypointensity raised the specificity to 100%. A serum AFP >15 ng/ml was associated with 95% specificity. CONCLUSION: Washout was observed in close to one-third of benign lesions, leading to an unacceptably low specificity for the diagnosis of HCC. The non-invasive diagnostic criteria proposed for cirrhotic patients cannot be extrapolated to patients with BCS. LAY SUMMARY: Washout on MRI is depicted in a significant proportion of benign nodules in patients with Budd-Chiari syndrome (BCS), limiting its value for the differentiation between benign and malignant lesions. Criteria proposed for the non-invasive diagnosis of hepatocellular carcinoma in patients with cirrhosis cannot be extrapolated to patients with BCS. Additional imaging findings and patient characteristics, including alpha-fetoprotein serum level, can help determine the probability of a nodule being HCC in patients with BCS.

Evidence for an Association Between Intrahepatic Vascular Changes and the Development of Hepatopulmonary Syndrome.

BACKGROUND: Hepatopulmonary syndrome (HPS) is characterized by an arterial oxygenation defect, defined by an increased alveolar-arterial oxygen gradient, induced by pulmonary vascular dilatations in the context of liver disease. The pathogenesis of HPS is poorly understood. Morphologic changes associated with HPS are unknown. This study aimed at describing imaging and pathology changes associated with HPS. METHODS: We performed a case-control study in candidates for transplant with suspicion of cirrhosis. Each patient with HPS (Pao2 ≤ 70 mm Hg) was matched to three control subjects for age, cause, and liver disease severity. Pretransplant thoracic and abdominal imaging and explanted livers were reviewed. RESULTS: CT scans and Doppler ultrasounds from 21 patients with HPS were compared with those from 63 control subjects. HPS was associated with a two- to threefold higher prevalence of obstructed intrahepatic portal branches, of slowed or hepatofugal portal blood flow, and of large abdominal portosystemic shunts. Hepatic artery diameter was also larger in patients with HPS. Explanted livers from 19 patients with HPS were compared with those from 57 control subjects. HPS was associated with a fourfold higher prevalence of portal venule thrombosis and a ninefold higher prevalence of extensive vascular proliferation within fibrous septa. Obstruction of centrilobular venules, sinusoidal dilatation, and liver parenchymal extinction were also more common in patients with HPS. CONCLUSIONS: HPS is associated with intrahepatic vascular changes and with features suggesting severe portal hypertension. These results raise the hypothesis that intrahepatic vascular changes precipitate the development of HPS, opening new therapeutic perspectives for HPS.

Peritoneal and pleural fluids may appear hyperintense on hepatobiliary phase using hepatobiliary MR contrast agents.

AIM: To describe the effect of hepatobiliary-specific MR imaging contrast agent (HBCA) administration on the signal intensity of peritoneal and pleural fluid effusions on T1-weighted MR images. MATERIALS AND METHODS: From October 2015 to May 2016 139 patients (mean 60±10 years old, 69 % males) with peritoneal or pleural effusions without biliary leakage who underwent HBCA-MRI (Gd-BOPTA or Gd-EOB-DTPA) at 1.5T and 3T were included from two centres. The fluid signal intensity was classified as hypo/iso/hyperintense before/after HBCA administration. The relative signal enhancement (RE) was calculated. RESULTS: On hepatobiliary phase (HBP), peritoneal fluids appeared hyper/isointense in 88-100 % and pleural effusions in 100 % of the patients following Gd-BOPTA administration. All fluids remained hypointense following Gd-EOB-DTPA. The signal intensity of fluids increased with both HBCA but RE was significantly higher following Gd-BOPTA (p=0.002 to <0.001). RE was correlated with HBP acquisition time-point (r=0.42, p<0.001 and r=0.50, p=0.033 for peritoneal and pleural fluids). CONCLUSION: The signal intensity of pleural and peritoneal fluids progressively increases following HBCA administration in the absence of biliary leakage. Due to its later hepatobiliary phase, this is more pronounced after Gd-BOPTA injection, leading to fluid hyperintensity that is not observed after Gd-EOB-DTPA injection. KEY POINTS: • Fluids appear hyper/isointense on HBP in most patients after Gd-BOPTA injection. • Fluids remain hypointense on HBP after Gd-EOB-DTPA injection. • RE of fluids increases with time after liver-specific Gd injection. • RE of fluids is higher in patients with chronic liver disease.

Diagnosis of Budd-Chiari syndrome.

Budd-Chiari syndrome (BCS) is defined by clinical and laboratory signs associated with partial or complete impairment of hepatic venous drainage in the absence of right heart failure or constrictive pericarditis. Primary BCS is the most frequent type and is a complication of hypercoagulable states, in particular myeloproliferative neoplasms. Secondary BCS involves tumor invasion or extrinsic compression. Most patients present with chronic BCS including a non-cirrhotic, dysmorphic, chronic liver disease with various degrees of fibrosis deposition. Acute BCS is rare, and patients present with hepatomegaly, ascites, and hepatic insufficiency. The diagnosis is based on imaging. Imaging features include (1) direct signs, in particular occlusion or compression of the hepatic veins and/or the inferior vena cava and venous collaterals and (2) indirect signs, in particular morphological changes in the liver with hypertrophy of the caudate lobe and delayed nodule formation. Ultrasound and magnetic resonance imaging are the gold standard for diagnosis. The aim of this review is to provide an overview of the role of imaging in the diagnosis of BCS.

Correlation of tumor response on computed tomography with pathological necrosis in hepatocellular carcinoma treated by chemoembolization before liver transplantation.

The purpose of this article was to compare the results of Response Evaluation Criteria in Solid Tumors (RECIST), modified Response Evaluation Criteria in Solid Tumors (mRECIST), and European Association for the Study of the Liver (EASL) criteria for the evaluation of tumor necrosis in patients treated with transarterial chemoembolization before liver transplantation (LT) for hepatocellular carcinoma. Response to treatment was evaluated on computed tomography scan by 2 independent readers based on RECIST, mRECIST, and EASL criteria, and compared with tumor necrosis assessed by explant pathology. Necrosis was defined as major when >90%. Factors associated with major necrosis were tested by multivariate analysis. Fifty-eight patients (53 males; mean age, 54 years; range, 31-64 years) were included with 88 nodules. Fifty-one (58%) nodules were shown to have major necrosis. Among them readers 1 and 2 identified a complete response (CR) according to RECIST, mRECIST, and EASL criteria in 2 (4%), 47 (92%), and 47 (92%), and 1 (2%), 45 (88%), and 45 (88%) nodules, respectively. However, 12-14 of 59 nodules classified as CR on mRECIST or EASL criteria were found to have intermediate or minor necrosis (overestimation in 20%-24% of the patients). Combining the classification of CR by mRECIST and EASL criteria and complete lipiodol deposition reduced the overestimation to 11%. Among 59 nodules classified with a CR according to mRECIST or EASL, those with complete lipiodol deposition (n = 36, 61%) had a higher rate of necrosis than those with incomplete lipiodol deposition (n = 23, 39%): 95% versus 68% and 95% versus 63% for reader 1 and 2, respectively. In conclusion, CR based on mRECIST/EASL combined with complete lipiodol deposition was better for identification of major tumor necrosis. Even in the presence of CR according to mRECIST/EASL, incomplete lipiodol deposition should be considered indicative of substantial viable tumor remnant. Liver Transplantation 22 1491-1500 2016 AASLD.

The cheating liver: imaging of focal steatosis and fatty sparing.

Focal steatosis and fatty sparing are a frequent finding in liver imaging, and can mimic solid lesions. Liver regional variations in the degree of fat accumulation can be related to vascular anomalies, metabolic disorders, use of certain drugs or coexistence of hepatic masses. CT and MRI are the modalities of choice for the noninvasive diagnosis of hepatic steatosis. Knowledge of CT and MRI appearance of focal steatosis and fatty sparing is crucial for an accurate diagnosis, and to rule-out other pathologic processes. This paper will review the CT and MRI techniques for the diagnosis of hepatic steatosis and the CT and MRI features of common and uncommon causes of focal steatosis and fatty sparing.

Management of large hepatocellular carcinoma by sequential transarterial chemoembolization and portal vein embolization: a systematic review of the literature.

INTRODUCTION: Currently, the treatment of HCC is multidisciplinary. Surgery remains the gold standard although the management of large hepatocellular carcinoma remains challenging. Hepatic resection is increasingly performed with ever-expanding indications. However, postoperative liver failure remains a major cause of death after major hepatic resections. The purpose of this review is to report the results of large hepatocellular carcinoma (>5 cm or more nodules in the same lobe) management using sequential transarterial chemoembolization (TACE) and portal vein embolization (PVE) before major liver resections. EVIDENCE ACQUISITION: A literature search was performed using PubMed, Scopus, and Web of Science (WoS) from cited English publications. The search was last conducted in December 2014. Search phrases included "hepatocellular carcinoma", "liver resection", "transarterial chemoembolization", and "portal vein embolization". Clinical and survival parameters were extracted. When there was more than one publication from the same surgical team and/or authors, only the last publication in chronological order was considered for the study. Case reports, abstracts, letters, editorials, and expert opinions were not considered for the drafting of the study. After application of selective criteria, only 4 original studies were analyzed. EVIDENCE SYNTHESIS: No meta-analyses were found in the search. Among the 4 selected publications, 3 originated from Asia and 1 from Europe. The total number of patients treated with the method considered was 171 (range: 18-71). The mean size of the tumor was >5 cm. The gain of volume of the future remnant liver (FRL) was higher in the group with TACE+PVE as compared to the group with PVE alone (12% vs. 8%). A major hepatectomy was carried out in 166 patients (97%). Mortality rate ranged between 0% and 11%. The 5-year overall survival was between 43% and 72% and the 5-year recurrence-free survival was between 37% and 61%. CONCLUSIONS: Sequential TACE+PVE prior to a major hepatectomy for HCC was feasible, safe, and with excellent 5-year overall survival rates reported to be between 43% and 72%.

Vascular complications following liver transplantation: A literature review of advances in 2015.

Although vascular complications (VCs) following orthotopic liver transplantation (OLT) seldom occur, they are the most feared complications with a high incidence of both graft loss and mortality, as they compromise the blood flow of the transplant (either inflow or outflow). Diagnosis and therapeutic management of VCs constitute a major challenge in terms of increasing the success rate of liver transplantation. While surgical treatment used to be considered the first choice for management, advances in endovascular intervention have increased to make this a viable therapeutic option. Considering VC as a rare but a major and dreadful issue in OLT history, and in view of the continuing and rapid progress in recent years, an update on these uncommon conditions seemed necessary. In this sense, this review comprehensively discusses the important features (epidemiological, clinical, paraclinical, prognostic and therapeutic) of VCs following OLT.

Post-hepatectomy liver failure: Should we consider venous outflow?

INTRODUCTION: Post-hepatectomy liver failure (PHLF) is one of the most serious complications of liver resection and is associated with high morbidity and mortality rates. PRESENTATION OF CASE: We report a case of PHLF involving clinical presentation of posthepatectomy-related 'small-for-size' syndrome (SFSS) secondary to obstructed venous outflow in the liver remnant, following extended right hepatectomy. DISCUSSION: PHLF is similar to SFSS in liver transplantation (LT) in terms of pathogenesis, clinical presentation and outcomes. Although inflow hypertension is clearly implicated in the pathogenesis of SFSS some authors have suggested that outflow obstruction is a potential pathogenic factor. CONCLUSION: The present case support the hypothesis that outflow obstruction could lead symptoms similar to SFSS.

Liver hilar abscesses secondary to gastrointestinal perforation by ingested fish bones: surgical management of two cases.

Several hepatobiliary complications secondary to gastrointestinal perforation after ingestion of a fish bone have been described in the literature, the most common being liver abscess, which can be potentially fatal. Treatment involves removal of the foreign body if possible (endoscopically or surgically), drainage of the abscess (radiologically or surgically), and appropriate antibiotic therapy. To our knowledge, no cases of hepatic hilar abscesses secondary to gastrointestinal perforation by a fish bone have been described in the literature. We report surgical management of two cases of abscess localized in the hepatic hilum secondary to the ingestion of fish bones.