Budd-Chiari syndrome (BCS): a challenging diagnosis not to be overlooked-single center report and pictorial essay.

Budd-Chiari syndrome (BCS) is a rare disease with a variable clinical presentation and often late diagnosis. Doppler ultrasonography (DUS) permits to determine the site of the obstructed venous tracts, the thrombotic or non-thrombotic nature of the obstruction with its morphologic features and the flow-pattern alterations. Other non-specific findings, which are seen in most of the other liver diseases, include ascites, hepatosplenomegaly and caudate hypertrophy. The aim of this study is to show our experience in BCS reporting retrospectively 15 cases referred to our hepatology center between 2017 and 2021. Four selected cases depict the extreme heterogeneous behaviour of BCS and highlight the importance of DUS as a diagnostic tool when there is a clinical suspicion. In patients, mainly young, who present with ascites and abdominal pain, BCS has to be considered and DUS is the first imaging technique to be performed to rule it out.

Medullary sponge kidney: unusual finding in kidney transplant recipient.

BACKGROUND: Medullary sponge kidney is generally considered a benign condition, gold standard for the diagnosis is urography but it has almost been replaced by UroCT that did not present the same sensibility. Although it is really rare, our sonography's findings were consistent with medullary sponge kidney in the transplanted kidneys. CASE PRESENTATION: A 45-year-old woman with a long history of double-kidney transplantation complained of frequent urinary tract infections, a history of vague loin pain and came to our attention for sonography follow-up. Her kidney function was normal, we did not find signs of infections in the transplanted kidneys and urinary findings were normal. Curiously, the transplanted kidneys came from a newborn and the patient received a double-kidney transplantation in order to guarantee a satisfactory renal function. CONCLUSIONS: Despite a long history of kidney transplantation, genetic disease should not be forgotten when symptoms and images recall to specific inherited alterations. Sonography has to be considered in diagnostic path of kidney cystic disease.

Selective Intra-procedural AAA sac Embolization During EVAR Reduces the Rate of Type II Endoleak.

OBJECTIVE: The pre-treatment presence of at least six efferent patent vessels (EPV) from the AAA sac and/or AAA thrombus volume ratio (VR%) <40% are considered to be positive predictive factors for persistent type II endoleak (ELIIp). The aim of the present study was to evaluate the effectiveness of sac embolization during EVAR in patients with pre-operative morphological risk factors (p-MRF) for ELIIp. METHODS: Patients undergoing EVAR and intra-procedural AAA sac embolization (Group A, 2012-2013) were retrospectively selected and compared with a control group of patients with the same p-MRF, who underwent EVAR without intra-procedural sac embolization (Group B, 2008-2010). The presence of ELIIp was evaluated by duplex ultrasound at 0 and 6 months, and by contrast enhanced ultrasound at 12 months. The association between AAA diameter, age, COPD, smoking, anticoagulant therapy, and AAA sac embolization with ELIIp was evaluated using multiple logistic regression. The primary endpoint was the effectiveness of the intra-procedural AAA sac embolization for ELIIp prevention. Secondary endpoints were AAA sac evolution and freedom from ELIIp and embolization related re-interventions at 6-12 months. RESULTS: Seventy patients were analyzed: 26 Group A and 44 Group B; the groups were homogeneous for clinical/morphological characteristics. In Group A the median number of coils positioned in AAA sac was 4.1 (IQR 1). There were no complications related to the embolization procedures. A significantly lower number of ELIIp was detected in Group A than in Group B (8/26 vs. 33/44, respectively, p < .001) at discharge, and this was confirmed at 6-12 months (7/26 vs. 30/44 respectively, p = .001, and 5/25 vs. 32/44, respectively, p < .001). On multivariate analysis, intra-procedural AAA sac embolization was the only factor independently associated with freedom from ELIIp at 6 (OR 0.196, 95% CI 0.06-0.63; p = .007) and 12 months (OR 0.098, 95% CI 0.02-0.35; p < .001). No differences in median AAA sac diameter shrinkage were detected between the two groups at 6-12 months (p = .42 and p = .58, respectively). Freedom from ELIIp related and embolization related re-interventions was 100% in both groups, at 6 and 12 months. CONCLUSION: Selective intra-procedural AAA sac embolization in patients with p-MRF is safe and could be an effective method to reduce ELIIp. Further studies are mandatory to support these results at long-term follow up.

Could four-dimensional contrast-enhanced ultrasound replace computed tomography angiography during follow up of fenestrated endografts? Results of a preliminary experience.

OBJECTIVE: To evaluate four-dimensional contrast-enhanced ultrasound (4D-CEUS) as an alternative imaging method to computed tomography angiography (CTA) during follow up of fenestrated endovascular aneurysm repair (FEVAR) for juxta- and para-renal abdominal aortic aneurysms (AAA). METHODS: Between October 2011 and March 2012, all consecutive patients who underwent FEVAR follow up were included in the study and evaluated with both 4D-CEUS and CTA. The interval between the two examinations was always ≤ 30 days. Endpoints were the comparison of postoperative AAA diameter, AAA volume, presence of endoleaks, revascularized visceral vessel (RVV) visualization, and patency. Comparative analysis was performed using Bland-Altman plots and McNemar's Chi-square test. RESULTS: Twenty-two patients (96% male, 4% female; mean age 74 ± 7 years; American Society of Anesthesiologists grade III/IV 82%/18%) were enrolled. Seventy-eight RVV (fenestrations: 60; scallops: 17; branches: 1) were analyzed. The mean AAA diameter evaluated by 4D-CEUS and CTA was 45 ± 10 mm (range 30-69 mm) and 48 ± 9 mm (range 32-70 mm), respectively. The mean difference was 3 ± 3 mm. The mean AAA volume evaluated by 4D-CEUS and CTA was 150 ± 7 cc (range 88-300 cc) and 159 ± 68 cc (range 80-310 cc), respectively. The mean difference was 7 ± 4 cc; a Bland-Altman plot revealed agreement in AAA diameter and volume evaluation (p < .01) between 4D-CEUS and CTA. The observed agreement for the detection of endoleaks was 95%. McNemar's Chi-square test confirmed that 4D-CEUS and CTA were equivalent (p > .05) at detecting endoleaks. The first segment of six (8%) RVVs (four renal and two superior mesenteric arteries) was not directly visualized by 4D-CEUS owing to obesity, but the contrast enhancement into the distal part of vessel or into the relative parenchyma gave indirect information about their patency. McNemar's Chi-square test demonstrated the superiority of CTA (p = .031) in visualizing RVVs. The patency of 77/78 RVVs was confirmed with both techniques. McNemar's Chi-square test confirmed that 4D-CEUS and CTA were equivalent in their ability to detect visceral vessel patency. CONCLUSIONS: The data suggest that 4D-CEUS is as accurate as CTA in the evaluation of postoperative AAA diameter and volume, endoleak detection, and RVV patency after FEVAR. Four-dimensional CEUS could provide hemodynamic information regarding RVVs, and reduce radiation exposure and renal impairment during follow up. Obesity limits the diagnostic accuracy of 4D-CEUS.

Measurement of shear wave velocity using acoustic radiation force impulse imaging is not hampered by previous use of ultrasound contrast agents.

PURPOSE: To assess the inter-observer reproducibility of acoustic radiation force impulse imaging (ARFI) between 2 skilled physicians and to evaluate if ultrasound contrast agents (UCA) affect the measurement of shear wave velocity (SWV) using ARFI. PATIENTS AND METHODS: 53 patients (29 males, 24 females, 59 ± 15 [22-84] years) who underwent contrast enhanced ultrasound (CEUS) examination were included. ARFI was performed on liver segment V by physicians A and B before CEUS, and by physician A within 4-6 minutes and 7-10 minutes after contrast injection. In a subgroup of 31 patients (15 males, 16 females, 57 ± 18 [22-84] years), ARFI was also performed on focal liver lesions (FLL) by physician A before CEUS, and within 4-6 minutes and 7-10 minutes after contrast injection. RESULTS: The SWV values obtained by physician A and B before CEUS yielded an intra-class correlation coefficient value of 0.913 (95% CI, 0.849-0.950). No significant differences were shown between the SWV values of liver segment V in all 53 patients and of FLL in the subgroup of 31 patients obtained before CEUS and that of within 4-6 minutes and 7-10 minutes after contrast injection (all P > 0.05). CONCLUSION: ARFI showed excellent inter-observer reproducibility between 2 skilled physicians. UCA did not affect the measurement of SWV in both liver parenchyma and FLL, at least when performed 4 minutes after the contrast injection. ARFI may become an additional tool in the differential diagnosis of FLL.

EchoScopy in scanning abdominal diseases: initial clinical experience.

BACKGROUND AND AIMS: The introduction of a new type of small handheld ultrasound device brings greater portability and affordability. The basic ultrasound approach with these handheld devices has been defined by European Federation of Societies of Ultrasound in Medicine and Biology (EFSUMB) as "EchoScopy". The current study aimed to assess the image quality, indications and limitations of a portable pocket "EchoScope" compared with a high-end ultrasound system in abdominal diseases. METHODS: 231 consecutive patients were included in this study. Physician A performed ultrasound examinations with a high-end ultrasound system (HEUS), then physician B performed the same ultrasound examination using an EchoScope (Vscan™). In patients with focal lesions, physician B also measured the largest diameters and graded the vascularity by using colour Doppler imaging (CDI) within the lesion on the same plane with both ultrasound systems. Compared with the excellent image quality using HEUS, the image quality of the EchoScope was graded as good, sufficient or non-sufficient. RESULTS: Out of all 231 patients, 167 had focal lesions, 19 patients were found with diffuse pathological findings, six with ascites, six after liver puncture and 33 without any pathological findings. The image quality of the pocket device was considered as being good or sufficient to delineate the pathology in 225/231 (97.4 %). The detection rate of the EchoScope for abdominal focal lesion was 162/167 (97 %), only five superficially located lesions could not be detected. Both systems showed agreement in determination of the best positioning to perform abdominal paracentesis and assessing complications after intervention. CONCLUSIONS: The investigated EchoScope displays a sufficient image quality, in some indications such as detection of focal lesions > 20 mm, detection of ascites, hydronephrosis and other pathological findings with comparable results to HEUS. We conclude that pocket sized EchoScopy devices have a promising future but the indications have to be determined by a prospective study.

Pancreas mobile.

BACKGROUND AND AIM: Up to now, little is known about the mobility of the pancreas due to changes in posture. The present study was conducted to assess the position of the pancreas in the left recumbent posture (endoscopy posture), the pancreatic mobility during changes of the posture from supine to endoscopy posture and also evaluates the possible factors associated with pancreatic mobility. METHODS: 199 patients with (68) or without chronic pancreatitis were examined using conventional gray-scale transabdominal ultrasound to image the position and mobility of the pancreatic head with reference to the vertebral spine and aorta in the supine and left recumbent position. In a subgroup of 75 consecutive healthy subjects regarding the pancreas, the exact moving distance of the central line of the pancreatic head from the supine to left recumbent position was obtained regarding the central line of the spine as the reference. Besides the 199 patients, 50 patients without pancreatic pathology were evaluated by endoscopic ultrasound with radial transducers. RESULTS: On conventional ultrasound in supine (left lateral) position the percentage of patients with right-aortal, pre-aortal, left-aortal pancreatic head were 91.0 % (49 %), 8.0 % (31 %) and 1.0 % (20 %), respectively. Significant movement of the pancreatic head in relation to the aorta was observed in 48 % of patients. The moving distance was correlated to the age, sex and the presence of chronic pancreatitis. Pancreas mobility was more pronounced in young healthy females. No association was found between the moving distance and the body mass index (BMI) and splenic size. On endoscopic ultrasound, the orientation of the pancreas with a view of more (or equal) than 180o was observed in 48 % and less than 180o in 52 % of patients. CONCLUSION: Pancreas mobile is a common phenomenon (about 50 %) which has not often been recognized in daily routine. This phenomenon is more likely in healthy young females. This knowledge might be of importance for improved interpretation of endoscopic ultrasound imaging examinations in patients with and without peripancreatic infiltration.

The pancreatic head is larger than often assumed.

Reference values for B-mode abdominal ultrasound are controversially discussed in the literature mainly due to the lack of data and non-standardized examination techniques. A cohort of 100 patients was prospectively examined. Patients with hepatobiliary diseases including gallstones, acute or chronic pancreatitis (n = 13) and non-adequate visualization (n = 10) were excluded from statistical analysis. Therefore, 77 patients were analyzed including 52 female and 25 male healthy subjects regarding the biliopancreatic system (median age ± SD: 56 ± 18 years [minimum: 22 and maximum 93 years]). The mean value of the cranio-caudal diameter of the pancreatic head was 49 ± 10 [26 - 77] mm(mean ± SD [minimum - maximum]) which is larger than usually reported except in one study. The size of the pancreatic head is often larger than assumed which is of importance to know to exclude pancreatic pathology below the level of the common bile duct.

Littoral cell angioma of the spleen.

We report for the first time on the contrast-enhanced ultrasound (CEUS) features of littoral cell angioma of the spleen (LCA). A patient presented with the incidental finding of splenomegaly which was investigated using modern ultrasound techniques. B-mode technique revealed heterogenous splenic parenchyma and small hypoechoic lesions up to 30 mm in size. Colour Doppler imaging revealed no specific vascularity. CEUS showed arterial hyper- and hypoenhancement with pronounced demarcation in the late phase. In an assumption of malignancy the lesions were biopsied using ultrasound guidance. Histology showed LCA. In this case report we discuss LCA in the light of new ultrasound techniques and present a review of the literature.

ARFI elastography in patients with chronic autoimmune liver diseases: A preliminary study.

OBJECTIVE: Acoustic radiation force impulse (ARFI) is a new software-based technique that evaluates liver stiffness during B-mode ultrasonography. The purpose of this study was to evaluate the accuracy of ARFI in distinguishing patients with chronic autoimmune liver disease from healthy subjects. MATERIAL AND METHODS: We enrolled 9 adult patients (8 women, 1 man; age 48.1 ± 12.8 years) with chronic autoimmune disease (primary biliary cirrhosis (PBC, n = 3), autoimmune hepatitis (AIH, n = 2), primary sclerosing cholangitis (PSC, n = 1) and overlap syndromes, (n = 3) who underwent a liver biopsy and 11 healthy volunteers (age 34.7 ± 10.4 years; 7 women, 4 men). Liver stiffness was evaluated and expressed as the shear wave velocity (SWV) in m/sec. We used a US scanner Siemens-Acuson S2000, evaluating the right liver lobe and the left liver lobe. RESULTS: THE SWV WAS SIGNIFICANTLY HIGHER IN CASES (RIGHT LOBE: 1.51 ± 0.44; left lobe: 1.57 ± 0.40) than in controls (right lobe: 1.08 ± 0.10; left lobe: 1.12 ± 0.13) (right lobe: P = 0.002; left lobe: P = 0.013). We found no significant correlation between right and left lobe SWVs in cases (P = 0.779) or controls (P = 0.385). The SWV cut-off that best distinguished cases from controls was 1.25 m/sec (accuracy: AUC=0.885; sensitivity: 70.6%; specificity: 95.5%). CONCLUSIONS: ARFI elastography is a noninvasive ultrasonographic technique that can differentiate healthy subjects from patients with fibrotic stages of chronic liver disease.

Real-time elastography as a noninvasive technique for quantification of fibrosis in patients with chronic viral liver disease: Preliminary findings.

INTRODUCTION: Real-time elastography (RTE) is a novel technique for measuring tissue elasticity. The aims of this study were to prospectively measure liver stiffness with RTE in patients with chronic viral hepatitis and to evaluate the possible correlation between RTE data and the extent of fibrosis based on liver biopsy findings (Ishak score). MATERIAL AND METHODS: Between February and October 2011, 26 patients (18M, 8F, mean age 41 ± 13 [standard deviation], range 22-62) with chronic viral hepatitis were prospectively evaluated with ultrasonography (US) that included RTE. All patients then underwent US-guided percutaneous liver biopsy (right lobe) for evaluation of fibrosis. Examinations were performed with a iU22 scanner (Philips, Bothell, WA, USA); a convex transducer (C5-1) was used for the US examination, and a linear transducer (L12-5) for RTE. In the RTE images, relative tissue stiffness is expressed according to a color scale with soft areas represented in green/red and hard areas in blue. Patients were examined in the supine position in suspended normal respiration; three loops of 20 RTE frames were recorded for each case. For each patient, we calculated the mean strain ratio (MSR) for the 3 loops. The Spearman correlation coefficient was used to assess correlation between the ASR and fibrosis stage (F) reflected by the Ishak score. RESULTS: The Spearman coefficient showed significant correlation between the MSR and F (Rho = 0.470, p = 0.015). CONCLUSIONS: RTE appears to be a useful tool for noninvasive evaluation of fibrosis in patients with chronic viral hepatitis although these findings need to be confirmed in larger case series.

Antibodies to SS-A/Ro-52kD and centromere in autoimmune liver disease: a clue to diagnosis and prognosis of primary biliary cirrhosis.

BACKGROUND: Primary biliary cirrhosis (PBC) may be associated with various rheumatological disorders. AIM: To investigate the frequency and significance of 'rheumatological' antinuclear antibodies in the field of autoimmune chronic liver disease, with special regard to PBC. METHODS: We studied 105 patients with PBC, 162 autoimmune liver disease controls (type 1 and 2 autoimmune hepatitis, primary sclerosing cholangitis), 30 systemic lupus erythematosus and 50 blood donors. Sera were tested for the presence of antibodies to extractable nuclear antigens (anti-ENA) by counterimmunoelectrophoresis, enzyme-linked and immunoblot (IB) assay, and for the presence of anti-centromere antibodies (ACA) by indirect immunofluorescence on HEp-2 cells and IB. RESULTS: The overall prevalence of IB-detected anti-ENA in PBC (30%) was higher than in type 1 autoimmune hepatitis (2.5%, P < 0.0001), type 2 autoimmune hepatitis (0%, P < 0.0001) and primary sclerosing cholangitis (11.5%, P = 0.006) and lower than in systemic lupus erythematosus (53%, P = 0.03). The most frequent anti-ENA reactivity in PBC was anti-SSA/Ro-52kD (28%). ACA were detected by IB in 21% PBC patients and never in the other subjects (P < 0.0001). Anti-SS-A/Ro/52kD positive PBC patients had at the time of diagnosis a more advanced histological stage (P = 0.01) and higher serum levels of bilirubin (P = 0.01) and IgM (P = 0.03) compared with negative ones. CONCLUSIONS: In the autoimmune liver disease setting, anti-SS-A/Ro-52kD and ACA have a high specificity for PBC and can thus be of diagnostic relevance in anti-mitochondrial antibodies negative cases. If confirmed in further studies with adequate follow-up, anti-SS-A/Ro-52kD antibodies might identify PBC patients with a more advanced and active disease.

Antinuclear antibodies giving the 'multiple nuclear dots' or the 'rim-like/membranous' patterns: diagnostic accuracy for primary biliary cirrhosis.

BACKGROUND: Serum antinuclear antibodies giving the 'multiple nuclear dots' or the 'rim-like/membranous' patterns are frequently detected by indirect immunofluorescence on HEp-2 cells in patients with primary biliary cirrhosis. AIM: To assess the accuracy of multiple nuclear dot and rim-like/membranous antinuclear antibodies for the diagnosis of primary biliary cirrhosis. METHODS: Sera from 4371 consecutive patients referred to our laboratory were analysed under code for antinuclear antibodies testing by indirect immunofluorescence on HEp-2 cells. RESULTS: Review of the clinical records of the 4371 patients allowed identification of 101 patients with antimitochondrial antibody-positive primary biliary cirrhosis and 22 with antimitochondrial antibody-negative variant. Multiple nuclear dot and/or rim-like/membranous patterns were found in 59 (1.3%) of the 4371 patients: 31 antimitochondrial antibody-positive primary biliary cirrhosis, 17 antimitochondrial antibody-negative primary biliary cirrhosis and 11 non-primary biliary cirrhosis. The specificity for primary biliary cirrhosis of both the antinuclear antibodies pattern was 99%. Positive predictive value and likelihood ratio for a positive test were 86% (95% CI: 72.7-94) and 221 (95% CI: 91.7-544) for multiple nuclear dot, 79% (95% CI: 62.2-90.1) and 132 (95% CI: 56.8-312.7) for rim-like/membranous, respectively. CONCLUSIONS: Multiple nuclear dot and rim-like/membranous antinuclear antibodies are rare findings. Their positivity strongly suggests the diagnosis of primary biliary cirrhosis, irrespective of antimitochondrial antibody status. The high specificity for primary biliary cirrhosis makes them a useful diagnostic tool especially in antimitochondrial antibody-negative patients.