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Chyluria is an uncommon medical condition resulting from an abnormal communication between the abdominal lymphatic system and the urinary tract, which results in the presence of chyle in the urine, making it appear milky white. Proper diagnosis is demonstrated by the concentration of urinary lipids. Worldwide, chyluria is most commonly associated with the parasite Wuchereria bancrofti. However, in Europe and North America, where the condition is rare, non-parasitic etiologies predominate. Identifying the cause and location of the uro-lymphatic communication is essential in guiding therapeutic management, but imaging the lymphatic channels remains a challenge. Magnetic resonance (MR) lymphography, a non-invasive free-breathing 3D high-resolution fast-recovery fast spin-echo sequence similar to that used for 3D MR cholangiopancreatography, may demonstrate the cause and location of an abnormal communication between the lymphatic system and urinary tract. In parasitic causes of chyluria, dilated lymphatics vessels communicating with the lymphatic system are demonstrated. In non-parasitic causes of chyluria channel type lymphatic malformations are the most common. Markedly dilated and dysplastic lymphatic vessels communicating with the urinary tract are demonstrated. In addition, other cystic or channel type lymphatic malformations such as thoracic, soft tissue or bone abnormalities may be observed. This review describes the abdominal lymphatic diseases leading to chyluria and presents the technique and images obtained with non-enhanced MR lymphography to enable radiologists in identifying and classifying uro-lymphatic fistulae.Critical relevance statement: Non-enhanced MR lymphography enables the identification and categorization of uro-lymphatic fistulae., (© 2023. The Author(s).)

Background & Aims: The low-phospholipid-associated cholelithiasis (LPAC) syndrome is a recently described peculiar form of cholelithiasis associated with the ATP-binding-cassette subfamily B, member 4 ( ABCB4 ) gene deficiency. The purpose of our study was to analyse the relationship between magnetic resonance (MR) features and the genetic status of ABCB4 in people with LPAC syndrome., Methods: A total of 233 individuals with proven LPAC syndrome were enrolled between January 2003 and June 2018 in a retrospective single-centre study. Inclusion criteria included availability of clinical files, MR images, and genetic data. MR images were analysed by consensus among 3 senior radiologists blinded to the status of ABCB4 gene mutation., Results: A total of 125 individuals (mean age at first MR imaging 40.8 years; 66% females; 48% ABCB4 variant) were included. MR abnormalities were found in 61 (49%) of the 125 individuals. Forty (67%) of the 60 individuals with an ABCB4 gene variant had MR abnormalities as compared with 21 (33%) of the 65 individuals without an ABCB4 gene variant (odds ratio [OR] 4.1, 95% CI 1.9-9.5, p  = 0.0001). Compared to individuals with no variant, individuals with an ABCB4 variant were more likely to show intrahepatic macrolithiasis (56 vs. 17%; OR 6.3, 95% CI 2.6-16.2, p <0.0001), bile duct dilatation (60 vs. 18%; OR 6.5, 95% CI 2.7-16.3, p <0.0001), and at least 1 MR feature of complication (35 vs. 15%; OR 2.9, 95% CI 1.1-7.8, p <0.05)., Conclusions: ABCB4 -related LPAC syndrome is associated with more frequent and severe hepatobiliary MR abnormalities. This finding strongly supports the major role of the ABCB4 gene in the pathogenesis of LPAC syndrome and highlights a genotype-phenotype association in this inherited disease with genetic heterogeneity., Lay Summary: ABCB4 -related LPAC syndrome associated with an ABCB4 gene variant demonstrates more frequent and severe hepatobiliary MR abnormalities. This finding supports the major role of the ABCB4 gene in the pathogenesis of LPAC syndrome., Competing Interests: The authors declare that there is no conflict of interest. Please refer to the accompanying ICMJE disclosure forms for further details., (© 2022 The Authors.)

Background & Aims: People with primary sclerosing cholangitis (PSC) have a variable and often progressive disease course that is associated with biliary and parenchymal changes. These changes are typically assessed by magnetic resonance imaging (MRI), including qualitative assessment of magnetic resonance cholangiopancreatography (MRCP). Our aim was to study the association of novel objective quantitative MRCP metrics with prognostic scores and patient outcomes., Methods: We performed a retrospective study including 77 individuals with large-duct PSC with baseline MRCP images, which were postprocessed to obtain quantitative measures of bile ducts using MRCP+™. The participants' ANALI scores, liver stiffness by vibration-controlled transient elastography, and biochemical indices were collected at baseline. Adverse outcome-free survival was measured as the absence of decompensated cirrhosis, liver transplantation (LT), or liver-related death over a 12-year period. The prognostic value of MRCP+-derived metrics was assessed by Cox regression modelling., Results: During a total of 386 patients-years, 16 cases of decompensation, 2 LTs, and 5 liver-related deaths were recorded. At baseline, around 50% of the patients were classified as being at risk of developing disease complications. MRCP+ metrics, particularly those describing the severity of bile duct dilatations, were correlated with all prognostic factors. Univariate analysis showed that MRCP+ metrics representing duct diameter, dilatations, and the percentage of ducts with strictures and/or dilatations were associated with survival. In a multivariable-adjusted analysis, the median duct diameter was significantly associated with survival (hazard ratio 10.9, 95% CI 1.3-90.3)., Conclusions: MRCP+ metrics in people with PSC correlate with biochemical, elastographic, and radiological prognostic scores and are predictive of adverse outcome-free survival., Lay Summary: In this study, we assessed in people with primary sclerosing cholangitis (PSC) the association of novel objective quantitative MRCP metrics automatically provided by a software tool (MRCP+) with prognostic scores and patient outcomes. We observed that MRCP+ metrics in people with PSC correlate with biochemical, elastographic, and radiological prognostic scores and are predictive of adverse outcome-free survival., Competing Interests: NC, SEM, QC, SL, CC, OC, and LA declare no conflict of interest related to this paper. CF and SF are employed by Perspectum Ltd. Please refer to the accompanying ICMJE disclosure forms for further details., (© 2022 The Authors.)

Purpose: The purpose of this study was to evaluate the efficacy of cone-beam computed tomography (CBCT) navigation to achieve percutaneous screw fixation (PSF) of pelvic bone metastases (PBM)., Materials and Methods: Thirty-five consecutive patients (12 men and 23 women; mean age, 62 ± 11.3 [SD]; range: 39-89 years) treated between 2019 and 2021 were retrospectively included. CBCT navigation software was systematically used. Manual drawing of the entry point (MDEP) was performed when CBCT automatic positioning failed. Influence of metastasis pattern, ablation, body mass index, number of screws, and MDEP on procedure duration (PD) and total Air Kerma (AK) was evaluated. Local pain was assessed before, one and six months after treatment. Variables were compared using Pearson correlation, Student t and Wilcoxon tests., Results: Seventy-five screws were inserted successfully (mean: 2.1 ± 1.1 [SD]; range: 1-5 per patient). CBCT automatic positioning was obtained for 41 screws (55%, 41/75), whereas 34 (45%, 34/75) required MDEP. Mean procedure duration, fluoroscopy time, kerma air product and AK were 73.3 ± 44.8 (SD) min (range: 19-233 min), 13.1 ± 9.5 (SD) min (range: 1.4-38.6 min), 73.8 ± 66.3 (SD) Gy.cm 2 (range: 11.6-303.7 Gy.cm 2 ) and 301.7 ± 242.1 (SD) mGy (range: 49.4-1111.5 mGy), respectively. Procedure duration was not significantly longer in patients with mixed or blastic PBM compared to those with lytic PBM or when performing ablation, and no significant correlations were observed with the number of screws inserted (P = 0.19), MDEP (P = 0.37) and BMI (P = 0.44). No adverse events were reported during the follow-up (median: 6 months; IQR: 6-6.5 months). Thirteen patients died during the follow-up related to cancer progression. Local pain decreased from 35 ± 32 (SD) mm (range: 0-10 mm) to 11 ± 20 (SD) mm (range: 0-80 mm) at one month (P = 0.001); and to 22 ± 23 (SD) mm (range: 0-60 mm) at six months (P = 0.001)., Conclusion: CBCT navigation allows to effectively performing PSF of PBM even in the presence of steep angulations., Competing Interests: Conflicts of Interest The authors have no conflicts of interest to declare. The authors did not receive support from any organization for the submitted work., (Copyright © 2022 Société française de radiologie. Published by Elsevier Masson SAS. All rights reserved.)

The purpose of this retrospective review was to evaluate SpineJack implantation in cancer-related vertebral compression fractures in 13 consecutive patients (mean age, 62.8 years ± 18.8). A total of 36 devices were inserted at 20 levels (13 [65%] lumbar and 7 [35%] thoracic vertebrae), with a mean Spinal Instability Neoplastic Score of 9.1 ± 2.1. Vertebral height restoration was observed in 10 levels (50%), with a mean height restoration of 5.6 mm ± 2.2 (interquartile range [IQR], 4-7.5). A total of 6 cement leakages were observed in 3 (23%) patients without clinical consequences. No severe adverse events were observed. One adjacent fracture occurred. Average pain scores on the visual analog scale significantly improved from 5.5 ± 1.8 (IQR, 4-7) preoperatively to 1.5 ± 2.2 (IQR, 0-3.3) at 1 month (P < .01) and to 1.5 ± 1.3 (IQR, 0.3-2.8) at 6 months (P < .01). In this small cohort, SpineJack offered pain relief in cancer-related fractures without an observed increase in adverse events., (Copyright © 2021 SIR. Published by Elsevier Inc. All rights reserved.)

Background and objectives: Cancer-related vertebral compression fractures (VCF) may cause debilitating back pain and instability, affecting the quality of life of cancer patients. To further drive cement deposition during vertebroplasty, the aims of this restrospective case series study were to report the feasibility, safety and short term efficacy (≤6 months) of percutaneous vertebral fixation in cancer-related vertebral compression fractures using various intravertebral implants. Methods: All consecutive cancer patients treated with percutaneous vertebral fixation for VCF were retrospectively included. Various devices were inserted percutaneously under image guidance and filled by cement. Descriptive statistics were used and a matched paired analysis of pain scores was performed to assess for changes following interventions. Results: A total of 18 consecutive patients (12 women (66.6%) and 6 men (33.3%); mean age 59.7 ± 15.5 years) were included. A total of 42 devices were inserted in 8 thoracic and 16 lumbar vertebrae. Visual analogue scale measurement significantly improved from 5.6 ± 1.8 preoperatively to 1.5 ± 1.7 at 1 week ( p < 0.01) and to 1.5 ± 1.3 at 6 months ( p < 0.01). No severe adverse events were observed, but three adjacent fractures occurred between 1 week and 5 months after implantation. Conclusions: Percutaneous vertebral fixation of cancer-related VCF is feasible and safe and allows pain relief.

Ergonomics in interventional radiology has not been thoroughly evaluated. Like any operators, interventional radiologists are exposed to the risk of work-related musculoskeletal disorders. The use of lead shielding to radiation exposure and the lack of ergonomic principles developed so far contribute to these disorders, which may potentially affect their livelihoods, quality of life, and productivity. The objectives of this review were to describe the different situations encountered in interventional radiology and to compile the strategies both available to date and in development to improve ergonomics.

Background & Aims: Patients affected by hepatocellular carcinoma (HCC) represent a vulnerable population during the COVID-19 pandemic and may suffer from altered allocation of healthcare resources. The aim of this study was to determine the impact of the COVID-19 pandemic on the management of patients with HCC within 6 referral centres in the metropolitan area of Paris, France., Methods: We performed a multicentre, retrospective, cross-sectional study on the management of patients with HCC during the first 6 weeks of the COVID-19 pandemic (exposed group), compared with the same period in 2019 (unexposed group). We included all patients discussed in multidisciplinary tumour board (MTB) meetings and/or patients undergoing a radiological or surgical programmed procedure during the study period, with curative or palliative intent. Endpoints were the number of patients with a modification in the treatment strategy, or a delay in decision-to-treat., Results: After screening, n = 670 patients were included (n = 293 exposed to COVID, n = 377 unexposed to COVID). Fewer patients with HCC presented to the MTB in 2020 ( p  = 0.034) and fewer had a first diagnosis of HCC (n = 104 exposed to COVID, n = 143 unexposed to COVID, p  = 0.083). Treatment strategy was modified in 13.1% of patients, with no differences between the 2 periods. Nevertheless, 21.5% vs. 9.5% of patients experienced a treatment delay longer than 1 month in 2020 compared with 2019 ( p <0.001). In 2020, 7.1% (21/293) of patients had a diagnosis of an active COVID-19 infection: 11 (52.4%) patients were hospitalised and 4 (19.1%) patients died., Conclusions: In a metropolitan area highly impacted by the COVID-19 pandemic, we observed fewer patients with HCC, and similar rates of treatment modification, but with a significantly longer treatment delay in 2020 vs.  2019., Lay Summary: During the coronavirus disease 2019 (COVID-19) pandemic era, fewer patients with hepatocellular carcinoma (HCC) presented to the multidisciplinary tumour board, especially with a first diagnosis of HCC. Patients with HCC had a treatment delay that was longer in the COVID-19 period than in 2019., Competing Interests: JCN received a research grant from Bayer for INSERM UMR1138. The other authors declare no conflicts of interest that pertain to this study. Please refer to the accompanying ICMJE disclosure forms for further details., (© 2020 The Author(s).)

Background: The authors' purpose was to evaluate the results of axillary lymph node transplantation with noncontrast magnetic resonance lymphography in 15 patients with secondary upper limb lymphedema., Methods: Fifteen female patients with lymphedema following breast cancer treatment underwent lymph node transplantation. Noncontrast magnetic resonance lymphography was obtained with a free-breathing three-dimensional fast spin-echo sequence. Image analysis included criteria both before surgery (i.e., severity of lymphedema graded as absent, mild, moderate, or severe; involvement of the muscular compartment; and distal dilated lymphatic vessels) and after surgery (i.e., visualization of the site of transplantation; visualization of transplanted lymph nodes; and severity of lymphedema with regard to pretransplantation severity, namely, improvement, stability, or aggravation). Clinically, circumferential measures were performed at four different levels., Results: Follow-up magnetic resonance examinations were performed at least 6 months after lymph node transplantation, with a longest follow-up time of 42 months. In two patients, no lymphedema was visualized before lymph node transplantation with magnetic resonance lymphography. In the other 13 patients, lymphedema was mild in four patients, moderate in five patients, and severe in the other four patients. After lymph node transplantation, an improvement of upper limb lymphedema was observed in seven of nine patients with moderate or severe upper limb lymphedema. In the 11 patients in whom comparison of magnetic resonance lymphography with circumferential measurement was available, evaluations were concordant in 10 cases., Conclusion: Noncontrast magnetic resonance lymphography may be used as an objective technique to analyze the results of lymph node transplantation., Clinical Question/level of Evidence: Therapeutic, IV.

Chylothorax is a rare cause of pleural effusion, secondary to accumulation of lymph in the pleural space. Diagnosis is based on the triglyceride and cholesterol content of pleural fluid obtained with thoracentesis. Because the lymphatic system plays an essential role in fat absorption and immune response, lymphatic leak associated with chylothorax may cause life-threatening malnutrition and immunodeficiency. Chylothorax is usually described as traumatic or nontraumatic. The main cause of chylothorax is traumatic, typically postsurgical, secondary to iatrogenic direct puncture of the thoracic duct during thoracic surgery. Causes of nontraumatic chylothorax include a wide range of differential diagnoses. Lymphoma and thoracic malignancies are the most common causes and are responsible for chylothorax by extrinsic compression or invasion of the thoracic duct. Other rare causes include primary and secondary diffuse lymphatic diseases, responsible for chylothorax by lymphatic vessel wall dysfunction. Imaging the lymphatic system remains a challenge in the days of modern imaging. Nonenhanced MR lymphography is a noninvasive technique based on heavily T2-weighted sequences, thus enabling visualization of the lymphatic circulation. This technique allows diagnosis and differential diagnosis, evaluation of disease severity, and guidance of therapeutic management in nontraumatic chylothorax. Furthermore, it may offer radiologic classification of primary lymphatic diseases on the basis of morphologic features of lymphatic vessels. The authors describe the anatomy and physiology of the thoracic lymphatic system, present the technique of nonenhanced MR lymphography, and discuss pathophysiologic mechanisms and imaging features in different causes of nontraumatic chylothorax. © RSNA, 2020.

Background & Aims: Primary sclerosing cholangitis (PSC) has a variable, often progressive, course. Magnetic resonance cholangiography (MRC) is used in the diagnosis of PSC. Magnetic resonance risk scoring systems, called Anali without and with gadolinium, are used to predict disease progression, determined by radiologic factors. We aimed to assess the prognostic value of Anali scores in patients with PSC and validate our findings in a separate cohort., Methods: We performed a retrospective study of patients with large-duct PSC (internal cohort, 119 patients in France; external cohort, 119 patients in Canada, Italy, and the United Kingdom). All the first-available MRC results were reviewed by 2 radiologists and the Anali scores were calculated as follows: Anali without gadolinium = (1× dilatation of intrahepatic bile ducts) + (2× dysmorphy) + (1× portal hypertension); Anali with gadolinium = (1× dysmorphy) + (1× parenchymal enhancement heterogeneity). The primary end point was survival without liver transplantation or cirrhosis decompensation. The prognostic value of Anali scores was assessed by Cox regression modeling., Results: During a total of 549 patient-years for the internal cohort and 497 patient-years for the external cohort, we recorded 2 and 8 liver transplantations, 4 and 3 liver-related deaths, and 26 and 25 cirrhosis decompensations, respectively. In the univariate analysis, factors associated with survival without liver transplantation or cirrhosis decompensation in the internal cohort were as follows: serum levels of bilirubin, aspartate aminotransferase, alanine aminotransferase, γ-glutamyl transferase, alkaline phosphatase, albumin, and Anali scores. Anali scores without and with gadolinium identified patients' survival without liver transplantation or cirrhosis decompensation with a c-statistic of 0.89 (95% CI, 0.84-0.95) and 0.75 (95% CI, 0.64-0.87), respectively. Independent prognostic factors identified by multivariate analysis were Anali scores and bilirubinemia. The prognostic value of Anali scores was confirmed in the external cohort., Conclusions: In internal and external cohorts, we found that Anali scores, determined from MRC, were associated with outcomes of patients with PSC. These scores might be used as prognostic factors., (Copyright © 2019 AGA Institute. Published by Elsevier Inc. All rights reserved.)

Objectives: Magnetic resonance (MR) risk scores and liver stiffness (LS) have individually been shown to predict clinical outcomes in primary sclerosing cholangitis (PSC). The aim of this study was to assess their complementary prognostic value., Methods: Patients with PSC from 3 European centers with a 3-dimensional MR cholangiography available for central reviewing and a valid LS measurement assessed by vibration-controlled transient elastography by FibroScan performed within a 6-month interval were included in a longitudinal retrospective study. The MR score (Anali) without gadolinium (Gd) was calculated according to the formula: (1 × dilatation of intrahepatic bile ducts) + (2 × dysmorphy) + (1 × portal hypertension). The primary end point was survival without liver transplantation or cirrhosis decompensation. The prognostic values of LS and Anali score without Gd were assessed using Cox proportional hazard models., Results: One hundred sixty-two patients were included. Over a total follow-up of 753 patient-years, 40 patients experienced an adverse outcome (4 liver transplantations, 6 liver-related deaths, and 30 cirrhosis decompensations). LS and Anali score without Gd were significantly correlated (ρ = 0.51, P < 0.001) and were independently associated with the occurrence of an adverse outcome. Optimal prognostic thresholds were 10.5 kPa for LS and 2 for the Anali score without Gd. Hazard ratios (95% confidence interval) were 2.07 (1.06-4.06) and 3.78 (1.67-8.59), respectively. The use in combination of these 2 thresholds allowed us to separate patients into low-, medium-, and high-risk groups for developing adverse outcomes. The 5-year cumulative rates of adverse outcome in these 3 groups were 8%, 16%, and 38% (P < 0.001), respectively., Discussion: The combined use of MRI and vibration-controlled transient elastography permits easy risk stratification of patients with PSC.

Objectives: To describe the MR features and prognosis of patients with an uncommon complication of primary sclerosing cholangitis (PSC) characterized by a spontaneous perforation of the common bile duct (CBD) resulting in a peri-biliary collection and a pseudo-cystic appearance of the CBD., Methods: A single-center cohort of 263 patients with PSC who had at least two MRIs between 2003 and 2022 and a minimum follow-up of 1 year was retrospectively analyzed. MRI data (characteristics of CBD perforation and MR features of PSC) and clinical data were assessed. Analysis of survival without liver transplantation according to type of PSC (classical or CBD spontaneous perforation) was performed according to the Kaplan-Meier method and the curves were compared using the Log-Rank test., Results: A total of nine (3.4%) PSC patients (5 males) had perforation of the CBD with a median age at diagnosis of 18 years compared to 33 years for the control group (p = 0.019). The peri-biliary collections were variable in appearance (fusiform or pedunculated), with a diameter ranging from 5 to 54 mm. All nine patients showed intra- and extra-hepatic bile duct involvement, dysmorphia, and high ANALI scores. The clinical course was characterized by numerous complications in most patients, and five patients (56%) underwent liver transplantation at a median time of 5 years from diagnosis, compared to 40 patients (16%) in the control group (p = 0.02)., Conclusion: The spontaneous perforation of the common bile duct is an uncommon complication of primary sclerosing cholangitis that affects young patients and is associated with a poor prognosis., Clinical Relevance Statement: This uncommon complication of primary sclerosing cholangitis with perforation of the common bile duct resulting in a peri-biliary collection and a pseudo-cystic appearance of the common bile duct is characterized by a poor prognosis in younger patients., Key Points: • Among 263 patients with primary sclerosing cholangitis (PSC), nine patients (3.6%) had an uncommon complication characterized on MRI by perforation of the common bile duct (CBD). • This perforation of the CBD was responsible in all nine cases for the formation of a peri-biliary collection, giving a pseudo-cystic appearance to the CBD. • The spontaneous perforation of the common bile duct is an uncommon complication of primary sclerosing cholangitis that affects young patients with a poor prognosis., (© 2024. The Author(s), under exclusive licence to European Society of Radiology.)

Purpose: The purpose of the present study was to analyse the performance of non-contrast MR lymphography for the classification of primary lower limb lymphoedema in 121 consecutive patients with 187 primary lower limb lymphoedemas.Materials and Methods: 121 consecutive patients with clinically diagnosed primary lower limb lymphoedema underwent non-contrast MR lymphography with a free-breathing 3D fast spin-echo sequence with a very long TR/TE (4000/884 ms). MR examinations were retrospectively reviewed for severity of lymphoedema (absent, mild, moderate, severe) and characteristics of inguinal lymph nodes and iliac and inguinal lymphatic trunks graded as aplasic (no lymph nodes or lymphatic trunks), hypoplasic (less lymph nodes or lymphatic trunks), normal and hyperplasic (more lymph nodes or more and/or dilated trunks).Results: There was an excellent correlation between clinical stage and severity of lymphoedema (Cramer's V of 0,73 (p < 0.001)). Differentiation was feasible between inguinal lymphatic vessel aplasia (21%), hypoplasia (15%), normal pattern (53%) and hyperplasia (11%). Severe lymphoedema was observed in 46% of aplasic patterns and in 37% of hyperplasic patterns, but in only 15% of hypoplasic patterns and never observed in normal patterns (p < 0.001).Conclusion: Non-contrast MR lymphography is able to classify primary lower limb lymphoedemas into hyperplasic, aplasic, hypoplasic and normal patterns.Key Points: • Non-contrast MR lymphography is able to classify primary lower limb lymphoedemas. • Lymphoedema can be classified in hyperplasic, aplasic, hypoplasic and normal patterns. • Non-contrast MR lymphography can optimize clinical management of primary lower limb lymphoedemas. [ABSTRACT FROM AUTHOR]

This review shows the images obtained with non-contrast MR lymphography in different pathologic conditions affecting the liver. Non-contrast MR lymphography is obtained with a free-breathing 3D high spatial resolution fast-recovery fast spin-echo sequence similar to that used for 3D MR cholangiopancreatography. The liver is the largest lymph-producing organ generating approximately half of the body's lymphatic fluid and is the most important part of the lymphatic system from a functional point of view. Therefore, understanding the anatomy, physiology, and physiopathology of the lymphatics of the liver is important. However, its anatomy and pathology are relatively unknown because of the absence of commonly used imaging techniques. We describe the anatomy, the physiology, and the pathophysiology of the lymphatic system of the liver and the possibility of identifying dilated lymphatic vessels in various liver diseases and conditions. Disruption of normal lymphatic structure and function is observed in various disease conditions. Liver lymph flow is directly correlated with portal venous pressure. Therefore, a dilatation of liver lymphatics is observed in portal hypertension as well as in increased pressure in hepatic veins. After liver transplantation, ligation of lymphatic vessels at the hilum reduces chylous ascites and results in lymphatic dilatation which is easily observed. In severe long-standing biliary stenosis, dilated lymphatic vessels are commonly demonstrated with non-contrast MR lymphography. In hepatocellular carcinoma, intrahepatic cholangiocarcinoma, and some metastases, lymphatic vessels are abundant in the immediate vicinity of the tumour. These various lymphatic abnormalities can be demonstrated with non-contrast MR lymphography.Key Points • Anatomy and pathology of the lymphatics of the liver are relatively unknown, partly because of lack of current imaging technique. • Non-contrast MR lymphography is obtained with a free-breathing 3D high spatial resolution fast spin-echo sequence similar to that used for 3D MR cholangiopancreatography. • Non-contrast MR lymphography may participate to the understanding of several abnormal liver conditions including portal hypertension, biliary diseases, and malignant hepatic tumours.

Background: The aim of this study was to identify predictive criteria of improvement after endoscopic treatment (ET) for severe strictures of extrahepatic bile ducts in patients with primary sclerosing cholangitis (PSC)., Methods: PSC patients who had at least one ET for severe stricture were included. Features of magnetic resonance cholangiography (MRC), performed before ET, were evaluated according to a standard model of interpretation, and a radiologic qualitative score of probability of improvement after ET was built. Score 3 (likely) was given in case of severe common bile duct (CBD) stricture with marked dilatation without severe strictures of upstream ducts, Score 1 (unlikely) was given in case of severe multiple strictures of secondary ducts without biliary dilatation and Score 2 (undeterminate) was given to an intermediate pattern. The response to ET was assessed at 2 months (T2-response) from the last ET and at 12 months (T12-response) from inclusion., Results: Thirty-one patients were included. All had severe stricture (reduction ≥ 75% of the diameter) of CBD and 50% had severe stricture of right and/or left hepatic duct (LHD) at MRC before ET. According to the qualitative score, 16 patients had Score 3, 7 had Score 1 and 9 had Score 2. T12-response was obtained in 50% of patients. In univariate analysis, short LHD strictures, bilirubin, transaminases, pruritus and Score 3 were associated with T12-response. Increased bilirubin and transaminases were independent predictive factors of T12-response (HR 24, 95% CI: 3.4-170.4, P = 0.001 and 23.8, 95% CI: 3.4-169.4, P = 0.002, respectively)., Conclusion: MRC, together with biochemical features, may contribute to identify the PSC patients who are likely to be improved after ET for severe strictures of extrahepatic bile ducts., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Aims: To evaluate the prognostic value of cystic dilatation (CD) of the intrahepatic biliary ducts in patients with primary sclerosing cholangitis (PSC)., Methods: A single-center cohort of 205 patients with PSC from 2003 to 2016 was analysed. CD was defined by quantitative and qualitative criteria. Radiological and clinical courses were assessed. A Kaplan-Meier analysis was used to estimate cumulative survival without liver transplantation (LT) from the date of PSC diagnosis. A log-rank test was performed to compare survival time of PSC patients with and without CD., Results: A total of 15 (7.3%) PSC patients (12 males) with a median age of 23 years at diagnosis had CD. Five patients had one CD; seven patients had two or three CDs; and three patients had diffuse CD. CDs ranged in small diameter size from 12 to 32 mm. Radiological evolution of CD was markedly variable. However, a radiological worsening of PSC over time was observed in all patients. The clinical course was characterized by the occurrence of complications in most patients. Half of the patients with CD underwent LT at a median time of 40 months from diagnosis of CD and the median survival time from PSC diagnosis was significantly lower than in PSC without CD (10.7 vs. 23.4 years; HR 3.8, 95% confidence interval: 1.7-8.3, p = 0.001)., Conclusions: CD in PSC is an unusual condition that mostly affects young patients. It is characterized by a rapid, unfavorable course and constitutes a significant prognostic factor., Key Points: • Cystic dilatation of the intrahepatic biliary ducts affects young patients with primary sclerosing cholangitis and is characterized by a markedly variable radiological evolution. • Biliary wall inflammation, found in explanted livers, could be a key feature in the pathogenesis of cystic dilatation. • Cystic dilatation of the intrahepatic biliary ducts is characterized by an unfavorable course and constitutes a significant prognostic factor of primary sclerosing cholangitis.

Background: Imaging markers of biliary disease in primary sclerosing cholangitis (PSC) have potential for use in clinical and trial disease monitoring. Herein, we evaluate how quantitative magnetic resonance cholangiopancreatography (MRCP) metrics change over time, as per the natural history of disease., Methods: Individuals with PSC were prospectively scanned using non-contrast MRCP. Quantitative metrics were calculated using MRCP+ post-processing software to assess duct diameters and dilated and strictured regions. Additionally, a hepatopancreatobiliary radiologist (blinded to clinical details, biochemistry and quantitative biliary metrics) reported each scan, including ductal disease assessment according to the modified Amsterdam Cholangiographic Score (MAS)., Results: At baseline, 14 quantitative MRCP+ metrics were found to be significantly different in patients with PSC (N = 55) compared to those with primary biliary cholangitis (N = 55), autoimmune hepatitis (N = 57) and healthy controls (N = 18). In PSC specifically, baseline metrics quantifying the number of strictures and the number and length of bile ducts correlated with the MAS, transient elastography and serum ALP values (p < 0.01 for all correlations). Over a median 371-day follow-up (range: 364-462), 29 patients with PSC underwent repeat MRCP, of whom 15 exhibited quantitative changes in MRCP+ metrics. Compared to baseline, quantitative MRCP+ identified an increasing number of strictures over time (p < 0.05). Comparatively, no significant differences in biochemistry, elastography or the MAS were observed between timepoints. Quantitative MRCP+ metrics remained stable in non-PSC liver disease., Conclusion: Quantitative MRCP+ identifies changes in ductal disease over time in PSC, despite stability in biochemistry, liver stiffness and radiologist-derived cholangiographic assessment (trial registration: ISRCTN39463479)., (© 2024 The Authors. Alimentary Pharmacology & Therapeutics published by John Wiley & Sons Ltd.)