Protective potential of the gallbladder in primary sclerosing cholangitis

Background & Aims: Gallbladder enlargement is common in patients with primary sclerosing cholangitis (PSC). The gallbladder may confer hepatoprotection against bile acid overload, through the sequestration and cholecystohepatic shunt of bile acids. The aim of this study was to assess the potential impact of the gallbladder on disease features and bile acid homeostasis in PSC.Methods: Patients with PSC from a single tertiary center who underwent liver MRI with three-dimensional cholangiography and concomitant analyses of serum bile acids were included. Gallbladder volume was measured by MRI and a cut-off of 50 ml was used to define gallbladder enlargement. Bile acid profiles and PSC severity, as assessed by blood tests and MRI features, were compared among patients according to gallbladder size (enlarged vs. normal-sized) or presence (removed vs. conserved). The impact of cholecystectomy was also assessed in the Abcb4 knockout mouse model of PSC.Results: Sixty-one patients with PSC, all treated with ursodeoxycholic acid (UDCA), were included. The gallbladder was enlarged in 30 patients, whereas 11 patients had been previously cholecystectomized. Patients with enlarged gallbladders had significantly lower alkaline phosphatase, a lower tauro-vs. glycoconjugate ratio and a higher UDCA vs. total bile acid ratio compared to those with normal-sized gallbladders. In addition, gallbladder volume negatively correlated with the hydrophobicity index of bile acids. Cholecystectomized patients displayed significantly higher aspartate aminotransferase and more severe bile duct strictures and dilatations compared to those with conserved gallbladder. In the Abcb4 knockout mice, cholecystectomy caused an increase in hepatic bile acid content and in circulating secondary bile acids, and an aggravation in cholangitis, inflammation and liver fibrosis.Conclusion: Altogether, our findings indicate that the gallbladder fulfills protective functions in PSC.Impact and implications: In patients with primary sclerosing cholangitis (PSC), gallbladder status impacts on bile acid homeostasis and disease features. We found evidence of lessened bile acid toxicity in patients with PSC and enlarged gall-bladders and of increased disease severity in those who were previously cholecystectomized. In the Abcb4 knockout mouse model of PSC, cholecystectomy causes an aggravation of cholangitis and liver fibrosis. Overall, our results suggest that the gallbladder plays a protective role in PSC.& COPY; 2022 The Authors. Published by Elsevier B.V. on behalf of European Association for the Study of the Liver (EASL). This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/)

Letter: the use of magnetic resonance scores (Anali) for risk stratification in PSC

International audienceGBCA gadolinium-based contrast agent MR magnetic resonance MRC magnetic resonance cholangiography PSC primary sclerosing cholangitis VCTE vibration controlled transient elastography Dear editor, We read with interest the review written by Mahzar and Russo about non-invasive prognostic tests for primary sclerosing cholangitis (PSC) 1. Although rare, PSC is a serious disease, associated with potentially lethal complications, notably cirrhosis and cholangiocarcinoma 2. Liver transplantation is the only curative treatment for PSC and median transplant-free survival is approximately 12-21 years after diagnosis 3. Identification of prognostic factors is essential for tailoring the follow-up strategies and testing new therapeutic modalities in homogeneous groups of PSC patients. As detailed in this review, over the last twenty years, different teams across the world, including ours, have identified several noninvasive prognostic tests for PSC patients 1. First, our team demonstrated in a large retrospective study that liver stiffness measured by vibration-controlled transient elastography (VCTE) was an independent predictive marker of survival in PSC patients 4. Next, we built 2 magnetic resonance (MR) risk scores (with and without gadolinium-based contrast agent (GBCA) administration) 5 , called Anali scores, and showed that they were able to predict adverse outcome-free survival (defined by survival without liver transplantation or cirrhosi

Non-contrast 3D MR lymphography of retroperitoneal lymphatic aneurysmal dilatation: a continuous spectrum of change from normal variants to cystic lymphangioma

OBJECTIVE: Our objective was to demonstrate the characteristic features of retroperitoneal lymphatic aneurysmal dilatation with three-dimensional (3D) magnetic resonance (MR) lymphography. CONCLUSION: Three-dimensional MR lymphography demonstrates that retroperitoneal lymphatic aneurysmal dilatation exhibits a continuous spectrum of change from normal variants to lymphatic aneurysmal dilatation and so-called cystic lymphangioma. MAIN MESSAGE: Non-contrast MR lymphography with very heavily T2-weighted fast spin echo sequences is a useful non-invasive technique without the need of contrast medium injection to obtain a unique evaluation of the lymphatic system. To prove the lymphatic origin of a cystic formation, it is essential to demonstrate the communication with retroperitoneal lymphatic vessels; 3D MR lymphography demonstrates that retroperitoneal lymphatic aneurysmal dilatation exhibits a continuous spectrum of change from normal variants to lymphatic aneurysmal dilatation and so-called cystic lymphangioma

Post-traumatic massive hand lymphedema fully cured by vascularized lymph node flap transfer

Post-traumatic localized hand lymphedema is a rare situation and its diagnosis may be difficult, causing lack of care leading to failure of care. Our case study is of two young women with massive post-traumatic hand lymphedema who were treated for algodystrophy for 2 years, and whose bandages and physiotherapy were unsuccessful. Major social and psychological consequences due to difficulty with diagnosis and management resulting in inappropriate tests and therapeutic treatment were prescribed due to these issues. Noncontrast magnetic resonance lymphography revealed complete lymphatic vessel blockage in the hand and wrist. A vascularized lymph node flap harvested at the groin level was transferred to the elbow level 1 month after local dermolipectomy. These procedures resulted in the restoration of lymphatic flow. Both patients were definitely cured, and they returned to normal life within 6 months after surgery. Lymph node flap transfer associated with dermolipectomy may cure massive localized lymphedema in selected cases

Non-contrast MR lymphography of the lymphatic system of the liver NON-CONTRAST MR LYMPHOGRAPHY OF THE LYMPHATIC SYSTEM OF THE LIVER

International audienceThis 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