Review

Ischemic cholangiopathy

La médiolyse artérielle segmentaire (SAM) est une vasculopathie rare, non athérosclérotique et non inflammatoire, affectant principalement les vaisseaux splanchniques de taille moyenne.

Nous rapportons le cas d’un patient porteur de multiples anévrismes artériels viscéraux, ayant présenté un hémopéritoine et un choc hémorragique. Le diagnostic de SAM a été retenu après avoir écarté les autres causes de vascularite.

Malgré sa rareté et l’absence de critères diagnostiques standardisés, la SAM doit être diagnostiquée rapidement car elle peut menacer le pronostic vital. La démarche diagnostique doit permettre de différencier la SAM des vascularites inflammatoires et infectieuses, ainsi que de la dysplasie fibromusculaire. Lorsque les patients présentent un anévrisme rompu, la stratégie thérapeutique repose essentiellement sur le traitement endovasculaire.

Nous rapportons le cas d’un patient avec des pseudo-anévrismes hépatiques rompus traités par embolisation de l’artère hépatique propre, et chez qui le diagnostic de SAM a été retenu au bout des investigations.

Segmental arterial mediolysis (SAM) is a rare, non-atherosclerotic and non-inflammatory vasculopathy, affecting mainly the medium-sized abdominal splanchnic vessels.

We report the case of a patient with multiple visceral artery aneurysms who presented with hemoperitoneum and hemorrhagic shock. The diagnosis of SAM was retained after ruling out other causes of vasculitis.

Despite its rareness and the absence of standard diagnostic criteria, SAM needs to be diagnosed quickly, as this condition can be life threatening. Diagnosis assessment should lead to distinguish SAM from inflammatory and infectious vasculitis, as well as fibromuscular dysplasia. When patients present with ruptured aneurysms, the therapeutic strategy is essentially based on endovascular treatment.

We report the case of a patient with a presumptive diagnosis of segmental arterial mediolysis, with ruptured hepatic pseudoaneurysms managed successfully by coil embolization of the hepatic artery.

In the liver, biliary epithelial cells (BECs) line intrahepatic bile ducts (IHBDs) and are primarily responsible for modifying and transporting hepatocyte-produced bile to the digestive tract. BECs comprise only 3% to 5% of the liver by cell number but are critical for maintaining choleresis through homeostasis and disease. To this end, BECs drive an extensive morphologic remodeling of the IHBD network termed ductular reaction (DR) in response to direct injury or injury to the hepatic parenchyma. BECs are also the target of a broad and heterogenous class of diseases termed cholangiopathies, which can present with phenotypes ranging from defective IHBD development in pediatric patients to progressive periductal fibrosis and cancer. DR is observed in many cholangiopathies, highlighting overlapping similarities between cell- and tissue-level responses by BECs across a spectrum of injury and disease. The following core set of cell biological BEC responses to stress and injury may moderate, initiate, or exacerbate liver pathophysiology in a context-dependent manner: cell death, proliferation, transdifferentiation, senescence, and acquisition of neuroendocrine phenotype. By reviewing how IHBDs respond to stress, this review seeks to highlight fundamental processes with potentially adaptive or maladaptive consequences. A deeper understanding of how these common responses contribute to DR and cholangiopathies may identify novel therapeutic targets in liver disease.

Pancreatic ductal adenocarcinoma (PDAC) is the most prevalent pancreatic neoplasm accounting for more than 90% of pancreatic malignancies. Surgical resection with adequate lymphadenectomy remains the only available curative strategy for patients with PDAC. Despite improvements in both chemotherapy regimen and surgical care, body/neck PDAC still conveys a poor prognosis because of the vicinity of major vascular structures, including celiac trunk, which favors insidious disease spread at the time of diagnosis. Body/neck PDAC involving the celiac trunk is considered locally advanced PDAC in most guidelines and therefore not eligible for upfront resection. However, a more aggressive surgical approach (i.e., distal pancreatectomy with splenectomy and en-bloc celiac trunk resection [DP-CAR]) was recently proposed to offer hope for cure in selected patients with locally advanced body/neck PDAC responsive to induction therapy at the cost of higher morbidity. The so-called “modified Appleby procedure” is highly demanding and requires optimal preoperative staging as well as appropriate patient preparation for surgery (i.e., preoperative arterial embolization). Herein, we review current evidence regarding DP-CAR indications and outcomes as well as the critical role of diagnostic and interventional radiology in patient preparation before DP-CAR, and early identification and management of DP-CAR complications.

Ischemic cholangiopathy is a process of bile duct injury that might result from peribiliary vascular plexus (PBP) thrombosis and remains a dreaded complication in liver transplantation from donors after circulatory death (DCD). The aim of this study was to propose a mechanical method of clot destruction to clear microvascular thrombi in DCD livers before transplantation.

Sonothrombolysis (STL) is a process by which inertial cavitation of circulating microbubbles entering an ultrasound field create a high-energy shockwave at a microbubble-thrombus interface, causing mechanical clot destruction. The effectiveness of STL in DCD liver treatment remains unclear. We carried out STL treatment during normothermic, oxygenated, ex vivo machine perfusion (NMP), introducing microbubbles into the perfusate with the liver enveloped in an ultrasound field.

The STL livers showed reduction in hepatic arterial and PBP thrombus and decreases in hepatic arterial and portal venous flow resistance, reduced parenchymal injury as measured by aspartate transaminase release and oxygen consumption, and improved cholangiocyte function. Light and electron microscopy showed reduction of hepatic arterial and PBP thrombus in STL livers compared with controls and preserved hepatocyte structure, sinusoid endothelial morphology, and biliary epithelial microvilli.

In this model, STL improved flow and functional measures in DCD livers undergoing NMP. These data suggest a novel therapeutic approach to treat PBP injury in DCD livers, which may ultimately increase the pool of grafts available to patients awaiting liver transplantation.