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Some hepato-biliary cancers require major liver resections. Post hepatectomy liver failure is a complication that occurs when the remnant liver cannot maintain its synthetic and excretory functions. To overcome this issue, portal vein embolization has been developed to induce future remnant liver hypertrophy preoperatively. However, up to 20% of patients cannot proceed to the hepatectomy due to insufficient hypertrophy or tumor progression in the interval between the embolization and the planned surgery. Liver venous deprivation (LVD) is a technique that combine ipsilateral portal and hepatic vein embolization. With this technique, the hypertrophy seems to be faster and more important, with low complications rate and no mortality associated with the procedure.Certains cancers hépatobiliaires nécessitent des résections hépatiques majeures. L’insuffisance hépatocellulaire est une complication postopératoire avec un risque de mortalité qui survient lorsque le foie restant ne peut maintenir ses fonctions de synthèse et d’excrétion. L’embolisation de la veine porte a été développée pour induire une hypertrophie du futur foie restant en préopératoire afin d’éviter cette complication. Cependant, 20 % des patients ne peuvent pas bénéficier de l’hépatectomie en raison d’une hypertrophie insuffisante ou d’une progression de la maladie oncologique dans l’intervalle entre l’embolisation et la chirurgie. L’embolisation portale et sus-hépatique combinée est une technique qui consiste à combiner durant le même geste l’embolisation de la veine porte et de la veine sus-hépatique ipsilatérale. Par cette technique, l’hypertrophie semble alors plus rapide et importante, avec peu de complications et aucune mortalité en lien avec la procédure.

Some hepato-biliary cancers require major liver resections. Post hepatectomy liver failure is a complication that occurs when the remnant liver cannot maintain its synthetic and excretory functions. To overcome this issue, portal vein embolization has been developed to induce future remnant liver hypertrophy preoperatively. However, up to 20% of patients cannot proceed to the hepatectomy due to insufficient hypertrophy or tumor progression in the interval between the embolization and the planned surgery. Liver venous deprivation (LVD) is a technique that combine ipsilateral portal and hepatic vein embolization. With this technique, the hypertrophy seems to be faster and more important, with low complications rate and no mortality associated with the procedure.

Abnormalities of umbilical-portal circulation are rare pathologies whose detection points in screening ultrasound are poorly taught. It can present as an unusual looking portal sinus, an abnormal trajectory of the umbilical vein, an anechoic intrahepatic image or more rarely as cardiomegaly. This can also be detected in the context of investigations of fetus with intrauterine growth retardation. Subsequently, the starting point of the diagnostic approach is based on the following dichotomy: does the umbilical vein penetrate or not into the liver, followed by systematic analysis of the trajectory and size of the umbilical-portosystemic vessels with color Doppler. Determining the prognosis of this abnormality, which varies according to the type, is a major challenge and by further studying this disorder in this project, it will help define what surveillance is required and subsequently help decide the most appropriate place for delivery.

Portal vein aneurysms are rare, representing 3% of venous aneurysms, with about 200 cases described in the literature, probably underestimated.A 66-year-old man, suspect of splenomegaly, underwent an abdominal ultrasound and a thoraco-abdomino-pelvic CT scan showing a 40mm portal vein aneurysm. Final diagnosis was T-cell hemopathy. Five and six months later, abdominal imaging was stable, suggesting congenital origin due to lack of obliteration of the vitelline vein.Portal vein aneurysms are often asymptomatic and an incidental finding. Monitoring is recommended because of their potential complications (thrombosis, rupture of aneurysm, portal hypertension, adjacent organs compression), annually if asymptomatic or more frequently with sometimes a surgical management in case of clinical manifestations.

Portal vein thrombosis is frequently encountered in patients with cirrhosis. Increased indication for non-invasive imaging in patients with cirrhosis has dramatically increased the recognition of PVT. This has led to a large amount of studies on pathogenesis as well as the prognostic impact of portal vein thrombosis on natural history of cirrhosis. However, in clinical practice, several aspects of the management of portal vein thrombosis remain unclear. This practical review discusses the most recent data toward the management of portal vein thrombosis in cirrhosis, especially regarding : a) the value of etiological workup ; b) the impact of portal vein thrombosis on the natural history of cirrhosis, and c) the indications and modalities of anticoagulation therapy.La thrombose porte est une complication fréquente au cours de la cirrhose. Sa reconnaissance a largement augmenté, grâce au développement de moyens d’imagerie non invasifs et performants. Au cours des dernières années, la pathogénie de la thrombose porte au cours de la cirrhose, ainsi que sa valeur pronostique ont été largement étudiées. Cependant, en pratique clinique, la prise en charge des malades atteints de cirrhose et de thrombose porte n’est pas établie. Cet article discute les données récentes de la littérature concernant la gestion pratique de la thrombose porte au cours de la cirrhose : a) la nécessité d’un bilan de thrombophilie ; b) l’influence de la thrombose porte sur l’histoire naturelle de la cirrhose et c) les indications et les modalités du traitement anticoagulant.

International audience; Portal vein embolization consists of occluding a part of the portal venous system in order to achieve the hypertrophy of the non-embolized liver segments. This technique is used during the preoperative period of major liver resection when the future remnant liver (FRL) volume is insufficient, exposing to postoperative liver failure, main cause of death after major hepatectomy. Portal vein embolization indication depends on the FRL, commonly assessed by its volume. Nowadays, FRL function evaluation seems more relevant and can be measured by 99mTc labelled mebrofenin scintigraphy. Portal vein embolization procedure is mostly performed with percutaneous trans-hepatic access by using ultrasonography guidance and consists of embolic agent injection, such as cyanoacrylate, in the targeted portal vein branches with fluoroscopic guidance. It is a safe and well-tolerated technique, with extremely low morbi-mortality. Portal vein embolization leads to sufficient FRL hypertrophy in about 80% of patients, allowing them to undergo surgery from which they were initially rejected. The two main reasons of non-resection are tumor progression (?15% of cases) and FRL insufficient hypertrophy (?5% of cases). When portal vein embolization is not enough to obtain adequate FRL regeneration, hepatic vein embolization may potentiate its effect (liver venous deprivation technique).

Portal and/or splenic vein thrombosis (PSVT) is common after splenectomy. It can be a life-threatening complication, with a risk of bowel ischemia and portal hypertension. An early diagnosis allows an effective medical treatment and prevents life-threatening complications. There is no consensus regarding the benefit of systematic screening of patients after splenectomy for PSVT. We started in January 2012 a routine screening of PSVT after elective splenectomy. The aim of this study was to assess this policy.Since January 2012, all patients undergoing an elective splenectomy had an abdominal CT-scan on postoperative-day 7. Demographic data, pathology, type of surgery, platelet counts before and after surgery, outcome, results of medical imaging, and management of PSVT and its results were recorded.Over 3 years, 52 patients underwent an elective splenectomy. All of them had a CT-scan at postoperative-day 7. A PSVT was found in 11 patients (21.2 %). They were all asymptomatic. Lymphoma and splenomegaly were the main factors associated with PSVT in the univariate analysis. All patients with PSVT were treated with anticoagulation and no complication of PSVT occurred. The follow-up CT confirmed the efficacy of anticoagulation therapy in all patients.Routine screening of PSVT after elective splenectomy is warranted because it allows to start anticoagulant therapy and avoid further life-threatening complications. The incidence of PSVT is particularly high among patients operated on for lymphoma or with splenomegaly.

The gastrointestinal nerves are crucial in the sensing of nutrients and hormones and its translation in terms of control of food intake. Major macronutrients like glucose and proteins are sensed by the extrinsic nerves located around the portal vein walls, which signal to the brain and account for the satiety phenomenon they promote. Glucose is sensed in the portal vein by neurons expressing the glucose receptor SGLT3, which activates the main regions of the brain involved in the control of food intake. Proteins indirectly act on food intake by inducing intestinal gluconeogenesis and its sensing by the portal glucose sensor. The mechanism involves a prior antagonism by peptides of the μ-opioid receptors present in the portal vein nervous system and a reflex arc with the brain inducing intestinal gluconeogenesis. In a comparable manner, short chain fatty acids produced from soluble fibers act via intestinal gluconeogenesis to exert anti-obesity and anti-diabetic effects. In the case of propionate, the mechanism involves a prior activation of the free fatty acid receptor FFAR3 present in the portal nerves and a reflex arc initiating intestinal gluconeogenesis.

Vascular liver diseases include a group of rare, even exceptional, diseases: portal vein thrombosis, obliterative portal venopathy and Budd-Chiari syndrome. Here we will discuss in detail the least rare, portal vein thrombosis, of which the main complication (when recanalization was not reached during the acute-phase) is esophageal variceal bleeding. However, a prothrombotic condition (including myeloproliferative syndrome) is often the cause of portal vein thrombosis. Thereby, in certain cases, a long-term anticoagulation is necessary despite the bleeding risk. Aside from these rare diseases, vascular and thrombotic involvement participate in the pathophysiology of cirrhosis, and anticoagulant treatment might be beneficial in some patients.

Idiopathic non-cirrhotic portal hypertension is an under-estimated cause of portal hypertension. The diagnosis requires the exclusion of cirrhosis, common causes of chronic liver disease and venous obstruction of the portal and hepatic veins. It has been associated with various extra-hepatic conditions that are most frequently immunologic, prothrombotic, hematologic and toxic. The most frequent clinical complications are variceal hemorrhage and portal vein thrombosis. Complications of portal hypertension should be managed as in patients with cirrhosis.

Portal cavernoma follows a chronic occlusion of the portal vein. The long-term consequences of portal cavernoma are not well known. The objective of this study was to report the aetiology of the portal cavernoma and its natural course after excluding liver diseases causes.A single centre retrospective study based on the data collected from the radiology department of the Clermont-Ferrand hospital was conducted from 2000 to 2011. All the patients for whom an imagery found a portal cavernoma have been looked for excluding the patients having a liver disease whatever the aetiology and the Budd-Chiari syndrome.Thirty-two cases (18 women and 14 men) were selected. The mean age at diagnosis was 54.2 years and the mean follow-up period was 5.4 years. The discovery of a portal cavernoma was incidental for 8 cases. An aetiology was found for 24 cases: it was an haematological aetiology in 15 cases (10 myeloproliferative syndromes, 2 antiphospholid syndromes, 1 thalassemia major, 1 hyperhomocysteinemia, 1 prothrombin gene mutation), a general aetiology in 2 cases (1 coeliac disease, 1 pancreatic neoplasia), and a local inflammation in 7 cases. A dysmorphic aspect of the liver was noticed on medical imaging for 11 out of the 32 cases. A liver biopsy was performed in 4 patients and was normal for all of them. Sixteen patients developed oesophageal varices, 4 patients developed ascites, 3 developed asymptomatic biliary compression by the portal cavernoma, and the patient who had been followed for the longest time (15 years) developed an encephalopathy.In addition to its underlying etiology, the prognosis of portal is mainly related to the occurrence of oesophageal varices that may develop during the follow-up of the patients.

We report the management of patient with acute abdominal pain in the immediate post-partum caesarean section performed in the context of preeclampsia. Acute portal vein thrombosis is diagnosed, in a patient with no personal or family history of venous thrombosis. Blood tests showed a protein C and S deficiencies. Portal vein thrombosis is an extremely rare occurrence in pregnancy and postpartum.

Invasive group A streptococcal infections are potentially serious. The occurrence in the neonatal period and meningeal location are two unusual situations. The complications reported in the literature vary; we add the risk of thromboembolic events. We report the case of a newborn, admitted to our department at 22 days of life for late neonatal group A streptococcal meningitis and diffuse cerebral infarction lesions. Ultrasound and abdominal CT scan objectified the presence of portal vein thrombosis and cavernoma. Echocardiography, electrocardiogram, as well as coagulation and thrombophilia tests were normal. Progression was marked by the installation of cerebral atrophy and ventricular dilation without the appearance of signs of portal hypertension over 18 months. We therefore concluded in neonatal group A streptococcal meningitis complicated by multiple thrombosis that can be explained by the invasive properties and hypercoagulability characterizing group A beta-hemolytic streptococcus. However, the characteristics of the fetal circulation may explain the possibility of paradoxical cerebral embolism from portal thrombosis.

Congenital portosystemic shunts (CPS) are rare vascular malformations that lead to liver metabolic modifications. The aim of this study was to describe the management and outcome of CPS in children who were followed in the western region of France and to compare our experience to a literature review.This retrospective study reports nine children with CPS who were followed in the western region of France between 2008 and 2013.Among these nine children, six were detected by prenatal ultrasonography, one had cholestatic jaundice during the postoperative period, one had cholestatic jaundice during the first days of life, and one had dyspnea and fatigue. Two children had liver tumors. The CPS was intrahepatic for four children and extrahepatic for two children. Three others had a patent ductus venosus. Among the intrahepatic shunts that were discovered before the age of 2 years, three closed spontaneously. The five other shunts were closed surgically or by interventional radiology techniques, after angiography for four children. After closure of eight shunts, the outcome was favorable for six children. Two children presented complications after surgery: portal hypertension and portal thrombus.When CPS is suspected, the diagnosis should be confirmed and complications should be investigated. Shunt closure restores portal flow and prevents complications.

There is a long-recognized association between cancer and venous thromboembolism. Venous thrombosis is the most common paraneoplastic complication. We describe a case of rupture of esophageal varices in a patient with a paraneoplastic portal thrombosis. We highlight the links between venous thromboembolism and cancer and also discuss treatment and prognostic factors.

Thèse placée sous licence Creative Commons CC-by-nc-nd (Paternité-Pas d'utilisation commerciale-Pas de modification); We present an original anatomic study of the intrahepatic portal three, based on a 307 abdominal MSCT analysis. Portal vein division variations are frequent (23,45%) and easily recognizable on 3D MIP views. Their detection is imperative before an interventional procedure (portal vein embolization, TIPS creation) or a complex surgical procedure (partial liver resection, liver transplantation). This classification provides a guide to the operating technique and detect the contraindications: type 1 is favorable for all interventions; type 2 is less favorable: it doesn't represent a contraindication to interventional radiology procedure but it makes the surgical split of the liver more difficult; type 3 need changes in interventional and surgical procedures because ignoring it could lead to major complications; type 4 contraindicates any interventional technique on the liver.; Nous présentons une étude anatomique originale de l'arborisation portale intra-hépatique à partir de l'analyse de scanners abdominaux de 307 sujets. Les variations de division de la veine porte sont fréquentes (23,45%) et facilement identifiables sur des vues 3D MIP. Leur connaissance est impérative avant de décider d'un geste de radiologie interventionnelle (embolisation, réalisation de shunt porto-systémique) ou chirurgical (hépatectomie partielle, transplantation hépatique). La classification proposée permet d'orienter la technique et de repérer les contre-indications : le type 1 est favorable à tout geste technique ; le type 2 est moins favorable : il ne représente pas une contre-indication à l'embolisation mais complexifie la partition chirurgicale du foie ; le type 3 nécessite des modifications des procédures de radiologie interventionnelle et chirurgicales car sa méconnaissance expose à des complications dramatiques ; le type 4 contre-indique tout geste d'embolisation ou de partition du foie.

Notre but est de rapporter une observation particulière de thrombose de la veine porte survenant au décours d'une hépatite auto-immune type 1 non compliquée qui, à notre connaissance, n'a pas été rapportée auparavant. Il s'agit d'une patiente de 35 ans connue ayant une dermatose bulleuse auto immune (DBAI) type pemphigus confirmée histologiquement et immunologiquement qui fut explorée pour des coliques hépatiques avec élévations des transaminases et un épisode d'ictère spontanément résolutif. Les explorations ont permis de retenir le diagnostic d'une hépatite auto immune de type 1. Traitée par corticothérapie systémique à la dose de 1 mg/kg/j pour sa DBAI, l'évolution était favorable avec stabilisation simultanée de l'atteinte hépatique durant 19 ans. On découvre sur l'échographie abdominale de contrôle une thrombose partielle du tronc porte confirmée par le scanner X abdominal. Le bilan étiologique de cette thrombose est resté négatif. De même il n'y avait pas de signes cliniques, biologiques, endoscopiques ou radiologiques de cirrhose ni de dégénérescence maligne. Elle était efficacement antigoagulée par les antagonistes de la vitamine K. Dans notre observation le bilan étiologique; aussi exhaustif que possible, de cette thrombose est resté négatif, éliminant en particulier une cirrhose, une dégénérescence maligne et un syndrome des anti phospholipides associé et permettant de la rattacher directement à l'hépatopathie chronique auto immune.

Portal venous gas is a worrying radiological sign that was described for the first time in 1955 among newborn children suffering from necrotizing enterocolitis. It was often related to mesenteric ischemia which mortality remains very high. Now, as radiological explorations increase, portal venous gas is more and more described in less pejorative contexts, such as hydrogen peroxide intoxication. It is what happened to our patient who underwent a hydrogen peroxide colic enema followed by a major and transient portal venous gas onset. This solved quickly and spontaneously.

The purpose was to determine the efficacy and technical particularities related to the use of Amplatzer(®) Vascular Plugs (AVP) for preoperative portal vein embolization. Between 2005 and 2009, a total of 48 type I AVP were embolized into the portal venous system of 17 patients (51-83 years) prior to extended hepatic resection where the residual liver volume (RLV) was deemed sufficient (RLV35-40% in patients with underlying hepatocellular disease,25-30% in patients with normal liver). AVP were used alone in seven patients and combined to other embolization agents in 10 patients (coils: n=5, microparticles: n=1, resorbable gel foam: n=4). The procedure was technically successful in 100% of cases with immediate success rate of 94.1% (imcomplete embolization of a segmental branch of segment VIII). The procedure was well tolerated clinically in 94.1% of cases, and in 100% of cases based on laboratory values. The rate of recanalization on follow-up CT at 5 weeks (2-22) was 11.7%. The rate of complications, major (left portal vein thrombosis following right portal vein embolization) and minor (one case of portovenous fistula), was 11.7%. The rate of RLV growth was from +13 to +285 cm(3) (mean at +122 cm(3)), or +4.98 to +78.51% (mean at +33.3%) (hepatocellular carcinoma: mean of +30.7%, metastases: mean of +19.7%). The rate of surgical candicacy was 94.1% (two patients were excluded: insufficient growth of RLV, development of peritoneal carcinomatosis). AVP appear to be reliable and effective for the preoperative embolization of the portal vein, with low morbidity and sufficient growth of RLV.

Most of patients with hepatocellular carcinoma (HCC) cannot benefit from surgical therapies. Among nonsurgical options, only radiofrequency can challenge surgery for small size tumours. Conformal radiotherapy is likely highly efficient on solitary tumours, but controlled studies are warranted to conclude. Other options are purely palliative. Transarterial hepatic chemoembolization is the goal-standard for multifocal hepatocellular carcinoma and sorafenib for hepatocellular carcinoma with portal vein invasion, leading to modest but significant benefit on survival rates. Yttrium-90 radioembolization is under evaluation through controlled studies, and could be of major interest for multifocal hepatocellular carcinoma with or without portal venous invasion.

The liver is a large abdominal organ in the right hypondrium. Because of its anatomical situation, it is near many abdominal PTVs as well as some lower thoracic PVTs. The liver could also be at the same time the target (for irradiation of liver metastases or primary liver tumours) and organ at risk (OAR). Radiation-induced liver disease (RILD) is radiobiologically the normal tissue complication probability (NTCP), i.e., the clinical event limiting the total dose that could be delivered. This review describes radiobiological criteria justifying the NTCP data, and recommendations for conformal 3D radiotherapy and stereotactic liver irradiation.

Septic pylephlebitis or purulent thrombosis of the portal venous system generally results from a progressive extension of suppurated thrombophlebitis, secondary to an intrabdominal infection. Germs most often found are Escherichia coli and Streptococcus, isolation of Enterobacter cloacae is unusual. We report a particular observation of septic pylephlebitis associated with E. cloacae bacteremia, without biliary, digestive or pancreatic lesion on the CT-scan. The antibiotic sensitivity pattern of the isolated germ and the negative epidemiologic investigation pled in favour of community acquired infection. The infection resolved with antibiotics and anticoagulation, followed by total repermeation of the portal system.

We report a case of portal vein stenosis six months after previous orthotopic liver transplantation. The patient presented with mesenteric venous ischemia. He underwent successful percutaneous transhepatic portal vein angioplasty and stent placement.

Pylephlebitis or septic thrombophlebitis of the portomesenteric veins is a complication of intra-abdominal infections. The disease is rare in children and the diagnosis is often delayed. The morbidity of pylephlebitis is relatively low, although there is a risk of residual thrombosis. We report on 2 cases of pylephlebitis in a 12-year-old girl and a 13-year-old boy, following undiagnosed appendicitis. In the 1st case, the young girl had been misdiagnosed with Salmonella infection and was given antibiotics; in the 2nd case, the boy had retrocecal appendicitis that was clinically subacute. An accurate diagnosis was finally made in both cases by CT scan. Both children evolved satisfactorily following appendectomy, long-term antibiotics, and anticoagulation. Clinically, the severe sepsis associated with pylephlebitis is at the forefront. Physical examination is often normal and therefore of little help; the knowledge of a preceding abdominal infection leads to further radiological investigations. Biologically, there are pronounced signs of infection. CT is the preferred exam for diagnosing pylephlebitis, as it can also show the underlying cause of the intra-abdominal sepsis or possible complications. Doppler sonography is recommended more for follow-up of the portal vein thrombosis. Treatment of pylephlebitis associated with appendicitis always includes long-term antibiotics. An appendectomy is always performed either at the time of diagnosis or later. The need for anticoagulation therapy in children is controversial. However, most pediatricians recommend its use, beginning as soon as possible, to be continued until normalization of portal vein flow.

The association of cholelithiasis and portal cavernoma is rarely described in adult or pediatric patients. We report 2 cases of gallstone associated with portal cavernoma in 2 girls. The first one suffered from Evans syndrome associated with congenital immune deficiency. The portal cavernoma was discovered with gallstone after splenectomy indicated because of high steroid dependence. In the second case, the cavernoma complicated neonatal umbilical catheterism. The gallstone was asymptomatic and discovered on annual ultrasonography. Septicemia, profound thrombocytopenia, and acute anaemia led to rapid death in the first case. However, the progression was favourable under celioscopic treatment in the second one. Our original observations suggest systematically searching for gallstone in children with portal cavernoma.

It is rare for portal vein thrombosis to complicate colorectal liver metastases. However malignant portal vein thrombosis must be anticipated when considering hepatic resection. While this finding may influence long-term survival, it does not absolutely contraindicate hepatic resection. We report here a case of colorectal metastasis to the liver with associated macroscopic malignant portal vein thrombosis treated with hepatic resection; the patient is free from recurrence at 5-year follow-up.

Portal vein thrombosis is a major complication of splenectomy. Its frequency is underestimated because of asymptomatic cases. Mesenteric occlusion with intestinal infarcts is the first cause of mortality. Secondarily, in the absence of repermeabilisation, a portal hypertension can occur. We present in this study 4 cases of portal vein thrombosis in childhood. Portal vein thrombosis is frequent (8% of splenectomies) and may be asymptomatic. Doppler postoperative surveillance is justified. Thrombocytosis seems to be a determinant factor. Early diagnosis and treatment may reduce lethal outcome.

Pylephlebitis or septic portal thrombophlebitis is a rare but serious condition which may occur following intra-abdominal sepsis from any source. Sigmoid diverticulitis is one of the most common sources. Modern imaging modalities, particularly CT, have increased the recognition of this condition. Standard treatment consists of anticoagulation plus antibiotic therapy to cover anaerobic and gram negative organisms. The duration of anticoagulation therapy remains controversial. Sigmoid colectomy may be required in cases of perforated diverticulitis or failure of medical therapy.

Septic pylephlebitis is usually a complication of intraabdominal infection in the region drained by the portal venous system. We report two cases of portal and mesenteric venous thrombosis associated with Fusobacterium necrophorum bacteremia, which did not show any obvious intra-abdominal source of infection with noninvasive imaging procedures. In one case, early anticoagulation treatment was associated with repermeation of the portal vein and its right branch. As in Bacteroides bacteremia, portal and/or mesenteric venous thrombosis should be searched for in case of Fusobacterium bacteremia of unknown origin. Repermeation of the portal vein and relief of extrahepatic portal hypertension can be achieved in these cases with early anticoagulation.

Spontaneous intrahepatic portosystemic shunts are rare vascular anomalies that consist of a communication between the portal system and the systemic venous circulation. We report a case of a porto-caval shunt associated with chronic pericarditis.A 47-year-old patient with post pericarditis cirrhosis and without encephalopathy and hypoglycaemia. The shunt was tubular in its initial segment and aneurismal just before joining the vena cava.Spontaneous intrahepatic portosystemic shunt is a rare anomaly. Diagnosis can be made by Doppler ultrasound and helical CT.