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A 43-year-old-woman admitted to our department because of abdominal pain, abdominal distension, pain on both inner thighs and blurred vision lasting for 3 months. Pancytopenia and positive anti-dou...

Resumen La vena porta es un conducto que drena el flujo esplácnico al hígado y se puede ocluir por diferentes patologías, variando su presentación clínica de acuerdo con la causa de la obstrucción. Es muy importante diferenciar la trombosis portal asociada o no a la cirrosis, ya que su tratamiento y pronóstico es diferente. La trombosis venosa portal extrahepática es una condición netamente de origen vascular, y es la principal causa de trombosis portal en niños y adultos. Presentamos tres casos tratados con derivación meso-Rex, con seguimiento a 6 meses. Abstract The portal vein is a conduit that drains splanchnic flow to the liver, it can be occluded by different pathologies and its clinical presentation varies according to the cause of the obstruction. It is very important to differentiate portal thrombosis associated or not with cirrhosis, since its treatment and prognosis is different. Extrahepatic portal vein thrombosis (PEVT) is a condition of purely vascular origin, being the main cause of portal thrombosis in children and adults. We present three cases with meso-Rex shunt, with a 6-month follow-up.

Purpose. To study contrast enhancement patterns of hepatocellular carcinoma (HCC) in cases with portal thrombosis and without it.Materials and methods. 61 consecutive patients with HCC were included in the study and divided into two groups: 41 patient with portal vein thrombosis (main group) and 20 patients without thrombosis (control group). Each patient underwent a abdominal CE MDCT for the analysis of the lesion contrast enhancement.Results. The 1st – “classic” – contrast enhancement pattern with a peak at arterial phase was observed in 2 (5%) patients with HCC complicated by portal thrombosis and in 10 (50%) patients without thrombosis (p < 0.01). The 2nd – “hypervascular” pattern with a peak shifted in 60–120s after contrast agent administration and no wash out was observed in 14 (34%) patients in the main group and in 10 (50%) patients in the control group (p = 0.4). The 3rd pattern – inhomogeneous contrast enhancement of liver parenchyma in the territory of thrombosed vein without visible tumor was discovered in 17 (41%) cases (p < 0.01). The 4th pattern – peripheral enhancement in arterial and portal phase with insignificant central enhancement in delayed phase was visualized in 8 cases (20%, p = 0.072).Conclusion. Portal thrombosis, which complicates the course of HCC, significantly changes the patterns of tumor’s contrast enhancement. This should be taken into account when image analysis to avoid diagnostic errors.

INTRODUCTION: Sarcoidosis is a systemic granulomatous disease, characterized by the formation of non-necrotizing granulomas. Even though granulomas are frequently found in liver biopsy, related symptoms rarely occur. In the current article, a case report is pictured to increase the knowledge on portal hypertension in hepatic sarcoidosis. CLINICAL SITUATION: A 62-year-old female was diagnosed with variceal bleeding for which elastic banding was performed. The patient was admitted to the intensive care unit (ICU) as the bleeding persisted and she evolved in hemorrhagic shock. Liver ultrasound detected nodular hepatomegaly and partial portal thrombosis. Chest CT showed diffuse hilar adenopathies and interstitial micronodular lesion. Finally, PET-CT detected metabolic active liver, bone marrow, and upper and lower diaphragmatic adenopathies. CLINICAL RESOLUTION: Multidisciplinary discussion brought major advantages in rapid diagnosis and prompt effective treatment. Cirrhosis was diagnosed by liver nodularity onimaging and liver biopsy. Sarcoidosis diagnosis was supported by the biopsies of liver and lymph node, which yielded non-caseating granulomas infiltration. Chest CT scan and PET-CT were also consistent with this diagnosis. The complementary analysis excluded differential diagnosis. The patient was treated with high-dose methylprednisolone with notable clinical improvements and discharge from the ICU. CONCLUSION: Hepatic sarcoidosis can present as life-threatening bleeding due to variceal bleeding caused by portal hypertension. Differential diagnosis is broad when hepatic sarcoidosis is suspected. Therefore, a multidisciplinary discussion is warranted. Anatomopathological examination of two potentially involved organs should be considered to make the appropriate diagnosis. Further studies are requested to investigate the pathophysiological mechanism of portal hypertension.

Thrombosis of the portal vein (PVT) is generally seen in the setting of liver cirrhosis and to a lesser extent in the absence of cirrhosis. There is no clear guidance in relation to approaching treatment with anticoagulation in this condition. The professional societies and guidelines recommend treatment with traditional anticoagulation like low-molecular-weight heparin and vitamin-K antagonists in patients presenting with acute portal vein thrombosis. There is no clarity in relation to treatment in the setting of chronic PVT and in patients with cirrhosis. Also, the role of direct-acting oral anticoagulants (DOACs) that are becoming a preferred choice for anticoagulation for various other indications is not clear in the case of PVT. There are a very few studies in the medical literature that have investigated the role of DOACs in patients with PVT in different settings. Thus, we performed a systematic review of the literature to study the use of DOACs in PVT in patients with and without cirrhosis. The results of the available studies show that DOACS appears to be a promising choice for the treatment of patients with PVT. The availability of more data in the future along with better availability of the approved reversal agents for various DOACs is expected to make DOACS a preferred choice for the clinicians to treat patients with PVT.

Introduction:Treatment of perihilar cholangiocarcinoma (PHC) usually requires extended resection after inducing hypertrophy of the future liver remnant (FLR). Associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) can achieve rapid hypertrophy of the FLR. Though, due to significant morbidity and mortality, portal vein embolization (PVE) is considered gold standard. Despite remaining controversies, ALPPS might suit as reserve in patients who failed to achieve adequate hypertrophy of the FLR or suffered complications following PVE. We illustrate a rescue-ALPPS after inadvertent nontarget thrombosis of the FLR following PVE in a patient with PHC. Presentation of Case:A 67-year-old patient requiring right trisectionectomy for PHC Bismuth type IV suffered inadvertent nontarget portal thrombosis of the FLR following PVE. Subsequently, insufficient FLR hypertrophy prevented the planned surgical resection. ALPPS procedure with concomitant thrombectomy of the left portal vein was used as a rescue strategy for this patient. Discussion:Since ALPPS is associated with significant limitations, especially in patients with PHC, this approach remains controversial. However, surgery still remains the only curative option for patients with PHC and thus, in case of inadequate hypertrophy of the FLR or technical failure following PVE, these patients lack further treatment options. Recent technical refinements and methods of improved patient selection have the potential to emend outcomes of ALPPS in experienced centres. Conclusion:ALPPS should be considered as reasonable rescue strategy not only in case of insufficient hypertrophy of the FLR but also in the event of technical failure or complications following PVE, even in patients with PHC.

Introduction Portal vein thrombosis (PVT) is a relatively common finding in patients undergoing liver transplantation. Although the recommendation to prevent its recurrence is anticoagulation for a duration of 3 to 6 months, this is controversial. Aim The aim of our study was to determine the efficacy of oral anticoagulants (OAC) as prophylaxis for recurrent PVT after liver transplantation. Materials and Methods Our study included 215 liver transplant patients who underwent surgery in our center from January 2012 to August 2017. We selected all patients diagnosed with PVT either pre-transplantation (using Doppler echography or Angio-CT) or during transplant surgery. All patients with PVT were initially anticoagulated with low-molecular-weight heparin in the postoperative period; at discharge they received OAC for a duration of six months. Control Doppler ultrasound was performed at 3, 6, and 12 months post-transplantation. Results PVT was identified in 37 out of 215 patients (17.2%). PVT was diagnosed with a pre-transplant vascular study in 17 out of 37 cases (45.9%). All patients were anticoagulated with OAC (warfarin) for at least 6 months. There were no cases of recurrent thrombosis and no complications associated with anticoagulant treatment throughout the follow-up period. Conclusions The prevalence of portal thrombosis in liver transplant patients in our study was fairly high, at 17.2%. PVT was identified in nearly 50% of patients using high-quality vascular studies prior to transplant surgery. Anticoagulation with OAC for 6 months was effective in preventing a recurrence of thrombosis and there were no associated complications.

The digestive manifestations of a SARS-CoV-2 infection are varied and nonspecific. The appearance of portal thrombosis in these patients is very rare. Facing a patient with a diagnosis of acute portal thrombosis, we must rule out that the trigger is an intra-abdominal infectious process. We present the case of a patient diagnosed with severe pneumonia due to SARS-CoV-2 infection with elevated D-Dimer and a concomitant diagnosis of portal thrombosis not attributed to other causes.

A surgical technique to intra-operatively define segmental boundaries by US-guided bimanual liver compression has been described by the authors, but this procedure is contraindicated in case of portal tumor thrombus. A technique to overcome this limitation is described. A patient with a single hepatocarcinoma nodule and segment 8 (S8) portal branch thrombosis was submitted to the procedure. Anatomical demarcation of S8 was achieved by hilar clamping of the common hepatic artery, intravenous injection of indocyanine green (ICG), and fluorescence imaging analyses of the liver. The procedure was feasible and the demarcation of S8 was visible within 2 min from the iv injection of ICG in a counterstaining fashion. Then S8 segmentectomy was safely carried out. This novel approach seems feasible, providing a reliably anatomical and conservative removal of HCC with portal branch tumor thrombus.

Introduction: Portal vein thrombosis (PVT) may occur in non-cirrhotic and cirrhotic patients. It can be classified as acute (if a recent thrombus is present) and chronic (if portal cavernoma has developed). Patients can be symptomatic or may present signs and symptoms related to the development of portal hypertension. In rare cases bowel infarction may occur.Areas covered: This review provides an overview of the clinical presentation, complications, diagnostic challenges and available treatments for PVT in non-cirrhotic and cirrhotic patients (NCPVT).Expert opinion: Treatment of acute NCPVT aims at recanalizing the thrombosed veins and preventing intestinal infarction and portal hypertension. Anticoagulation should be started promptly and maintained for at least 6 months. Long-term anticoagulation should be implemented in the presence of underlying persistent thrombotic state. In chronic NCPVT, treatment aims at managing portal hypertension and portal cavernoma cholangiopathy and preventing new th...

BackgroundDifferentiation between benign and malignant portal vein thrombosis (PVT) in the setting of a hepatocellular carcinoma (HCC) is of paramount importance. Histological analysis is usually not carried out because of potential severe side effects of the percutaneous approach. Endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) may be safer and may guide the clinical management of patients with HCC.ObjectiveTo describe the feasibility of the EUS-FNA in a series of patients with HCC and PVT.Materials and methodsA chart review of patients with PVT was performed from 2014 to 2016 in three tertiary care hospitals of Spain. Patients with chronic liver disease and PVT with a suspicion of HCC referred for EUS-FNA were included. The impact of the EUS-FNA was assessed by comparing staging following the Barcelona Clinic Liver Cancer algorithm (BCLC) before and after EUS-FNA.ResultsOf 104 patients with PVT and chronic liver disease, 23 were considered candidates for EUS-FNA. Eight patients were referred for EUS-FNA. The technique was feasible in seven patients and FNA was positive in six patients. No side effects were reported. EUS-FNA upstaged six out of seven (85.7%) patients: one patient BCLC stage B, two patients BCLC stage A, and three patients in whom the HCC was not diagnosed before EUS-FNA of the PVT. A benign PVT was found in the explant of the only patient with a negative PVT.ConclusionEUS-FNA is a valuable technique in selected patients with chronic liver disease with PVT. It is feasible, safe, and may alter the clinical management in these patients.

Introduction La rupture de varices œsophagiennes liee a une hypertension portale d’origine bilharzienne reste l’etiologie majeure d’hemorragie digestive non ulcereuse a Madagascar. Le cavernome portal est parmi les autres etiologies possibles mais rares. Nos objectifs sont de mettre en exergue l’interet de connaitre cette pathologie et de discuter de la possibilite de prise en charge dans notre centre.

Present day increasing dependence on electricity, in both home and work environments, can lead, if the necessary precautions are not met, to a wide type of injuries, from cutaneous burns to important visceral lesions. We report the case of a 44 year-old male with cutaneous and severe visceral lesions, paresis and paresthesia of the right upper limb induced by a high energy electrical shock. No psychiatric manifestations were observed. This case followed an unusual course due to the particular evolution of the hepatic lesions, which included necrosis, abscesses, thrombosis and angiocholitis.

The chapter considers the principles, the techniques, the results of PEI for treating cirrhotic patients with HCC, and its current indications compared to those of RF, which is now considered the gold standard.

Hepatocellular carcinoma (HCC) is the most frequent primary cancer of the liver. Hepatic transplantation, surgical resection or local destruction are the only curatives options. Only 15% to 25% of patients are candidate to a curative treatment and most of them have unresectable HCC. Palliative treatment of these patients remains difficult in spite of several available therapeutic options. The presence of portal thrombosis, limits even more the therapeutic choices. The Yttrium-90 (Y-90) radioembolization seems to have good efficacy and safety even in case of portal thrombosis, and it can be used as a “bridge” treatment before a hepatic transplantation. We report the case of a patient having a locally advanced HCC, associated with a thrombosis portal, the treatment by radioembolization with Yttrium-90 allowed a regression of the portal thrombosis and a prolonged survival.

Collateral circulation secondary to liver cirrhosis may cause the development of large PSSs that may steal flow from the main portal circulation. It is important to identify these shunts prior to, or during the transplant surgery because they might cause an insufficient portal flow to the implanted graft. There are few reports of “steal flow syndrome” cases in pediatrics, even in biliary atresia patients that may have portal hypoplasia as an associated malformation. We present a 12-month-old female who received an uneventful LDLT from her mother, and the GRWR was 4.8. During the early post-operative period, she became hemodynamically unstable, developed ascites, and altered LFT. The post-operative ultrasound identified reversed portal flow, finding a non-anatomical PSS. A 3D CT scan confirmed the presence of a mesocaval shunt through the territory of the right gonadal vein, draining into the right iliac vein, with no portal inflow into the liver. The patient was re-operated, and the shunt was ligated. An intraoperative Doppler ultrasound showed adequate portal inflow after the procedure; the patient evolved satisfactorily and was discharged home on day number 49. The aim was to report a case of post-operative steal syndrome in a pediatric recipient due to a mesocaval shunt not diagnosed during the pretransplant evaluation. Fil: Rubio, Juan S.. Fundación Favaloro; Argentina Fil: Rumbo, Carolina. Fundación Favaloro; Argentina Fil: Farinelli, Pablo A.. Fundación Favaloro; Argentina Fil: Aguirre, Nicolás. Fundación Favaloro; Argentina Fil: Ramisch, Diego A.. Fundación Favaloro; Argentina Fil: Paladini, Hugo. Fundación Favaloro; Argentina Fil: D´Angelo, Pablo. Fundación Favaloro; Argentina Fil: Barros Schelotto, Pablo. Fundación Favaloro; Argentina Fil: Gondolesi, Gabriel Eduardo. Fundación Favaloro; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Medicina Traslacional, Trasplante y Bioingeniería. Fundación Favaloro. Instituto de Medicina Traslacional, Trasplante y Bioingeniería; Argentina

Background: Portal biliopathy refers to abnormalities of the biliary tract developing in relation to portal hypertension. Portosystemic splenorenal or mesenterico-caval shunting is a safe and effective method to relieve biliary obstruction in symptomatic patients but is unfeasible in cases of extensive thrombosis of the splenic and superior mesenteric veins. In such cases, a makeshift portosystemic shunt between a suitable portal varix and the caval system can be an interesting alternative. Methods: This study describes 3 patients admitted for symptomatic portal biliopathy caused by idiopathic portal cavernoma associated with extensive portal thrombosis. A makeshift portosystemic shunt was carried out after preoperative portal imaging had demonstrated the presence of a suitable splanchnic varix. Results: The makeshift portosystemic shunt was performed by direct anastomosis in 2 patients and by prosthetic interposition in 1 case. Shunting was between a splanchnic varix and the inferior vena cava in 2 cases and the left renal vein in 1 case. Postoperative morbidity was nil and follow-up ranging from 2 to 12 years showed good results with no recurrence of biliary obstruction. Conclusion: In patients presenting symptomatic portal biliopathy associated with extensive thrombosis of the portal system, a makeshift portosystemic shunt is preferable to repeated endoscopic procedures or intrahepatic biliodigestive bypass, provided that a suitable varix is available.

Resume Introduction. – L'infection a cytomegalovirus est souvent asymptomatique, les formes graves sont l'apanage de l'immunodeprime. Nous rapportons un nouveau cas d'hepatite aigue a cytomegalovirus (CMV) compliquee de thrombose portale chez un immunocompetent. Exegese. – Un homme de 29 ans presenta une hepatite aigue a CMV confirmee par la presence de la proteine pp65 et de l'ADN viral dans le sang. Elle fut compliquee d'une thrombose portable d'evolution rapidement favorable sous traitement anticoagulant. Aucune autre cause de thrombose portale ne fut decelee. Onze cas de thrombose majeure compliquant une infection aigue a CMV chez un immunocompetent ont ete rapportes dans la litterature anglosaxonne et francaise. L'absence de cause locale et generale, la resolution en l'absence de traitement anticoagulant, le risque eleve de thrombose chez les sujets seropositifs a la fois pour le virus de l'immunodeficience humaine et le CMV, et chez les transplantes renaux seropositifs pour le CMV suggerent une relation causale. Diverses hypotheses physiopathogeniques ont ete evoquees : presence d'anticorps antiphospholipides, absents dans notre observation, induction d'un phenotype procoagulant des cellules endotheliales infectees, induction d'une proliferation des cellules musculaires lisses. Conclusion. – L'infection aigue a CMV peut etre consideree comme une cause possible de thrombose majeure.

Resume L'evaluation morphologique et fonctionnelle du systeme porte est necessaire dans de nombreuses pathologies hepatiques ou pancreatiques pouvant avoir un retentissement portal. L'etude du systeme porte est egalement indiquee dans le bilan pretherapeutique et la surveillance des malades ayant une transplantation hepatique ainsi qu'au cours de la mise en place d'un shunt portosystemique intrahepatique par voie transjugulaire (TIPS). Le choix d'une technique doit etre raisonne, adapte a l'indication. L'echographie couplee au Doppler est l'examen de premiere intention dans le diagnostic et la surveillance du traitement des thromboses portales. La tomodensitometrie helicoidale utilisant plusieurs couronnes de detecteurs permet une approche morphologique et meme fonctionnelle des vaisseaux portes, en montrant l'etendue, l'extension aux vaisseaux splanchniques, les consequences et les causes d'une thrombose portale. L'imagerie par resonance magnetique (IRM) visualise aussi bien que la tomodensitometrie l'anatomie du systeme porte. Son interet reside dans une meilleure capacite a caracteriser les masses hepatiques et pancreatiques compliquees de thrombose portale d'ou son principal avantage par rapport a la tomodensitometrie. Apres transplantation hepatique, l'angio-IRM peut etre proposee dans la recherche de complications vasculaires arterielles et veineuses. Les techniques invasives (portographie percutanee transhepatique et portographie par voie transjugulaire) sont reservees a des indications particulieres.

Three cases of prehepatic portal vein thrombosis, complicated by the clinical manifestations of portal hypertension, were successfully treated by surgically created splanchnic–intrahepatic portal bypass. Two out of three patients had been previously submitted to liver transplantation. No significant morbidity was observed and long-term Doppler evaluations proved the patency of the venous grafts. Together with the technical aspects of the procedures, the possible role of this technique, primarily proposed by De Ville de Goyet in 1992, is discussed in relation to the available therapies for the extrahepatic portal vein thrombosis.

In percutaneous microwave coagulation therapy (PMCT), it is important to understand the three-dimensional anatomy of a hepatic tumor in order to allow careful aiming and sufficient tissue coagulation. Therefore, the effectiveness of PMCT under general anesthesia was examined. When PMCT was performed under general anesthesia, insertion and tissue coagulation could be performed safely even for the tumors in which percutaneous aiming is difficult, such as those in the caudate lobe or under the diaphragm. Pain was complained of by 5.8% of the patients treated under general anesthesia and 70% of those treated under local anesthesia. Portal thrombosis, biliary stenosis, and gastric and intestinal wall injury by the probe tended to occur more frequently in those treated under local anesthesia. The number of days in hospital after starting therapy was 6.9 ± 3.1 days for patients treated under general anesthesia, while it was 8.1 ± 6.6 days for those treated under local anesthesia.

OBJECTIVE To describe the frequency and the characteristics of hepatocellular carcinoma (HCC) cases that appeared in HIV/hepatitis C virus (HCV)-coinfected patients with previous sustained virological response (SVR) and to compare these cases to those diagnosed in patients without SVR. METHODS All HIV/HCV-coinfected patients diagnosed with HCC in 26 hospitals in Spain before 31 December 2012 were analyzed. Comparisons between cases diagnosed in patients with and without previous SVR were made. RESULTS One hundred and sixty-seven HIV/HCV-coinfected patients were diagnosed with HCC in the participant hospitals. Sixty-five (39%) of them had been previously treated against HCV. In 13 cases, HCC was diagnosed after achieving consecution of SVR, accounting for 7.8% of the overall cases. The median (Q1-Q3) elapsed time from SVR to diagnosis of HCC was 28 (20-39) months. HCC was multicentric and was complicated with portal thrombosis in nine and six patients, respectively. Comparisons with HCC cases diagnosed in patients without previous SVR only yielded a significantly higher proportion of genotype 3 infection [10 (83%) out of 13 cases versus 34 (32%) out of 107; P = 0.001)]. The median (Q1-Q3) survival of HCC was 3 (1-39) months among cases developed in patients with previous SVR, whereas it was 6 (2-20) months in the remaining individuals (P = 0.7). CONCLUSION HIV/HCV-coinfected patients with previous SVR may develop HCC in the mid term and long term. These cases account for a significant proportion of the total cases of HCC in this setting. Our findings reinforce the need to continue surveillance of HCC with ultrasound examinations in patients with cirrhosis who respond to anti-HCV therapy.

We present the case of a 16-year old woman with Alagille's syndrome, who had upper gastrointestinal bleeding due to rupture of esophageal varices secondary to presinusoidal portal hypertension without liver fibrosis. Portal thrombosis is a manifestation previously unreported in association to this syndrome.

To evaluate the complications and clinical outcomes of transcatheter arterial embolization (TAE) for symptoms related to severe arterioportal fistulas (APFs).Six patients (3 males, 3 females; mean age, 63.8 years; age range, 60-71 years) with chronic liver disease and severe APFs due to percutaneous intrahepatic treatment (n = 5) and portal vein (PV) tumor thrombosis of hepatocellular carcinoma (n = 1) underwent TAE for symptoms related to severe APFs [refractory ascites (n = 4), hemorrhoidal hemorrhage (n = 1), and hepatic encephalopathy (n = 1)]. Control of symptoms related to APFs and complications were evaluated during the follow-up period (range, 4-57 mo).In all patients, celiac angiography revealed immediate retrograde visualization of the main PV before TAE, indicating severe APF. Selective TAE for the hepatic arteries was performed using metallic coils (MC, n = 4) and both MCs and n-butyl cyanoacrylate (n = 2). Three patients underwent repeated TAEs for residual APFs and ascites. Four patients developed PV thrombosis after TAE. During the follow-up period after TAE, APF obliteration and symptomatic improvement were obtained in all patients.Although TAE for severe APFs may sometimes be complicated by PV thrombosis, TAE can be an effective treatment to improve clinical symptoms related to severe APFs.

We describe an elderly woman who presented with an unusual primary arterioportal fistula and cavernous transformation of the portal vein caused by portal thrombosis, which were subsequently managed with endovascular coil embolization and transjugular intrahepatic portosystemic shunt using 2 stents after balloon remodeling. This case shows a rarely seen condition in the elderly and a novel management strategy that should be considered in the management of this complex disease.

Background: A 35-year-old female presented with upper gastrointestinal tract bleeding. Fifteen years ago, the diagnosis of esophageal varices caused by idiopathic portal venous thrombus was made. Follow-up examinations revealed mild elevation of bilirubin levels and normal alphafetoprotein level on blood tests. Splenectomy had been performed 6 years previously due to portal hypertension caused by portal venous thrombus.

Resume Parmi les manifestations extradigestives habituelles des colopathies inflammatoires, les atteintes vasculaires sont rares, mais souvent d'une extreme gravite. La survenue d'une thrombose dans le territoire portal, souvent precipitee par une infection ou un traumatisme, en particulier chirurgical, reste exceptionnelle. Nous rapportons le cas d'une jeune patiente suivie pour une maladie de Crohn severe presentant une thrombose brutale de sa veine porte. Malgre un pronostic initial pejoratif, la patiente mene actuellement, 10 mois apres cet episode, une vie tout a fait normale. Il s'agit du troisieme cas decrit de thrombose porte au cours de la maladie de Crohn: nous discutons le role favorisant d'un deficit acquis en proteine S au cours d'une contraception estroprogestative.

Purpose The aim of this study was to analyze our experience with portal vein thrombosis after liver transplantation with a persistent splenorenal shunt. Materials and methods The study population included 780 liver transplantations from 1990 to 2009. We analyzed the existence of portal vein thrombosis in the immediate posttransplant period, selecting cases with a persistent splenorenal shunt requiring surgery. Results The incidence of posttransplant portal vein thrombosis was 1.41% (n = 11), of which 3 (27%) had a splenorenal shunt as a possible cause (0.38% of the total). Two cases required liver retransplantation due to portal vein thrombosis, and the third a thrombectomy. In all cases the shunt was also closed. During the early postoperative follow-up of these 3 patients, 2 needed repeat surgeries because of a new portal vein thrombosis (thrombectomy) in one and a bilioperitoneum in the other. After a median follow-up of 11 months, the patients showed a good evolution with no primary graft dysfunction. Discussion The portal steal phenomenon secondary to persistence of a splenorenal shunt promotes the occurrence of portal vein thrombosis. Although it is a rare cause of graft dysfunction, it must be treated early, because it can lead to a small-for-size syndrome.

We report a case of reversible hepatofugal portal flow after auxiliary partial orthotopic liver transplantation (APOLT) from a living donor in this study. On postoperative day 6, continuous hepatofugal portal flow was observed in the grafted liver without portal thrombosis and obstruction of the hepatic vein. Based on histological findings, acute rejection was the suspected cause. The normal portal venous flow was restored after steroid pulse and antithymocyte globulin (ATG) therapies. The patient was discharged on the 30th postoperative day. It was concluded that hepatofugal flow after liver transplantation is a sign of serious acute rejection, and can be successfully treated by anti-rejection therapy.

A man with abscess of the round ligament of the liver associated with acute obstructive suppurative cholangitis and portal thrombosis is reported. A 63-year-old man was admitted with epigastralgia and high fever. Blood tests showed elevation of hepato-biliary enzymes and coagulopathy consistent with acute obstructive suppurative cholangitis and disseminated intravascular coagulation. Computed tomography revealed a small abscess of the round ligament of the liver and left portal thrombosis. After endoscopic biliary stenting, antibiotics and thrombolytic therapy, the high fever, disseminated intravascular coagulation and portal thrombosis rapidly improved, and the round ligament abscess was also later resolved., Article, INTERNAL MEDICINE. 48(21):1885-1888 (2009)

Splanchnic vein thrombosis (SVT) is a severe complication of essential thrombocythemia (ET). No clear explanation has been given for the occurrence of thrombosis in this unusual site in patients with ET, but the existence of a specific association between unexplained SVT and the JAK2 mutation has been reported.The present study describes SVT (portal and splenic vein thrombosis) in a young woman as the first presenting symptom of latent ET. Extensive screening for thrombophilia was negative. Our patient in fact did not fulfill the WHO diagnostic criteria for myeloproliferative disease (MPD), while she had splenomegaly and developed features suggestive of latent ET during follow-up.In these patients with SVT, the detection of JAK2(V617F) mutation is diagnostic for masked MPD as could be documented by bone marrow histopathology. The presence of JAK2(V617F) mutation should be considered per se a prothrombotic state for cerebral, coronary and peripheral microvascular disturbances and for SVT but not for deep vein thrombosis. Anticoagulation is the treatment of choice for all SVT and proper treatment of the MPD is recommended in patients with SVT associated with the JAK2(V617F) mutation.

非常にまれな巨大な門脈一大静脈シャントにより, 猪瀬型肝性脳症を呈した症471に, シャント切除術を行い良好な結果を得た. 患者は55歳の男性で肝硬変と陣腫を伴い総ビリルビン2.6mg/dl, ICGR1544.5%白血球1,800, 血小板6.2X104でぁった, シャントは上腸間膜静脈より右腎静脈下の下大静脈に流入し, 血管径は3.4cmで右後腹膜腔全体に屈曲蛇行する巨大なものであった. 手術は陣摘出術とシャント血管切除を行った. 術中門脈圧に変化を認めず門脈血流は向肝性となった. 術後, 自血球, 血小板は著明に増加し, アンモニア, ICG, 胆汁酸, エンドトキシン等の諸検査成績も改善した. また, 門脈血栓症を併発したが抗凝固療法によリコントロールできた. 胃周囲血行郭清術はしなかったが, 食道静脈瘤の進展はみていない. 今後, 門脈大静脈シヤント症例には, 肝不全, 食道静脈瘤, 門脈血栓症の発症に注意して積極的に切除すべきである.

Portal and mesenteric vein thrombosis is a very uncommon complication of laparoscopic surgery, especially after anti-reflux procedures. We report the case of a twenty-year-old man with a history of alcohol and cocaine consumption. A Nissen fundoplication was performed. The patient received a single 20-mg dose of enoxaparin (Clexane ® , Aventis Pharma, Spain) two hours before surgery for antithrombotic prophylaxis. On the seventh postoperative day the patient had a portal and mesenteric venous thrombosis, which was confirmed at laparotomy, with both extensive small-intestine necrosis and partial colon necrosis. Despite anticoagulant therapy, the patient died 24 hours later. Surgical findings were confirmed at necropsy. Portal and mesenteric venous thrombosis is an uncommon but severe and even fatal complication after laparoscopic anti-reflux surgery. When other pro-thrombotic, predisposing conditions such as laparoscopic surgery and cocaine consumption are present, the usual prophylactic doses of low molecular weight heparin might not be sufficient to protect against this life-threatening complication.

Portal vein thrombosis may complicate splenectomy in patients with hemolytic anemia and myeloproliferative disease, whereas the frequency of portal vein thrombosis in case of trauma is not defined. A case of right portal vein thrombosis after splenectomy for trauma is reported in this paper. Hematologic workup did not reveal an underlying platelet or coagulation disorder. The patient was promptly anti-coagulated with complete recanalization of the portal vein. We conclude that mild symptoms, like abdominal pain and fever, after splenectomy should be investigated with a color Doppler ultrasonography to confirm or rule out a diagnosis of portal thrombosis and to anti-coagulate the patient with thrombosis, thus preventing bowel infarction and secondary portal hypertension. Routine postoperative color Doppler might also be justified in all postsplenectomy patients (without hematologic diseases) for early detection of a portal vein thrombosis.