Suprahepatic Veins Research Articles

Liver and pancreas transplantation in adult donor and recipients with situs inversus totalis: a case series and review of the literature

BackgroundSitus inversus totalis is a rare congenital anomaly characterized by a mirror-image orientation of abdominal, and in some cases, thoracic organs. Here, we report our situs inversus totalis transplantation experience and further review liver transplantations in adult recipients and donors with situs inversus totalis.Case presentationWe describe three cases with situs inversus totalis. The first case was liver transplantation in a recipient (a 61-year-old Iranian man) with situs inversus totalis, the second was a liver transplantation from a donor (a 52-year-old Iranian woman) with situs inversus totalis, and finally, for the first time, a simultaneous pancreas and kidney transplantation in a recipient (a 26-year-old Iranian man) with situs inversus totalis. In patient one, hepatectomy could be performed according to the standard method and on the basis of preoperative studies. Hepatic vein and arterial anastomosis were performed as in every other patient without situs inversus totalis. To prevent biliary complications, a Roux-en-Y hepaticojejunostomy was performed. In patient two, implantation time, suprahepatic vein, portal vein, arterial, and biliary reconstruction could be done as in any other case without situs inversus totalis. Plication of the right-sided diaphragm and fixation of the falciform ligament was done for our patient. In patient three, systemic drainage was preferred to portal flow for establishing the outflow drainage of the pancreas compared with otherwise normal patients.ConclusionAlthough situs inversus totalis is a rare condition, our reported techniques are suitable, considering advantages such as easier accessibility, more acceptable placement of the implanted organs regarding vascular variations, and the appropriate location of the allograft in the proximity of other organs.

A new variant in the heterotaxy polysplenia syndrome

Heterotaxy is a low incidence genetic disorder of multifactorial inheritance characterized by various abnormalities in the position of organs and vessels relative to the midline of the body. It is most often associated with fatal congenital heart defects at birth. As a result, only a small percentage of patients survive into adulthood. The following clinical case presents a female adult patient with abdominal pain secondary to choledocholithiasis. During the diagnostic workup, she was diagnosed with intestinal malrotation complicated by portal vein thrombosis, which later developed into portal hypertension. In addition to several anomalies (agenesis of the inferior vena cava with direct communication of the suprahepatic veins to the right atrium, polysplenia and shortening of the pancreas), a possible variant of heterotaxy syndrome was diagnosed.

Budd-chiari syndrome: case report

Budd-Chiari Syndrome (BCS) is a rare pathological condition characterized by an obstruction of the hepatic venous outflow. It can be classified as primary, due to an intra-luminal venous lesion, or secondary, resulting from extrinsic compression or invasion of the venous system. This study aims to report a case of Budd-Chiari Syndrome, emphasizing the importance of diagnosis and treatment in preventing progression to chronic liver disease. We report the case of a 47-year-old patient who presented to the emergency department with severe pain in the right hypochondriac region and abdominal distension. A total abdominal CT scan was performed and revealed thrombosis of the suprahepatic veins, confirming the diagnosis of BCS. The patient developed portal hypertension and signs of chronic liver disease. Treatment was administered with Spironolactone and Marevan. After six months of Marevan use, the patient experienced worsening of the esophageal varices and hematemesis, requiring esophageal variceal band ligation. Liver transplantation was not recommended as the patient's MELD score was 14. Therefore, an accurate diagnosis of BCS is crucial for appropriate therapeutic intervention in order to prevent the progression to chronic liver disease, with permanent damage to liver functionality and the patient’s quality of life.

Budd Chiari Syndrome Revealed by Spinal Cord Compression

Budd-Chiari syndrome results from obstruction of hepatic venous drainage, from the hepatic venules to the terminal part of the inferior vena cava, whatever the cause of the obstruction. It may be of primary or secondary origin. In our case, we report a case of Budd-Chiari syndrome with thrombosis of the terminal part of the inferior vena cava extending to the right suprahepatic vein, revealed by an unusual picture of functional impotence of all 4 limbs due to spinal cord compression caused by collateral venous circulation. Etiological assessment for budd chiari syndrome revealed a protein C deficiency, for which the patient was put on lifelong anticoagulation.

CONSTRICTIVE PERICARDITIS DURING SARS-COV-2 INFECTION: A CASE REPORT

Abstract In our Department we witnessed numerous cases of pericarditis at the end of summer 2023, including that of a 44-year-old patient suffering from rheumatoid arthritis with breath-taking chest pain and fever. Objectively no rubbing, but mild-moderate pericardial effusion. On the EKG diffuse anomalies of ventricular repolarization; increased inflammation indices with negative troponin; positive Sars-Cov-2 PCR. During hospitalization, despite the improvement of clinical parameters, dyspnea persisted. On the control echocardiogram, disappearance of the pericardial effusion, but thickening of the pericardial sheets associated with a "notch" of the interventricular septum and an anomalous Doppler profile in the suprahepatic vein. These findings were subsequently confirmed by cardiac MRI, with diagnostic criteria for constrictive pericarditis. On the basis of these data, therapy with high doses of NSAIDs and colchicine was established. The patient is currently followed periodically by our clinics and feels well. In the literature, constrictive pericarditis is generally secondary to chronic conditions; in this case the contribution of Sars-Cov-2 infection and rheumatoid arthritis cannot be excluded.

Effectiveness of a thrombin-gelatin flowable for treating severe liver bleeding: an experimental study

BackgroundCurrent scientific evidence has pointed out the relevance of hemostatic products for improving clinical outcomes in liver trauma, including increased survival rates and reductions in bleeding-related complications. The purpose of this study was to compare the use of the gelatin-thrombin flowable (Flowable) versus the standard technique of Packing in a new experimental liver injury model.MethodsTwenty-four swine were prospectively randomized to receive either Flowable or standard packing technique. We used a novel severe liver injury model, in which the middle and left suprahepatic veins were selectively injured, causing an exsanguinating hemorrhage. The main outcome measure was the percentage of lost blood volume.ResultsThe median total percentage of total blood volume per animal lost, from injury to minute 120, was significantly lower in the Flowable group (15.2%; interquartile range: 10.7–46.7%) than in the Packing group (64.9%; Interquartile range: 53.4–73.0%) (Hodges-Lehmann median difference: 41.1%; 95% CI: 18.9–58.0%, p = 0.0034). The 24-hour survival rate was significantly higher in the Flowable group (87.0%) than in the Packing group (0.0%) (Hazard ratio (HR) 0.08; 95% confidence interval 0.102 to 0.27; p < 0.0001). Mean-arterial pressure was significantly lower at minute 60 and 120 in the Flowable group than in the packing group (p = 0.0258 and p = 0.0272, respectively). At minute 120, hematocrit was higher in the Flowable than in the packing group (Hodges-Lehmann median difference: 5.5%; 95%CI: 1.0 to11.0, p = 0.0267). Finally, the overall-surgical-procedure was significantly shorter with Flowable than with Packing (Hodges-Lehmann median difference: 39.5 s, 95% CI: 25.0 to 54.0 s, p = 0.0004).ConclusionsThe use of the Flowable was more effective in achieving hemostasis, reducing blood loss, and improving survival rates than standard packing in a severe porcine-liver bleeding model.

Importance of prenatally diagnosed portosystemic vascular shunts in clinical outcomes

To report two cases of prenatal diagnosis of portosystemic vascular shunts and review the importance of this rare pathology in its neonatal and pediatric evolution. Case 1: pregnancy with fetal growth restriction, 2nd percentile, polyhydramnios, without fetal malformations and abnormal patterns on fetal Doppler. Abnormal blood flow through the ductus venous and abnormal venous communication in the liver were identified. Normal genetic study. Male newborn (NB) delivered at 36 weeks, because of severe fetal growth restriction, by emergency cesarean section. He evolved asymptomatic, with normal liver function, and did not continue follow-up. Case 2: pregnancy with fetal growth restriction < percentile 1. Agenesis of the ductus venous and abnormal communication between the portal vein and the left suprahepatic vein were identified with no other malformations or signs of heart failure. Severe SGA newborn was delivered by induction of labor at 35 weeks. He evolved asymptomatic. Normal complementary study. A home check-up at 2 months showed persistent vascular anomaly without systemic involvement. Even though in the cases presented there were no neonatal complications, this kind of malformations require a high index of suspicion in cases with fetal growth restriction, as well as a long-term multidisciplinary follow-up.

Construction and validation of a novel and severe hepatic injury model in swine focuses on research and training. Observational study

Some hepatic wound models have been developed in pigs with the aim of reproducing liver injury; however, the wound shape, severity, and outcome differ among them. The novel injury profile employed in this study differed from that used elsewhere for standardized, repeatable, reproducible, incising-penetrating, vascular, and severe injury in swine. It is made with a cutting object that penetrates deep into the hepatic parenchyma, always affecting the two suprahepatic veins at the point where they merge into the common trunk.The primary outcome was reproducibility and replicability of the surgical method.The secondary outcome was the analysis of some variables (blood loss, survival, and flow) to validate the model.•This novel method of liver injury provides a liver injury with the following characteristics: standardized, incise-penetrated, deep, bloody, and severe.•This model can be used for research (trauma, hepato-bilio-pancreatic, pharmaceutical) and training (damage control surgery).•Method name: Incising-Penetrating, Vascular and Severe Liver Injury Model in Swine.

Mesenchymal Hamartoma of the Liver Associated with Discontinuity of the Vena Cava

Primary liver tumors in children are infrequent, accounting for 0.5-2 % of all pediatric neoplasms. About two-thirds are malignant. The most common pediatric malignant liver tumours are, in order of frequency, hepatoblastoma, hepatocellular carcinoma, and embryonic sarcoma of the liver. Among benign tumors, the most frequent are hemangioendothelioma and mesenchymal hamartoma of the liver, and more rarely focal nodular hyperplasia and hepatocellular adenoma [1,2]. We report the case of an infant who was entrusted to us for the management of a large liver tumor. Radiology (CT scan and MRI), in addition to the elements in favor of a mesenchymal hamartoma of the right liver revealed a congenital anomaly associated, in this case, with the absence of the inferior vena cava in its intraabdominal portion. A right hepatectomy enlarged to segment IV was performed. The right renal vein drained into the right suprahepatic vein was preserved. Note that this tumor represents 50% of the total weight of the patient, which explains the difficulties and constraints that hindered the development and quality of life of the young patient.

C32 CONSTRICTIVE PERICARDITIS WITH PLATYPNEA ORTHODEOXIA

Abstract A 56–year–old woman is admitted to our centre for biventricular heart failure. In anamnesis she reports a submandibular phlegmon complicated by pleuropericarditis at the age of 13 years requiring repeated pericardiocentesis. In the last three years she has been hospitalised twice for ascites and discharged with a diagnosis of idiopathic liver cirrhosis. On admission she presented with peripheral oedema, ascites and hippocratic fingers; oxygen saturation is 80% in room air. On blood tests creatinine, transaminases, albumin, coagulation and NT–proBNP are normal, bilirubin is elevated. An echocardiography is performed showing a calcific spicola impinging the free wall of the right ventricle producing endocavitary obstruction; diffusely thickened pericardium, septal bounce, annulus reversus and expiratory reflux in the suprahepatic veins. Biventricular contractile function is normal and no significant valvulopathy is present. CT scan confirms the presence of diffuse pericardial calcifications, small liver and irregular profile with large ascitic effusion. Decongestive therapy is started with weight loss of 22 kg and regression of oedema but severe hypoxia persists with evidence of platypnea–orthodeoxia syndrome. An echocardiography is repeated showing passage of microbubbles in the left atrium from the sixth beat onwards, compatible with intrapulmonary shunt. A large arteriovenous malformation (AVM) of the right lower pulmonary branch is found at CT angiography, which is also revealed by targeted invasive angiography. Cardiac catheterisation and cardiac MRI confirmed the diagnosis of constrictive pericarditis. Percutaneous closure of the AVM with complete resolution of the hypoxia and subsequent pericardiectomy is performed. The patient is discharged on day XXVII with normal saturation on room air, in good haemodynamic compensation and with normal liver stasis parameters. In conclusion, constrictive pericarditis is a rare cause of heart failure and to our knowledge this is the second case described reporting a pulmonary AVM secondary to constrictive pericarditis.

Venae Cavae Anatomic Characteristics in Severe Tricuspid Regurgitation: Implications for Transcatheter Interventions

Venae Cavae Anatomic Characteristics in Severe Tricuspid Regurgitation: Implications for Transcatheter Interventions

P198 Intracardiac thrombosis and vascular involvement in Behçet's disease: two sides of the same coin?

Abstract Background/Aims Behçet’s disease (BD) is a relapsing vasculitis that can affect vessels of all sizes and kinds. Intracardiac thrombosis (ICT) is a rare but serious complication which often presents with vascular lesions. Yet, the link between these manifestations is not fully clarified. Our study aims to describe the profile, therapeutic management, and outcomes of BD patients with ICT. Secondly, it aims to shed some light on the relationship between ICT and vascular involvement in BD. Methods We retrospectively conducted a descriptive and monocentric study, including 531 Behçet’s patients admitted to our department between 2010 and 2022. All patients selected fulfilled the criteria of the International Study Group of Behçet’s Disease. Results Twelve patients were enrolled (2%). The sex ratio (M:F) was 9:1 and the mean age was 29 ± 6 years (20-41). ICT occurred as the revealing form of BD (50%), or after a median of 4 years since the disease onset (50%). The main symptoms were dyspnea (58%), chest pain (58%), hemoptysis (41%), and dry cough (33%). Concurrent BD flares were mainly mucocutaneous (80%). Inflammatory parameters were elevated with a median ESR of 48 mm/h and CRP of 52 mg/L. High levels of BNP and troponin were noted in 58% and 16% of the cases. Hemostasis parameters were within the normal range. On transthoracic echocardiogram, ICT was located in the right (100%), and left cavities (20%), with an extension to the inferior vena cava in half of the cases (50%). Multiple thrombi were visualized (n = 4). The associated cardiac lesions were tricuspid insufficiency (50%), pericardial effusion (41%), mitral insufficiency (16%), endomyocardial fibrosis (8%), myocarditis (8%), and ischemic coronaropathy (8%). Pulmonary hypertension (58%) and right dilatation (50%) were common. Concomitant pulmonary artery involvement was frequent (83%), consisting of thrombosis (70%) and aneurysms (50%). Six patients had a past history of lower extremity DVT, either as a single episode (n = 4) or recurrent episodes (n = 2). Vascular thrombosis was also found in the superior vena cava (n = 2), suprahepatic veins (n = 2), internal jugular vein (n = 1), pulmonary veins (n = 1), cerebral vein (n = 1), superior mesenteric vein (n = 1), and femoral artery (n = 1). Patients were treated with colchicine (100%), glucocorticoids (100%), and immunosuppressants (92%), mainly cyclophosphamide (n = 11), and a TNF-alpha inhibitor (n = 2). Six patients were under curative anticoagulation. A good response to therapy was observed in all patients. One case of relapse was reported. No deaths were recorded. Conclusion ICT usually occurs during the first years of BD onset in young male patients presenting with vascular lesions, especially of the pulmonary arteries and vena cava. We thus believe that, unlike common thrombotic diseases, ICT in BD is a direct consequence of the underlying vasculitis. The use of immunosuppressants, with or without anticoagulants, is generally associated with a favorable outcome. Disclosure S. Chadli: None. H. Khibri: None. S. Fari: None. N. Moatassim: None. W. Ammouri: None. M. Maamar: None. H. Harmouche: None. M. Adnaoui: None. Z. Tazi Mezalek: None.

P206 Uniqueness of venous thrombosis in Behçet's syndrome: a case series of 62 patients

Abstract Background/Aims Behçet’s syndrome (BS) is a complex inflammatory disorder frequently observed in the so-called “Silk Road”. Venous thrombosis (VT) is common in BS and known to be a predictive factor of future severe manifestations of the disease. Our work aims to describe the clinical and imaging characteristics of VT in BS, in order to shed the light on its specific features for early diagnosis and management of these patients. Methods We conducted a retrospective, descriptive and monocentric study, including 200 BS patients admitted to our internal medicine department between 2014 and 2021. All patients fulfilled the International Study Group criteria for Behçet’s disease. Results VT was found in 62 patients (31%). The mean age was 29 ± 10.8 (18-61) and the sex ratio (M:F) was 7.85:1. Family history of BS was documented in 5% of the cases. VT was either the revealing manifestation of the syndrome (56%) or occurred after a median of 3.5 years (1; 6.5) following the disease onset. Four patients had superficial venous thrombosis (SVT) mostly affecting the superficial femoral vein (98%). All patients had deep vein thrombosis (DVT), visualised in the lower extremities (58%), superior vena cava (21%), inferior vena cava (16%), cerebral veins (13%), and suprahepatic veins (5%). Atypical sites of DVT occurred in the renal (n = 1), axillary (n = 1), central retinal (n = 1), and dorsal penile (n = 1) veins. DVT was observed in one vein (55%), two veins (27%), or multiple (3 to 7) veins (16%). Thrombi sizes ranged from 10 to 40 mm, being willingly extended (55%) and strongly adherent to the vessel walls (95%). Concomitant arterial lesions were found in 34% of the patients, mainly in the pulmonary arteries (57%). They consisted of aneurysms (52%), thrombosis (43%), stenosis (17%), and pseudoaneurysms (9%). Cardiac lesions were also observed (11%), including right intracardiac thrombosis (n = 4), coronary artery thrombosis (n = 2), pericarditis (n = 1), and myocarditis (n = 1). The other extravascular involvements associated were mucocutaneous (100%), ocular (42%), neurological (29%), articular (24%), and digestive (23%). Fever was reported in 4 cases. All patients were treated with colchicine and glucocorticoids. Immunosuppressive therapy was prescribed in 75% of the cases: cyclophosphamide (n = 31), azathioprine (n = 12), and TNF alpha inhibitor (n = 3). Curative anticoagulation was also administered (98%). Two patients underwent surgery: thrombectomy (n = 1) and embolectomy (n = 1). Relapse occurred in 27% of the patients. No death was recorded. Conclusion VT is widely considered to be the hallmark of BS as well as a major risk factor for future severe vascular and cardiac involvements. Due to its inflammatory pathogenesis, it has specific clinical and imaging features. Better knowledge of these characteristics could help early establish the diagnosis of BS, thus improving patient outcomes. Disclosure S. Chadli: None. H. Khibri: None. R. Benachour: None. S. Firdaous Fari: None. N. Moatassim: None. W. Ammouri: None. M. Maamar: None. H. Harmouche: None. M. Adnaoui: None. Z. Tazi Mezalek: None.

Portal hypertension and pancreatic cancer, TIPSS as a bridge to a safe resection

Background: Pancreaticoduodenectomy (PD) is a complex operation associated with high morbidity, especially in the setting of hepatic fibrosis/cirrhoses and portal hypertension. Portal hypertension is a near certain contraindication for PD and may preclude patients with resectable malignancy from a curative operation. Transjugular intrahepatic portosystemic shunt (TIPSS) is an artificial path between the portal vein and suprahepatic veins for decreasing the portal pressure, defined as a hepatic venous pressure gradient > 5mmHg. TIPS can be used as a bridge to facilitate safe performance of PD. We reviewed our experience over the past decade at a single institution.

Vascular Behçet syndrome: from pathogenesis to treatment.

Behçet syndrome is a rare, chronic inflammatory disease of unknown aetiopathogenesis, most commonly presenting with mucocutaneous and ocular manifestations. Vascular involvement, most frequently superficialvein and deepvein thrombosis, can occur in up to 50% of patients with Behçet syndrome. Venous thrombosis at atypical sites (inferior and superior vena cava, suprahepatic veins with Budd-Chiari syndrome, portal vein, cerebral sinuses and right atrium and/or ventricle) and arterial involvement (mostly in situ thrombosis and aneurysms of the pulmonary arteries, as well as aneurysms of the abdominal aorta, and peripheral and visceral arteries) are also unique features of Behçet syndrome. Behçet syndrome is considered a natural model of inflammation-induced thrombosis in humans, with an impaired immune-inflammatory response rather than traditional cardiovascular risk factors contributing to thrombogenesis. Specifically, neutrophil hyperactivation and neutrophil-mediated mechanisms of damage directly promote endothelial dysfunction, platelet activation and thrombogenesis in Behçet syndrome. This unusual pathogenesis directly determines the treatment approach, which relies mostly on immunosuppressants rather than anticoagulants for treatment of thrombosis and for secondary prevention. This Review discusses the main histopathological, pathogenetic and clinical aspects of vascular Behçet syndrome, addressing their implications for therapeutic management. Future perspectives in terms of pathogenetic studies, disease monitoring and treatment strategies are also discussed.

Utility of microporous polysaccharide hemospheres in severe hepatic trauma: Experimental study of hemostatic strength and ease of use

BackgroundAnimal studies confirm the utility of hemostatics against standard packing following severe liver injury. We investigated the efficacy and ease of use of novel microporous polysaccharide hemosphere (MPH) compounds (Perclot®, Baxter) in the treatment of severe hepatic hemorrhage in pig. MethodsPigs were randomized to one of two equal treatment groups: MPH compounds (n=12) and Standard Packing (n=12). All animals underwent standardized surgical devascularization of the suprahepatic veins (grade V) to induce severe hepatic injury. Measures relating to the hemostatic success were monitored at 12, 60, 120 minutes and 24 hours post injury. ResultsAnimals allocated to treatment with MPH compounds were associated with higher survival rates at 24 hours than those undergoing standard packing: 66.7% vs, 0%, respectively (p=0.001). At 120 minutes MPH compounds were also associated with reduced blood loss, median (IQR) 1.16 (0.60) vs. 10.19 (5.77) mL/Kg, respectively: p<0.001) as well as higher invasive mean arterial pressures (iMAP) median (IQR) 39.12 (11.29) vs. 25.75 (14.28) mmHg, respectively: p=0.14) and hemoglobin levels median (IQR) 5.45 (2.50) vs. 6.45 (1.73) g/dL, respectively p=0.127). Overall, the application of MPH compounds required nearly half the time of standard packing median (IQR) 32.92 (6.51) vs. 67.75 (14.66) sec, respectively: p<0.001). ConclusionsThe data suggests that the use of MPH works in a severe hemorrhage in the liver of pigs, improving many variables in comparison to standard packing, including survival, blood loss and speed of application and we conclude that this offers a potential alternative for the treatment of hepatic injury. Further work is needed to corroborate these findings.

Severe polycystic liver disease: An unsual cause of chronic Budd-Chiari Syndrome

Autosomal dominant polycystic kidney disease is a hereditary disease, characterized by the development of cysts in the renal parenchyma with extra-renal manifestations. Liver damage is rarely a source of complications. A Budd-Chiari syndrome could occur following the compression of the supra-hepatic veins by the cysts. It is an exceptional mechanical complication. We present a case of a 54 year old woman, with a diagnostic of an asymptomatic autosomal dominat polycystic kidney disease since childhood was admitted to our hospital due to significantly increased abdominal girth. The physical examination showed grade III ascites. A paracentesis for relief at admission disclosed an exudative fluid. A abdominal computed tomographic scan showed multiples cystic lesions in the kidneys and liver, with a large hepatic cyst responsible for compression of the suprahepatic veins and the inferior vena cava resulting in chronic Budd-Chiari syndrome. The treatment was radiological drainage followed by percutaneous sclerosis of cysts to alleviation of the compression. Unfortunately, the patient died a few days after an intraperitoneal cystic rupture.

Invading of Renal Cell Carcinoma in Inferior Vena Cava and Right Atrium with a Huge Metastatic Thrombus

BACKGROUND: Renal cell carcinoma (RCC) with cavoatrial involvement represents a major surgical challenge. Several surgical techniques for the treatment of these tumors have been proposed, but due to a small number of patients and limited follow-up, substantial controversy about the best operative management still exists. CASE REPORT: A 54-year-old woman, with no previous comorbidities, comes to the emergency room with low back pain, weight loss, and edema of the lower legs that computed tomography revealed a massive infiltrative expansive formation in the abdomen that affected practically the entire right kidney, measuring 8.2 cm × 7.6 cm that invaded the collecting system and was in close contact with the right hepatic lobe and the head of the pancreas. During the intervention, infiltration of the renal vein on this side is found, and the inferior vena cava (IVC) that extends in its intrahepatic part, up to the junction of the suprahepatic veins, with almost complete closure of the lumen accompanied by the presence of retroperitoneal lymph nodes, with size up to 10 mm. CONCLUSION: Advanced extension of RCC can occur with no apparent symptoms and be detected incidentally. In rare circumstances, atypical presentation of RCC should be considered in a patient presenting with the right atrial mass detected by echocardiography. RCC with IVC and right atrium extension is a complex surgical challenge, but excellent results can be obtained with proper patient selection, meticulous surgical techniques, and close perioperative patient care.

Arterial and venous involvement in Behçet's syndrome: a narrative review.

Behçet syndrome (BS) is a unique type of vasculitis that affects veins and arteries of all sizes, leading to recurrent vascular events, mostly venous thrombosis. The prevalence of venous thromboembolism in BS patients ranges between 15 and 40%. Thrombosis is usually an early manifestation leading to diagnosis of BS in up to 40% of patients. BS is per se a model of inflammation-induced thrombosis. The primary autoimmune response activates lymphocytes that in turn produce a cytokine cascade that activates neutrophils, which modify the secondary structure of fibrinogen making it less susceptible to plasmin-induced lysis. This leads to endothelial dysfunction, platelet activation and overexpression of tissue factor leading to inflammatory thrombi, usually attached to the wall. The pathogenesis of thrombosis is especially relevant to direct the specific treatment, that is based on immunosuppression rather than anticoagulation. Superficial vein thrombosis (SVT) and deep vein thrombosis (DVT) are the most common form of thrombosis in BS, but thrombosis in atypical sites (cava vein, suprahepatic veins, intracardiac thrombus) and arterial involvement can also occur. We assessed the latest update of the European League Against Rheumatism recommendations for the management of BS. Vascular Behçet treatment is usually based of immunosuppressants, and the role of anticoagulation remains controversial. The use of interventional and surgical procedures should be carefully evaluated, due to the risk of triggering a vascular pathergy phenomenon.

Nonsyndromic impacted triple supernumerary teeth in the maxilla, including single dens in dente malformation: A case report with a two-year follow-up

Introduction. A percutaneous transhepatic approach has been used to place tunneled catheters for hemodialysis in the inferior vena cava. This route through the suprahepatic vein could be used to place a tunnelled catheter for permanent hemodialysis without complications and with an excellent permeability rate. Case report. From 2011 to 2020 at the Military Medical Academy, we treated 4 patients with the transhepatic central venous catheter for hemodialysis. All of them had exhausted approaches during the period of hemodialysis. Arterio-venous fistulas thrombosed on the arms, subclavian vein thrombosis bilaterally or superior and inferior vena cava thrombosis, as well as bilateral iliac and femoral vein thrombosis were present as complications of longterm hemodialysis through femoral catheters. Peritoneal dialysis was not possible. One patient needed a scroll catheter since the hemodialysis did not have a good outcome, and one patient needed a thrombolysis of catheter due to its malfunction. The other two patients have been on hemodialysis without complications for 300 and 1,650 days, respectively. Conclusion. Transhepatic venous access under ultrasound and radioscopic guidance is a simple and safe method. It is an acceptable alternative for permanent hemodialysis catheters when other venous accesses are exhausted and when it is performed by a well-trained team.