Extrahepatic Portal Vein Thrombosis Research Articles

Extraordinary cause of abdominal pain in sickle-cell disease patient: a case report

Background: The coexistence of extrahepatic portal vein thrombosis (EHPVT) and sickle-cell disease (SCD) was documented in a young adult patient who was presented with vague gastrointestinal symptoms, including right upper quadrant abdominal pain associated with fever and jaundice. Diagnosing EHPVT in children can be challenging due to ambiguous presentation and the lack of sufficient literature, particularly in those with SCD. Case Presentation: A five-year-old boy with homozygous sickle-cell (SS) disease presented to the emergency department with subjective fever and abdominal pain. The patient was managed conservatively with intravenous (IV) fluids, antibiotics, and analgesics. After admission, the patient was found to have hypoalbuminemia, anemia, and thrombocytopenia. Abdominal ultrasound showed moderate ascites with bowel loop thickness and preserved vasculature. Later, the boy developed increased abdominal pain. Abdominal computed tomography (CT) with contrast showed hepatic, portal, superior mesenteric and splenic veins thrombosis. Conclusion: Venous thrombosis is rare in SS disease, and its occurrence in this disease raises the possibility of a common pathogenesis and adds another pathology to the causes of abdominal pain in sickle-cell disease.

Portosinusoidal Vascular Disorder: A Heretofore Unrecognized Manifestation of Sickle Cell Disease?

Portosinusoidal vascular disorder (PSVD) is a recently proposed histopathologic entity that encompasses a spectrum of often subtle hepatic microvascular lesions and related microarchitectural abnormalities. Clinical manifestations may arise years after histologic diagnosis and include extrahepatic portal vein thrombosis and portal hypertension. While the histopathologic features of PSVD have been associated with numerous clinical conditions, most notably prothrombotic/vasculopathic disorders, PSVD has not yet been described in sickle cell disease. This gap is striking given the central role of microvascular dysfunction in sickle cell disease and well-described patterns of hepatic injury and dysfunction in this population. This case series is the first to explore the prevalence and pathogenesis of PSVD in sickle cell disease. Forty-one diagnostically adequate liver biopsies from patients with sickle cell disease were identified across the archives of 5 tertiary medical centers. All biopsies exhibited at least 1 histopathologic feature associated with PSVD (mean 3.8 features/case). Overall, 90.2% of patients met the criteria for a diagnosis of PSVD based on the presence of specific histopathologic and/or clinical findings. Immunohistochemical stains for von Willebrand factor, CD34, and glutamine synthetase were performed on 36 cases (87.8%). Aberrant (centrilobular sinusoidal) CD34 and von Willebrand factor staining was present in 97.2% and 86.1% of cases, respectively. Glutamine synthetase reactivity was at least mildly decreased in zone 3 hepatocytes in 52.8% of cases. We posit that chronic erythrocyte sickling results in dysfunction and remodeling of the portal microvasculature, culminating in regression of zone 3 hepatocytes. The presence of PSVD may explain, at least in part, the hepatic dysfunction observed in this patient population. These patients may also benefit from extended clinical surveillance for portal hypertension and other complications. While subtle and prone to overdiagnosis, the features of PSVD should be carefully considered when interpreting liver biopsies from patients with sickle cell disease.

PORTAL VEIN THROMBOSIS – A STUDY OF RISK FACTORS, CLINICAL PROFILE, COMPLICATIONS AND MANAGEMENT

Background: The portal vein is formed by the conuence of the splenic and superior mesenteric veins. Occlusion of the portal vein by thrombus (portal vein thrombosis [PVT]) typically occurs in patients with cirrhosis and/or prothrombotic disorders/ pancreatitis. Patients with acute PVT have the sudden onset of portal venous occlusion due to thrombus. Patients with acute PVT have not yet developed features of chronic PVT, such as collateral circulation (e.g., cavernous portal transformation) or portal hypertension. Chronic PVT develops when acute doesn't resolve with formation of collaterals. In patients with suspected acute PVT, we typically obtain a contrast-enhanced abdominal computed tomography (CECT) to conrm the diagnosis, evaluate for predisposing conditions (eg, intra-abdominal infection), assess the extent of the thrombosis and the anatomy of collaterals, and detect evidence of intestinal infarction. Doppler and MRI are other alternative imaging modalities. The primary management of acute portal vein thrombosis (PVT) is anticoagulation and, when possible, treatment of predisposing condition. The goal of anticoagulation is to prevent extension of the clot and to allow for recanalization, so that intestinal infarction and portal hypertension do not develop. Unlike chronic PVT, where the role of anticoagulation in patients with cirrhosis is unclear, studies suggest anticoagulation for acute PVT is benecial for patients with cirrhosis. However, because patients with cirrhosis may have esophageal varices, we typically screen for varices prior to initiating anticoagulation. 60 cases of extrahepatic portal vein thrombosis or intrahepatic Methods: portal vein thrombosis were included. All registered diagnoses were based on either ultrasound with Doppler, CT-angiography or MRI. In our study, Ris Results: k factors were established in 24 cases (80%). When including all risk factors, 16 cases (53.3%) had local risk factor, and 8 cases (26.6%) had a systemic risk factor. Anatomical location was in 26 cases (86%)extrahepatic, (14%) intrahepatic. In addition, patients with extrahepatic PVT also had intrahepatic thrombosis (38%) and/or in the splenic vein (23%). 66% had oesophageal varices, 53% gastric varices, 46% portal hypertensive gastropathy, 26% variceal haemorrhage, and 40% ascites. Most patients had a combination of local and systemic risk Conclusion: factors for PVT. Partial/ complete recanalization is more in patients treated with anticoagulation therapy, and that regression of varices was more in patients who were treated with active endoscopy combined with beta blockers.

Extra-hepatic portal vein thrombosis in children: Single center experience.

We aimed to discuss our experience in management of children with extra-hepatic portal vein thrombosis (EHPVT). This retrospective cohort study included 62 children with EHPVT. All patients' records were reviewed. The patients' socio-demographic data, post-natal history, disease presentation and clinical examination were collected. Data from laboratory investigations - complete blood count, liver function tests, renal function tests, abdominal ultrasound/Doppler studies, upper endoscopic findings and treatment regimens - were collected whenever available. Of the 62 patients, 62.9% were male and 37.1% were female. The mean age at disease presentation was 3.5 ±2.7 years. The main initial clinical presentation of the disease was hematemesis and/or melena (30 cases; 48.4%). History of umbilical catheterization (UVC) was present in 60% of cases. The thrombophilia profile was assessed in 17 patients, of whom 12 (70.6%) were found to have a coagulation disorder. Splenomegaly was present in 91.7% of the patients. Hematological abnormalities in the form of cytopenias were present in most cases. Ultrasound revealed the presence of collaterals in 76.2%. Upper endoscopy showed the presence of varices in 45 cases, all of which needed endoscopic intervention, while in 11 cases the varices were either low grade or absent and thus were subjected only to medical treatment with propranolol and 6 cases were lost to follow-up. Splenectomy was done in only one case and 2 cases underwent the Rex operation. Variceal bleeding is the most common clinical presentation of EHPVT in children. UVC is still the main etiological factor of EHPVT in our cohort especially with presence of thrombophilic disorder.

Porto-sinusoidal vascular disorder.

It is well established that portal hypertension can occur in the absence of cirrhosis, as reported in patients with immune disorders, infections and thrombophilia. However, similar histological abnormalities primarily affecting the hepatic sinusoidal and (peri)portal vasculature have also been observed in patients without portal hypertension. Thus, the term porto-sinusoidal vascular disorder (PSVD) has recently been introduced to describe a group of vascular diseases of the liver featuring lesions encompassing the portal venules and sinusoids, irrespective of the presence/absence of portal hypertension. Liver biopsy is fundamental for PSVD diagnosis. Specific histology findings include nodular regenerative hyperplasia, obliterative portal venopathy/portal vein stenosis and incomplete septal fibrosis/cirrhosis. Since other conditions including alcohol-related and non-alcoholic fatty liver disease, or viral hepatitis, or the presence of portal vein thrombosis may occur in patients with PSVD, their relative contribution to liver damage should be carefully assessed. In addition to histology and clinical diagnostic criteria, imaging and non-invasive tests such as liver and spleen stiffness measurements could aid in the diagnostic workup. The introduction of PSVD as a novel clinical entity will facilitate collaborative studies and investigations into the underlying molecular pathomechanisms encompassed by this term.

Intestinal arteriovenous malformations: a diagnostic and therapeutic challenge

Arteriovenous malformations (AVM) of the intestine are rare causes of gastrointestinal bleeding. Diagnosis depends upon accurate interpretation of imaging features and histopathology to differentiate them from other vascular malformations. Presentation of colonic AVM with mucous diarrhea and pain is rare and poses diagnostic challenges. Usually, patients presenting with bleed need surgical intervention. The current report discussed one patient with chronic liver disease, extrahepatic portal vein thrombosis with small bowel AVM and two patients with colonic AVM, highlighting management strategies adopted.

Functional side-to-side splenorenal shunts to treat extrahepatic portal vein thrombosis in children

BackgroundSurgical shunts are commonly used to manage complications resulting from extrahepatic portal vein thrombosis (EHPVT) in children. We describe a single-center experience utilizing a functional Side-to-Side Splenorenal Shunt (fSRS), created using either an enlarged inferior mesenteric vein (IMV) or left adrenal vein (LAV). MethodsPediatric patients with isolated EHPVT who were poor candidates for a Rex shunt and who underwent a fSRS procedure at our institution between 2003 and 2020 were reviewed. The pre/post shunt portosystemic gradient change, rates of early and late complications, postoperative shunt patency, and mortality were evaluated. ResultsTwelve EHPVT patients (mean age of 6.1 years) underwent a fSRS procedure. The mean portosystemic gradient change for the cohort was −11.7 mmHg (±4.9). There were no cases of recurrent variceal bleeding or episodes of shunt thrombosis reported after fSRS procedures. ConclusionsSurgical shunts continue to be an important adjunct in the treatment of complications related to EHPVT. The functional Side-to-Side Splenorenal Shunt is a safe alternative that is easy to perform, involves minimal dissection and requires only a single anastomosis.

Extrahepatic Portal Vein Thrombosis in Childhood: Risk Factors, Clinical Manifestations, and Management

Objective: Extrahepatic portal vein thrombosis (EHPVT) is a common cause of portal hypertension in children. The aim of the present study was to identify the clinical manifestations and the risk factors for development of EHPVT in pediatric patients. Subjects and Methods: This was a single-center retrospective cohort study. A total of 12 children (6 boys and 6 girls) took part in the study. We noted the clinical presentations and the predisposing risk factors for development of EHPVT in all patients. In addition, as all of them had undergone an esophagogastroduodenoscopy for detection and grading of esophageal varices as part of the treatment algorithm, we analyzed the endoscopic findings and the therapeutic approach. Results: The median age of subjects at diagnosis was 3.5 years (range: 1–17 years). The most frequent initial clinical manifestation was upper gastrointestinal bleeding (6 cases, 50.0%) followed by splenomegaly (3 cases, 25.0%). The most frequent systemic risk factor for EHPVT was presence of inherited prothrombotic disorder (10 cases, 83.3%), and the most common local risk factor for EHPVT was umbilical vein catheterization (5 cases, 41.7%). Esophageal varices were revealed in all the study participants, and in the most cases, they were grade ≥2. Propranolol was used as primary or secondary prophylaxis in 7 children (58.3%), and in 5 children (41.7%), a shunt was performed (Meso-Rex bypass in 3 children and splenorenal shunt in 2 children). Conclusion: Patients with known systemic or local risk factors for EHPVT are indicated for proactive ultrasound screening for early diagnosis and timely management.

Use of the Rex Shunt in Adults for Left Portal Vein Reconstruction

Introduction: To report an expanded use of Rex shunt (RS) for portal vein (PV) reconstruction in adults. The RS was developed to treat children with extrahepatic portal vein thrombosis (EHPVT) following liver transplantation or with a healthy liver. Method: An RS was used to achieve portal reperfusion during various liver surgeries when a standard porto-left portal anastomosis was deemed technically unsatisfactory or incompatible with right-sided R0 onco-surgical resection. Results: This study involved 5 male and 6 female patients (median age, 59 years). The RS was feasible in all 11 consecutive attempts, including hepatectomy for malignancy combined with PV resection in 9 (82%) patients where the left lobe or liver was left in place.A reinforced Gore-Tex graft was used in 10 patients and an allogenic iliac vein was used in 1 patient.Within 90 days, 1 patient with a patent shunt had died of pulmonary embolism, and 1 patient had died of diffuse PVT with multiple organ failure.In 4 patients, postoperative complications included ascites, biliary fistula, and shunt thrombosis.At a median 13.5-month follow-up, 7 patients with a patent shunt remained alive. A systematic review of RS in adults identified 11 cases in 8 reports performed mainly for EHPVT (9 patients). In 2 patients, postoperative complications involved transient ascites and abdominal bleeding requiring surgical revision.Postoperative mortality was nil. The median long-term patency rate was 24 months. Conclusion: Use of the RS in adults is straightforward, offering a supplementary technical option for left portal reconstruction in patients with malignancy or EHPVT,provided the downstream portal system is normal.

Extrahepatic Portal Vein Thrombosis, an Important Cause of Portal Hypertension in Children.

One of the most important causes of portal hypertension among children is extrahepatic portal vein thrombosis (EHPVT). The most common risk factors for EHPVT are neonatal umbilical vein catheterization, transfusions, bacterial infections, dehydration, and thrombophilia. Our study aimed to describe the clinical manifestations, treatment, evolution, and risk factors of children with EHPVT. Methods: We analyzed retrospectively all children admitted and followed in our hospital with EHPVT between January 2011–December 2020. The diagnosis was made by ultrasound or contrast magnetic resonance imaging. We evaluated the onset symptoms, complications, therapeutic methods, and risk factors. Results: A total of 63 children, mean age 5.14 ± 4.90 (33 boys, 52.38%), were evaluated for EHPVT during the study period. The first symptoms were upper gastrointestinal bleeding (31 children, 49.21%) and splenomegaly (22 children, 34.92%). Thrombocytopenia was present in 44 children (69.84%). The most frequent risk factors were umbilical vein catheterization (46 children, 73.02%) and bacterial infections during the neonatal period (30 children, 47.62%). Protein C, protein S, antithrombin III levels were decreased in 44 of the 48 patients tested. In 42 of these cases, mutations for thrombophilia were tested, and 37 were positive. Upper digestive endoscopy was performed in all cases, revealing esophageal varices in 56 children (88.89%). All children with gastrointestinal bleeding received an octreotide infusion. In 26 children (41.27%), variceal ligation was performed, and in 5 children (7.94%), sclerotherapy. Porto-systemic shunt was performed in 11 children (17.46%), and Meso-Rex shunt was done in 4 children (6.35%). The evolution was favorable in 62 cases (98.41%). Only one child died secondary to severe sepsis. Conclusions: EHPVT is frequently diagnosed in the last period in our region due to the increased use of umbilical vein catheterization. Furthermore, genetic predisposition, neonatal bacterial infections, and prematurity certainly play an important role in this condition. A proactive ultrasound assessment of children with risk factors for EHPVT should be encouraged for early diagnosis and treatment.

Trombosis venosa portal extrahepática, manejo quirúrgico con derivación meso-Rex. Serie de 3 casos

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.

Sugiura modificado: una alternativa para pacientes no-cirróticos con trombosis de la porta extrahepática y sangrado variceal recurrente

Una de las consecuencias más graves de la trombosis de la vena porta extrahepática es la hipertensión portal con sangrado variceal recurrente. Una vez falla la ligadura endoscópica de las várices y el eje esplenoportal no se encuentra permeable, la devascularización tipo Sugiura modificado puede ser la única alternativa. Se ha documentado su uso en pacientes con cirrosis, pero hay poca información en personas no cirróticas. En este artículo se describe una serie de 4 casos de pacientes no cirróticos, en los cuales se realizó dicho procedimiento. Los pacientes fueron seguidos durante 12 meses y ninguno presentó episodios de resangrado de las várices esofágicas, ni tampoco se requirió la ligadura de las várices residuales. Esta cirugía se perfila como una alternativa terapéutica para este tipo de pacientes.

Pediatric living donor liver transplantation with non-anatomical portal vein reconstruction in idiopathic extrahepatic portal vein thrombosis

A 13-year-old boy had a history of recurrent hematemesis and melena since the age of 2 years. Owing to recurrent bleeding, he had undergone several endoscopic ligation procedures and had received regular blood transfusions over the last 7 years. Doppler ultrasonography showed extrahepatic portal vein thrombosis, while the hepatic vein was not visualized. Abdominal computed tomography confirmed cavernous transformation in the liver hilum. Liver biopsy revealed fibrosis. A diagnosis of extrahepatic portal vein obstruction (EHPVO) with portal hypertension was established. Living donor liver transplantation (LDLT) was performed owing to recurrent gastrointestinal bleeding and liver fibrosis. The liver graft was obtained from the patient's father, utilizing segments 2, 3, and 4 (graft-to-recipient weight ratio: 0.84%). Portal vein reconstruction using native internal jugular vein and external iliac vein was achieved with non-anatomical anastomosis due to the lack of availability of sizable superior mesenteric vein. The postoperative course was uneventful. We successfully performed LDLT with unusual portal vein reconstruction in a patient with EHPVO. Multiple interposition grafts were used to obtain adequate inflow from the splenorenal shunt. LDLT remains the last resort in such cases as other surgical procedures (such as Meso-Rex bypass) are not feasible.

An analysis on the use of Warren’s distal splenorenal shunt surgery for the treatment of portal hypertension at the University Hospitals Leuven

Introduction Extrahepatic portal vein thrombosis (PVT) is the most common cause of portal hypertension (PH), particularly in children. PH-related manifestations include refractory variceal bleeding, splenomegaly and ascites. Albeit more rarely performed, the distal splenorenal shunt (Warren’s shunt) has proven to be effective in selectively decompressing the collateral circulation. The aim of our study was to describe our experience with the distal splenorenal shunt and to determine the long-term effect on PH-related side-effects. Methods Distal splenorenal shunt operations performed at our institution between 2000 and 2014 were reviewed for: age, male/female ratio, children/adults ratio, body mass index, indications, grade of PVT (Yerdel classification), maximal shunt-flow velocity, shunt patency and thrombosis, re-intervention for variceal bleeding and survival. Complications of PH (esophageal variceal bleeding and ascites) were compared pre- versus post-operatively (last follow-up). Paired student t-test and fisher’s exact were applied for pre- versus post-operative comparison. Results are reported as median [range]. Results Fourteen patients with PVT and refractory complications of PH underwent distal splenorenal shunt surgery. Age was 15 years [4.5–66]. Male/female ratio was 7/7. PVT -grade was 2 [1–4]. Follow-up was 3 [0.5–14]. All shunts were patent (100%) with no shunt thrombosis (0%) at last follow-up. There was no re-intervention for variceal bleeding (0%) and survival at last follow-up was 100%. Occurrence of esophageal variceal bleeding was higher pre-operatively (57%) than postoperatively (0%) (p = .0032) and also the incidence of ascites was higher pre-operatively (79%) than postoperatively (0%) (p < .0001). Conclusions Based on our experience, the distal splenorenal shunt can be considered a valuable surgical technique for PVT-induced PH, with excellent post-operative prevention of complications of PH.

Portal Vein Thrombosis and Budd-Chiari Syndrome as the Initial Symptom of Polycythemia Vera and Hyperhomocysteinemia

Portal Vein Thrombosis (PVT), commonly associated with cirrhosis of liver and thrombophilia, is one of the causes of severe abdominal pain. In the absence of non-cirrhotic non-malignant extrahepatic portal vein thrombosis, Myeloproliferative Disease (MPD) and an underlying thrombotic disorder should always be suspected and investigated. Hyperhomocysteinemia has been well-documented to increase the risk of arterial thrombotic events, peripheral arterial disease, and stroke. It is also a risk factor for deep-vein thrombosis. In the general population, association with portal vein thrombosis is very unusual, and only a few cases have been reported. We describe a case of Polycythemia Vera (PV) and hyperhomocysteinemia presenting with severe abdominal pain due to portal vein thrombosis. The patient underwent phlebotomy and was prescribed life-long anticoagulant, aspirin, vitamin B6, vitamin B12, and folic acid, then referred to a hematologist.

Splanchnic vein closures

Closures in the splanchnic venous system (SVS) represent abroad medical problem. Anatomically, individual or even multiple sections of SVS may be affected at the same time. Main sections of SVS include the venous liver outflow system, the portal vein, and the upper mesenteric vein and its basin. Thrombosis is clearly the predominant cause of closure. The closures can present as acute, subacute, chronic occult or chronic manifest. The main pathological and anatomical units are the Budd-Chiari syndrome (BCS), extrahepatic portal vein obstruction (EHPVO) and mesenteric vein thrombosis (MVT). Advanced laboratory, imaging and intervention methods substantially modify the approach to prevention, diagnosis and treatment; surgical approach also plays arole. The problem of SVS closures is interdisciplinary.

MR angiography and 2-D phase-contrast imaging for evaluation of meso-rex bypass function.

The meso-Rex bypass restores blood flow to the liver in patients with extrahepatic portal vein thrombosis. Stenosis occurs in some cases, causing the reappearance of portal hypertension. Complications such as thrombocytopenia present on a spectrum and there are currently no guidelines regarding a threshold for endovascular intervention. While Doppler ultrasound (US) is common for routine evaluation, magnetic resonance (MR) angiography with two-dimensional phase-contrast MRI (2-D PC-MRI) may improve the assessment of meso-Rex bypass function. To determine the feasibility and utility of MR angiography with 2-D PC-MRI in evaluating children with meso-Rex bypass and to correlate meso-Rex bypass blood flow to markers of portal hypertension. MR angiography and 2-D PC-MRI in meso-Rex bypass patients were retrospectively analyzed. Minimum bypass diameter was measured on MR angiography and used to calculate cross-sectional area. Meso-Rex bypass blood flow was measured using 2-D PC-MRI and divided by ascending aortic flow to quantify bypass flow relative to systemic circulation. Platelet and white blood cell counts were recorded. Correlation was performed between minimum bypass area, blood flow and clinical data. Twenty-five children (median age: 9.5years) with meso-Rex bypass underwent MR angiography and 2-D PC-MRI. The majority of patients were referred to imaging given clinical concern for complications. Eighteen of the 25 patients demonstrated >50% narrowing of the bypass cross-sectional area. The mean platelet count in 19 patients was 127K/μL. There was a significant correlation between minimum cross-sectional bypass area and bypass flow (rho=0.469, P=0.018) and between bypass flow and platelet counts (r=0.525, P=0.021). Two-dimensional PC-MRI can quantify meso-Rex bypass blood flow relative to total systemic flow. In a cohort of 25 children, bypass flow correlated to minimum bypass area and platelet count. Two-dimensional PC-MRI may be valuable alongside MR angiography to assess bypass integrity.

Imaging of the intrahepatic portal vein in children with extrahepatic portal vein thrombosis — Comparison of magnetic resonance imaging and retrograde portography

PurposeExtrahepatic portal vein thrombosis (EPVT) is one major cause of portal hypertension in children. Surgical reinstallation of portal venous flow can be achieved in patients with patent intrahepatic portal venous system/Rex recess. Our study aimed to compare the ability of magnetic resonance imaging (MRI) and retrograde portography (RP) to assess patency of the intrahepatic portal venous system in children with EPVT. MethodsAll pediatric patients with EPVT who were examined with contrast enhanced MRI (1.5 T) and invasive RP between 2013 and 2017 were included in this retrospective study. Medical records were reviewed for demographic, biochemical and clinical data. Patency of the Rex recess as detected by MRI and RP was retrospectively reviewed. ResultsSixteen children (7.6 ± 5.0 years) with EPVT were included. Sensitivity, specificity, positive and negative predictive value for the detection of patent Rex recess by MRI compared to RP were 55%, 57%, 63% and 50%. Diagnostic accuracy was 56%. Diagnostic failure of MRI compared to RP was explained by the following: I. Problems differentiating collaterals from portal venous vessels II. Incapability showing dynamic blood flow in compromised portal venous flow III. Poor spatial resolution, especially in small children. ConclusionRP is a reliable method for the visualization of the Rex recess and the intrahepatic portal venous system in children with EPVT, whereas MRI has shown to be unsuitable for the assessment of the intrahepatic portal vein in these patients. In the preoperative setup, we recommend both procedures, RP and MRI for the visualization of the intrahepatic portal venous system, and the extrahepatic vessels, respectively. Level of evidenceLevel III.

Long-Term Azathioprine Use and the Development of Non-Cirrhotic Portal Hypertension

Introduction: Non-cirrhotic portal hypertension (NCPH) is caused by intra- and extrahepatic etiologies. NCPH presents with signs of portal hypertension, especially variceal bleeding and splenomegaly. Extrahepatic portal vein thrombosis, nonalcoholic steatohepatitis, and primary sclerosing cholangitis are the most prevalent causes in the Western world. In rare cases, immunosuppressive drugs, particularly azathioprine (AZA), 6-mercaptopurine, and 6-thioguanine (TG), have been implicated in nodular regenerative hyperplasia, an idiopathic form of NCPH. Here we describe a case of NCPH with obstructive portal venopathy possibly induced by long-term AZA use. Case: A 44-year-old male with type I diabetes and Crohn's Disease was referred to our hepatology clinic for management of presumed cirrhosis. At the age of 40, after 20 years on AZA, EGD demonstrated grade II esophageal varices. Ultrasound revealed increased liver echogenicity and marked heterogeneity, as well as portal vein dilatation and splenomegaly. Beta-blocker therapy was initiated and AZA was discontinued. Adalimumab was started but was switched to vedolizumab one year later due to coccidioidomycosis infection. Labs were significant for thrombocytopenia, leukopenia, and microcytic anemia. MRI on presentation to our facility demonstrated splenomegaly and patent portal veins but no signs of cirrhosis. Ultrasound-guided liver biopsy demonstrated dense portal fibrosis and focal obliteration of portal vein branches. Portal vein branches were absent from portal tracts, and outlet venules were dilated (Figure 1). There was no evidence for clinical, radiologic, or histological cirrhosis or fatty liver disease.2250 Figure 1. Absence of portal veins from portal tracts with dilatations of the outlet venules (“herniated” portal veins, arrows), consistent with obstructive portal venopathy (A. H&E stain x200; B. Trichrome stain x100).Discussion: While the pathogenesis of AZA-induced NCPH remains uncertain, there is evidence that glutathione depletion, which is toxic to sinusoidal endothelial cells, leads to veno-occlusive disease. Whether occlusive venopathy is attributable to 6-TG toxicity alone remains unclear. Portal fibrosis and obstructive venopathy on histology in the absence of vascular occlusion on imaging suggest that portal hypertension in our patient was heralded by intrahepatic changes. Damage to endothelium by 6-TG represents one possible mechanism; however, it is just as feasible that an autoimmune process led to NCPH in this patient. We thus propose further research into possible associations between AZA and NCPH.

Role and safety of human thrombin injection for the treatment of bleeding gastric varices.

Human thrombin appears to be a new effective tool in the armamentarium of management of bleeding gastric varices, but there arepaucity of data on its use. Thus, we share our experience with human thrombin in the treatment of bleeding gastric varices. It was a prospective interventional study conducted between September 2015 and December 2017. Patients with upper gastrointestinal bleeding from gastric varices were included, while patients with previous history of cyanoacrylate glue injection or band ligation were excluded. A total of 20 patients including 13 males (mean age 32.65 [18-52] years) presenting with gastric variceal bleeding requiring endoscopic injection of human thrombin were studied. The underlying diagnosis was cirrhosis in 8 patients, and extrahepatic portal vein thrombosis, noncirrhotic portal fibrosis, and chronic pancreatitis in 6, 4, and 2 patients, respectively. Isolated gastric varices were found in 6 patients while 14 patients had gastroesophageal varices (GOV) (GOV1-3, GOV2-11). Patients received 1 to 3 sessions (mean = 1.3) of thrombin with a mean total dose of 700IU (range = 500-2000IU). Mean follow up was 16.8months (range 3-28months). Hemostasis in the acute setting was successfully managed in all the 20 patients on initial presentation. On serial follow up, 4 out of 20 patients required repeat endoscopic session for gastric varices. No thrombin injection-related complication was recorded. Endoscopic therapy with thrombin appears safe and effective in the management of bleeding gastric varices.