Presinusoidal Portal Hypertension Research Articles

Noninvasive diagnosis of liver fibrosis in schistosomiasis japonica: Opportunities and challenges

Noninvasive diagnosis of liver fibrosis in schistosomiasis japonica: Opportunities and challenges

Hepatosplenic schistosomiasis: an uncommon cause of liver disease in developed countries.

Schistosomiasis is a parasitic infection caused by trematode species of the genus Schistosoma. It is prevalent in tropical regions of Africa, Asia and South America, being rare in Europe, where it is usually diagnosed in immigrants and tourists from endemic areas. It has different clinical forms of presentation. Hepatosplenic schistosomiasis produces periportal fibrosis, which can progress to presinusoidal portal hypertension, with all its associated complications. We present the case of a 43-year-old female patient from the Philippines who was referred to gastroenterology consultation due to liver enzyme alteration with a predominantly cholestatic pattern. An aetiological study was performed, with negative results. An abdominal ultrasound revealed signs of chronic liver disease, with transient elastography of 9.5 kPa. A percutaneous liver biopsy was performed, with histological findings consistent with infestation by schistosome eggs, receiving treatment with praziquantel and subsequently verifying its eradication with a stool test.

A Comparative Cross-Sectional Study of Coagulation Profiles and Platelet Parameters of Schistosoma mansoni-Infected Adults at Haik Primary Hospital, Northeast Ethiopia.

Introduction Schistosoma mansoni is an intravascular parasite that interacts with all components of the host blood. Nearly, 10% of S. mansoni-infected patients progress to severe hepatosplenic Schistosomiasis is characterized by periportal fibrosis, obstruction of intrahepatic veins, presinusoidal portal hypertension, and splenomegaly. Thus, this study aimed to compare the basic coagulation profiles and platelet parameters of S. mansoni-infected adults and noninfected individuals as controls at Haik Primary Hospital, Northeast Ethiopia. Methods A comparative cross-sectional study was conducted at Haik Primary Hospital from April to June 2021. The diagnosis and intensity of S. mansoni infection was determined using the Kato–Katz technique. The coagulation profiles and platelet parameters were analyzed using coagulation and hematology analyzers. Data were analyzed using SPSS version 26.0. The Kolmogorov–Smirnov and Shapiro–Wilk tests were done to check the distribution of continuous variables. The Mann–Whitney U test was used to compare the coagulation profiles and platelet parameters. Spearman's rank-order correlation was done to assess the correlation between the intensity of infection and coagulation profiles and platelet parameters. In all comparison, a P value <0.05 was considered statistically significant. Result In this study, a total of 180 study participants (90 S. mansoni-infected adults and 90 controls) were included. Of the total S. mansoni-infected adults, 55.6%, 28.9%, 33, and 15.6% had light, moderate, and heavy intensity of infections, respectively. All S. mansoni-infected study participants had prolonged prothrombin time (PT) and international normalized ratio (INR). Moreover, about 80% of S. mansoni-infected adults had prolonged activated partial thromboplastin time (APTT). Thrombocytopenia was found in 26.7% of the S. mansoni-infected adults. The Mann–Whitney U test showed a statistically significant difference in coagulation profiles between S. mansoni-infected adults and healthy controls (P-value ≤0.001). The Kruskal–Wallis H-test showed a significant difference in PT, APTT, and INR between the intensity of infection and healthy controls (P-value <0.05). Conclusion This study showed a prolonged coagulation time in S. mansoni-infected individuals. Thus, screening of schistosomiasis patients for hemostatic abnormalities and treating the underlying disorder is crucial.

A case of agnogenic intrahepatic arterioportal fistula complicated by gastric ulcer bleeding and progressive portal thrombosis

AbstractArterioportal fistulas (APFs) are defined as vascular communications between the systemic arteries and portal circulation. APFs are classified as congenital or acquired, large or small, intrahepatic or extrahepatic, and traumatic or spontaneous. The consistent abnormal connection may result in severe presinusoidal portal hypertension, leading to gastrointestinal bleeding (GIB), ascites, elevated liver function enzymes, heart failure, diarrhea, and even pancreatitis or hematobilia in some unusual cases. Indeed, less than half reported cases have GIB, predominately caused by variceal rupture. Peptic ulcer bleeding has rarely been reported in the setting of APFs. Herein, we describe a case in which intrahepatic APF manifested as gastric ulcer bleeding, progressive portal thrombosis, and massive ascites, with these symptoms successfully relieved by endovascular treatment.

A110 FEASIBILITY OF EUS-GUIDED PORTAL PRESSURE GRADIENT MEASUREMENT WITHOUT DEEP SEDATION: A PATH TO ACCURATE PORTAL PRESSURE DETERMINATION

AimsHepatic venous pressure gradient (HVPG) is the gold standard for the diagnosis and staging of portal hypertension. Previous studies have shown that it underestimates pre-sinusoidal portal hypertension. Endoscopic ultrasound-guided portal pressure gradient (EUS-PPG) can safely measure direct portal vein pressure (PVP) and bridge this diagnostic gap. However, EUS-PPG has so far been performed with patients under deep sedation which can alter HVPG measurement and lead to misclassification of portal hypertension. Ketamine, a conscious sedation agent, has minimal effect on portal hemodynamics. We present our center’s experience with EUS-PPG under ketamine and low dose midazolam.MethodsWe retrospectively reviewed consecutive patients undergoing EUS-PPG with conscious sedation using ketamine and low-dose midazolam (<0.02 mg/kg) at the McGill University Health Centre from February to May 2021. Patients were placed in the left lateral position. Hepatic vein and portal vein were located through EUS via a trans-gastric/hepatic approach. A through the scope 25-gauge needle attached to a manometer was advanced through the liver to measure hepatic vein pressure (HVP) and portal vein pressure (PVP). Three measurements were sampled per vessel and mean pressure differences calculated to obtain PPG. PPG was considered reliable if the differences between the values was no more than 1mmHg.ResultsThree patients underwent EUS-PPG for evaluation of pre-sinusoidal portal hypertension. Cirrhosis was excluded in all patients based on recent liver biopsy or transient elastography. The first patient is a 69-year-old man with splenomegaly and recanalization of the paraumbilical vein on imaging. He received 80 mg of ketamine and no midazolam. HVPG measured was 1 mmHg. EUS-PPG was successful with PPG of 6 mmHg (HVP= 7 mmHg, and PVP= 13 mmHg). The second patient is a 42-year-old woman with previous sleeve gastrectomy and known with portal cavernoma. She received 120 mg of ketamine and 1 mg of midazolam. EUS-PPG was technically difficult due to respiratory movements. The measurement was not considered reliable as differences in PPG were as high as 2mmHg. In this case, PPG was 0mmHg (HVP= 15mmHg, and PVP= 15mmHg). The third patient, a 74-year-old man with hepatic steatosis and splenomegaly received 70 mg of ketamine and 1 mg of midazolam. EUS-PPG was successful (HVP= 3mmHg, PVP= 3mmHg) yielding a PPG 0mmHg. All patients tolerated the procedure well with no procedural or sedation-related complicationsConclusionsOur early experience suggests that EUS-PPG can be successfully and safely performed in patients under conscious sedation with ketamine and low-dose midazolam. This combination may avoid deep sedation with high dose midazolam or propofol which are known to alter accuracy of HVPG. Measurement of HVP (left) and PVP (right)Funding AgenciesNone

Endoscopic Ultrasound-guided Portal Pressure Gradient Measurement Is Technically Successful and Safe Without Deep Sedation: A Proof-of-Concept Case Series

The hepatic venous pressure gradient (HVPG), which is the difference between the wedged (WHVP) and free hepatic vein pressures (FHVP), is the gold standard to evaluate portal hypertension in patients with cirrhosis 1 Bosch J Abraldes JG Berzigotti A et al. The clinical use of HVPG measurements in chronic liver disease. Nat Rev Gastroenterol Hepatol. 2009; 6: 573-582 Crossref PubMed Scopus (436) Google Scholar . Unfortunately, there currently exists a gap in the evaluation of patients with pre-sinusoidal portal hypertension, where HVPG is known to underestimate portal pressure. Endoscopic ultrasound-guided portal pressure gradient measurement (EUS-PPG) has recently brought back the possibility of safely obtaining direct portal vein pressure (PVP) and represents a long-awaited less-invasive diagnostic tool for patients with suspected pre-sinusoidal portal hypertension.

A228 WHEN ASCITES &amp; VARICEAL BLEEDING ARE NOT FROM CIRRHOSIS: A CASE OF MUTIPLE ARTERIOPORTAL FISTULAE CAUSING PORTAL HYPERTENSION

Abstract Background Portal hypertension is usually due to increased resistance from cirrhosis. However, pressures can also be elevated due to increased flow. Aims To describe a peculiar case of non-cirrhotic portal hypertension. Methods A case report and literature review was performed. Results A 47-year-old previously well man presented with a 6 month history of rapidly progressive weight loss, ascites and variceal bleed. Workup ruled out common causes of primary liver disease. Initial imaging demonstrated a heterogenous liver, splenomegaly, ascites, patent hepatic/portal veins and multiple poorly defined low-density hepatic lesions with the largest measuring 2.1 cm. Transient elastography was 7.3 kPa (F1-mild fibrosis). At transjugular liver biopsy, hepatic venogram ruled out Budd-Chiari and hepatic vein pressure gradient was normal at 3–4 mmHg. Histology unfortunately showed hemangioma. A percutaneous liver biopsy suggested nodular regenerative hyperplasia, minimal fibrosis and mild cholestasis. Given worsening ascites, hyponatremia and 7 months of rapidly progressive decline, transjugular intrahepatic portosystemic shunt (TIPSS) was inserted. Intra-procedure, portal vein pressure was noted to be 51 mmHg, with a portosystemic gradient of 42 mmHg. Although numerous abdominal CT and MRI did not show AV shunting, ultrasound post-TIPSS showed hepatic pseudoaneurysms &amp; arterioportal fistulae (APF). Direct angiogram showed numerous hepatic pseudoaneurysms and intrahepatic fistulae making embolization impossible. CT showed no evidence of pseudoaneurysms or fistulae outside of the liver. Workup for autoimmune rheumatological diseases and congenital telangiectatic syndromes were negative. Given the high pressures being directed through the new TIPSS, right heart failure is an ongoing concern. APF are rarely encountered causes of presinusoidal portal hypertension, with communications most commonly arising from the hepatic (65%) &amp; splenic arteries (11%) &amp; the portal vein. Causes include traumatic (28%), iatrogenic (16%), vascular/telangiectatic malformations (15%), tumors (15%), aneurysms (14%) &amp; congenital disease. Endovascular embolization can be used to treat single lesions. In complex cases with mulitple APF, surgery and/or liver transplantation may be required. Conclusions We report a rare case of non-cirrhotic portal hypertension due to increased flow rather than increased resistance secondary to APF. Funding Agencies None

S2747 A Detailed Travel History Solved a Medical Mystery

INTRODUCTION: Taking a detailed history is often a clinical challenge. Asking the right question related to the presenting symptoms in the key. At the Minneapolis VA, we have encountered an asymptomatic patient who had incidental finding of “portal hypertension” on imaging. Yet, he had an extensive negative workup. At the time of his EGD, a more detailed history was taken during medical records review, the his own history finally solved the mystery. CASE DESCRIPTION/METHODS: 66 y/o male with incidental findings of “portal hypertension” from a CT scan presented for a diagnostic EGD. A recent radiology report described a cluster of varices near the gastric fundus and a spleen size of 15 cm. Subsequent evaluation by the GI specialty clinic failed to identify a cause; the workup included alcohol use history, medication review, autoimmune markers, viral serologies, genetic testing, metabolic markers, lipid studies, iron studies, tumor markers, liver function tests, clotting factors, and fibroscan. Just prior to EGD, a detailed history revealed a Vietnam deployment in 1970. The patient further described a life threatening event. He was sick for almost a year. The upper endoscopy subsequently showed no varices and no gastropathy. DISCUSSION: Falciparum malaria is the most severe form of malarial disease caused by plasmodium falciparum. This disease is responsible for over 400,000 death globally in 2018. Over 228 MILLION cases of malaria were reported by the WORLD HEALTH ORGANIZATION (WHO) in 2018. Plasmodium falciparum is classified as Group 2A carcinogen by the WHO. This organism is transmitted by infected female anopheline mosquitos. The eventual multiple organ failure and death is caused by the obstruction of the body’s microcirculation by the organism particles. In this case, the CT findings were caused by temporary pre-sinusoidal portal hypertension until the malaria was cured several months later. As a result, permanent enlarged spleen and permanent dilated veins were discovered on the CT scan several decades later. The primary disease was cured 40 years ago. It is imperative to understand the history, pathophysiology, and the clinical sequelae of common tropical diseases when taking care of patients that travel throughout the world. In today’s western medical practice, we frequently forget the old diseases that still cause wide range of destruction in other parts of the world. We can avoid wasting medical resources by asking the right question.

Porto-Sinusoidal Vascular Disease Associated to Oxaliplatin: An Entity to Think about It.

Portal sinusoidal vascular disease is a presinusoidal cause of portal hypertension (PHT) of unknown etiology, characterized by typical manifestations of PHT (esophageal varices, ascites, portosystemic collaterals), plaquetopenia and splenomegaly with a gradient of portal pressure slightly increased, according to the presinusoidal nature of the PHT. A few cases in the literature have shown a relationship between oxaliplatin and the development of presinusoidal portal hypertension, years after the chemotherapy for colorectal cancer (therefore, different to sinusoidal obstruction syndrome). There are three mechanisms through which oxaliplatin can cause sinusoidal damage: (1) damage at the level of endothelial cells and stimulates the release of free radicals and depletion of glutathione transferase, with altering the integrity of the sinusoidal cells. The damage in the endothelial sinusoidal cells allows to erythrocytes to across into the Dissé space and formation of perisinusoidal fibrosis, (2) the appearance of nodular regenerative hyperplasia is favored by the chronic hypoxia of the centrilobular areas and, finally, (3) oxaliplatin can generate an obliteration of the blood capillaries and zones of parenchymal extinction. These three facts can develop, in a minority of cases, the appearance of a presinusoidal increase of portal pressure, which typically appears years after the completion of chemotherapy and sometimes is underdiagnosed until variceal bleeding, ascites or encephalopathy appear. The knowledge of this pathology is essential to be able to perform an early diagnostic and consult to the hepatologist.

Pancreatic Retroperitoneal Fibrosis Masquerading as a Pancreatic Mass: A Diagnostic Quandary

Retroperitoneal fibrosis (RPF) is a cryptic condition affecting 0.1 per 100,000 patients per year. The pathophysiologic etiology is believed to be autoimmune in nature, resulting in fibrotic tissue deposition within multiple organs. This case highlights an unusual primary pancreatic manifestation of an already exceedingly rare disease. The patient is a 45 year-old male with no significant past medical history who presented with painless jaundice. Computerized tomography revealed a 3 cm pancreatic-head mass encasing the vasculature and ductal system. An endoscopic ultrasound with fine-needle-biopsies (EUS-FNB) of the mass and endoscopic retrograde cholangiopancreatography (ERCP) were performed. The EUS characterized the lesion as a 34 x 28 mm ill-defined heterogeneous mass with multiple collateral blood vessel in the pancreas, suggestive of underlying portal hypertension, correlating with an endoscopic finding of esophageal varices. On ERCP, the common bile duct was dilated to 14mm with a shelf-like distal stricture, treated with a 10 French, 5cm plastic biliary stent. Initial fine needle biopsy results, while inconclusive, could not rule out malignancy or autoimmune pancreatitis. Repeat EUS-FNB with 7 needle passes again returned with non-malignant findings. He subsequently underwent laparoscopic biopsy of the pancreatic mass which revealed sclerosing collagenous fibrosis, displaying lymphoplasmacytic inflammation, suggestive of RPF. Infectious and malignant etiologies for RPF were excluded. He was started on steroids with symptomatic improvement and mass stabilization on subsequent imaging. Through extensive review of the literature, RPF encasing the pancreas without ureteral or periaortic involvement has scarcely been documented. In this case, the mass effect of the fibrosis resulted in biliary obstruction and pre-sinusoidal portal hypertension, and highlights the difficulty in diagnosing an exceedingly rare condition masquerading as pancreatic malignancy. This patient had a protracted course involving multiple sub-specialties, numerous endoscopic procedures, and ultimately a diagnostic surgery. The seemingly elusive diagnosis of RPF conclusively requires histologic certainty prior to treatment. When finally diagnosed, steroids are the mainstay of therapy for RPF.1424_A.tif Figure 1: Dense fibrosis with keloid-type collagen fibers1424_B.tif Figure 2: PET scan showing avid FDG uptake at pancreatic lesion1424_C.tif Figure 3: No Caption available.

Canine and feline abdominal arterioportal communications can be classified based on branching patterns in computed tomographic angiography.

Arterioportal vascular anomalies are communications between the splanchnic arteries and the portal system that represent a rare cause of presinusoidal portal hypertension in small animals. There is little information concerning the imaging findings of arterioportal communications in small animals and no classification could be found for radiologists and surgeons. The aims of this retrospective descriptive multicentric study were to describe the computed tomographic characteristics of arterioportal communications in a group of cats and dogs, and to propose a classification based on computed tomography (CT) angiographic anatomy. Computed tomography databases from multiple veterinary hospitals were searched for cats and dogs with a diagnosis of arterioportal communication. A total of 36 animals (33 dogs, three cats) met the inclusion criteria. There were 32 intrahepatic arterioportal malformations and four extrahepatic fistulae. The intrahepatic arterioportal malformations were classified as right divisional (11/32) and left divisional (21/32), and the left divisional were subclassified as left medial (16/21) and left lateral (4/21). One patient showed multiple intrahepatic arterioportal communications with concomitant left medial and left lateral conformations. Two patients with intrahepatic arteriovenous malformation showed concomitant congenital intrahepatic shunts. The proposed anatomical classification based on CT angiography could allow veterinary radiologists to have a more systematic approach and help improve the radiologist-surgeon communication.

\u03b2-Blockers Improve Presinusoidal Portal Hypertension

BackgroundPresinusoidal portal hypertension is a clinically important cause of gastric and gastroesophageal varices. Whereas β-blockers have an established prophylactic role against bleeding from esophageal and gastric varices in portal hypertension due to cirrhosis, the effect on presinusoidal portal hypertension is unknown.AimsTo evaluate the hemodynamic effect of β-blockers in non-cirrhotic patients with presinusoidal portal hypertension.MethodsWe measured the blood pressure gradient from spleen pulp to free hepatic vein in 12 patients with presinusoidal portal hypertension by combined hepatic vein catheterization and spleen pulp puncture while on and off β-blocker treatment (random sequence).ResultsThe β-blockers reduced the gradient from a mean off-treatment value of 32 mm Hg to a on-treatment value of 26 mm Hg (P < 0.05) with a reduction of at least 20% in five patients (42%).Conclusionsβ-blocker treatment caused a clinically significant reduction in the pressure gradient from spleen pulp to the free hepatic vein. This finding supports the recommendation for prophylactic β-blockage in patients with presinusoidal portal hypertension.

PORTAL PRESSURE DECREASE AFTER ESOPHAGOGASTRIC DEVASCULARIZATION AND SPLENECTOMY IN SCHISTOSOMIASIS: LONG-TERM VARICES BEHAVIOR, REBLEEDING RATE, AND ROLE OF ENDOSCOPIC TREATMENT

Schistosomiasis is an endemic health problem affecting about four million people. The hepatosplenic form of the disease is characterized by periportal hepatic fibrosis, pre-sinusoidal portal hypertension and splenomegaly. Liver function is preserved, being varices bleeding the main complication of the disease. The surgical treatment used in the majority of centers for the prevention of rebleeding is esophagogastric devascularization and splenectomy. Most authors reported better results with the association of surgical and postoperative endoscopic treatment. The aim of this study was to compare the intra operative portal pressure decrease and esophageal varices behavior and rebleeding rates in patients submitted to surgical and postoperative endoscopic treatment after long-term follow-up. A retrospective study of 36 patients with schistosomiasis with, at least, one previous bleeding from esophageal varices rupture submitted to esophagogastric devascularization and splenectomy, added to endoscopic varices postoperative treatment was performed. Patients were stratified according to the intra operative portal pressure decrease in two groups: reduction below and above 30%. Long-term varices presence, size and bleeding recurrence were evaluated. Regarding varices behavior, no significant influence was observed in both groups of portal pressure fall. Regarding bleeding recurrence, despite three times more frequent in the group with lower portal pressure fall, no significant difference was observed. All patients were submitted to postoperative endoscopic treatment. Esophageal varices banding, rather than portal pressure decrease, seems to be the main responsible factor for good results after combination of two therapies (surgery and endoscopy) for patients with portal hypertension due to schistosomiasis; further studies are necessary to confirm this hypothesis.

Liver atrophy and regeneration in noncirrhotic portal vein thrombosis: Effect of surgical shunts.

The goal of the study is to characterize the relationship between portal vein thrombosis (PVT) and hepatic atrophy in patients without cirrhosis and the effect of various types of surgical shunts on liver regeneration and splenomegaly. Patients without cirrhosis with PVT suffer from presinusoidal portal hypertension, and often hepatic atrophy is a topic that has received little attention. We hypothesized that patients with PVT have decreased liver volumes, and shunts that preserve intrahepatic portal flow enhance liver regeneration. Sixty-four adult and pediatric patients with PVT who underwent surgical shunt placement between 1998 and 2011 were included in a retrospective study. Baseline liver volumes from adult patients were compared with standard liver volume (SLV) as well as a group of healthy controls undergoing evaluation for liver donation. Clinical assessment, liver function tests, and liver and spleen volumes from cross-sectional imaging were compared before and after surgery. A total of 40 patients received portal flow-preserving shunts (32 mesoportal and 8 selective splenorenal), whereas 24 received portal flow-diverting shunts (16 nonselective splenorenal and 8 mesocaval). Baseline adult liver volumes were 26% smaller than SLV (1248 versus 1624 cm3 ; P = 0.02) and 20% smaller than the control volumes (1248 versus 1552 cm3 ; P = 0.02). Baseline adult spleen volumes were larger compared with controls (1258 versus 229 cm3 ; P < 0.001). Preserving shunts were associated with significant increase in liver volumes (886 versus 1131 cm3 ; P = 0.01), whereas diverting shunts were not. Diverting shunts significantly improved splenomegaly. In conclusion, we have demonstrated that patients without cirrhosis with PVT have significant liver atrophy and splenomegaly. Significant liver regeneration was achieved after portal flow-preserving shunts. Liver Transplantation 24 881-887 2018 AASLD.

Idiopathic portal hypertension with regard to thiopurine treatment.

Idiopathic portal hypertension (IPH) is an infrequent adverse reaction to the use of thiopurines that tends to be overlooked. Herein, we present a patient with ileocolic Crohn's disease treated with azathioprine who presented ascites, esophageal varices and splenomegaly without any signs of liver cirrhosis. A portal hemodynamics study revealed a normal portosystemic gradient compatible with presinusoidal portal hypertension. Finally, IPH was diagnosed after a liver biopsy. IPH secondary to thiopurines is due to a 6-thioguanine nucleotide (6-TGN)-dependent reaction and occurs predominantly between three months and three years after the start of treatment. The onset is usually insidious and thrombocytopenia is the first manifestation. The definitive diagnosis is obtained by liver biopsy.

Assessment of contrast-enhanced ultrasonography of the hepatic vein for detection of hemodynamic changes associated with experimentally induced portal hypertension in dogs.

OBJECTIVE To assess the use of contrast-enhanced ultrasonography (CEUS) of the hepatic vein for the detection of hemodynamic changes associated with experimentally induced portal hypertension in dogs. ANIMALS 6 healthy Beagles. PROCEDURES A prospective study was conducted. A catheter was surgically placed in the portal vein of each dog. Hypertension was induced by intraportal injection of microspheres (10 to 15 mg/kg) at 5-day intervals via the catheter. Microsphere injections were continued until multiple acquired portosystemic shunts were created. Portal vein pressure (PVP) was measured through the catheter. Contrast-enhanced ultrasonography was performed before and after establishment of hypertension. Time-intensity curves were generated from the region of interest in the hepatic vein. Perfusion variables measured for statistical analysis were hepatic vein arrival time, time to peak, time to peak phase (TTPP), and washout ratio. The correlation between CEUS variables and PVP was assessed by use of simple regression analysis. RESULTS Time to peak and TTPP were significantly less after induction of portal hypertension. Simple regression analysis revealed a significant negative correlation between TTPP and PVP. CONCLUSIONS AND CLINICAL RELEVANCE CEUS was useful for detecting hemodynamic changes associated with experimentally induced portal hypertension in dogs, which was characterized by a rapid increase in the intensity of the hepatic vein. Furthermore, TTPP, a time-dependent variable, provided useful complementary information for predicting portal hypertension. IMPACT FOR HUMAN MEDICINE Because the method described here induced presinusoidal portal hypertension, these results can be applied to idiopathic portal hypertension in humans.

Abnormal Liver Stiffness Measurement in a Patient with Waldenstrom Macroglobulinemia: Nuances in Interpreting the Findings

Background: Liver stiffness measurement (LSM) using vibration-controlled transient elastography (VCTE) provides an estimate of liver fibrosis, non-invasively, in a quick and efficient manner. Its use and interpretation of LSM is limited. In the current case, we report our findings of abnormal LSM and liver histology in a patient with Waldenstrom Macroglobulineima (WM) with evidence of portal hypertension. Case: A 84-year-old white man with history notable for WM and history of pulmonary embolism on chronic anticoagulation presented with new-onset ascites. His blood work revealed isolated elevation in alkaline phosphatase (AST 14 U/L, ALT 7 U/L, ALP 173 U/L, GGT 275 u/L and total bilirubin 0.6 mg/dL) with a platelet count was 208 k/cumm. Blood work for competing etiology was negative for viral, metabolic, and autoimmune hepatitis (negative viral hepatitides, normal iron studies, normal alpha-1-antitrypsin level, negative anti-smooth muscle antibody and negative anti-mitochondrial antibody with normal immunoglobulins (other than presence of IgM kappa monoclonal protein) ruling out primary biliary cirrhosis). Ultrasound with dopplers showed cirrhotic appearing liver, cholelithiasis, mild ascites not amenable to diagnostic paracentesis, and patent portal veins. MRCP showed normal appearing liver, mild ascites, and gallbladder sludge without biliary dilation. Evaluation with VCTE after paracentesis revealed elevated LSM at 12.2 with IQR% of 9%. Pressure measurements during transjugular liver biopsy noted normal hepatic vein pressure gradient (HVPG) of 6 mmHg (corrected sinusoidal pressure 10 mmHg (RA 1 mmHg, IVC 5 mmHg, free HV 5 mmHg, wedge 11 mmHg), consistent with pre-sinusoidal portal hypertension. Liver histology showed nodularity in the absence of significant fibrosis. Reticulin stain confirmed nodularity and showed areas of hepatic trabecular compression and others with trabecular thickening. Focal mild sinusoidal dilatation was seen. The portal tracts are largely unremarkable with patchy mild chronic inflammation and minimal ductular reaction. Congo red stain was negative as well ruling out amyloidosis. Conclusion: This case highlights the rare presentation of non-cirrhotic portal hypertension with WM and the false increase in LSM due to infiltration with M protein.

Left Ventricular Dilation and Pulmonary Vasodilatation after Surgical Shunt for Treatment of Pre-Sinusoidal Portal Hypertension.

ObjectiveThe aim of this study was to prospectively investigate the long-term cardiovascular and pulmonary hemodynamic effects of surgical shunt for treatment of portal hypertension (PH) due to Schistosomiasis mansoni.LocationThe University of São Paulo Medical School, Brazil; Public Practice.MethodsHemodynamic evaluation was performed with transesophageal Doppler and contrast-enhanced echocardiography (ECHO) on twenty-eight participants with schistosomal portal hypertension. Participants were divided into two groups according to the surgical procedure used to treat their schistosomal portal hypertension within the last two years: group 1—distal splenorenal shunt (DSRS, n = 13) and group 2—esophagogastric devascularization and splenectomy (EGDS, n = 15).ResultsThe cardiac output (5.08 ± 0.91 L/min) and systolic volume (60.1 ± 5.6 ml) were increased (p = 0.001) in the DSRS group. DSRS participants had a significant increase (p < 0.0001) in their left ventricular end-systolic and end-diastolic diameters as well as in their left ventricular end-diastolic and end-systolic volumes (p < 0.001) compared with the preoperative period. No statistically significant difference was found in the patients who underwent EGDS. ECHO revealed intrapulmonary vasodilatation (IPV) in 18 participants (64%), 9 DSRS and 9 EGDS (p > 0.05).ConclusionsThe late increase in the cardiac output, stroke volume and left ventricular diameters demonstrated left ventricular dilatation after a distal splenorenal shunt. ECHO revealed a greater prevalence for IPV in patients with schistosomiasis than has previously been described in patients with PH from liver cirrhosis.

Value of portal venous system radiological indices in predicting esophageal varices.

IntroductionPortal hypertension results from increased resistance to portal blood flow and has the potential complications of variceal bleeding and ascites. The splenoportal veins increase in caliber with worsening portal hypertension, and partially decompress by opening a shunt with systemic circulation, ie, a varix. In the event of portosystemic shunting, there is a differential decompression across the portal vein and splenic vein (portal vein > splenic vein), with a resultant decrease in the ratio of portal vein diameter to that of splenic vein. Portal vein to splenic vein diameter ratio and gradient could be valuable tools in predicting the presence of portosystemic shunting.MethodsWe retrospectively reviewed patients with cirrhosis who underwent esophagogastroduodenoscopy (EGD) for variceal screening and had a computerized tomogram (CT) of the abdomen within 6 months of the index endoscopic study, between January 2009 and December 2013. Patients on nonselective beta blockers, patients with presinusoidal portal hypertension (portal vein thrombosis or extrinsic compression), and patients who had undergone portosystemic shunting procedures (transjugular intrahepatic portosystemic shunt [TIPS]) or balloon-occluded retrograde transvenous obliteration (BRTO) were excluded from the study. Splenic and portal vein diameters were measured (in mm) just proximal and distal to the splenomesenteric venous confluence, respectively.ResultsA total of 164 patients were included in the study; of these, 60% (n=98) were male and 40% (n=66) were female. The mean age of the study population was 58.7 years. A total of 126 patients (77%) had varices, while 38 patients (33%) did not. The mean Model for End-Stage Liver Disease (MELD) score was 5.9 for those who had varices as compared with 7.03 for those who did not. The mean of ratios of portal vein to splenic vein diameters in patients with varices was 1.27 (±0.2), while it was 1.5 (±0.23) in those without varices. This difference was statistically significant (P<0.001). The mean of the gradients between the portal vein and splenic vein diameters was 2.7 (±2) mm for patients with varices as compared with 5 (±1.8) mm in those without varices. This difference was also statistically different (P<0.001). These correlations were statistically significant even after controlling for age, sex, and MELD. These radiological indices also had statistically significant correlations with the presence of gastric varices (P=0.018 for the ratio and P=0.01 for the gradient). A discriminant function analysis was performed that generated the equation: D = 2.68 (ratio of portal vein to splenic vein diameters) + 0.187 (gradient of portal vein to splenic vein diameters, in mm) − 4.152. This equation had a very high sensitivity, of 95%, but low specificity, of 26.3%, in predicting the presence of esophageal varices.ConclusionBoth venous diameter ratio (portal vein size/splenic vein size) and venous diameter gradient in mm (portal vein size – splenic vein size) calculated from CTs of the abdomen were good predictors of presence of esophageal varices. These parameters might be useful in stratifying patients at risk of developing esophageal varices who are poor candidates for endoscopic evaluation.

Non-cirrhotic portal hypertension – Concept, diagnosis and clinical management

Non-cirrhotic portal hypertension (NCPH) is mainly related to vascular disorders in the portal system, granuloma formation with periportal fibrosis or genetic alterations affecting the hepatobiliary system. For the diagnosis of the so-called idiopathic NCPH, it is essential to rule out chronic liver diseases associated with progression to cirrhosis as viral hepatitis B and C, alcoholic and non-alcoholic fatty liver, autoimmune disease, hereditary hemochromatosis, Wilson's disease as well as primary biliary cirrhosis and primary sclerosing cholagitis. This mini review will focus on the most common types of NCPH, excluding the idiopathic NCPH. Primary Budd-Chiari syndrome, characterized by obstruction of hepatic venous outflow, must be distinguished from sinusoidal obstruction syndrome, a cause of portal hypertension associated with exposure to toxic plants or therapeutic agents. Noninvasive imaging methods usually help the diagnosis of both Budd-Chiari syndrome and portal thrombosis, the later a relatively frequent cause NCPH. Clinical presentation and management of these vascular disorders are evaluated. Schistosomiasis, a worldwide spread endemic parasitic disease, may evolve to severe forms of the disease with huge spleen and gastroesophageal varices due to presinusoidal portal hypertension. Although management of acute upper gastrointestinal bleeding is similar to that of cirrhosis, prevention of rebleeding differs. Instead of portosystemic shunt procedures, the esophagogastric devascularization with splenectomy is the accepted surgical alternative. Its association with endoscopic therapy is suggested to be the best option for PH due to schistosomiasis. In conclusion, the prompt diagnosis of the disorder leading to non-cirrhotic portal hypertension is essential for its correct management.