Management of splanchnic vein thrombosis.

The expression splanchnic vein thrombosis encompasses Budd-Chiari syndrome and portal vein thrombosis. These disorders have common characteristics: they are both rare diseases which can cause portal hypertension and its complications. Budd-Chiari syndrome and portal vein thrombosis in the absence of underlying liver disease share many risk factors, among which myeloproliferative neoplasms represent the most common; a rapid comprehensive work-up for risk factors of thrombosis is needed in these patients. Long-term anticoagulation is indicated in most patients. Portal vein thrombosis can also develop in patients with cirrhosis and in those with porto-sinusoidal vascular liver disease. The presence and nature of underlying liver disease impacts the management of portal vein thrombosis. Indications for anticoagulation in patients with cirrhosis are growing, while transjugular intrahepatic portosystemic shunt is now a second-line option. Due to the rarity of these diseases, studies yielding high-grade evidence are scarce. However, collaborative studies have provided new insight into the management of these patients. This article focuses on the causes, diagnosis, and management of patients with Budd-Chiari syndrome, portal vein thrombosis without underlying liver disease, or cirrhosis with non-malignant portal vein thrombosis.

Benign Hepatic Nodules in Patients With Primary Extrahepatic Portal Vein Obstruction: Clinical and Magnetic Resonance Imaging Features.

Background/objective: To retrospectively evaluate the magnetic resonance imaging (MRI) features of benign hepatic nodules in patients with extrahepatic portal vein obstruction (EHPVO) and assess predictable features for their development. Methods: This retrospective observational study included 18 diagnosed patients of EHPVO who underwent contrast enhanced abdominal MRI at our Institute between June 2016 and May 2017, and who could be followed up for at least two years. The patients with liver nodules formed the study group (n = 8; 4 males, 4 females; mean age: 26.1 ± 10.9 years) and patients without liver nodules were controls (n = 10; 3 males, 7 females; mean age: 24.2 ± 15.1 years). Liver nodules were confirmed as benign by either biopsy or stability on follow up imaging. MRI features of liver nodules were assessed. Clinical details and imaging data of the study group were compared with controls to assess predictable features. Results: There was no statistically significant difference in age, gender, clinical characteristics and upper gastrointestinal endoscopic findings between the study and control groups. The size of the lienorenal collateral, left renal vein and superior mesenteric vein were significantly larger in the study group (P < 0.05). In the study group, the majority had multiple hepatic nodules with most of them being isointense on T1 (18/35; 51.4%) and T2-weighted images (16/35; 45.7%) and showing restriction of diffusion (21/35; 60%). All (n = 35) lesions showed arterial phase hyperenhancement and none showed washout in the venous phase. The patients in the control group did not develop any liver nodules during the follow-up period. Conclusion: Liver nodules in patients with EHPVO are likely to be benign and have characteristic MRI features. Significantly larger lienorenal collateral, left renal vein and superior mesenteric vein were associated with the development of these nodules.

Long-Term Outcomes of Endoscopic Management of Patients with Symptomatic Portal Cavernoma Cholangiopathy with No Shuntable Veins for Surgery or Failed Surgery.

Background and Aim: Shunt surgery is the definitive treatment for symptomatic patients with portal cavernoma cholangiopathy (PCC), but few patients are non-surgical candidates or fail to improve even after surgery. This study aims to analyze the long-term outcomes of endoscopic therapy in these patients. Methods: Retrospective review of a prospectively maintained database of all patients with symptomatic PCC managed with endoscopic retrograde cholangiography (ERC) followed by stent placement. Outcomes studied included number of biliary interventions, complications, resolution of stricture, development of decompensation and mortality. Results: Thirty-five patients (68.6% males, median age = 35 years) with a median follow-up duration of 46 months (12-112) were included in the analysis. Presentation was only jaundice in 51.4% cases while one-third (37.1%) of the patients presented with cholangitis. Patients underwent a total of 363 endoscopic sessions with a median of 9 procedures (3-29) per patient. Hemobilia was the most common complication of the procedure (6.06%). Ten (28.5%) patients required frequent stent exchanges. Patients who required frequent stent exchanges had higher number of cholangitis episodes and hospitalization. Secondary biliary cirrhosis developed in 4 (11.4%) patients and 2 (5.7%) patients had mortality. Of the 5 (14.3%) patients who were given a stent free trial, 3 patients required restenting due to redevelopment of symptoms. Conclusion: Patients with PCC without shuntable veins for surgery or those who failed to improve after surgery can be managed long-term with repeated endoscopic intervention with a slightly increased risk of non-fatal hemobilia.

Portal vein recanalisation alone to treat severe portal hypertension in non-cirrhotic patients with chronic extrahepatic portal vein obstruction.

Background & Aims: We aimed to evaluate long-term outcome of patients with chronic non-cirrhotic extrahepatic portal vein obstruction (CNC-EHPVO) who underwent portal vein recanalisation (PVR) without transjugular intrahepatic portosystemic shunt (TIPS) insertion and to determine factors predicting PVR failure and stent occlusion. Methods: This retrospective monocentric study included all patients who underwent PVR without TIPS insertion in the context of CNC-EHPVO between the years 2000 and 2019. Primary patency was defined by the absence of a complete stent occlusion on follow-up imaging. Results: A total of 31 patients underwent PVR with a median follow-up of 52 months (24-82 months). Indications were gastrointestinal bleeding (n = 13), abdominal pain attributed to CNC-EHPVO (n = 7), prior to abdominal surgery (n = 4), and others (n = 7). Technical success was obtained in 27 patients. PVR failure was associated with extension within the intrahepatic portal veins (p = 0.005) and recanalisation for abdominal pain (p = 0.02). Adverse events occurred in 6 patients with no mortality. Anticoagulation was administered in 21 patients after technical success of PVR. In patients with technical success, 5-year primary patency was 73% and was associated with improved muscle mass (p = 0.007) and decreased spleen volume (p = 0.01) at 1 year. Furthermore, 21 (78%) patients with PVR technical success were free of portal hypertension complication at 5 years. Conclusions: PVR without TIPS insertion was feasible and safe in selected patients with CNC-EHPVO and portal hypertension with past or expected complications. Primary patency at 5 years was obtained in 3 of 4 patients with technical success of PVR and was associated with a control of complications of CNC-EHPVO. PVR was associated with improvement of sarcopenia and decreased spleen volume at 1 year. Lay summary: Patients with chronic obstruction of the portal vein and without cirrhosis or malignancy can develop complications related to the high pressure in the venous system. The present study reports long-term favourable outcome of patients in whom the obstruction was treated with stents.

Predictors of in-hospital Outcomes in Patients With Cirrhosis and Coronavirus Disease-2019.

Background: Coronavirus disease-2019 (COVID-19) cases continue to increase globally. Poor outcomes in patients with COVID-19 and cirrhosis have been reported; predictors of outcome are unclear. The existing data is from the early part of the pandemic when variants of concern (VOC) were not reported. Aims: We aimed to assess the outcomes and predictors in patients with cirrhosis and COVID-19. We also compared the differences in outcomes between the first wave of pandemic and the second wave. Methods: In this retrospective analysis of a prospectively maintained database, data on consecutive cirrhosis patients (n = 221) admitted to the COVID-19 care facility of a tertiary care center in India were evaluated for presentation, the severity of liver disease, the severity of COVID-19, and outcomes. Results: The clinical presentation included: 18 (8.1%) patients had compensated cirrhosis, 139 (62.9%) acute decompensation (AD), and 64 (29.0%) had an acute-on-chronic liver failure (ACLF). Patients with ACLF had more severe COVID-19 infection than those with compensated cirrhosis and AD (54.7% vs. 16.5% and 33.3%, P < 0.001). The overall mortality was 90 (40.7%), the highest among ACLF (72.0%). On multivariate analysis, independent predictors of mortality were high leukocyte count, alkaline phosphatase, creatinine, child class, model for end-stage liver disease (MELD) score, and COVID-19 severity. The second wave had more cases of severe COVID-19 as compared to the first wave, with a similar MELD score and Child score. The overall mortality was similar between the two waves. Conclusion: Patients with COVID-19 and cirrhosis have high mortality (40%), particularly those with ACLF (72%). A higher leukocyte count, creatinine, alkaline phosphatase, Child class, and MELD score are predictors of mortality.

Celiac Disease and Portal Hypertension: A Causal Association or Just a Coincidence?

INTRODUCTION: Celiac disease (CD) has been linked to portal hypertension (PHT) of varied etiology, but the causality association has never been proved. We aim to study the prevalence of CD in patients of PHT of different etiology. METHODS: A prospective observational study was conducted from June 2017 to December 2018 involving all the cases of PHT of varied etiology. Consecutive patients of PHT with chronic liver disease (CLD) of defined etiology like ethanol, viral hepatitis (B or C), Budd-Chiari syndrome (BCS), autoimmune-related cirrhosis, and cryptogenic CLD (cCLD) (group A) and those with noncirrhotic PHT (NCPHT), which included noncirrhotic portal fibrosis (NCPF) and extrahepatic portal vein obstruction (EHPVO) (group B), were screened for CD by IgA anti-tTG antibody followed by duodenal biopsy in serology-positive patients. RESULTS: Out of a total of 464 patients, group A constituted 382 patients, CLD related to ethanol (155), cCLD (147), hepatitis B (42), hepatitis C (21), autoimmune (10), and BCS (7), whereas 82 patients were in group B with NCPF (64) and EHPVO (18). Total 29 patients were diagnosed with CD in both groups, 17 in group A (4.5%) and 12 in group B (14.6%). In group A, 13 patients with cCLD, two with HBV-related CLD, one with BCS, and one with autoimmune-related CLD were concomitantly diagnosed as CD. In group B, CD was diagnosed in 12 patients of NCPF (11) and EHPVO (1). Liver histology showed chronic hepatitis in two patients and was normal in three patients. CONCLUSION: CD is common in PHT of different etiology, especially in cCLD, NCPH and autoimmune hepatitis; however, the etiological basis for this association is still to be defined. The likelihood of CD is higher in liver disease than the general population, and these patients should be screened for CD.

Evaluation of Splenic Stiffness in Patients of Extrahepatic Portal Vein Obstruction Using 2D Shear Wave Elastography: Comparison with Intra-Operative Portal Pressure.

BACKGROUND/AIMS: To compare splenic stiffness (SS) with intra-operative portal pressures (PPs) in patients of extrahepatic portal vein obstruction (EHPVO). METHODS: Twenty-one patients (14 males; 7 females) of mean age 20.4 years with clinical and sonographic diagnosis of EHPVO were included in this approved prospective study. Endoscopy for esophageal varices (EV) was done in all patients followed by ultrasonographic 2D shear wave elastography (SWE) of spleen. Three values were taken at different areas of spleen avoiding major vessels and mean was calculated. Intra-operative PP was measured from an omental vein during proximal spleno-renal shunt surgery. The PP was compared and correlated with SS along with other parameters. RESULTS: The mean SS was 46.04 ± 8.0 kPa and the mean PP was 33.29 ± 4.1 mmHg. There was no significant correlation between PP and SS (P = 0.61) and between grades of EV and SS (P = 0.38). Significant correlation was seen between grades of EV and PP (0.04). SS also did not show significant correlation with splenic size or duration of disease. CONCLUSION: SS measured by 2D SWE did not correlate with PP and thus may not help in predicting gastrointestinal bleed in patients of EHPVO.

Pregnancy with portal hypertension.

Even though pregnancy is rare with cirrhosis and advanced liver disease, but it may co-exist in the setting of non-cirrhotic portal hypertension as liver function is preserved but whenever encountered together is a complex clinical dilemma. Pregnancy in a patient with portal hypertension presents a special challenge to the obstetrician as so-called physiological hemodynamic changes associated with pregnancy, needed for meeting demands of the growing fetus, worsen the portal hypertension thereby putting mother at risk of potentially life-threatening complications like variceal hemorrhage. Risks of variceal bleed and hepatic decompensation increase many fold during pregnancy. Optimal management revolves round managing the portal hypertension and its complications. Thus management of such cases requires multi-speciality approach involving obstetricians experienced in dealing with high risk cases, hepatologists, anesthetists and neonatologists. With advancement in medical field, pregnancy is not contra-indicated in these women, as was previously believed. This article focuses on the different aspects of pregnancy with portal hypertension with special emphasis on specific cause wise treatment options to decrease the variceal bleed and hepatic decompensation. Based on extensive review of literature, management from pre-conceptional period to postpartum is outlined in order to have optimal maternal and perinatal outcomes.

Portal cavernoma cholangiopathy - history, definition and nomenclature.

Biliary changes secondary to portal hypertension, especially in portal cavernoma secondary to extrahepatic portal vein obstruction have long been described in literature under different names by various authors. Most of the times these changes are asymptomatic and discovered on imaging, but can occasionally cause obstructive jaundice. There is no consensus on the appropriate nomenclature and definition of this entity. This article reviews the history of portal hypertensive biliopathy and the Indian Association for the Study of Liver Working Party consensus definition and nomenclature for it.

Imaging of portal cavernoma cholangiopathy.

Portal cavernoma cholangiopathy (PCC) refers to the biliary changes which occur in the setting of extrahepatic portal vein obstruction and secondary portal cavernoma formation. The main radiological findings include the vascular changes in the form of portosystemic collaterals and biliary changes in the form of extrinsic impressions and strictures. Till date, conventional cholangiography has been the gold standard for the diagnosis of PCC. However, it is an invasive procedure and is associated with complications. At present there is a transition towards non-invasive modalities like ultrasound (US), computed tomography (CT) and magnetic resonance imaging (MRI). The recent advances in these modalities provide an excellent delineation of both the vascular and the biliary changes non-invasively in a short time. The findings of PCC using these newer modalities are not so well described in literature. The findings of PCC also overlap with malignant conditions of biliary tract such as cholangiocarcinoma and compression of biliary tract by malignant adenopathies. In this article we describe the vascular and biliary changes associated with PCC on US, CT and MRI. We also describe the imaging findings using each modality along with their advantages and disadvantages.

Collateral pathways in portal hypertension.

Presence of portosystemic collateral veins (PSCV) is common in portal hypertension due to cirrhosis. Physiologically, normal portosystemic anastomoses exist which exhibit hepatofugal flow. With the development of portal hypertension, transmission of backpressure leads to increased flow in these patent normal portosystemic anastomoses. In extrahepatic portal vein obstruction collateral circulation develops in a hepatopetal direction and portoportal pathways are frequently found. The objective of this review is to illustrate the various PSCV and portoportal collateral vein pathways pertinent to portal hypertension in liver cirrhosis and EHPVO.

Noncirrhotic portal hypertension.

Portal hypertension is characterized by an increase in portal pressure (> 10 mmHg) and could be a result of cirrhosis of the liver or of noncirrhotic diseases. When portal hypertension occurs in the absence of liver cirrhosis, noncirrhotic portal hypertension (NCPH) must be considered. The prognosis of this disease is much better than that of cirrhosis. Noncirrhotic diseases are the common cause of portal hypertension in developing countries, especially in Asia. NCPH is a heterogeneous group of diseases that is due to intrahepatic or extrahepatic etiologies. In general, the lesions in NCPH are vascular in nature and can be classified based on the site of resistance to blood flow. In most cases, these disorders can be explained by endothelial cell lesions, intimal thickening, thrombotic obliterations, or scarring of the intrahepatic portal or hepatic venous circulation. Many different conditions can determine NCPH through the association of these various lesions in various degrees. Many clinical manifestations of NCPH result from the secondary effects of portal hypertension. Patients with NCPH present with upper gastrointestinal bleeding, splenomegaly, ascites after gastrointestinal bleeding, features of hypersplenism, growth retardation, and jaundice due to portal hypertensive biliopathy. Other sequelae include hyperdynamic circulation, pulmonary complications, and other effects of portosystemic collateral circulation like portosystemic encephalopathy. At present, pharmacologic and endoscopic treatments are the treatments of choice for portal hypertension. The therapy of all disorders causing NCPH involves the reduction of portal pressure by pharmacotherapy or portosystemic shunting, apart from prevention and treatment of complications of portal hypertension.

Progression of noncirrhotic portal hypertension in a pediatric population.

BACKGROUND/OBJECTIVES: The optimal management of children with noncirrhotic portal hypertension is controversial. Some groups suggest early and aggressive surgical intervention, while others report long-term success with conservative management. METHODS: We conducted a retrospective study of 26 patients with noncirrhotic portal hypertension treated at our institution. We compared platelet counts, white blood cell (WBC) counts, spleen size, hospital admissions, gastrointestinal bleeds, and longitudinal trends of specific clinical parameters using standard univariate and time-trend analytic techniques. RESULTS: Mean age at the time of diagnosis was 5.2 years. Portal vein thrombosis was found in 84.6% of patients (n=22). There was one mortality related to malignancy. There was not a progression of hypersplenism in patients that did not receive a shunt and conversely, we did not notice a significant decrease in spleen size following shunt surgery (P=0.2). Platelet and WBC counts trended downward among patients managed medically, while platelets increased and WBC counts remained stable in surgical patients. There was a significant decrease in hospital admissions for gastrointestinal bleeding following surgical intervention in the shunt group compared with nonshunt (P=0.0009). CONCLUSION: While our analysis was limited given small sample sizes and selection bias, it suggests that the majority of pediatric patients with noncirrhotic portal hypertension will do well long-term without surgical intervention.