Extrahepatic Portal Vein Obstruction Patients Research Articles

Abdominal surgery in patients with chronic noncirrhotic extra hepatic portal vein obstruction: A multicenter retrospective study.

In patients with noncirrhotic chronic extra-hepatic portal vein obstruction (EHPVO), data on morbimortality of abdominal surgery are scarce. We retrospectively analyzed the charts of 76 patients (78 interventions) with EHPVO undergoing abdominal surgery within the VALDIG network. Fourteen percent of the patients had ≥1 major bleeding (unrelated to portal hypertension) and 21% had ≥1 Dindo-Clavien grade ≥3 postoperative complication within 1 month after surgery. Fifteen percent had ≥1 portal hypertension related complication within 3 months after surgery. Three patients died within 12 months after surgery. An unfavorable outcome (i.e. ≥1 above-mentioned complications or death) occurred in 37% of the patients and was associated with a history of ascites and with non-wall, non-cholecystectomy surgical intervention: 17% of the patients with none of these features had an unfavorable outcome, versus 48% and 100% when one or both features were present, respectively. We then compared 63/76 EHPVO patients with 126 matched (2:1) control patients without EHPVO but with similar surgical interventions. As compared with control patients, incidence of major bleeding (p<0.001) and portal-hypertension related complication (p<0.001) was significantly higher in patients with EHPVO, but not that of grade ≥3 postoperative complication nor of death. The incidence of unfavorable post-operative outcome was significantly higher in patients with EHPVO than in those without (33% vs. 18%, p=0.01). Patients with EHPVO are at high-risk of major peri- or postoperative bleeding and postoperative complications, especially in those with ascites or undergoing surgery other than wall surgery or cholecystectomy.

Navigating portal hypertension: Unlocking safe passage to healthy pregnancy in EHPVO.

Pregnancy is associated with hyperdynamic circulatory state and increased risk of portal hypertension related complications in patients with extra-hepatic portal vein obstruction (EHPVO). We aim to study the impact of EHPVO on pregnancy-related outcomes with focus on subset of patients with UGIB (upper GI bleed). Retrospective analysis of obstetric, maternal and neonatal outcomes of patients with EHPVO registered between January 2006 and December 2022. Forty-five patients were included. Forty-five healthy females with low-risk pregnancies formed the control group. Adverse obstetric and neonatal outcomes were comparable between EHPVO and control group (22% vs. 28.6%; p > .05; low birth weight/ small for gestational age 17.8% vs. 36%, p = .0918 and 14.2% vs. 10%, p = .5698 respectively). Adverse outcomes were similar in patients with and without history of UGIB (26.3% vs. 19.4%, p = .0814; 17.8% vs. 36%, p = .0918; 14.2% vs. 10%, p = .5698). There was no maternal mortality in both the groups. A total of 7% pregnancies in EHPVO patients were complicated by ascites. EHPVO pregnancies have successful obstetric and neonatal outcomes with adequate management of portal hypertension.

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

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. 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. 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. 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.

Meso-Rex bypass versus portosystemic shunt for the management of extrahepatic portal vein obstruction in children: systematic review and meta-analysis.

Extrahepatic portal vein obstruction (EHPVO) is a major cause of non-cirrhotic portal hypertension in children. Surgical procedures for EHPVO include portosystemic shunts (PSS) and meso-Rex bypass (MRB). We conducted a systematic review and meta-analysis to compare the effectiveness of MRB versus PSS in EHPVO patients. A systematic literature search was performed using four databases. Articles reporting EHPVO and comparing patients who received MRB and PSS were included in the analysis. We retrieved 851 papers, of which five observational studies met the inclusion criteria. There was no difference in shunt complications, mortality, or gastrointestinal bleeding after surgery between MRB and PSS in the meta-analysis. MRB had increased shunt complications compared with PSS in the non-comparative studies. MRB had a potential advantage over PSS in long-term prognosis in one comparative study. Overall, the quality of the evidence was low. Based on available data, our meta-analysis indicates that MRB does not increase shunt complications, mortality, or gastrointestinal bleeding after surgery. The present study did not reveal superiority for either MRB or PSS. The paucity of well conducted trials in this area justifies future multicenter studies and studies that examine long-term outcomes.

Correlation of endoscopic findings with Doppler ultrasound in portal hypertension in children.

Aim of the studyTo determine the correlation of the endoscopic findings with portal Doppler and ultrasound (USG) in children with suspected portal hypertension (PHT).Material and methodsEighty children with extrahepatic portal vein obstruction (EHPVO) and chronic liver disease (CLD) were included in this retrospective study conducted over a period of 1 year. All patients underwent upper gastrointestinal (GI) endoscopy and Doppler.ResultsThe etiology was EHPVO in 30 (37.5%) patients, biliary atresia in 12 (15%), Budd-Chiari syndrome in 11 (13.7%), Wilson’s disease in 10 (12.5%), idiopathic CLD in 8 (10%), autoimmune hepatitis in 4 (5%), glycogen storage disease (GSD) in 3 (3.8%), non-alcoholic liver disease (NAFLD) in 1 (1.3%) and systemic lupus erythematosus (SLE) in 1 (1.3%) patient. Fifty-three (66.25%) patients had esophageal varices on endoscopy, of whom 3 (3.8%) had associated gastric varices. Portal hypertensive gastropathy (PHG) was present in 30 (37.5%) patients, of whom 10 (12.5%) had severe PHG. Forty-one (51.3%) patients had PHT on Doppler (κ correlation 0.43). Kappa correlation was 0.43 in patients with biliary atresia, 0.31 in Budd-Chiari syndrome, 0.23 in idiopathic CLD, 0.21 in CLD, and 0.05 in Wilson’s disease. All (100%) EHPVO patients and 39 (78%) CLD patients had PHT on USG. Endoscopic findings of PHT were seen in 24 (80%) EHPVO patients and 29 (58%) CLD patients. All patients with EHPVO had cavernous transformation of the portal vein on Doppler. For patients with CLD, the common Doppler findings were collaterals seen in 35 patients and reversal of flow in 12 patients.ConclusionsDoppler ultrasound followed by endoscopy should be used to diagnose PHT in children. In children with biliary atresia, Doppler ultrasound may miss changes of PHT.

Surgical Management of Portal Biliopathy – A Prospective Analysis.

Background: Portal biliopathy denotes intrahepatic and extra hepatic biliary ductal abnormalities in portal hypertension. It is usually associated with extra hepatic portal vein obstruction (EHPVO). These patients are also prone to develop obstructive jaundice as a result of strictures and/or choledocholithiasis. Surgical management of obstructive jaundice in such patients becomes difficult in the presence of these collaterals. The aim of the study is to prospectively analyze the approach to management of patients with Symptomatic portal biliopathy. Subjects and Methods: The study was conducted at Narayana Medical College &amp; Hospital, Chintareddy Palem, Nellore, Andhra Pradesh on surgical management of the patients of EHPVO with portal biliopathy presenting to the surgical clinic of this tertiary referral center between November 2016 and October 2017. The data was analyzed for presentation, clinical features, imaging and results of surgical management. Results: During the study period, total of 44 patients of EHPVO were referred for surgical management. Of these 14 patients (9 males, mean age 34.6 years) were diagnosed to have portal biliopathy. Ten patients had prior history of variceal bleed which was managed endoscopically. Jaundice was the most common symptom followed by right upper quadrant pain and recurrent cholangitis. Four patients had prior unsuccessful endoscopic management. ERCP/ MRCP was used for delineation of the biliary tree, which showed irregularity (14 patients), dominant strictures (8 patients), filling defects (5 patients), and intrahepatic biliary dilatation (7 patients). Proximal splenorenal shunt (PSRS) was performed in 13 patients. While in 1 patient peroperatively liver was found to be grossly nodular, hence gastro-esophageal devascularization with simultaneous biliary drainage was done. Of the 13 patients who underwent PSRS, all patients were intensively followed for 4-6 weeks with history and liver function tests. After 6 weeks, five patients showed clinical as well as biochemical improvement and they are being followed up regularly. Eight patients had persistent symptoms and abnormal liver function tests. These were the patients with dominant stricture and choledocholithiasis. Of these, 6 patients underwent Roux-en-Y hepaticojejunostomy. The average blood transfusion requirement at second surgery was 1 unit. Postoperative complications were minimal with no mortality. One patient and was lost to follow up and the remaining one is awaiting second surgery (RYHJ). Over a follow up of 3-28 months the patients are asymptomatic and well. Conclusion: Portal biliopathy with symptomatic biliary obstruction needs intervention. Surgical decompressive shunt followed by biliary drainage is the best possible treatment. While for most of the early biliary changes shunt alone is effective, patients with dominant stricture will need a biliary diversion which can be safely performed following Porto systemic shunt without increase in morbidity or mortality.

Pathological abnormalities in splenic vasculature in non-cirrhotic portal hypertension: Its relevance in the management of portal hypertension.

BACKGROUNDPortal hypertension (PH) is associated with changes in vascular structure and function of the portosplenomesenteric system (PSMS). This is referred to as portal hypertensive vasculopathy. Pathological abnormalities of PSMS has been described in the literature for cirrhotic patients. Raised portal pressure and hyperdynamic circulation are thought to be the underlying cause of this vasculopathy. In view of this, it is expected that pathological changes in splenic and portal vein similar to those reported in cirrhotic patients with PH may also be present in patients with non-cirrhotic PH (NCPH).AIMTo investigate pathological abnormalities of splenic vein in patients with NCPH, and suggest its possible implications in the management of PH.METHODSA prospective observational study was performed on 116 patients with NCPH [Extrahepatic portal vein obstruction (EHPVO): 53 and non-cirrhotic portal fibrosis (NCPF): 63] who underwent proximal splenorenal shunt (PSRS), interposition shunt or splenectomy with devascularization in JIPMER, Pondicherry, India, a tertiary level referral center, between 2011-2016. All patients were evaluated by Doppler study of PSMS, computed tomography porto-venogram and upper gastrointestinal endoscopy. An acoustic resonance forced impulse (ARFI) scan and abdomen ultrasound were done for all cases to exclude cirrhosis. Intraoperative and histopathological assessment of the harvested splenic vein was performed in all. The study group was divided into delayed and early presentation based on the median duration of symptoms (i.e. 108 mo).RESULTSThe study group comprising of 116 patients [77 (66%) females and 39 (34%) males] with NCPH had a median age of 22 years. Median duration of symptoms was 108 mo. The most common presentation in both EHPVO and NCPF patients was upper gastrointestinal bleeding (hematemesis and melena). The ARFI scan revealed a median score of 1.2 (1.0-1.8) m/s for EHPVO and 1.5 (0.9-2.8) m/s for NCPF. PSRS was performed in 84 patients (two of whom underwent interposition PSRS using a 10 mm Dacron graft); splenoadrenal shunt in 9; interposition mesocaval shunt in 5; interposition 1st jejunal to caval shunt in 1 patient and devascularization with splenectomy in 17 patients. Median pre-splenectomy portal pressure was 25 (range: 15-51) mm Hg. In 77% cases, the splenic vein was abnormal upon intraoperative assessment. Under macroscopic examination, wall thickening was observed in 108 (93%), venous thrombosis in 32 (28%) and vein wall calcification in 27 (23%) cases. Upon examination under a surgical magnification loupe, 21 (18%) patients had intimal defects in the splenic vein. Histopathological examination of veins was abnormal in all cases. Medial hypertrophy was noted in nearly all patients (107/116), while intimal fibrosis was seen in 30%. Ninety one percent of patients with intimal fibrosis also had venous thrombosis. Vein wall calcification was found in 22%, all of whom had intimal fibrosis and venous thrombosis. The proportion of patients with pathological abnormalities in the splenic vein were significantly greater in the delayed presentation group as compared to the early presentation group.CONCLUSIONPathological changes in the splenic vein similar to those in cirrhotic patients with PH are noted in NCPH. We recommend that PH in NCPH be treated as systemic and pulmonary hypertension equivalent in the gastrointestinal tract, and that early aggressive therapy be initiated to reduce portal pressure and hemodynamic stress to avoid potential lethal effects.

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

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. 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. 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. 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.

Recanalization of Chronic Extrahepatic Portal Vein Obstruction in Pediatric Patients Using a Minilaparotomy Approach.

Extrahepatic portal vein obstruction (EHPVO) is the most frequent cause of portal hypertension in children. Some patients are not amenable to meso-Rex bypass and alternative surgeries do not restore physiologic flow. We aim to demonstrate the feasibility and safety of minilaparotomy for recanalization of chronic EHPVO. This 2013-2015 single-center, retrospective review included pediatric patients with chronic EHPVO who underwent minilaparotomy, mesenteric vein access, and attempted recanalization of the occluded portal vein. Outcomes included portal patency, resolution of variceal bleeding, size and number of varices, spleen size, and platelet count. There were 6 EHPVO patients. The median age was 9.9 years and median duration of EHPVO was 7 years (3-16 years). EHPVO etiologies were liver transplantation (50%), idiopathic (33%), and umbilical vein catheterization (17%). Four patients (67%) had successful portal vein recanalization and stenting. At last follow-up [median 3.1 years (2.2-4.3 years)] all successfully recanalized patients had patent portal vein stents and resolution of varices and variceal bleeding. The median reduction in spleen size was 26%, with improvement in platelet counts (50-310/μL). The 2 patients with an idiopathic etiology may have never had a main extrahepatic portal vein based on imaging, and both were unable to be recanalized. Recanalization and stenting of a prolonged occlusion of the portal vein via a minilaparotomy approach is feasible, safe, and may provide an alternative to shunt surgery or endoscopic therapy in selected patients.

Long-Term Results after Diversion Surgery in Extrahepatic Portal Vein Obstruction.

Extrahepatic portal vein obstruction (EHPVO) is a frequent cause of noncirrhotic portal hypertension in children. The aim of this study is to analyze long-term results after diversion surgery. Retrospective review of EHPVO patients who underwent diversion surgery analyzing number of platelets, leukocytes, prothrombin activity, splenomegaly, and gastrointestinal bleeding 10 years after surgery. Thirty-three patients were evaluated, mostly males (64%) and presenting with gastrointestinal bleeding (46%). Mesoportal shunt (Rex) was performed in 19 patients, mesocaval in 7, distal splenorenal in 7, and proximal splenorenal in 3. While comparing mesoportal shunt to the other portosystemic shunts, an increase in platelets was found with every technique, but it was clearly higher in mesoportal shunt. The highest increase was 6 months after surgery (p = 0.0015) as well as prothrombin activity (p = 0.0003). Leukocytes level also increased without statistical significance. Spleen size (cm) and spleen size Z score (SSAZ) decreased significantly 6 months after mesoportal shunt (p = 0.0168). Before surgery, over 94% patients suffered gastrointestinal bleeding, which reduced significantly afterward with bleeding episodes in only four (12%) of them. Diversion surgery in EHPVO, especially mesoportal shunt of Rex, improves hepatic function (prothrombin activity), reduces hypersplenism (platelets, leukocytes, and spleen size), and decreases gastrointestinal bleeding episodes.

Bleeding risk of variceal band ligation in extrahepatic portal vein obstruction is not increased by oral anticoagulation.

Noncirrhotic nontumoral extrahepatic portal vein obstruction (EHPVO) is the second leading cause of portal hypertension (PHT) and is mainly related to prothrombotic disorders. Patients with EHPVO often require prolonged oral anticoagulation therapy (OAT) together with variceal band ligation (VBL) to prevent thrombosis recurrence and PHT-related bleeding, respectively. The benefit-risk balance of VBL in this context remains unknown. We aimed to assess upper gastrointestinal bleeding (UGB) risk and variceal eradication efficacy in EHPVO patients undergoing a VBL program without stopping OAT. All patients with EHPVO treated (group A) or not (group B) with OAT and undergoing the VBL program were included between 2001 and 2010 in two tertiary French liver centers. We compared the incidence, source, and severity of UGB and variceal eradication efficacy. All EHPVO patients were then matched 1 : 1 with compensated cirrhotic patients with PHT not receiving OAT (group C) to compare UGB incidence and VBL efficacy. Forty-three EHPVO patients (30 with and 13 without OAT) and 43 cirrhotic patients were included for a total of 471 VBL sessions. The incidence of UGB was similar between group A (nine episodes/121 sessions) and group B (6/130), and tended to be higher in EHPVO patients (group A and B) than in cirrhotic patients (2/220). There was no difference between groups when considering bleeding source or severity and variceal eradication efficacy (84%). VBL can be performed safely and efficiently without stopping anticoagulation therapy in EHPVO patients.

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

To compare splenic stiffness (SS) with intra-operative portal pressures (PPs) in patients of extrahepatic portal vein obstruction (EHPVO). 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. The mean SS was 46.04±8.0kPa and the mean PP was 33.29±4.1mmHg. 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. SS measured by 2D SWE did not correlate with PP and thus may not help in predicting gastrointestinal bleed in patients of EHPVO.

Long Term Outcomes of Patients with Significant Biliary Obstruction Due to Portal Cavernoma Cholangiopathy and Extra-Hepatic Portal Vein Obstruction (EHPVO) With No Shuntable Veins

The natural history of portal cavernoma cholangiopathy (PCC) in patients with significant biliary obstruction (SBO) who cannot undergo shunt surgery, is not known. We therefore, analyzed data of patients of extra-hepatic portal venous obstruction (EHPVO) with PCC. Prospectively recorded details of 620 (age 21.2 [11.4] years; 400 [65%] males) patients with primary EHPVO were reviewed. Outcomes (hepatic decompensation/mortality) of patients with PCC and SBO without shuntable veins were noted at follow up of 7 [4-11] years. Ninety-seven of 620 (15.6% [60 men]) EHPVO patients had PCC-SBO. Of these 57 did not have shuntable veins. The median duration from any index symptom to symptomatic PCC was 7 (0-24) years and from index bleed to symptomatic PCC was and 12 (5-24) years, respectively. Thirteen patients underwent endoscopic retrograde cholangiography; nine repeatedly over 7 (4-10) years. Decompensation was seen in 5 patients. Presentation other than variceal bleed was associated with hepatic decompensation (5/19 versus 0/38, P=0.003). Majority of patients with PCC-SBO do not have shuntable veins, and may have good long-term outcomes. Patients presenting with variceal bleed have low chance of decompensation. Symptomatic PCC appears to be a late event in EHPVO.

A study on demographic and clinical profile of children with extra hepatic portal venous obstruction and with special reference to thrombophilic factors

Background: Extra-hepatic portal vein obstruction (EHPVO) due to portal vein thrombosis is an important cause of portal hypertension in several region including India. The cause of thrombosis in these patients remains unclear. Objective of the study was to study the demographic features, etiology, clinical, laboratory findings with special reference to thrombophilic factors like protein C, protein S and antithrombin III deficiency in children with EHPVO.Methods: The prospective analysis of 62 patients of EHPVO (&lt;14 years of age) was done in the Department of Hepatology, SCB medical College, Cuttack. After detailed history, clinical examination, Ultrasound abdomen /color Doppler and Upper GI endoscopy, the subjects were analyzed for any deficiency of thrombophilic factors like protein C, protein S and antithrombin III.Results: A total of 62 patients (37 Male, 25 Female) with mean age of 8.3+3.1 years were studied. Growth retardation was present in the form of wasting (alone) 20.9%, stunting (alone) 25.8% and both wasting and stunting was found in 9.8% cases. History of neonatal, umbilical sepsis and umbilical vein catheterization was found in 15.9% and 10.2% of cases respectively. Haemorrhage from oesophageal varices was prevalent symptoms in 85.9% patients. Splenomegaly was found in 91.9% patients and ascites in 9.4% patients. 47 patients studied for protein C, S and antithromibin III. 14 patients were found to have thrombophilia: protein C deficiency in 9, protein S deficiency in 8, Antithrombin III deficiency in 6.Conclusions: The etiology of EHPVO in the majority of patients remain still unclear. It is commonly associated impaired somatic growth. The risk of EHPVO increases in the presence of thrombophilia, resulting from deficiency of naturally occurring anticoagulant proteins like Protein C, Protein S and Antithrombin III.

2D Shear Wave Elastography of Liver in Patients with Primary Extrahepatic Portal Vein Obstruction

AimsTo evaluate liver stiffness (LS) in patients of primary extrahepatic portal vein obstruction (EHPVO) using 2D shear wave elastography (SWE) and compare it with healthy volunteers. MethodsFifty patients (mean age: 22.4 years) of EHPVO and 25 healthy volunteers were included in the study. Liver function tests and viral markers were done in both groups and endoscopy in EHPVO group, followed by ultrasonography and SWE of liver. Liver elastography was done with patients/volunteers in supine position through right intercostal space. The LS for right lobe of liver was recorded in kilopascals. Three such measurements were taken and the mean of both groups were compared. The variables were also correlated with mean LS using Pearson's correlation coefficient in EHPVO group. ResultsThere was no significant difference in the mean LS in patients of EHPVO (5.96kPa) and healthy volunteers (5.47kPa) (P=0.093). There was no significant correlation between LS with duration of symptoms, hematemesis, esophageal varices, total bilirubin, serum alkaline phosphatase and aspartate aminotranferase levels in EHPVO group. ConclusionSWE of liver may be used as a simple additional tool in the diagnosis of patients of EHPVO who show LS values similar to normal liver.

Splanchnic vein thrombosis and myeloproliferative neoplasms: molecular-driven diagnosis and long-term treatment.

Splanchnic vein thrombosis (SVT) encompasses Budd-Chiari syndrome (BCS), extrahepatic portal vein obstruction (EHPVO), and mesenteric vein thrombosis. Philadelphia-negative myeloproliferative neoplasms (MPNS) are the leading systemic cause of non-cirrhotic and non-malignant SVT and are diagnosed in 40% of BCS patients and one-third of EHPVO patients. In SVT patients the molecular marker JAK2 V617F is detectable up to 87% of those with overt MPN and up to 26% of those without. In the latter, other MPN molecular markers, such as mutations in JAK2 exon 12, CALR and MPL genes, are extremely rare. Immediate anticoagulation with heparin is used to treat acute patients. Upon clinical deterioration, catheter-directed thrombolysis or a transjugular intrahepatic portosystemic shunt is used in conjunction with anticoagulation. Orthotopic liver transplantation is the only reliable option in BCS patients with a lack of a response to other treatments, without contraindication due to MPN. Long-term oral anticoagulation with vitamin K-antagonists (VKA) is recommended in all SVT patients with the MPN-related permanent prothrombotic state; the benefits of adding aspirin to VKA are uncertain. Cytoreduction is warranted in all SVT patients with an overt MPN, but its appropriateness is doubtful in those with molecular MPN without hypercythaemia.

High patency of proximal splenorenal shunt: A myth or reality ? – A prospective cohort study

BackgroundProximal splenorenal shunt (PSRS) is a well-accepted surgical procedure for non-cirrhotic portal hypertension (NCPH). Though a patent shunt is important for good long term outcome, there are very few studies on patency of these shunts. We analysed shunt patency using dynamic computed tomographic (CT) portography and compared it with other modalities. MethodsFrom 2004 to 2014, 50 patients with PSRS were evaluated prospectively for shunt patency using dynamic CT portography, clinical parameters and ultrasound Doppler. ResultsThe causes of NCPH were extrahepatic portal vein obstruction (EHPVO) in 38 patients and non-cirrhotic portal fibrosis (NCPF) in 12 patients. The shunt patency rate using clinical parameters, ultrasound Doppler and dynamic CT portography were 70%, 40% and 60% respectively. Clinical parameters overestimated while ultrasound Doppler underestimated the shunt patency rate. Dynamic portography had 100% correlation with conventional angiography in the five patients when this was done. The site of shunt could be demonstrated convincingly by dynamic CT portography. The shunt patency rate decreased over time. It was 64%, 60% and 43% in <1 year, 1–5 years and >5 years respectively. Our NCPF patients had a greater shunt patency rate compared to EHPVO patients (9/12 vs. 21/38) though the difference was not significant. Only size of the splenic vein had a significant impact on the shunt patency rate on statistical analysis. ConclusionsDynamic CT portography is useful for evaluation of shunt patency. Proximal splenorenal shunts have a high blockage rate which has hitherto not been reported.

Well-tolerated portal hypertension and favorable prognosis in adult patients with extrahepatic portal vein obstruction in Japan.

To evaluate the clinical features and prognoses in adult patients with extrahepatic portal vein obstruction (EHO) from the aspect of portal hypertension during the last 20years in Japan. There were 40 EHO patients (aged 21-77years; mean ± standard deviation [SD], 54.6 ± 15.0). Clinical findings and prognoses were examined retrospectively during the median observation period of 71.6months. Twenty-two patients (55%) showed positive signs of portal hypertension; 18 with esophageal varices (F0, one; F1, eight; F2, nine), two with gastric varices (F1, one; F2, one) and seven with mild ascites. Multivariate analysis showed that platelet count and spleen size were significant factors for the presence of gastroesophageal varices, with odds ratios of 0.989 (95% confidence interval [CI], 0.980-0.997; P = 0.011) for platelet count and 1.003 (95% CI, 1.001-1.005; P = 0.003) for spleen size. Ten of 20 patients with gastroesophageal varices received primary prophylaxis and only one patient (10%) showed variceal recurrence. The cumulative overall survival rate was 100% at 1year, 94.2% at 3-7years and 68.7% at 10years. The cumulative survival rates did not differ between the patients with and without gastroesophageal varices, with and without ascites, and patterns of portal cavernoma at baseline. Forty-five percent of adult EHO patients in Japan were free from signs of portal hypertension, and platelet count and spleen size are predictive for identifying patients with gastroesophageal varices. EHO patients with gastroesophageal varices show favorable prognoses comparable to those without, if primary/secondary prophylaxis was performed appropriately.

Extra-hepatic portal vein obstruction in children is not associated with growth impairment

Purpose – The purpose of this paper is to assess nutritional status among children with extra-hepatic portal vein obstruction (EHPVO). Design/methodology/approach – Forty six children with documented EHPVO with age and sex matched siblings and controls were selected from the Department of Gastroenterology, Sawai Man Singh Hospital, Jaipur. Nutritional assessment was done by anthropometry and dietary recall method (24-h dietary recall method). Height for age and BMI for age were compared with WHO standards. Findings – An equal percentage of EHPVO patients, siblings and controls (4.34 percent) were stunted. Nearly 23.9 percent EHPVO patients and their siblings and 30.4 percent of controls were severely wasted. Similar proportion of subjects had MAMA&lt;5th percentile. Dietary intake was similar between the study groups. Growth velocity was similar among the groups after one-year follow-up. Originality/value – Data from India regarding EHPVO are very scarce from India. One study from Sarin et al. in 1992 reported EHPVO-affected children to be growth retarded. The present study, in contradiction to this study postulates that the affected children may not be growth retarded because of the disease but heredity and environmental factors may play a role.

Revisiting Extra Hepatic Portal Vein Obstruction in Children from the North Indian Gangetic Plain

To study the demographic features, etiology, clinical, radiology and laboratory findings in children with Extra hepatic portal vein obstruction (EHPVO) from the North Indian Gangetic Plain. A prospective analysis of 53 patients of EHPVO (<14y of age) was done. Data for clinical presentation, laboratory workup, endoscopic procedures, growth and development, management and outcome were analyzed. A total of 53 patients (32 male, 21 female) with mean age of 8.66 ± 3.32 y at presentation were included. Growth retardation was present in 30 (56.60%) patients at time of diagnosis. The most common presenting symptoms were upper gastrointestinal bleeding (86.80%) and an incidental finding of splenomegaly (13.2%). Hematological parameters of hypersplenism were present in 9 (17%) patients. Protein C (PC), Protein S (PS), antithrombin III (AT) deficiency were found in 8 (20%), 4 (10%) and 6 (15%) of the patients respectively in 40 patients tested. On first endoscopy, esophageal varices were present in all patients. Endoscopic sclerotherapy (EST)/band ligation (EBL) was performed in 46 bleeder patients and success rate was 83.3% for sclerotherapy and 90% for band ligation. The etiology of EHPVO in the majority of patients remains elusive. It results in impaired somatic growth. Sclerotherapy and endoscopic banding are effective means for treatment for bleeding varices. It is still not clear whether deficiency of anticoagulant proteins is a primary event or secondary to disease process.