Extrahepatic Portal Venous Obstruction Research Articles

A clinico-etiological profile of adult extrahepatic portal venous obstruction in patients from Northern India. Is it a novel subset of occult extrahepatic portal vein obstruction?

Background: Extrahepatic portal vein obstruction (EHPVO) is caused by occlusion and cavernomatous transformation of the portal vein. The EHPVO is well characterized in children. However, the same is not valid for adults. We aimed to evaluate the clinico-etiological profile along with the management of adult patients. Aims and Objectives: We evaluated the clinico-etiological profile along with the management and outcomes of adult patients presenting with EHPVO. Materials and Methods: In a descriptive, observational study, patients between ages 15 and 75 years diagnosed with EHPVO on ultrasonography abdomen were included in the study. Liver cirrhosis and malignancy were considered as an exclusion criterion. There was evaluation of clinical and imaging findings along with biochemical analysis, workup for thrombophilia, treatment given to the patients, and follow-up. Results: Out of total 109 patients enrolled in the study, the median age of patients was ±40.25. Main clinical features were dyspepsia, abdominal discomfort, and splenomegaly. Out of 109, 27.5% patients showed fatty liver and 5.5% had both splanchnic vein thrombosis and fatty liver. Idiopathic EHPVO in majority of patients (80%), adult EHPVO with polycythemia rubra vera in 7.33% patients, adult EHPVO with heterozygous MTHFR mutation in 7.33% patients, and adult EHPVO with antiphospholipid antibody in 3.66%. No mortality was seen in this 5-year observational study and majority of the patients, i.e., 96 (88.1%) did not require any treatment. Conclusion: Relatively benign nature of adult EHPVO was found in the selected group of patients. Majority of the cases in our study were idiopathic and few had positive thrombophilia profile.

Effectiveness of anticoagulant therapy using heparin combined with Plavix after Rex shunt.

Rex shunt is an optimal surgery for the treatment of extra-hepatic portal venous obstruction (EHPVO) in children. Anticoagulant therapy has been used to keep the patency of the bypass vein in the Rex shunt. This study was to investigate the effectiveness of anticoagulant therapy using heparin combined with Plavix in improving the prognosis and shunt patency of Rex shunt. From January 2010 to September 2019, 51 children with EHPVO underwent a portal cavernoma- Rex shunt. Based on whether using the anticoagulant therapy after the Rex shunt, all patients were divided into two groups: the anticoagulant group and the non-anticoagulant group. The diameter and flow velocity of the bypass vein were measured by the post-operative ultrasound, which was used to calculate the flow volume of the bypass vein (FV) and standard portal venous flow (SPVF). The bypass venous flow index (BVFI) was used to evaluate the ability of portal blood into the liver through the bypass vein after the Rex shunt, which was a ratio of FV to SPVF. The incidence of post-operative re-bleeding, the postoperative patency rate of the bypass vein, the remission rate of postoperative hypersplenism, the remission rate of postoperative esophagogastric varices and the BVFI were compared between the two groups. Of the 51 patients, 12 patients in the anticoagulant group were treated with heparin combined with Plavix after Rex shunt; 39 patients in the non-anticoagulant group were not treated with any anticoagulant therapy. 8 of 51 patients suffered from postoperative re-bleeding, of whom 6 patients with thrombosis of the bypass vein and 2 patients with anastomotic stenosis of the bypass vein. All 8 patients with re-bleeding belonged to the non-anticoagulant group. The remission rate of hypersplenism was no significant difference between the two groups after surgery (91% vs. 58%, P = 0.100). However, 3 patients without hypersplenism before surgery suffered from hypersplenism after surgery, who belonged to the non-anticoagulant group. There was no significant difference in the remission rate of esophagogastric varices (33% vs. 46%, P = 1.000). The BVFI of the anticoagulant group was significantly higher than that of the non-anticoagulant group (5.71 ± 5.89 vs. 1.1 ± 1.52, P = 0.003). Anticoagulant therapy using heparin combined with Plavix plays an important role in maintaining the patency of the bypass vein, which improved the portal blood flow into the liver through the bypass vein after the Rex shunt.

Prevalence of minimal hepatic encephalopathy in extra hepatic portal venous obstruction patients following proximal splenorenal shunt surgery

Prevalence of minimal hepatic encephalopathy in extra hepatic portal venous obstruction patients following proximal splenorenal shunt surgery

CT Arterioportography for Complex Portomesenteric Venous Interventions

AbstractAuthors report the use of computed tomography arterioportography (CTAP) in the diagnosis and endovascular treatment of portomesenteric venous occlusion. Three patients (2 male and 1 female; median age: 51 years) with an extrahepatic portal or mesenteric venous occlusion and variceal bleeding were included in this institutional review boards-approved retrospective study. CTAP was performed with an angiographic catheter positioned in the proximal superior mesenteric artery, and CT was obtained during the venous phase following the intra-arterial injection of contrast material. CTAP provided excellent opacification and delineation of portomesenteric veins, including occlusion length, intravascular webs, venous collaterals, and bleeding varices. This enabled preoperative planning for complex recanalization or extra-anatomic bypass to treat extrahepatic portal and mesenteric venous obstruction. There were no complications related to CTAP.

A History of Umbilical Vein Catheterization Does Not Preclude Children from a Successful Meso-Rex Bypass.

Umbilical vein catheterization (UVC) can cause portal venous thrombosis, leading to the development of extrahepatic portal venous obstruction (EHPVO) and portal hypertension (PHT). The feasibility of the Meso-Rex bypass (MRB) for the treatment of EHPVO in patients with a history of UVC has been questioned. We compared the feasibility of performing an MRB in patients with or without a history of previous UVC. A retrospective review of patients with EHPVO and known UVC status explored for a possible MRB at our institution was performed (1997-2022). Patients were categorized in two groups: with (UVC(+)) or without (UVC(-)) a history of UVC for comparison. A p-value less than 0.05 was considered significant. One hundred and eighty-seven patients were included (n = 57 in UVC(+); n = 130 in UVC(-)). Patients in the UVC group were significantly younger at surgery and the incidence of prematurity was higher. Other risk factors for the development of EHPVO were similar between the groups, but only history of UVC could predict the ability to receive MRB (odds ratio [OR]: 7.4 [3.5-15.4]; p < 0.001). The success rate of MRB was significantly higher in patients with no history of UVC (28/57 [49.1%] in UVC(+) vs. 114/130 [87.7%] in UVC(-); p < 0.001). However, MRB patency at discharge (25/28 [89.3%] in UVC(+) vs. 106/114 [94.7%] in UVC(-); p = 0.3) was equally high in both groups. Our results indicate that a history of UVC is not a contraindication to MRB. Half of the patients were able to successfully receive an MRB. Patients with symptomatic PHT from EHPVO should not be excluded from consideration for MRB based on UVC history.

Surgical outcome of extrahepatic portal venous obstruction: Audit from a tertiary referral centre in Eastern India.

Extra hepatic portal venous obstruction (EHPVO) is the most common cause of portal hypertension in Indian children. While endoscopy is the primary modality of management, a subset of patients require surgery. This study aims to report the short- and long-term outcomes of EHPVO patients managed surgically. All the patients with EHPVO who underwent surgery between August 2007 and December 2021 were retrospectively reviewed. Postoperative complications were classified after Clavien-Dindo. Binary logistic regression in Wald methodology was used to determine the predictive factors responsible for unfavourable outcome. Total of 202 patients with EHPVO were operated. Mean age of patients was 20.30 ± 9.96 years, and duration of illness, 90.05 ± 75.13 months. Most common indication for surgery was portal biliopathy (n = 59, 29.2%), followed by bleeding (n = 50, 24.8%). Total of 166 patients (82.2%) had shunt procedure. Splenectomy with esophagogastric devascularization was the second most common surgery (n = 20, 9.9%). Nine major postoperative complications (Clavien-Dindo > 3) were observed in 8 patients (4.0%), including 1 (0.5%) operative death. After a median follow-up of 56 months (15-156 months), 166 patients (82.2%) had favourable outcome. In multivariate analysis, associated splenic artery aneurysm (p = 0.007), isolated gastric varices (p = 0.004), preoperative endoscopic retrograde cholangiography and stenting (p = 0.015), and shunt occlusion (p < 0.001) were independent predictors of unfavourable long-term outcome. Surgery in EHPVO is safe, affords excellent short- and long-term outcome in patients with symptomatic EHPVO, and may be considered for secondary prophylaxis.

Ascites and Chronic Liver Disease in Children.

Development of ascites in children with chronic liver disease is the most common form of decompensation. It is associated with a poor prognosis and increased risk of mortality. A diagnostic paracentesis should be performed in liver disease patients with- new-onset ascites, at the beginning of each hospital admission and when ascitic fluid infection (AFI) is suspected. The routine analysis includes cell count with differential, bacterial culture, ascitic fluid total protein and albumin. A serum albumin-ascitic fluid albumin gradient of ≥1.1g/dL confirms the diagnosis of portal hypertension. Ascites has been reported in children with non-cirrhotic liver disease like acute viral hepatitis, acute liver failure and extrahepatic portal venous obstruction. The main steps in management of cirrhotic ascites include dietary sodium restriction, diuretics and large-volume paracentesis. Sodium should be restricted to maximum of 2mEq/kg/d (max 90mEq/d) of sodium/day. Oral diuretic therapy comprises of aldosterone antagonists (e.g., spironolactone) with or without loop-diuretics (e.g., furosemide). Once the ascites is mobilized, the diuretics should be gradually tapered to the minimum effective dosage. Tense ascites should be managed with a large-volume paracentesis (LVP) preferably with albumin infusion. Therapeutic options for refractory ascites include recurrent LVP, transjugular intrahepatic porto-systemic shunt and liver transplantation. AFI (fluid neutrophil count ≥250/mm3) is an important complication, and requires prompt antibiotic therapy. Hyponatremia, acute kidney injury, hepatic hydrothorax and hernias are the other complications.

Portosystemic Shunt Surgery for Extrahepatic Portal Venous Obstruction Beyond Endoscopic Variceal Eradication: Two Decades of Pediatric Surgical Experience

This exclusively surgical series on pediatric extrahepatic portal venous obstruction (EHPVO) defines surgical indications beyond endoscopic eradication of esophageal varices (EEEV), the selection of an appropriate surgical procedure, and the long-term post-surgical outcome. EHPVO management protocol at the reporting institute has been endotherapy untilesophageal variceal eradication and surgery for select adverse sequelae manifesting after EEEV. One hundred and thirty-nine EHPVO cases underwent surgery for the following indications in combination: i) massive splenomegaly with severe hypersplenism (n=132, 95%); ii) growth retardation (GR, n=95, 68%); iii) isolated gastric (IGV) and ectopic varices (n=49, 35%); iv) Portal cavernoma cholangiopathy (PCC) (n=07, 5%). A portosystemic shunt (PSS) was performed in 119 (86%) cases. Types of PSS performed were as follows: central end-to-side splenorenal shunt with splenectomy (n=104); side-to-side splenorenal shunt (n=4); mesocaval shunt (n=1); inferior mesenteric vein (IMV) to left renal vein shunt (n=2); IMV to inferior vena cava shunt (n=3); H-graft interposition splenorenal shunt (n=1); spleno-adrenal shunt (n=3); makeshift shunt (n=1). Esophagogastric devascularization (n=20, 14%) was opted for only for non-shuntable anatomy. At a median follow-up (FU) of 41 (range: 6-228) months, PSS block was detected in 13 (11%) cases, with recurrent variceal bleeding in 4 cases. PCC-related cholestasis regressed in 5 of 7 cases. Issues of splenomegaly were resolved, and growth z-scores improved significantly. Endotherapy for secondary prophylaxis untilEEEV has resulted in a shift in surgical indications for EHPVO. Beyond EEEV, surgery was indicated predominantly for non-variceal sequelae, namely massive splenomegaly with severe hypersplenism, GR, and PCC. Varices warranted surgery infrequently butmore often from sites less amenable to endotherapy, i.e., IGV and ectopic varices. The selection of PSS was tailored to anatomy and surgical indications. On long-term FU post surgery, PSS block was detected in 13% of patients. PCC-related cholestasis regressed in 71%, and issues of splenomegaly resolved with significantly improved growth Z scores.

Effectiveness of Rex shunt for improving the abnormal portal hemodynamics and portal venous pathology in EHPVO animal model.

To investigate the feasibility of the re-patent EHPVO (r-EHPVO) as an animal model of Rex shunt and the effectiveness of Rex shunt in improving abnormal portal hemodynamics and portal venous pathology of EHPVO. A total of 18 New Zealand white rabbits were randomly divided into three groups: normal control (NC) group, extrahepatic portal venous obstruction (EHPVO) group, and r-EHPVO group. The main portal vein was dissected only in the NC group. The main portal vein was narrowed by a cannula in the EHPVO group. The cannula narrowing the main portal vein was removed to restore the portal blood flow into the liver on day 14 in the r-EHPVO group. The portal pressure, splenic size, blood flow velocity, and diameter of the portal vein were measured on days 14 and 28. The shear stress (SS) and circumferential stress (CS) of the portal vein were calculated. The proximal end of the main portal vein was collected on day 28 for further pathological analysis, and the thickness and area of the intima and media were measured by Image J software. The portal pressure, splenic size, SS, CS, intima and media thickness, the ratio of intimal to medial area (I/M), and the ratio of intimal area to the sum of intimal and medial area (I/I + M) were compared among the three groups. The correlation between SS and intimal thickness and between CS and medial thickness were analyzed. On day 28, the portal pressure of the EHPVO group was significantly higher than that of the NC and r-EHPVO groups, but no significant difference was detected in the portal pressure between r-EHPVO and NC groups. The length and thickness of the spleen in the EHPVO and r-EHPVO groups were significantly higher than those in the NC group (P < 0.01) but were significantly lower in the r-EHPVO group than those in the EHPVO group (P < 0.05). The SS was significantly lower in the EHPVO group than in NC and r-EHPVO groups (P < 0.05) but was significantly higher in the NC group than in the r-EHPVO group (P = 0.003). The CS was significantly higher in the EHPVO and r-EHPVO groups than that in the NC group (P < 0.05) but was significantly lower in the r-EHPVO group than that in the EHPVO group (P < 0.001). The intimal thickness, I/M, and I/I + M of the EHPVO group were significantly higher than those of the NC and r-EHPVO groups (P < 0.05), but no significant difference was observed between the NC and r-EHPVO groups (P > 0.05). The SS is negatively related to intimal thickness (r = - 0.799, P < 0.001). The r-EHPVO model is feasible as an animal model of the Rex shunt. The Rex shunt could be beneficial to improving the abnormal portal hemodynamic and portal venous intimal hyperplasia by restoring the portal blood flow into the liver.

Imaging Approach to Portal Hypertension

Abstract Increase in portal venous pressure (termed portal hypertension) is seen in a variety of liver diseases. Imaging tests are useful to detect portal hypertension and identify its cause. Noninvasive tests like abdominal ultrasound and Doppler studies are routinely done in clinical practice for this indication. Cross-sectional studies like computed tomography and magnetic resonance imaging are especially useful to delineate morphological abnormalities in the liver. Invasive tests like assessment of hepatic venous pressure gradient are done less frequently for specific indications. Distinctive imaging findings help differentiate the different causes of portal hypertension like cirrhosis and vascular liver disorders like noncirrhotic portal hypertension, extrahepatic portal venous obstruction, and Budd–Chiari syndrome. Radiological interventions are increasingly used to treat complications of portal hypertension like refractory ascites or refractory bleeding from gastroesophageal varices.

Bibliometric Analysis and Visualization of the Extrahepatic Portal Venous Obstruction Publication Landscape.

A scientometric analysis was conducted to characterize the global research publications in extrahepatic portal venous obstruction (EHPVO), and state-of-the-art visualization graphics were generated to provide insight into specific bibliometric variables. The Web of Science database was accessed for research productivity and bibliometric variables of countries, institutions, authors, journals, and content analysis of top-20 cited documents were performed. Collaborative networks and co-occurrence of keywords map were generated using VOSviewer software. Two hundred and sixteen records were retrieved with an annual growth rate of 2.53%. India is the leading country in productivity (n = 4339), followed by the USA and China. Post Graduate Institute of Medical Education and Research, Chandigarh, was the top productive institute. Sarin SK was the most prolific author, having the highest citations received and h-index. The hotspot topics were "portal hypertension," "cirrhosis," "children," "biliopathy/cholangiopathy," "liver fibrosis," and "liver transplantation" as per keyword co-occurrence networking. J Gastroenterol Hepatol had the most publications of EHPVO research as well the h-index. Regarding collaborative network mapping, the USA and Primignani M were the significant nodes among country and author, respectively. EHPVO research publication volume is low but is gradually progressing with dominant contributions from Indian institutes and authors. Most highly cited articles are of low level of evidence, and multi-institutional collaborative research can be the way forward.

Assessment of Rex vein patency in children with Extra hepatic Portal Venous Obstruction

Assessment of Rex vein patency in children with Extra hepatic Portal Venous Obstruction

Extrahepatic portal vein occlusion presenting with vascular, biliary, and visceral complications evaluated on multidetector computed tomography

Extrahepatic portal venous obstruction, predominantly a disease of childhood, is a chronic and long-standing disease characterized by thrombosis of the portal vein, leading to multiple collateral formation and other associated complications owing to long-standing nature of the disease. We present the case of a 22-year-old male with symptoms and signs of gastrointestinal bleed and pain in the left upper abdomen with similar episodes in the past.

S3305 A Unique Case of Extra Hepatic Portal Venous Obstruction Managed by Meso-Rex Shunt

S3305 A Unique Case of Extra Hepatic Portal Venous Obstruction Managed by Meso-Rex Shunt

S1476 Efficacy and Clinical Outcomes of Endoscopic Variceal Ligation With N-butyl-2-cyanoacrylate in Pediatric Patients in a Tertiary Care Center—A Retrospective Study

Introduction: Acute variceal bleeding (AVB) from gastroesophageal varices is a life-threatening complication in patients with portal hypertension. Bleeding from gastric varices is less common and treatment options are not standardized. Endoscopic variceal obliteration (EVO) with N-butyl-2-cyanoacrylate glue or balloon-occluded retrograde transvenous obliteration (BRTO) are the 2 most common treatment modalities and information on these are limited to adult series. There is paucity of information about gastric varices and its treatment in children. Methods: We conducted a retrospective cohort study among pediatric patients (1-18 years) who presented to our center with AVB from gastric varices. Between 2011-2021, we identified 538 among 4539 cases (12%) of portal hypertension with bleeding from gastric varices. Of these only 14 cases (2.6%) had a diagnosis of gastric variceal bleeding in children. Details of 13 patients were extracted with non-availability of records of 1 patient. Cyanoacrylate glue in 0.5-1 ml were injected in to the gastric varices. Patients were evaluated for efficacy, safety and complications associated with the treatment. Endoscopy was repeated after 3 weeks for assessment of variceal size and need for repeat glue injection. Results: The mean age of the patients was 13.3±5.2, with males (54%) and females (46%). The mean age at first presentation to hospital with gastrointestinal bleeding was 8.7±3.6, with hematemesis (62%) being the most common presentation. On endoscopy, 8 patients had type 1 gastro-esophageal varices and 3 patients had type 2 gastroesophageal varices. All 13 patients had stigmata of recent gastric variceal bleeding. The most common cause of portal hypertension was extra hepatic portal venous obstruction (EHPVO) (54%). The mean age at glue therapy treatment was 11.3±5.3. While immediate hemostasis was achieved in all 13 patients (100%) with gastric variceal bleeding, re-bleeding was seen among 3 patients, which occurred after 1 month of glue therapy. 1-3 sessions were required for obliteration of gastric varices. There was one case of death among the 13 children: a patient with decompensated Wilson’s disease. We did not encounter complications like anaphylactic shock, treatment-associated infection, gastric perforation, and distant emboli. Conclusion: The experience from our series suggests that endoscopic vascular obliteration using cyanoacrylate glue injection is safe and effective in children with gastric variceal bleeding.

S1836 Six Years of Evolution With Portal Cavernomatosis and Portal Biliopathy

Introduction: In patients with Portal Biliopathy (PB), It is paramount to engage several branches of gastroenterology to interject the aggressive evolution of this condition. Case Description/Methods: Our patient is a Man of 45 years old. Medical history of pancreatic divisum, chronic pancreatitis, biliary litho extraction, cholecystectomy, use of endoprosthesis, and pancreatic conduct stenosis. On CT scans in February 2015 he presented a pancreatitis Baltazar E, generating a perfusion defect, and multiple collaterals, in Abril 2015 portal cavernomatosis, and by January 2018 the collateral veins provoked biliary ectasis. During the next 2 years, he presented two events of mild cholangitis. To solve the choledochal impingement, on October 8, 2019, it took place a portal vein angioplasty with a balloon. The direct extrahepatic portal vein pressure was 30 mmHg pre-plasty, diminishing to 22 mmHg post-plasty. Posterior to the intervention, he had a new severe healthcare-associated colangitis plus acute pancreatitis, with a 7 mm dilatation only in the intrahepatic biliary pathway. To improve the biliary stasis, by CPRE it was placed a metallic stent covered with a Wallflex 10 mm x 8 cm. By February 2020, a CT scan still showed a permeable porta in the confluence, yet the portal prosthesis was not visible. Finally in September 2021, without clinic traits of portal hypertension, events of cholestasis, and improvement in hepatic function. (Figure) Discussion: In the biliary tract, venous drainage passes through two venous plexus named, the epicholedochal venous plexus of Saint and the paracholedochal venous plexus of Petren. In portal biliopathy, changes are a result of dilatation of veins of the plexus of Saint, causing bile duct impingement. It has been hypothesized that this lead to fibrous scarring of porta hepatis, and ischemic injury to the bile ducts resulting in stricture formation and caliber irregularity with upstream dilatation. Several studies have shown that bile duct changes occur in 81–100% of patients with extrahepatic portal vein obstruction, but only 5–30% of patients present symptoms of biliary obstruction. In adults conditions such as thrombophilia, intra-abdominal infection, malignancy, myeloproliferative disorders and cirrhosis have been implicated PB is mainly a feature of extrahepatic portal venous obstruction, patients presenting with symptoms require an integrated management addressing biliary obstruction and portal hypertension in tandem. However, the relief of both conditions is only temporary.Figure 1.: Top. Initial axial CT scan following the known event of acute pancreatitis, showing acute splenic-portal venous thrombosis demonstrated by unenhanced and distended portal vein (yellow arrow in a). The middle image three months later shows the formation of small collateral vessels in the hepatic hilium, (yellow arrow in b), and also notes new-onset ascites due to impaired liver function. Coronal reformat image two years later, points to the focal area of chronic portal thrombosis, yellow arrow in (c) with more prominent appearance collateral vessels “cavernomatosis” (asterisk), several perfusion defects in the liver parenchyma, and marked splenomegaly. Middle. DSA images, in (a) transhepatic percutaneous venography with opacification of intrahepatic portal vasculature with a sudden interruption in the site of thrombosis (yellow arrow in a). (b) splenic approach with opacification of the extrahepatic portal vein and aneurismatic collateral vessels due to cavernomatosis and upstream thrombotic interruption (yellow arrow in b). (c) final imagen after portal vein angioplasty demonstrates adequate and complete opacification of the portal vein and its branches. Bottom. ERCP in (a) shows intrahepatic bile duct dilatation and impingement and (b) after stent placement with diminished dilatation.

Portal cavernoma cholangiopathy: Update and recommendations on diagnosis and management.

Portal cavernoma cholangiopathy is defined as an obstruction of the biliary system due to distended veins surrounding bile ducts that mainly occur in patients with extrahepatic portal venous obstruction. The periductal venous plexuses encircling the ducts can cause morphological changes which may or may not become symptomatic. Currently, non-invasive techniques such as ultrasonography, computed tomography, magnetic resonance cholangiopancreatography, and dynamic contrast enhanced magnetic resonance images are being used to diagnose this disorder. Only a few patients who have symptoms of biliary obstruction require drainage which might be accomplished using endoscopic stenting, decompression of the portal venous system usually via a lienorenal shunt, a difficult direct hepaticojejunostomy, and rarely a liver transplant.

OUTCOMES OF PROXIMAL SPLENORENAL SHUNT SURGERY IN EXTRA HEPATIC PORTAL VEIN OBSTRUCTION (EHPVO) – EXPERIENCE FROM A TERTIARY CENTER IN GUJARAT, INDIA.

Background – Extra hepatic portal vein obstruction commonly presents with variceal bleeding and splenomegaly. Management with endoscopic means provide temporary palliation. A proximal splenorenal shunt is a denitive procedure with good results. Our aim was to evaluate the outcomes of splenectomy with proximal splenorenal shunt in patients with extrahepatic portal venous obstruction. Methods – The records of all patients with diagnosis of EHPVO who were operated for splenectomy with proximal splenorenal shunt during the period from January 2014 to august 2018 were analysed. Total of 34 patients with EHPVO were operated for splenectomy with proximal splenorenal shunt over the study period. Outcomes were evaluated in term of rebleeding, variceal regression shunt thrombosis and post shunt encephalopathy. Results – All the patients were below 30 years age. Shunt surgery lead to reversal of various degree of cytopenias and hypersplenism. Variceal regression was seen from large size (grade III and IV) to small size (grade II) in 74% of patients. We had low incidence of shunt block (5.88%), variceal rebleeding (2.94%) and no mortality. None of the patients developed encephalopathy during follow-up. Conclusion- Splenectomy with proximal splenorenal shunt is a single time safe procedure, with low morbidity and mortality; having low rates of variceal rebleeding and encephalopathy; with low cost in teaching hospital, thus can be considered as the main treatment approach in patients with EHPVO.

Rex Shunt for Extra-Hepatic Portal Venous Obstruction in Children

Rex shunt, which was first put in use in 1992, has been considered as an ideal surgical method for the treatment of extra-hepatic portal venous obstruction (EHPVO) due to its reconstruction of the hepatopetal portal blood flow. However, despite its long tradition, there are only a few reports about the application and advances in Rex shunt for the treatment of EHPVO in children. In this paper, we summarized the literature related to Rex shunt and discussed the new advances of Rex shunt in the following aspects: surgical method of Rex shunt, the indications of Rex shunt, the strengths of Rex shunt, the effectiveness of Rex shunt, factors affecting the efficacy of Rex shunt, methods that improve the prognosis of Rex shunt, and treatment strategy for recurrence after Rex shunt.

Ultrasound and Computed Tomography Features of Non Cirrhotic Portal Hypertension caused due to Non Cirrhotic Portal Fibrosis and Extrahepatic Portal Vein Obstruction: A Retrospective Study

Introduction: Non Cirrhotic Portal Hypertension (NCPH) encompasses a wide range of disorders that are characterised by increased portal pressure with the absence of cirrhosis of liver. The disorders are classified anatomically based on the site of blood flow obstruction into prehepatic, hepatic and posthepatic causes. These numerous and variable aetiological factors and the lack of standardised diagnostic criteria, makes NCPH under-recognised both clinically and pathologically and often being falsely labelled as cryptogenic cirrhosis. Therefore, it is important for radiologists to be aware of the imaging features that constitute NCPH for its early recognition, so that appropriate management can be carried out. Aim: To assess the distinct ultrasound and Computed Tomography (CT) features of the two main causes of NCPH: Non Cirrhotic Portal Fibrosis (NCPF) and Extrahepatic Portal Venous Obstruction (EHPVO). Materials and Methods: This retrospective observational study included all radiologically diagnosed cases of NCPH who underwent ultrasound and CT examination at the Institution between June 2020-June 2021. A total of 10 patients met the inclusion criteria. The various imaging features of NCPF and EHPVO were analysed and tabulated. Results: The study showed male predominance with males and females being seven and three, respectively. The mean age of the patients in the study was 35.1 years. The common cause in both NCPF and EHPVO was idiopathic. The most characteristic imaging finding in EHPVO was cavernous transformation of the portal vein seen in all cases and that in NCPF being dilatation of the spleno-portal axis and mural thickening of the main portal vein seen in all the cases. Conclusion: The present study demonstrated the characteristic imaging features of NCPF and EHPVO which are leading cause of NCPH, facilitating radiologist to identify the conditions and not label cases with Portal Hypertension (PHT) as cryptogenic cirrhosis.