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

Purpose: 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. Methods: 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. Results: 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). Conclusions: 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.

Successful management of portal hypertension with splenomegaly and cavernous transformation of the portal vein: a rare case report.

Portal hypertension, often stemming from liver cirrhosis or vascular anomalies, can result in cavernous transformation of the portal vein, a rare condition associated with biliary obstruction, variceal hemorrhage, and splenomegaly. This case report details a unique occurrence of portal hypertension, splenomegaly, and cavernous transformation of the portal vein successfully managed through splenectomy and spleno-renal shunt. A 30-year-old female with a history of portal hypertension, portal gastropathy, and splenomegaly presented with left upper quadrant abdominal pain. She had previously undergone esophageal variceal ligation and required intermittent blood transfusions. Additional complications included easy bruising, heavy menstrual bleeding, and a prior episode of hematemesis. Clinical assessment confirmed splenomegaly, while a CT scan confirmed the diagnosis. A tailored surgical approach was chosen, leading to splenectomy and spleno-renal shunt.

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

Introduction: 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. Materials and Methods: 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. Results: 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. Conclusion: 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.

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.

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.

Safety of endoscopic N-Butyl-2 Cyanoacrylate injection for the treatment of bleeding gastric varices in children.

OBJECTIVE: To determine the safety and efficacy of N-butyl 2-cyanoacrylate in bleeding gastric varices in children. METHODS: This retrospective observational study was conducted in the Department of Gastroenterology and Pediatric Surgery in Liaquat National Hospital Karachi between January 2010 and January 2017. Gastric fundal varices were obliterated in pediatric population with single shot of N-butyl-2 Cyanoacrylate 0.50ml diluted with 0.50ml of Lipoidal with use of forward-viewing video endoscope with 22-gauge needle. The primary outcome was primary hemostasis, Secondary outcome was complications, re-bleeding and mortality. RESULTS: Thirty patients was included in the study, 18(60%) were male with mean age of 7.12± 2.9 years. Non cirrhotic portal hypertension was the most common etiology in 15 (50%) patients, followed by liver cirrhosis secondary to hepatitis B and D co-infection in 6(20%) patients. Primary hemostasis was achieved in 29 (96.7%), while 3(10%) patients developed re-bleeding after 48 hours, and hemostasis was achieved after second session of endoscopic obliteration. Abdominal pain and fever developed in 3(10%) patients which was managed conservatively. Mortality was observed in 1(3%) of cases due to sepsis after shunt surgery. CONCLUSION: Endoscopic fundal varix obliteration with N Butyl-2 cyanoacrylate was safe and effective in treatment of gastric variceal hemorrhage in children.

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.

AIMS: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

Portal vein thrombosis.

Portal vein thrombosis is an important cause of portal hypertension. PVT occurs in association with cirrhosis or as a result of malignant invasion by hepatocellular carcinoma or even in the absence of associated liver disease. With the current research into its genesis, majority now have an underlying prothrombotic state detectable. Endothelial activation and stagnant portal blood flow also contribute to formation of the thrombus. Acute non-cirrhotic PVT, chronic PVT (EHPVO), and portal vein thrombosis in cirrhosis are the three main variants of portal vein thrombosis with varying etiological factors and variability in presentation and management. Procoagulant state should be actively investigated. Anticoagulation is the mainstay of therapy for acute non-cirrhotic PVT, with supporting evidence for its use in cirrhotic population as well. Chronic PVT (EHPVO) on the other hand requires the management of portal hypertension as such and with role for anticoagulation in the setting of underlying prothrombotic state, however data is awaited in those with no underlying prothrombotic states. TIPS and liver transplant may be feasible even in the setting of PVT however proper selection of candidates and type of surgery is warranted. Thrombolysis and thrombectomy have some role. TARE is a new modality for management of HCC with portal vein invasion.

Portal hypertension: A critical appraisal of shunt procedures with emphasis on distal splenorenal shunt in children.

BACKGROUND: Extrahepatic portal venous obstruction (EHPVO) is the most common cause of pediatric portal hypertension. We analyzed the investigative protocol and results of portosystemic shunts in this group of patients. MATERIALS AND METHODS: A total of 40 consecutive children aged below 12 years operated with a diagnosis of extra-hepatic portal hypertension formed the study group. Historical data and clinical data were collected. All patients underwent upper gastrointestinal endoscopy, ultrasound Doppler and computed tomographic portogram pre-operatively and post-operatively. Results with respect to shunt patency, hypersplenism and efficacy of different radiological investigations were collected. RESULTS: A total of 40 patients, 28 boys and 12 girls constituted the study group. Lienorenal shunt (LRS) was performed in 14 patients; distal splenorenal shunt in 21 patients and side-to-side lienorenal shunt in 4 patients, inferior mesenteric renal shunt was performed in 1 patient. Follow-up ranged from 36 to 70 months. At a minimum follow-up of 3 years, 32 (80%) patients were found to have patent shunts. Patent shunts could be visualized in 30/32 patients with computer tomographic portogram (CTP) and 28/32 with ultrasound. Varices regressed completely in 26/32 patients and in the rest incomplete regression was seen. Spleen completely regressed in 19/25 patients. Hypersplenism resolved in all patients with patent shunts. CONCLUSIONS: Portosystemic shunting in children with EHPVO is a viable option. While long-term cure rates are comparable with sclerotherapy, repeated hospital visits are reduced with one time surgery. Pre-operative and post-operative assessment can be performed with complimentary use of ultrasound, CTP and endoscopy.

Endoscopic retrograde cholangiography in portal cavernoma cholangiopathy - results from different studies and proposal for uniform terminology.

Portal cavernoma cholangiopathy (PCC) refers to a constellation of secondary changes in the biliary tree in patients with chronic portal vein (PV) thrombosis and portal cavernoma formation. These findings of PCC are seen in the extra-hepatic bile duct(s), with or without involvement of the 1st or 2nd degree intra-hepatic bile ducts. Of all patients with chronic PV thrombosis, cholangiographic features of PCC are found in 80%-100%. The biliary changes are symptomatic in a smaller proportion of 5%-38% patients. Choledocholithiasis and hepatolithiasis occur in 5%-20%, independent of the occurrence of cholelithiasis. We review the published literature on cholangiographic description of PCC. We also propose standardized nomenclature for the cholangiographic findings, namely: extrinsic impressions/indentations, shallow impressions, irregular ductal contour, stricture (s), upstream dilatation, filling defects, bile duct angulation, and ectasia.

Natural history of portal cavernoma cholangiopathy.

The natural history of portal cavernoma cholangiopathy (PCC) is poorly defined and poorly understood. It develops early after acute portal vein thrombosis (PVT) if there is failure of recanalization. In PCC, the likelihood of progression of biliary abnormalities after 1 year is extremely low. The natural history of PCC is conveniently divided into asymptomatic and symptomatic stages. The majority of patients with PCC are asymptomatic and are detected incidentally on imaging. Limited data suggest that asymptomatic PCC is static or only slowly progressive in the initial stages. However, most workers agree that, overall, PCC is a slowly progressive disease. Symptomatic PCC represents a late stage in its natural history. Finding strictures with dilatation at cholangiography is associated with a higher risk of developing symptoms of PCC. Onset of symptoms is often precipitated by the development of biliary sludge or calculi and treating calculi usually relieves symptoms for prolonged periods of time. Clinical presentations include biliary pain, obstructive jaundice, acute cholangitis, acute cholecystitis, or other presentations of gallstone disease. Progressive liver dysfunction and secondary biliary cirrhosis can develop in a minority of patients.

Hepatic artery Doppler indices in children with extra hepatic portal vein obstruction.

UNLABELLED: Doppler measurement provides information on the hemodynamics in the hepatic artery and the portal venous system. AIM: To study the hepatic artery hemodynamics in children with extra hepatic portal vein obstruction. MATERIALS AND METHODS: Hepatic artery indices were studied using Doppler indices in 15 children (<12 years) with extra hepatic portal hypertension (EHPVO) and obliterated esophageal varices. The hepatic artery resistive index, the arterial acceleration time and the acceleration index were used to determine the flow pattern within the hepatic artery. Controls were 15 healthy age-sex matched children, belonging to the same socioeconomic strata in absence of liver disease. RESULTS: The mean age of the children was 8.43 ± 3.2 years and male female ratio was 4:1. All the children had obliterated esophageal varices. The hepatic artery resistive index in the children with EHPVO was normal and similar to controls. The hepatic arterial early systolic acceleration index was significantly higher in cases compared to controls (436 ± 290 vs 214 ± 100; P value <0.004). The hepatic arterial acceleration time though low in the cases (86 ± 35 cm/s) was not statistically different from the controls (128 ± 14 cm/s). CONCLUSION: There was a significant increase in hepatic arterial early systolic acceleration in children with chronic EHPVO. The latter may be responsible for an increase in hepatic arterial in flow velocity in a slow flow system despite a normal resistive index.

Preditores não invasivos de varizes esofágicas em crianças e adolescentes com hepatopatia crônica ou obstrução extra-hepática da veia porta / Non-invasive predictors of esophageous varices in children and adolescents with chronic liver disease or extrahepatic portal venous obstruction

OBJETIVO: Identificar preditores não invasivos de varizes esofágicas em crianças e adolescentes com hepatopatia crônica ou obstrução extra-hepática da veia porta. PACIENTES E MÉTODOS: Estudo transversal que incluiu 53 crianças e adolescentes com hepatopatia crônica ou obstrução extra-hepática da veia porta, sem antecedente de hemorragia digestiva ou tratamento de varizes esofágicas, com até 20 anos de idade. Dois grupos foram formados: grupo I (35 pacientes com hepatopatia crônica) e grupo II (18 com obstrução extra-hepática da veia porta). Foram realizados hemograma, razão normalizada internacional, albumina, bilirrubina total, ultrassonografia de abdome e endoscopia digestiva alta. O índice esplênico foi determinado dividindo a dimensão esplênica pelo valor do limite superior da normalidade. As variáveis foram comparadas quanto à presença ou não de varizes esofágicas através de análise univariada (testes qui-quadrado, exato de Fischer e de Wilcoxon) e multivariada (regressão logística). A acurácia foi determinada a partir da área sob a curva ROC. RESULTADOS: As varizes esofágicas foram observadas em 48,5% dos pacientes do grupo I e em 83,3% do grupo II. Plaquetopenia (p = 0,0015), esplenomegalia (p = 0,0003) e a razão plaquetas/índice esplênico (p = 0,0007) se mostraram indicadores preditivos de varizes esofágicas entre os pacientes do grupo I. Após análise multivariada, a plaquetopenia (odds ratio = 21,7) se manteve como um indicador independente da presença de varizes esofágicas entre os pacientes com hepatopatia crônica. CONCLUSÃO: O número de plaquetas, o índice esplênico e a razão plaquetas/índice esplênico se mostraram preditivos de varizes esofágicas em crianças e adolescentes com hepatopatia crônica. Não foram encontrados preditores de varizes esofágicas entre os pacientes com obstrução extra-hepática da veia porta.

OBJECTIVE: To identify non-invasive predictors of esophageal varices in children and adolescents with chronic liver disease or extrahepatic portal venous obstruction (EHPVO). METHODS: 53 patients younger than 20 years with chronic liver disease or EHPVO and no history of bleeding or prophylactic treatment of esophageal varices (EV) were assessed. They were divided into 2 groups: group I (35 with chronic liver disease) and group II (18 with EHPVO). Their blood count, international normalized ratio (INR), albumin, bilirubin, abdominal ultrasonography and upper endoscopy results were taken. A splenic index was determined by dividing the patients' spleen dimension by its uppermost limit according to their age. The variables were compared to EV presence or not. Univariate (chi-square test, Fischer's exact test and Wilcoxon exact test) and multivariate (logistic regression) analyses were performed. A receiver operating characteristic (ROC) curve was constructed and the area under the ROC curve was calculated. RESULTS: EV were observed in 48.5% of group I patients and in 83.3% of group II patients. Low platelet count (p = 0.0015), splenomegaly (p = 0.0003) and splenic index (p = 0.0007) were statistically significant predictors of EV among group I patients. The multivariate analysis showed low platelet count (odds = 21.7) as an independent predictor of EV in patients with chronic liver disease. CONCLUSION: Platelet count, splenic index and platelet-splenic index ratio were predictors of EV in children and adolescents with chronic liver disease. There were no EV predictors among group II patients.

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.

Extrahepatic portal venous obstruction: The effects of early ligation of splenic artery during splenectomy.

AIM: To objectively demonstrate the gain in blood volume and blood components following early ligation of splenic artery during splenectomy and splenorenal shunts in children with extra hepatic portal venous obstruction (EHPVO). METHODS: Twenty-eight children (20 males and 8 females, mean age: 9.9 (+/-3.2) years) with EHPVO and hypersplenism were recruited. We followed a protocol of systematically locating and ligating the splenic artery first, followed by a 30-minute waiting period to allow the massive spleen to decongest via the splenic vein and venous collaterals and then completing the splenectomy by standard procedure. No intravenous fluid was administered during this 30-minute period. Blood samples were drawn just prior to splenic artery ligation and soon after splenectomy for the estimation of hematological and biochemical parameters. RESULTS: We noticed a highly significant increase in the hemoglobin, hematocrit, leukocyte, platelet, and RBC counts by early ligation of the splenic artery (p < 0.0004). The gain in hemoglobin and hematocrit was equivalent to a transfusion of atleast 100-150 ml of packed RBC. The increase in platelet count was equivalent to a platelet transfusion of atleast 4 units of platelet concentrates in an adult. There is a positive correlation between the splenic weight and the platelet gain (p= 0.0568) and the splenic volume on preoperative imaging and the platelet gain (p= 0.0251). CONCLUSION: Early ligation of the splenic artery during splenectomy results in passive splenic decongestion and thereby a significant gain in blood components. This protocol appears to be a feasible blood conservation method to avoid blood transfusions in this group of hypersplenic EHPVO patients.