Extrahepatic portal vein obstruction in children

INTRODUCTION AND IMPORTANCE: Extrahepatic portal vein obstruction (EHPVO) with portal hypertension is rare in children. Intestinal varices as new collaterals accompanying portal hypertension are very rare. PRESENTATION OF CASE: We report an unusual case of a 12-year-old boy with EHPVO with gastrointestinal bleeding from ectopic jejunal varices, without any gastroesophageal varices. DISCUSSION: Portal hypertension is the most common cause of EHPVO...

Extrahepatic portal vein obstruction (EHPVO) is a major cause of portal hypertension (PH) in children. Portal vein thrombosis (PVT) is the most common cause accounting for up to 75% of cases in developing countries. Upper gastrointestinal bleeding is the most dreaded and commonest presentation of portal hypertension. Successful treatment of paediatric PH, though challenging is performed in resource constraint countries. Cases: Five children presented over three years to a tertiary hospital in Ghana, with massive upper gastrointestinal bleeding...

BACKGROUND: Extrahepatic portal vein obstruction (EHPVO) causes portal hypertension in noncirrhotic children. Among surgical treatments, it is unclear whether the meso-Rex shunt (MRS) or portosystemic shunt (PSS) offers lower post-operative morbidity and superior patency over time. Our objective was to evaluate long-term outcomes comparing MRS and PSS for pediatric patients with EHPVO. METHODS: A systematic review was conducted of articles reporting children undergoing surgical shunts for EHPVO from 1/2000-2/2020...

BACKGROUND: Surveillance endoscopy to detect varices needing treatment (VNT) is important to prevent bleeding and morbidity in portal hypertension. In adult and pediatric cirrhosis, platelet count and liver stiffness measurement (LSM) are useful in selecting patients for endoscopy. Such recommendations do not exist for extrahepatic portal vein obstruction (EHPVO). Splenic stiffness measurement (SSM) has been studied in adult and pediatric EHPVO with conflicting results and methodological errors...

The etiology of portal hypertension (pHTN) in children differs from that of adults and may require different management strategies. We set out to review the etiology, management, and natural history of pHTN at a pediatric liver center. From 2008 to 2018, 151 children and adolescents with pHTN were identified at a free-standing children's hospital. Patients were stratified by etiology of pHTN (intrahepatic disease [IH], defined as cholestatic disease and fibrotic or hepatocellular disease; extrahepatic disease [EH], defined as hepatic vein obstruction and prehepatic pHTN)...

Portal vein thrombosis (PVT) is the most common cause of extrahepatic portal vein obstruction in children. Upper gastrointestinal (GI) bleeding and splenomegaly are the most common presenting features. PVT diagnosis is usually delayed in the absence of upper GI bleeding and many children undergo a hematologic work-up due to splenomegaly and signs of hypersplenism. Here, we present a case of a 2-year-old girl who had pancytopenia and splenomegaly. The hematologic work-up including a bone marrow aspirate was unrevealing and she was thought to have viral-induced bone marrow suppression and severe iron deficiency anemia...

Chronic non cirrhotic extrahepatic portal vein obstruction (EHPVO) refers to the cavernomatous transformation of the portal vein (the so-called "portal cavernoma") which occurs following acute thrombosis of the portal vein in the absence of recanalization. In adults, EHPVO mainly occurs following thrombosis, while in children it may be related to congenital malformations and/or neonatal umbilical venous catheterization. However, 50% of the cases of EHPVO remain idiopathic [1]. Risk factors and associated diseases should be investigated (chapter 1)...

PURPOSE: To report our initial experience with splenectomy and proximal spleno-left portal shunt as an alternative to the standard Rex shunt, when not applicable, in children with Extrahepatic Portal Vein Obstruction (EHPVO). METHODS: Patients from March 2015 till September 2018, with EHPVO not suitable for Rex shunt or whose caregivers refused to consent for Internal Jugular Vein (IJV) dissection were assessed and prepared for splenectomy with proximal spleno-left portal shunt...

Extrahepatic portal vein obstruction (EHPVO) is the most common cause of pediatric portal hypertension and can cause life-threatening variceal bleeding. Meso- Rex shunt (MRS) is a surgical procedure that restores physiological portal venous blood flow to the liver by using a graft to connect the superior mesenteric vein and the left portal vein within the Rex recess, and can relieve variceal bleeding and other complications associated with EHPVO. Although the MRS is regarded as an optimal and potentially curative treatment with good long-term patency, graft thrombosis or failure due to unknown causes is not rare, prompting the need for further research on the risk factors of graft failure or poor patency...

PURPOSE: The aim of the study was to test the feasibility of spleen stiffness measurement (SSM) by two-dimensional shear wave elastography (2D-SWE) and compare data on its diagnostic use with upper gastrointestinal endoscopy in children with extrahepatic portal hypertension (EHPH) before and after surgery. METHODS: A total of 44 children were included in this study [34 children with EHPH (main group)] and 10 controls (7.57 ± 1.22 years), who underwent ultrasonography including SSM by 2D-SWE...

INTRODUCTION: Changes in the coagulation profile in children with liver disease and extrahepatic portal vein obstruction or shunt result in both bleeding and thrombosis. Routine coagulation tests do not accurately predict bleeding risk, as they are not sensitive to changes in anticoagulant factors. The thrombin generation assay could be suitable for describing the overall balance of coagulation in children with liver disease. This study aims to characterise the mechanism of thrombin generation in this population, focusing on prothrombin conversion and thrombin inhibition...

OBJECTIVE: This study investigated the morphologic changes of portal cavernoma in children with extrahepatic portal venous obstruction and explored the relationship with prognosis. METHODS: From February 2008 to October 2017, there were 107 patients with cavernous transformation of the portal vein admitted to our hospital. Rex shunts were performed in 99 cases, Warren shunts in 7 cases, and laparoscopic splenic vessel ligation in 1 case. Intraoperative superior mesenteric venography was used to determine the structure of the portal venous system...

OBJECTIVES: Outcomes of pediatric-onset portal hypertension are poorly defined. We aimed to assess population-based long-term outcomes of pediatric-onset portal hypertension complicated by varices. METHODS: All children with esophageal varices (n = 126) were identified from 14,144 single nationwide referral center endoscopy reports during 1987 to 2013, and followed up through national health care and death registers. A questionnaire was sent to survivors (n = 94) of whom 65 (69%) responded...

BACKGROUND AND AIMS: The medical literature on colonic changes in children with extrahepatic portal venous obstruction (EHPVO) is limited. We evaluated EHPVO children for prevalence, nature, and relation of colonic changes with disease duration, extent of splenoportal axis (SPA) thrombosis, portal hypertensive gastropathy (PHG), and esophageal varices (EVs). The correlation between histologic and endoscopic changes was studied. METHODS: Subjects were evaluated by colonoscopy with ileoscopy and biopsy sampling, clinico-laboratory profiles, and SPA imaging...

OBJECTIVES: This study aimed to evaluate factors associated with upper digestive hemorrhage and primary and secondary endoscopic prophylaxis outcomes in children with extrahepatic portal vein obstruction. METHODS: This observational and prospective study included 72 children with extrahepatic portal vein obstruction who were followed from 2005 to 2017. Risk factors associated with upper digestive hemorrhage and the results of primary and secondary prophylaxis of these patients were evaluated...

Idiopathic portal hypertension (IPH) and extrahepatic portal venous obstruction (EHPVO) are prototype noncirrhotic causes of portal hypertension (PHT), characterized by normal hepatic venous pressure gradient, variceal bleeds, and moderate to massive splenomegaly with preserved liver synthetic functions. Infections, toxins, and immunologic, prothrombotic and genetic disorders are possible causes in IPH, whereas prothrombotic and local factors around the portal vein lead to EHPVO. Growth failure, portal biliopathy, and minimal hepatic encephalopathy are long-term concerns in EHPVO...

INTRODUCTION: In children with extrahepatic portal vein obstruction or those who develop portal vein thrombosis after liver transplant, the use of Meso-Rex Bypass (MRB) creates a more physiological state by redirecting mesenteric blood flow back into the intrahepatic portal system via a venous conduit. PRESENTATION OF CASE: A 3-year-old female with biliary atresia associated with polysplenia syndrome and a surgical history of Kasai portoenterostomy procedure, and an ABO incompatible whole liver transplant...

Extrahepatic portal vein obstruction (EHPVO) is the most common cause of upper gastrointestinal bleeding in children. It is defined as thrombosis of the extrahepatic portal vein with or without extension to the intrahepatic portal veins. The Meso-Rex shunt is the gold standard treatment in children with favorable anatomy since it restores physiological portal liver reperfusion. This is achieved by rerouting the splanchnic venous blood through an autologous graft from the superior mesenteric vein (SMV) into the Rex recess of the left portal vein, curing portal hypertension by doing so...

PURPOSE: 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. METHODS: 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...

Cirrhosis is a complex process in which the architecture of the liver is replaced by structurally abnormal nodules due to cirrhosis. Cirrhosis frequently leads to the development of portal hypertension. In children, portal hypertension may be caused by a wide range of etiologies, including extrahepatic portal vein obstruction, biliary atresia, alpha 1 antitrypsin deficiency, and autoimmune hepatitis. Gastroesophageal varices and ascites are two of the complications of portal hypertension likely to cause morbidity and mortality...