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Extrahepatic portal vein obstruction in children

Hanaa Mostafa El-Karaksy, Omneya Afifi, Azza Bakry, Ann Abdel Kader, Noha Saber
BACKGROUND: Minimal hepatic encephalopathy (MHE) is not associated with overt neuropsychiatric symptoms but rather with subtle changes in psychometric and/or neurophysiologic tests. We aimed to diagnose MHE in children with extrahepatic portal vein obstruction (EHPVO) and to evaluate the effect of lactulose on MHE. METHODS: A prospective study was carried out on 30 patients with EHPVO (21 males; mean age 10±2.5 years). The study was carried out in the Pediatric Hepatology Unit, Cairo University Pediatric Hospital, Cairo, Egypt, between 2011 and 2013...
February 2017: World Journal of Pediatrics: WJP
Toufeeq Ahmad Mir, Raiz Ahmad Misgar, Bashir Ahmad Laway, Omar Javed Shah, Zafar Amin Shah, Showkat Ali Zargar
OBJECTIVE: Growth retardation is common in children with extrahepatic portal vein obstruction (EHPVO) and growth hormone (GH) resistance may play a dominant role. The aim of this study was to ascertain growth parameters and growth-related hormones in children with EHPVO, comparing with controls and to study the response of shunt surgery on growth parameters. MATERIALS AND METHODS: The auxological and growth-related hormone profile (GH; insulin-like growth factor binding protein-3 [IGFBP-3] and IGF-1) of thirty children with EHPVO were compared with controls...
November 2016: Indian Journal of Endocrinology and Metabolism
Parisá Khodayar-Pardo, Andrés Peña Aldea, Ana Ramírez González, Adela Meseguer Carrascosa, Cristina Calabuig Bayo
Extrahepatic portal vein obstruction, although rare in children, is a significant cause of portal hypertension (PHT) leading to life-threatening gastrointestinal bleeding in the pediatric age group. PHT may also lead to other complications such as hyperesplenism, cholangyopathy, ascites, and even hepatopulmonary syndrome and portopulmonary hypertension that may require organ transplantation. Herein we report the case of an asymptomatic 11-month-old infant wherein a hepatomegaly and cavernous transformation of the portal vein was detected by liver ultrasound...
May 2016: Case Reports in Gastroenterology
Gloria Chocarro, Paloma Triana Junco, Eva Dominguez, María Virginia Amesty, Vanesa Nuñez Cerezo, Francisco Hernandez, Javier Murcia, Juan Antonio Tovar, Manuel Lopez Santamaria
OBJECTIVE: The mesoportal shunt (MPS) and liver transplantation (LT) have changed the scenario of extrahepatic portal vein obstruction (EHPVO) since the MPS, the only "curative" technique, can now be offered in asymptomatic patients and also thrombotic complications of LT have increased the incidence of EHPVO. MATERIAL AND METHODS: A retrospective study of patients undergoing surgery for EHPVO was conducted between 1990 and 2015. An analysis was done for the shunt permeability and clinical evolution over time...
February 2016: European Journal of Pediatric Surgery
Benjamin L Shneider, Jean de Ville de Goyet, Daniel H Leung, Anshu Srivastava, Simon C Ling, Mathieu Duché, Patrick McKiernan, Riccardo Superina, Robert H Squires, Jaime Bosch, Roberto Groszmann, Shiv K Sarin, Roberto de Franchis, George V Mazariegos
UNLABELLED: Approaches to the management of portal hypertension and variceal hemorrhage in pediatrics remain controversial, in large part because they are not well informed by rigorous clinical studies. Fundamental biological and clinical differences preclude automatic application of approaches used for adults to children. On April 11-12, 2015, experts in the field convened at the first Baveno Pediatric Satellite Meeting to discuss and explore current available evidence regarding indications for MesoRex bypass (MRB) in extrahepatic portal vein obstruction and the role of primary prophylaxis of variceal hemorrhage in children...
April 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
Hanaa M El-Karaksy, Nehal El-Koofy, Nabil Mohsen, Heba Helmy, Nevian Nabil, Mortada El-Shabrawi
BACKGROUND AND AIM: Extrahepatic portal vein obstruction (EHPVO) is an important cause of portal hypertension in children. The aim of this study was to describe the clinical presentation, possible risk factors, upper gastrointestinal endoscopic findings, and treatment modalities of children with EHPVO. METHODS: After ethical approval of our study protocol by our institution review board, we analyzed available data from medical records of patients with EHPVO presenting to the Pediatric Hepatology Unit, Cairo University Pediatric Hospital, Egypt, for a period of 15 years from January 1996 to December 2010...
January 2015: Journal of Pediatric Gastroenterology and Nutrition
Patrick McKiernan, Mona Abdel-Hady
Portal hypertension is one of the most serious complications of childhood liver disease, and variceal bleeding is the most feared complication. Most portal hypertension results from cirrhosis but extra hepatic portal vein obstruction is the single commonest cause. Upper gastrointestinal endoscopy endoscopy remains necessary to diagnose gastro-esophageal varices. Families of children with portal hypertension should be provided with written instructions in case of gastrointestinal bleeding. Children with large varices should be considered for primary prophylaxis on a case-by-case basis...
May 2015: Expert Review of Gastroenterology & Hepatology
Vibhor V Borkar, Ujjal Poddar, Niraj Kumari, Suruchi Singh, Raja Roy, Surender K Yachha
OBJECTIVE: We prospectively studied children with portal hypertension (PHT) for portal hypertensive duodenopathy (PHTD) and small bowel intestinal permeability (SIP) with the objectives of defining histopathological parameters for PHTD and to find out whether any association existed among structural changes, SIP, and nutritional status. METHOD: SIP was assessed by using lactulose and mannitol sugar probes in 31 children with PHT (cirrhosis n = 15 and extrahepatic portal venous obstruction n = 16) and 15 healthy children as controls...
February 2015: Journal of Pediatric Gastroenterology and Nutrition
Lorenzo D'Antiga, Patrizia Dacchille, Clementina Boniver, Sara Poledri, Sami Schiff, Lucia Zancan, Piero Amodio
OBJECTIVE: In children with noncirrhotic extrahepatic portal vein obstruction (EHPVO), minimal hepatic encephalopathy (MHE) was reported in a few series, but neither is it routinely investigated nor does consensus about its diagnosis exist. In this prospective observational study we aimed at detecting the prevalence of MHE in children with EHPVO and providing a practical diagnostic protocol. METHODS: A consecutive sample of 13 noncirrhotic children (age range 4-18 years) with EHPVO underwent a screening for MHE based on level of fasting ammonia, quantified electroencephalogram (EEG) evaluation, and a wide battery of 26 psychometric tests exploring learning ability, abstract reasoning, phonemic and semantic fluency, selective attention, executive functions, short-term verbal and visual memory, long-term verbal memory, and visuopractic ability...
December 2014: Journal of Pediatric Gastroenterology and Nutrition
F Guérin, V Bidault, E Gonzales, S Franchi-Abella, G De Lambert, S Branchereau
BACKGROUND: Meso-Rex bypass (MRB) and portosystemic surgical shunt (PSS) are both used to treat extrahepatic portal vein obstruction (EHPVO) in children. The aim of this study was to analyse the outcome of MRB and PSS to select patients who could benefit from a prophylactic MRB. METHODS: This single-centre retrospective study of children who underwent either MRB or PSS for EHPVO was conducted between 1996 and 2010. Details of patient demographics and preoperative evaluation were collected...
November 2013: British Journal of Surgery
Seung Jin Kim, Kyung Mo Kim
Variceal bleeding results in significant morbidity and mortality in both children and adults. The guidelines for the management of variceal bleeding are well established in adults but not in children as there have been insufficient pediatric studies of this disorder. In addition, the adult guidelines for treatment of variceal bleeding cannot be applied directly to children as the etiology and natural course of this disease differs between children and adults. Examples of recommended treatments in children include endoscopic variceal ligation as secondary prophylaxis for biliary atresia whereas a meso-Rex shunt operation for extrahepatic portal vein obstruction...
March 2013: Pediatric Gastroenterology, Hepatology & Nutrition
Roberta V de Alcantara, Roberto M Yamada, Sílvia R Cardoso, Maria de Fátima, C P Servidoni, Gabriel Hessel
OBJECTIVE: The aim of this study was to identify ultrasonographic predictors of esophageal varices (EVs) in children and adolescents with chronic liver disease (CLD) and extrahepatic portal venous obstruction (EHPVO). METHODS: This study evaluates 53 patients younger than 20 years with CLD or EHPVO and no history of bleeding or prophylactic EVs treatment. They were divided into 2 groups: group I (35 with CLD) and group II (18 with EHPVO). Splenorenal shunt (SS), gallbladder wall varices, gallbladder wall thickening (GT), and lesser omental thickness (LOT) were compared with the presence of EVs, gastric varices, and portal hypertensive gastropathy (PHG)...
December 2013: Journal of Pediatric Gastroenterology and Nutrition
Daniele Alberti, Mara Colusso, Maurizio Cheli, Paolo Ravelli, Amedeo Indriolo, Sergio Signorelli, Stefano Fagiuoli, Lorenzo D'Antiga
BACKGROUND: The management of extrahepatic portal vein obstruction (EHPVO) in children is controversial. We report our experience with a prospective evaluation of a stepwise protocol based on severity of portal hypertension and feasibility of mesoportal bypass (MPB). METHODS: After diagnosis, children with EHPVO underwent surveillance endoscopies and received nonselective β-blockers (NSBBs) or endoscopic variceal obliteration (EVO) when large varices were detected...
November 2013: Journal of Pediatric Gastroenterology and Nutrition
Rukhmi Bhat, Timothy B Lautz, Riccardo A Superina, Robert Liem
BACKGROUND/PURPOSE: Extrahepatic portal vein obstruction (EHPVO) is an important cause of chronic portal hypertension in children. Although usually idiopathic in etiology, genetic and acquired thrombophilia have been implicated in EHPVO. Meso-Rex bypass is increasingly used to treat EHPVO in children. OBJECTIVE: The objective of this study is to assess the relationship of postoperative anticoagulation strategies and thrombophilic risk factors to the development of bypass thrombosis following the meso-Rex bypass...
May 2013: Journal of Gastrointestinal Surgery: Official Journal of the Society for Surgery of the Alimentary Tract
Timothy B Lautz, Lisa A Keys, Joseph C Melvin, Joy Ito, Riccardo A Superina
BACKGROUND: Consequences of extrahepatic portal vein obstruction (EHPVO) include variceal bleeding and hypersplenism due to portal hypertension, as well as metabolic abnormalities secondary to impaired portal venous circulation. The purpose of this study was to compare the effectiveness of meso-Rex bypass and portosystemic shunt (PSS) for reversing these symptoms in children with EHPVO. STUDY DESIGN: All children with idiopathic EHPVO evaluated for potential meso-Rex bypass at a single institution between 1997 and 2010 were reviewed...
January 2013: Journal of the American College of Surgeons
Aleksandar Lj Sretenovic, Vojislav Perišić, Zoran Krstić, Dragana Vujović, Polina Pavićević, Dejana Stanisavljević, Božina Radević
PURPOSE: To establish the importance of shunt surgery combined with partial resection of the spleen for selected pediatric patients with extra-hepatic portal vein obstruction (EHPVO), enormous splenomegaly and severe hypersplenism. Severe hypersplenism is often refractory to treatment with endoscopic sclerotherapy or band ligation and shunt surgery; however, to our knowledge, this is the first such study to be published. METHODS: Distal splenorenal shunt with partial resection of the spleen was performed in 16 of 60 children treated for EHPVO in the Gastroenterology Department of our hospital...
May 2013: Surgery Today
Victor Lewa Bandika, Elizabeth A Goddard, Ronalda D De Lacey, Robin Alexander Brown
BACKGROUND: The outcome of sclerotherapy for bleeding oesophageal varices may be influenced by injection technique. In a previous study at our institution, sclerotherapy was associated with a high re-bleeding rate and oesophageal ulceration. Embolisation of the injection tract was introduced in an attempt to reduce injection-related complications. METHODS: To determine the outcome and effectiveness of injection tract embolisation in reducing injection-related complications, we retrospectively reviewed a series of 59 children who underwent injection sclerotherapy for oesophageal varices (29 for extrahepatic portal vein obstruction (EHPVO) and 30 for intrahepatic disease) in our centre...
September 11, 2012: South African Medical Journal, Suid-Afrikaanse Tydskrif Vir Geneeskunde
Dominik Ketelsen, Steven W Warmann, Juergen F Schaefer, Peter Haber, Joerg Fuchs, Claus D Claussen, Klaus Brechtel
Extrahepatic portal vein (PV) obstruction (EHPVO) is a rare disorder in early childhood with unknown incidence and mostly unknown etiology. In children with EHPVO, the hepatopedal flow of the mesenteric venous blood is hindered by an obstruction of the PV resulting in prehepatic portal hypertension. The curative treatment with a meso-Rex shunt connects the superior mesenteric vein to the left PV by a venous autograft. Despite good primary patency, reocclusion of a meso-Rex bypass and its treatment can be challenging...
September 2012: Journal of Pediatric Surgery
Agustín M Cárdenas, Monica Epelman, Kassa Darge, Elizabeth B Rand, Sudha A Anupindi
OBJECTIVE: The objective of this article is to illustrate the imaging features of patients with extrahepatic portal venous obstruction who are evaluated before or after a Rex shunt surgery. CONCLUSION: The Rex shunt is a potentially curative surgical procedure that reestablishes physiologic hepatopetal portal flow. It is typically accomplished by interposing a vascular conduit between the superior mesenteric vein to the still patent intrahepatic portal venous system...
May 2012: AJR. American Journal of Roentgenology
Chiara Grimaldi, Jean de Ville de Goyet, Valerio Nobili
The main causes of intrahepatic portal hypertension in children are cirrhosis and congenital hepatic fibrosis. Non cirrhotic portal hypertension in children is mostly due to extrahepatic portal vein obstruction. In half of cases, no underlying disorder is found. The meso-Rex bypass is the preferred treatment, when it is possible. The closest to the portal vein the obstruction, the highest the risk of esophagogastric varices.
June 2012: Clinics and Research in Hepatology and Gastroenterology
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