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https://www.readbyqxmd.com/read/29668568/transjugular-intrahepatic-portosystemic-shunt-insertion-for-the-management-of-portal-hypertension-in-children
#1
Lauren Johansen, Patrick McKiernan, Khalid Sharif, Simon McGuirk
OBJECTIVES: To describe our 20-year experience with transjugular intrahepatic portosystemic shunt (TIPSS) procedures for children with resistant portal hypertension (PHTN). METHODS: Retrospective review of all children that had a TIPSS performed at Birmingham Children's Hospital from 1 January 1995 - 1 January 2015. RESULTS: 40 children underwent 42 attempted TIPSS for resistant PHTN with recurrent variceal bleeding (n = 35), refractory ascites (n = 4), and hypersplenism (n = 1)...
April 17, 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29622386/long-term-outcome-of-transjugular-intrahepatic-portosystemic-shunt-for-portal-hypertension-in-autosomal-recessive-polycystic-kidney-disease
#2
Sarah Verbeeck, Djalila Mekhali, David Cassiman, Geert Maleux, Peter Witters
BACKGROUND: Autosomal recessive polycystic kidney disease (ARPKD) with congenital hepatic fibrosis (CHF) causes portal hypertension and its complications. A transjugular intrahepatic portosystemic shunt (TIPSS) could serve as a symptomatic treatment for portal hypertension-related symptoms in these children. AIMS: To study the effect of TIPSS on portal hypertension, liver and kidney function and the long term complications. MATERIALS AND METHODS: We report on 5 children with CHF treated with a TIPSS to manage severe portal hypertension related symptoms...
March 15, 2018: Digestive and Liver Disease
https://www.readbyqxmd.com/read/29470353/clinical-efficacy-of-percutaneous-transhepatic-portal-vein-angioplasty-for-late-onset-portal-vein-stenosis-in-pediatric-liver-transplant-patients
#3
Kushal B Naik, C Matthew Hawkins, Anne E Gill, Nitika A Gupta
BACKGROUND: Stenosis of the portal vein (PV) is a rare complication after liver transplantation (LT) in pediatric patients and it adversely affects outcomes. We reviewed the safety and efficacy of percutaneous transhepatic balloon angioplasty (PTBA) as a treatment for post-LT late-onset portal vein stenosis (PVS). METHODS: 318 patients between the ages of 0-21 years received a LT from 2001 to 2016 at this tertiary center. 21 children were evaluated for PVS using percutaneous portal venography...
February 20, 2018: Transplantation
https://www.readbyqxmd.com/read/29464506/idiopathic-portal-hypertension-and-extrahepatic-portal-venous-obstruction
#4
REVIEW
Rajeev Khanna, Shiv Kumar Sarin
BACKGROUND: Idiopathic portal hypertension (IPH) and extrahepatic portal venous obstruction (EHPVO) are non-cirrhotic vascular causes of portal hypertension (PHT). Variceal bleed and splenomegaly are the commonest presentations. AIM: The present review is intended to provide the existing literature on etiopathogenesis, clinical profile, diagnosis, natural history and management of IPH and EHPVO. RESULTS: IPH and EHPVO are both characterized by normal hepatic venous pressure gradient, moderate to massive splenomegaly with preserved liver synthetic functions...
February 2018: Hepatology International
https://www.readbyqxmd.com/read/29457852/successful-management-of-living-donor-liver-transplantation-for-biliary-atresia-with-single-ventricle-physiology-from-peri-transplant-through-total-cavopulmonary-connection-a-case-report
#5
Yohei Yamada, Ken Hoshino, Takayuki Oyanagi, Ryohei Gatayama, Jun Maeda, Nobuyuki Katori, Yasushi Fuchimoto, Taizo Hibi, Masahiro Shinoda, Kentaro Matsubara, Hideaki Obara, Ryo Aeba, Yuko Kitagawa, Hiroyuki Yamagishi, Tatsuo Kuroda
Children with single ventricle physiology have complete mixing of the pulmonary and systemic circulations, requiring staged procedures to achieve a separation of these circulations, or Fontan circulation. The single ventricle physiology significantly increases the risk of mortality in children undergoing non-cardiac surgery. As liver transplantation for patients with single ventricle physiology is particularly challenging, only a few reports have been published. We herein report a case of successful LDLTx for an 8-month-old pediatric patient with biliary atresia, heterotaxy, and complex heart disease of single ventricle physiology...
May 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29297972/prognostic-scoring-systems-and-outcome-of-endovascular-radiological-intervention-of-chronic-budd-chiari-syndrome-in-children
#6
Sumit K Singh, Moinak Sen Sarma, Rajanikant Yadav, Sheo Kumar, Raghunandan Prasad, Surender K Yachha, Anshu Srivastava, Ujjal Poddar
BACKGROUND AND AIM: Prognostic scoring systems (PSS) have not been validated in children with chronic Budd-Chiari syndrome (BCS). We aimed to analyse the long-term outcome of radiological intervention (RI) and validate the PSS in children. METHODS: Chronic BCS children were analysed in four subgroups: (i) SI: successful intervention (primary or secondary stent patency) (ii) PO: poor outcome (refractory stent block or requirement of liver transplantation), (iii) NU: naïve unintervened (awaiting RI) and (iv) DBI: died before intervention...
January 3, 2018: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/29243189/congenital-portosystemic-venous-shunt
#7
REVIEW
M Papamichail, M Pizanias, N Heaton
Congenital portosystemic venous shunts are rare developmental anomalies resulting in diversion of portal flow to the systemic circulation and have been divided into extra- and intrahepatic shunts. They occur during liver and systemic venous vascular embryogenesis and are associated with other congenital abnormalities. They carry a higher risk of benign and malignant liver tumors and, if left untreated, can result in significant medical complications including systemic encephalopathy and pulmonary hypertension...
March 2018: European Journal of Pediatrics
https://www.readbyqxmd.com/read/29183422/congenital-vascular-anomalies-of-the-liver
#8
A Tyraskis, N Durkin, M Davenport
Congenital vascular anomalies of the liver include a range of malformations of the portal venous, hepatic arterial and venous systems. Congenital portosystemic shunts and arteriovenous malformations make up the two most frequent such malformations. While infantile haemangiomas of the liver, endothelial tumours characterised by vascular proliferation should also be considered, as a proportion of them form prenatally. Evidence to support treatment strategies for these infants and children has been mainly based on small case series...
November 6, 2017: South African Medical Journal, Suid-Afrikaanse Tydskrif Vir Geneeskunde
https://www.readbyqxmd.com/read/29055931/congenital-shunts-of-the-portal-venous-system-case-series-of-uncommon-shunts
#9
Liliana Santos, Susana Nobre, Nadia Laezza, Catarina Cunha, Isabel Gonçalves, Maria F Lopes
BACKGROUND AND AIMS: Congenital shunts of the portal venous system are rare entities that can present in children with clinical heterogeneity. To evaluate the clinical course of children with uncommon shunts presenting to our institution and examine the available literature on this topic. Medical records of children with rare forms of congenital shunts were retrospectively reviewed for demographics, symptoms, management, and outcome between 2003 and 2016. RESULTS: Three female patients with congenital shunts, including a congenital mesenterico-portal Rex shunt (n = 1) and congenital portosystemic shunts (CPSS) (n = 2), were referred for surgical evaluation between ages 4 and 9...
November 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28983709/late-complications-of-biliary-atresia-hepatopulmonary-syndrome-and-portopulmonary-hypertension
#10
Frederick M Karrer, Bradley J Wallace, Arturo E Estrada
Children with biliary atresia (BA) following Kasai portoenterostomy have a high risk for portal hypertension, however, while variceal and hemorrhagic complications have been more commonly studied, less frequent but no less possibly devastating complications of hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPH) remain less well understood. HPS and PPH both occur in a setting of portal hypertension, however, paradoxically patients with HPS develop pulmonic vasculature dilation leading to shunting and hypoxia, while those with PPH develop an opposite progression of pulmonary vasoconstriction eventually leading to cor pulmonale and decompensation...
December 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28940454/choledochal-cyst-with-17q12-chromosomal-duplication
#11
Radana Kotalova, Petra Dusatkova, Jana Drabova, Lenka Elblova, Tomas Dedic, Ondrej Cinek, Jan Lebl, Stepanka Pruhova
The 17q12 chromosomal region carries the HNF1B gene, mutations of which cause various conditions. When searching for HNF1B/17q12 rearrangements among children with biliary atresia and/or choledochal cysts, we identified a male proband carrying a 17q12 duplication spanning 1698 kb that included 24 genes from TBC1D3C to HNF1B. The boy presented with cholestatic jaundice at the age of 2 weeks due to a choledochal cyst sized 15 ×12 mm (type Ia according to the Todani classification). He underwent a shunt surgery consisting of a hepaticojejunostomy using Roux-en-Y loop at the age of 2 months, which led to a permanent relief of cholestasis...
January 2018: Annals of Human Genetics
https://www.readbyqxmd.com/read/28741138/trans-splenic-access-for-portal-venous-interventions-in-children-do-benefits-outweigh-risks
#12
Sheena Pimpalwar, Ponraj Chinnadurai, Alberto Hernandez, Kamlesh Kukreja, Shakeel Siddiqui, Henri Justino
BACKGROUND: The primary concern of trans-splenic access for portal interventions is the risk of life-threatening intraperitoneal bleeding. OBJECTIVE: To review the clinical indications and efficacy and evaluate the risk factors for intraperitoneal bleeding during trans-splenic portal interventions in children. MATERIALS AND METHODS: A retrospective review of consecutive patients who underwent trans-splenic portal interventions at a tertiary care pediatric institution between March 2011 and April 2017 was performed...
July 24, 2017: Cardiovascular and Interventional Radiology
https://www.readbyqxmd.com/read/28632515/p4-stump-approach-for-intraoperative-portal-vein-stenting-in-pediatric-living-donor-liver-transplantation-an-innovative-technique-for-a-challenging-problem
#13
Chao-Long Chen, Yu-Fan Cheng, Viola Huang, Ting-Lung Lin, Yi-Chia Chan, Hsin-You Ou, Chee-Chien Yong, Shih-Ho Wang, Chih-Che Lin
OBJECTIVE: The aim of this study was to evaluate the utility of the P4 stump stenting approach for treating portal vein (PV) complications in pediatric living donor liver transplantation (LDLT). BACKGROUND: PV complications cause significant morbidity and mortality in pediatric LDLT. Biliary atresia in the backdrop of pathological PV hypoplasia and sclerosis heightens the complexity of PV reconstruction. The authors developed a novel approach for intraoperative PV stenting via the graft segment 4 PV stump (P4 stump) to address this challenge...
March 2018: Annals of Surgery
https://www.readbyqxmd.com/read/28570120/characterization-of-the-coagulation-profile-in-children-with-liver-disease-and-extrahepatic-portal-vein-obstruction-or-shunt
#14
William Beattie, Maria Magnusson, Winita Hardikar, Paul Monagle, Vera Ignjatovic
INTRODUCTION: Chronic liver disease causes a disruption of procoagulant and anticoagulant factors, resulting in a fragile state, prone to increased rates of both bleeding and thrombosis. Currently, there is limited literature describing the changes observed in pediatric liver disease and extrahepatic portal vein obstruction or shunt. This study aimed to describe the changes that occur in children with chronic liver disease and extrahepatic portal vein obstruction or shunt. MATERIALS AND METHODS: We measured the concentration and activity of key procoagulant and anticoagulant factors in children with liver disease, children with extrahepatic portal vein obstruction or shunt, and healthy children...
March 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/28386718/globus-pallidus-mr-signal-abnormalities-in-children-with-chronic-liver-disease-and-or-porto-systemic-shunting
#15
Sylviane Hanquinet, Claire Morice, Delphine S Courvoisier, Vladimir Cousin, Mehrak Anooshiravani, Laura Merlini, Valérie A McLin
OBJECTIVES: Detection of subclinical hepatic encephalopathy in children is difficult. We aimed to assess the changes in imaging of the central nervous system in children with chronic liver disease using MR imaging, diffusion, and1 H -spectroscopy. METHODS: Forty three children with chronic liver disease and/or porto-systemic shunting (111.4±56.9 months) and 24 controls (72.0±51.8 months) underwent brain MRI/spectroscopy on a 1.5T to examine T1, T2, ADC, Cho/Cr, ml/Cr, Glx/Cr ratio spectroscopy in the globus pallidus...
October 2017: European Radiology
https://www.readbyqxmd.com/read/28353468/primary-budd-chiari-syndrome-in-children-king-s-college-hospital-experience
#16
Susana Nobre, Rajeev Khanna, Natalie Bab, Eirini Kyrana, Sue Height, John Karani, Pauline Kane, Nigel Heaton, Anil Dhawan
Primary Budd-Chiari syndrome is a rare cause of liver disease in children in the western world. Here we present a retrospective review of children with Primary Budd-Chiari syndrome presenting from January 2001 to November 2015 to our hospital. Seven children were identified. Their presentation was mostly chronic. All had predisposing factors for thrombosis and were started on anticoagulation. Radiological interventions (2 transjugular intrahepatic portosystemic shunts and 1 hepatic vein stenting), liver transplant and mesocaval shunt were done in 3, 2, and 1 patients, respectively; 1 child underwent bone marrow transplantation following transjugular intrahepatic portosystemic shunts and 1 child was managed only medically...
July 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28099341/laparoscopic-distal-splenoadrenal-shunt-for-the-treatment-of-portal-hypertension-in-children-with-congenital-hepatic-fibrosis-a-case-report
#17
Jin-Shan Zhang, Wei Cheng, Long Li
BACKGROUND: The distal splenorenal shunt is an effective procedure for the treatment of portal hypertension in children. However, there has been no report about laparoscopic distal splenorenal shunt in the treatment of portal hypertension in children. METHODS: From December 2015 to August 2016, 4 children with upper gastrointestinal bleeding underwent laparoscopic distal splenoadrenal shunt. Portal hypertension and splenomegaly were demonstrated on the preoperative computed tomography (CT) and sonography...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28003725/neonatal-cholestasis-as-initial-presentation-of-portosystemic-shunt-a-case-report
#18
Güzide Doğan, Fatih Düzgün, Serdar Tarhan, Yeliz Çağan Appak, Erhun Kasırga
Congenital intrahepatic portosystemic shunts are rare in children. Portosystemic venous malformations are characterized by extreme clinical variability. We report a full-term 33-day-old male infant presenting with neonatal jaundice. On physical examination, he had generalized icterus and the liver was palpable 3.5 cm below the right costal margin. He had no other symptoms. Laboratory tests showed AST 632 U/L, ALT 198 U/L, total bilirubin 12.1 mg/dL, conjugated bilirubin 10.2 mg/dL, alkaline phosphatase 753 U/L, GGT 47 U/L and glucose 67 U/L...
December 2016: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/27896406/evaluation-of-rex-shunt-on-cavernous-transformation-of-the-portal-vein-in-children
#19
Ruo-Yi Wang, Jun-Feng Wang, Xiao-Gang Sun, Qian Liu, Jia-Long Xu, Qi-Gang Lv, Wei-Xiu Chen, Jin-Liang Li
BACKGROUND: Children with cavernous transformation of the portal vein (CTPV) develop severe complications from prehepatic portal hypertension, such as recurrent variceal bleeding and thrombocytopenia. In this study, we reported the results of 30 children with symptomatic CTPV that were treated by a Rex shunt. The effectiveness of this surgical approach was evaluated. METHODS: A retrospective review was performed of 30 children aged between 3 and 18 years with CTPV, who underwent a Rex shunt between 2008 and 2015...
April 2017: World Journal of Surgery
https://www.readbyqxmd.com/read/27775284/-congenital-portosistemic-shunt-importance-of-early-treatment
#20
N Vicente, M Pérez, R Gander, A Segarra, C Leganés, J Bueno
AIM: Congenital portosistemic shunt (CPSS) is an uncommon condition that can cause serious complications such as encephalopathy and liver tumors at risk of malignant degeneration. Occlusion of the shunt by surgery or interventional radiology can prevent and even improve such complications. In some cases, liver transplantation is the only curative option. We describe our experience with this condition. PATIENTS AND METHODS: Between 1992 and 2013, eight children (four male and four female) were diagnosed with CPSS (four extrahepatic and four intrahepatic) in our center, of which seven were diagnosed after 2007...
April 15, 2015: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
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