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Diaphragmatic hernia managment

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https://www.readbyqxmd.com/read/29333839/-pa%C3%A3-ister-kiuian-syndrome-in-a-mexican-mestizo-patient-case-report
#1
Paola Mendelsberg-Fishbein, Constanza García-Delgado, Linda B Muñoz-Martínez, Maura Robledo-Cayetano, Leonardo J Mejía-Marín, Luis E Martínez-Barrera, Mabel Cerrillo-Hinojosa, Verónica F Moran-Barroso
Pallister-Killian syndrome is caused by a tetrasomy 12p mosaicism and is characterized by facial dysmorphism, pigmentary skin anomalies, congenital heart defects, diaphragmatic hernia, epilepsy and mental retardation. The diagnosis is complex as the cytogenetic analysis in blood is usually normal, requiring karyotyping in other tissues, therefore the clinical suspicion is critical to guide the diagnostic tests and the patient requires an interdisciplinary clinical evaluation regarding the several manifestation of the syndrome...
February 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29305406/early-population-based-outcomes-of-infants-born-with-congenital-diaphragmatic-hernia
#2
Anna-May Long, Kathryn J Bunch, Marian Knight, Jennifer J Kurinczuk, Paul D Losty
PURPOSE: This study aims to describe short-term outcomes of live-born infants with congenital diaphragmatic hernia (CDH) and to identify prognostic factors associated with early mortality. DESIGN: A prospective population cohort study was undertaken between April 2009 and September 2010, collecting data on live-born infants with CDH from all 28 paediatric surgical centres in the UK and Ireland using an established surgical surveillance system. Management and outcomes are described...
January 4, 2018: Archives of Disease in Childhood. Fetal and Neonatal Edition
https://www.readbyqxmd.com/read/29274787/expert-surgical-consensus-for-prenatal-counseling-using-the-delphi-method
#3
Loren Berman, Jordan Jackson, Kristen Miller, Rebecca Kowalski, Paul Kolm, Francois I Luks
BACKGROUND: Pediatric surgeons frequently offer prenatal consultation for congenital pulmonary airway malformation (CPAM) and congenital diaphragmatic hernia (CDH); however, there is no evidence-based consensus to guide prenatal decision making and counseling for these conditions. Eliciting feedback from experts is integral to defining best practice regarding prenatal counseling and intervention. METHODS: A Delphi consensus process was undertaken using a panel of pediatric surgeons identified as experts in fetal therapy to address current limitations...
November 28, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29250478/an-unusual-complication-of-congenital-diaphragmatic-hernia
#4
E Tian Tan, Keren Sloan, Kokila Lakhoo
A term newborn was referred to our unit with a postnatal diagnosis of a right-sided congenital diaphragmatic hernia (CDH). She was managed with high-frequency oscillatory ventilation, inotropic support, and nitric oxide, with planned surgical repair when she was medically optimized. On day 6 of life, there was an acute deterioration causing difficulty maintaining adequate ventilation and the infant requiring increasing analgesia and paralysis, especially during abdominal examination. A repeat X-ray showed distended bowel loops in the right hemithorax when compared with previous films raising suspicion of bowel obstruction...
January 2017: European Journal of Pediatric Surgery Reports
https://www.readbyqxmd.com/read/29237145/current-practices-in-the-management-of-congenital-diaphragmatic-hernia-patients-requiring-extracorporeal-membrane-oxygenation-results-of-an-international-survey-of-pediatric-surgeons
#5
Sophia Abdulhai, Ian C Glenn, Neil L McNinch, Todd A Ponsky, Avraham Schlager
INTRODUCTION: There is little consensus on optimal management for congenital diaphragmatic hernia extracorporeal membrane oxygenation (CDH ECMO) patients. Meaningful comparisons of the various approaches have been limited due to the low number of cases in institutions. In addition, the multidisciplinary reliance and rigid institutional framework of ECMO serve to further limit exposure to alternative practices. The goal of this study is to survey the international pediatric surgery community to describe the current practice trends...
December 13, 2017: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
https://www.readbyqxmd.com/read/29233624/first-and-second-trimester-screening-for-fetal-structural-anomalies
#6
REVIEW
Lindsay Edwards, Lisa Hui
Fetal structural anomalies are found in up to 3% of all pregnancies and ultrasound-based screening has been an integral part of routine prenatal care for decades. The prenatal detection of fetal anomalies allows for optimal perinatal management, providing expectant parents with opportunities for additional imaging, genetic testing, and the provision of information regarding prognosis and management options. Approximately one-half of all major structural anomalies can now be detected in the first trimester, including acrania/anencephaly, abdominal wall defects, holoprosencephaly and cystic hygromata...
December 9, 2017: Seminars in Fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29212620/one-year-outcome-for-congenital-diaphragmatic-hernia-results-from-the-french-national-register
#7
François Barrière, Fabrice Michel, Anderson D Loundou, Virginie Fouquet, Elsa Kermorvant, Sébastien Blanc, Elisabeth Carricaburu, Amélie Desrumaux, Odile Pidoux, Alexis Arnaud, Nicolas Berte, Thierry Blanc, Frederic Lavrand, Guillaume Levard, Isabelle Rayet, Sylvain Samperiz, Anne Schneider, Marie-Odile Marcoux, Norbert Winer, Yann Chaussy, Valérie Datin-Dorriere, Quentin Ballouhey, Aurélien Binet, Charles Muszynski, Jean Breaud, Armelle Garenne, Laurent Storme, Julia Boubnova
OBJECTIVE: To evaluate the status of congenital diaphragmatic hernia (CDH) management in France and to assess predictors of adverse outcomes. STUDY DESIGN: We reviewed the first-year outcome of all cases of CDH reported to the French National Register in 2011. RESULTS: A total of 158 cases were included. Of these, 83% (131) were prenatally diagnosed, with a mortality rate of 39% (44 of 112) for live born infants with a known outcome at hospital discharge...
December 4, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/29209510/milrinone-in-congenital-diaphragmatic-hernia-a-randomized-pilot-trial-study-protocol-review-of-literature-and-survey-of-current-practices
#8
REVIEW
Satyan Lakshminrusimha, Martin Keszler, Haresh Kirpalani, Krisa Van Meurs, Patricia Chess, Namasivayam Ambalavanan, Bradley Yoder, Maria V Fraga, Holly Hedrick, Kevin P Lally, Leif Nelin, Michael Cotten, Jonathan Klein, Stephanie Guilford, Ashley Williams, Aasma Chaudhary, Marie Gantz, Jenna Gabrio, Dhuly Chowdhury, Kristin Zaterka-Baxter, Abhik Das, Rosemary D Higgins
Background: Congenital diaphragmatic hernia (CDH) is commonly associated with pulmonary hypoplasia and pulmonary hypertension (PH). PH associated with CDH (CDH-PH) is frequently resistant to conventional pulmonary vasodilator therapy including inhaled nitric oxide (iNO) possibly due to right and left ventricular dysfunction. Milrinone is an intravenous inotrope and lusitrope with pulmonary vasodilator properties and has been shown anecdotally to improve oxygenation in PH. We developed this pilot study to determine if milrinone infusion would improve oxygenation in neonates ≥36 weeks postmenstrual age (PMA) with CDH...
2017: Maternal Health, Neonatology and Perinatology
https://www.readbyqxmd.com/read/29193315/review-shows-that-implementing-a-nationwide-protocol-for-congenital-diaphragmatic-hernia-was-a-key-factor-in-reducing-mortality-and-morbidity
#9
REVIEW
L Storme, J Boubnova, S Mur, L Pognon, D Sharma, E Aubry, R Sfeir, P Vaast, T Rakza, A Benachi
The French Rare Disease Reference Center for Congenital Diaphragmatic Hernia (CDH) was created in 2008, to implement a national protocol for fetuses and children with this serious condition. Neonatal mortality from CDH is 30-40%, mainly due to pulmonary hypoplasia and persistent pulmonary hypertension, and half of those who live have high respiratory, nutritional and digestive morbidity. CDH management requires long-term and specialised multidisciplinary care. It has been well established that a standardised management protocol improves the prognosis of children with CDH...
November 28, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/29177588/treatment-of-giant-paraesophageal-hernia-pro-laparoscopic-approach
#10
REVIEW
B Dallemagne, G Quero, A Lapergola, L Guerriero, C Fiorillo, S Perretta
PURPOSE: Giant paraesophageal hernias (GPEH) are relatively uncommon and account for less than 5% of all primary hiatal hernias. Giant Secondary GPEH can be observed after surgery involving hiatal orifice opening, such as esophagectomy, antireflux surgery, and hiatal hernia repair. Surgical treatment is challenging, and there are still residual controversies regarding the laparoscopic approach, even though a reduced morbidity and mortality, as well as a shorter hospital stay have been demonstrated...
November 25, 2017: Hernia: the Journal of Hernias and Abdominal Wall Surgery
https://www.readbyqxmd.com/read/29169875/current-and-future-antenatal-management-of-isolated-congenital-diaphragmatic-hernia
#11
REVIEW
Francesca Maria Russo, Paolo De Coppi, Karel Allegaert, Jaan Toelen, Lennart van der Veeken, George Attilakos, Mary Patrice Eastwood, Anna Louise David, Jan Deprest
Congenital diaphragmatic hernia is surgically correctable, yet the poor lung development determines mortality and morbidity. In isolated cases the outcome may be predicted prenatally by medical imaging. Cases with a poor prognosis could be treated before birth. However, prenatal modulation of lung development remains experimental. Fetoscopic endoluminal tracheal occlusion triggers lung growth and is currently being evaluated in a global clinical trial. Prenatal transplacental sildenafil administration may in due course be a therapeutic approach, reducing the occurrence of persistent pulmonary hypertension, either alone or in combination with fetal surgery...
November 21, 2017: Seminars in Fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29150031/thoracic-surgery-in-the-pregnant-patient
#12
REVIEW
Brian Whang
Thoracic surgeons are sometimes asked to consult on the management of a patient who is pregnant. Conditions commonly encountered are empyema, spontaneous pneumothorax, and diaphragmatic hernia. Lung cancer is rarely seen in pregnancy, but its incidence is rising. Diagnostic imaging and perioperative management involve the navigation of fetal risks and nuances in maternal physiology. Shared decision making within a multidisciplinary framework will optimally guide the course of management.
February 2018: Thoracic Surgery Clinics
https://www.readbyqxmd.com/read/29138242/role-of-ecmo-in-congenital-diaphragmatic-hernia
#13
REVIEW
Merrill McHoney, Philip Hammond
Congenital diaphragmatic hernia (CDH) is typified morphologically by failure of diaphragmatic development with accompanying lung hypoplasia and persistent pulmonary hypertension of the newborn (PPHN). Patients who have labile physiology and low preductal saturations despite optimal ventilatory and inotropic support may be considered for extracorporeal membrane oxygenation (ECMO). Systematic reviews into the benefits of ECMO in CDH concluded that any benefit is unclear. Few randomised trials exist to demonstrate clear benefit and guide management...
November 14, 2017: Archives of Disease in Childhood. Fetal and Neonatal Edition
https://www.readbyqxmd.com/read/29117038/development-and-validation-of-extracorporeal-membrane-oxygenation-mortality-risk-models-for-congenital-diaphragmatic-hernia
#14
Yigit S Guner, Danh V Nguyen, Lishi Zhang, Yanjun Chen, Matthew T Harting, Peter Rycus, Ryan Barbaro, Matteo Di Nardo, Thomas V Brogan, John P Cleary, Peter T Yu
The purpose of our study was to develop and validate extracorporeal membrane oxygenation (ECMO)-specific mortality risk models for congenital diaphragmatic hernia (CDH). We utilized the data from the Extracorporeal Life Support Organization Registry (2000-2015). Prediction models were developed using multivariable logistic regression. We identified 4,374 neonates with CDH with an overall mortality of 52%. Predictive discrimination (C statistic) for pre-ECMO mortality model was C = 0.65 (95% confidence interval, 0...
November 7, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/29106923/impact-of-objective-echocardiographic-criteria-for-timing-of-congenital-diaphragmatic-hernia-repair
#15
Scott Deeney, Lisa W Howley, Maggie Hodges, Kenneth W Liechty, Ahmed I Marwan, Jason Gien, John P Kinsella, Timothy M Crombleholme
OBJECTIVE: To assess the impact of specific echocardiographic criteria for timing of congenital diaphragmatic hernia repair on the incidence of acute postoperative clinical decompensation from pulmonary hypertensive crisis and/or acute respiratory decompensation, with secondary outcomes including survival to discharge, duration of ventilator support, and length of hospitalization. STUDY DESIGN: The multidisciplinary congenital diaphragmatic hernia management team instituted a protocol in 2012 requiring the specific criterion of echocardiogram-estimated pulmonary artery pressure ≤80% systemic blood pressure before repairing congenital diaphragmatic hernias...
October 26, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/29078158/iatrogenic-gastric-perforation-in-a-misdiagnosed-case-of-late-presenting-congenital-diaphragmatic-hernia-report-of-an-avoidable-complication
#16
Pradeep Kajal, Namita Bhutani, Mohit Goyal, Poonam Kamboj
INTRODUCTION: Congenital diaphragmatic hernia (CDH) is a defect in diaphragm which usually presents with severe respiratory distress in neonatal period. PRESENTATION OF CASE: We present a case of congenital diaphragmatic hernia presenting at an age of 2.5 years in a male child. It was misdiagnosed as a case of pyothorax for which chest tube was attempted on left side resulting in iatrogenic gastric perforation. The patient was managed by early and prompt surgery...
October 12, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/29054892/transthoracic-littre-s-hernia-presenting-with-faecopneumothorax-following-perforation-of-the-meckel-s-diverticulum-a-late-complication-of-oesophagectomy
#17
Charles West, Karen Erskine, Khaled Hamdan
A Littre's hernia is an unusual phenomenon where a Meckel's diverticulum protrudes through a potential abdominal opening. We wish to present a unique case of a 79-year-old man with respiratory distress following a fall from standing, initially managed as a haemothorax. After a chest drain was placed, bowel contents were drained from the pleural cavity and he was taken to theatre. He had a history of minimally invasive oesophagectomy for cancer and had subsequently developed a diaphragmatic hernia. A blind ending diverticulum with a perforation at its tip was found in the left oblique lung fissure that was subsequently confirmed histologically as a perforated Meckel's diverticulum...
October 20, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29052830/diaphragmatic-rupture-a-single-institution-experience-and-literature-review
#18
Carlo Corbellini, Stefano Costa, Tiberio Canini, Roberta Villa, Ettore Contessini Avesani
BACKGROUND: Diaphragmatic rupture (DR) is a rare and potentially life-threatening event caused by trauma or spontaneously. DR occasionally occurs several months after the injury. Chest X-ray and computed tomography are the most effective diagnostic methods. Delay in DR diagnosis occurs frequently. This study aimed to examine and improve our understanding of the etiology, clinical presentation, and management of DR. METHODS: This study was performed at the Emergency and General Surgery Department of Fondazione I...
September 2017: Ulusal Travma Ve Acil Cerrahi Dergisi, Turkish Journal of Trauma & Emergency Surgery: TJTES
https://www.readbyqxmd.com/read/29037794/ventricular-performance-is-associated-with-need-for-extracorporeal-membrane-oxygenation-in-newborns-with-congenital-diaphragmatic-hernia
#19
COMPARATIVE STUDY
Gabriel Altit, Shazia Bhombal, Krisa Van Meurs, Theresa A Tacy
OBJECTIVE: To compare echocardiography (ECHO) findings of patients with congenital diaphragmatic hernia (CDH) who required extracorporeal membrane oxygenation (ECMO) to non-ECMO treated patients. STUDY DESIGN: We reviewed clinical and ECHO data of newborns with CDH born between 2009 and 2016. Exclusions included major anomalies, genetic syndromes, or no ECHO prior to ECMO. Pulmonary hypertension was assessed by ductal shunting and tricuspid regurgitant jet. Speckle tracking echocardiography (STE) assessed function by quantifying deformation...
December 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/29030926/sternal-malformations-and-anesthetic-management
#20
REVIEW
John H Nichols, Viviane G Nasr
Shamberger and Welch classify sternal malformations into four types: thoracic ectopia cordis, cervical ectopia cordis, thoraco-abdominal ectopia cordis, and cleft sternum. Cleft sternum is the most common subset, with a reported incidence of 1 in 50,000 to 100,000 live births, representing 0.15% of all anterior chest wall malformations. Acostello et al further classify cleft sternum into complete or partial (superior, medium, inferior) with a simple superior partial cleft sternum being by far the most common with an orthotopic heart, intact pericardium, and normal skin coverage...
November 2017: Paediatric Anaesthesia
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