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Esophagic atresia

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https://www.readbyqxmd.com/read/28528013/congenital-h-type-tracheoesophageal-fistula-a-multicenter-review-of-outcomes-in-a-rare-disease
#1
Sara C Fallon, Jacob C Langer, Shawn D St Peter, KuoJen Tsao, Caroline M Kellagher, Dave R Lal, Jill S Whitehouse, Diana L Diesen, Michael D Rollins, Elizabeth Pontarelli, Marcus M Malek, Corey W Iqbal, Jeffrey S Upperman, Charles M Leys, Mark L Wulkan, Sarah J Hill, Martin L Blakely, Timothy D Kane, David E Wesson
OBJECTIVE: To perform a multicenter review of outcomes in patients with H-type tracheoesophageal fistula (TEF) in order to better understand the incidence and causes of post-operative complications. BACKGROUND: H-type TEF without esophageal atresia (EA) is a rare anomaly with a fundamentally different management algorithm than the more common types of EA/TEF. Outcomes after surgical treatment of H-type TEF are largely unknown, but many authoritative textbooks describe a high incidence of respiratory complications...
May 11, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28514381/-complications-and-long-term-results-of-delayed-esophagoezophagostomy-for-esophageal-atresia
#2
A Yu Razumovsky, A B Alkhasov, O G Mokrushina, M A Chundokova, N V Kulikova, A G Gebekov, S A Gebekova
AIM: To evaluate complications and long-term results of delayed esophagoesophagostomy in children with esophageal atresia (EA). MATERIAL AND METHODS: 165 EA children were operated at the Filatov Municipal Children's Hospital #13 for the period 2006-2016. Primary esophageal anastomosis was performed in 136 (82.4%) children with tracheoesophageal fistula. In 5 (3%) neonates with non-fistulous EA esophago- and gastrostomy were made for further coloesophagoplasty. Other 24 (14...
2017: Khirurgiia
https://www.readbyqxmd.com/read/28503544/update-on-foregut-molecular-embryology-and-role-of-regenerative-medicine-therapies
#3
REVIEW
Silvia Perin, Conor J McCann, Osvaldo Borrelli, Paolo De Coppi, Nikhil Thapar
Esophageal atresia (OA) represents one of the commonest and most severe developmental disorders of the foregut, the most proximal segment of the gastrointestinal (GI) tract (esophagus and stomach) in embryological terms. Of intrigue is the common origin from this foregut of two very diverse functional entities, the digestive and respiratory systems. OA appears to result from incomplete separation of the ventral and dorsal parts of the foregut during development, resulting in disruption of esophageal anatomy and frequent association with tracheo-oesophageal fistula...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28499713/long-term-outcomes-of-oesophageal-atresia-without-or-with-proximal-tracheooesophageal-fistula-gross-types-a-and-b
#4
A I Koivusalo, S J Sistonen, H G Lindahl, R J Rintala, M P Pakarinen
PURPOSE: Because of an extended gap between esophageal pouches a variety of methods are employed to treat oesophageal atresia (OA) without (type A) or with (type B) proximal tracheooesophageal fistula. This retrospective observational study describes their single centre long-term outcomes from 1947 to 2014. METHODS: Of 693 patients treated for OA 68 (9.7%) had type A (n=58, 8.3%) or B (n=10, 1.4%). Hospital records were reviewed. Main outcome measures were survival and oral intake...
May 1, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28492107/aborted-tracheo-esophageal-fistula-in-a-neonate
#5
John M Hallett, Victoria Bryant, David Drake, Simi George, Manasvi Upadhyaya
Esophageal atresia (EA) is an uncommon congenital anomaly which is often associated with a tracheoesophageal fistula. An isolated EA is a rarer anomaly and its diagnosis has implications for the ongoing treatment and outcome of the infant. For the first time, we report a case of a premature newborn with a pure EA and a tracheal diverticulum, containing both respiratory and esophageal mucosa. We have termed this an aborted trachea-esophageal fistula. Recognition of these very rare variations of foregut anomalies may contribute to our understanding of their pathogenesis...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28489830/-clinical-and-laboratory-characteristics-of-patients-with-pulmonary-hypertension-and-pulmonary-vascular-complications-hospitalized-at-the-instituto-nacional-de-salud-del-ni%C3%A3-o
#6
Alexis Jose Ormeño Julca, Carlos Melchor Alvarez Murillo, Pedro Miguel Amoretti Alvino, Angel Aladino Florian Florian, Rosa Aurora Castro Johanson, Maria Danisa Celi Perez, Olga Rocío Huamán Prado
The hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHN) are distinct pulmonary vascular complications of portal hypertension (PHT) and are associated with increased morbidity and mortality. OBJECTIVES: To describe the clinical and laboratory characteristics of patients with pulmonary hypertension and pulmonary vascular complications hospitalized at the Instituto Nacional de Salud del Niño. MATERIALS AND METHODS: We included patients with HTP hospitalized from January 2012 to June 2013 and that during its evolution progressed with SHP or HTPP...
January 2017: Revista de Gastroenterología del Perú: órgano Oficial de la Sociedad de Gastroenterología del Perú
https://www.readbyqxmd.com/read/28487849/impedance-testing-in-esophageal-atresia-patients
#7
REVIEW
Maheen Hassan, Hayat Mousa
Esophageal atresia patients are predisposed to gastroesophageal reflux as a result of the altered esophageal anatomy and motility. These patients experience significant morbidity from gastroesophageal reflux. As a result, an effective way to diagnose and monitor for reflux is crucial. pH-metry is able to quantify acid burden, ensure that acid suppression is adequate during long-term follow-up, and correlate acid reflux to symptoms. pH with impedance is additionally able to detect non-acid reflux as well as volume clearance, both of which also correlate with patient symptoms...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28475726/development-and-pilot-testing-of-a-condition-specific-instrument-to-assess-the-quality-of-life-in-children-and-adolescents-born-with-esophageal-atresia
#8
M Dellenmark-Blom, K Abrahamsson, J H Quitmann, R Sommer, S Witt, J Dingemann, S Flieder, L Jönsson, V Gatzinsky, M Bullinger, B M Ure, C Dingemann, J E Chaplin
The survival rate of children with esophageal atresia has today reached 95%. However, children are at risk of chronic morbidity related to esophageal and respiratory dysfunction, and associated anomalies. This study describes the pilot testing of a condition-specific health-related quality-of-life instrument for children with esophageal atresia in Sweden and Germany, using a patient-derived development approach consistent with international guidelines. Following a literature review, standardized focus groups were conducted with 30 Swedish families of children with esophageal atresia aged 2-17 years...
July 1, 2017: Diseases of the Esophagus: Official Journal of the International Society for Diseases of the Esophagus
https://www.readbyqxmd.com/read/28473972/importance-of-an-international-registry-for-and-collaborative-research-on-esophageal-atresia
#9
Frédéric Gottrand, Delphine Ley, Laurent Michaud, Rony Sfeir
Esophageal atresia (EA) is a rare congenital defect. Data on EA prevalence, management, and long-term outcome are lacking because the available data come from small retrospective series from tertiary referral centers. An international multicenter registry would provide strong epidemiological data from large population-based cohorts on EA prevalence and incidence, treatment, long-term morbidity, and prognosis and would thus provide accurate data for evaluation of the current guidelines for EA management. The future challenge of the new international network on EA, which was created in 2013, is to promote the creation of a collaborative database and further studies...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28471144/therapeutic-effect-of-one-stage-esophageal-replacement-with-stomach-in-newborns-in-the-treatment-of-long-gap-esophageal-atresia
#10
Zhandong Zeng, Hongwei Zhang, Fengli Liu
No abstract text is available yet for this article.
May 3, 2017: Minerva Pediatrica
https://www.readbyqxmd.com/read/28451032/our-experience-in-two-cases-of-type-iv-laryngotracheoesophageal-cleft-ltec-with-a-diagnosis-of-antenatal-esophageal-atresia
#11
Kaan Sonmez, Ramazan Karabulut, Zafer Turkyilmaz, Canan Turkyilmaz, Berrin Isik, Sibel Eryilmaz, Kıvanc Seref, Ebru Ozcan, Gul Meral Hosgoren, Abdullah Can Basaklar
Laryngotracheoesophageal clefts (LTECs) are rare congenital defects that are often accompanied by additional anomalies. The major issues in the treatment of these patients are intraoperative exposure insufficiency, technical difficulty of the operation, and anesthesia problems originating from the respiratory tract. Problems originating from mechanical ventilation and respiratory tract, eating disorders and relapse of fistula are among the problems encountered following surgery. Most of the time, concomitant additional anomalies also worsen the clinical picture...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28446132/pcsk5-is-required-in-the-early-cranio-cardiac-mesoderm-for-heart-development
#12
Dorota Szumska, Milena Cioroch, Angela Keeling, Annik Prat, Nabil G Seidah, Shoumo Bhattacharya
BACKGROUND: Loss of proprotein convertase subtilisin/kexin type 5 (Pcsk5) results in multiple developmental anomalies including cardiac malformations, caudal regression, pre-sacral mass, renal agenesis, anteroposterior patterning defects, and tracheo-oesophageal and anorectal malformations, and is a model for VACTERL/caudal regression/Currarino syndromes (VACTERL association - Vertebral anomalies, Anal atresia, Cardiac defects, Tracheoesophageal fistula and/or Esophageal atresia, Renal & Radial anomalies and Limb defects)...
April 26, 2017: BMC Developmental Biology
https://www.readbyqxmd.com/read/28439200/use-of-pericardium-to-repair-anastomotic-leak-after-esophageal-atresia-surgery-experience-with-one-case
#13
Ying Liyang, Gao Zhan, Zhang Zewei, Qi Jianchuan, Wang Wei, Liu Xiwang
Diverse therapies for the management of anastomotic leakage after esophageal atresia repair have been reported with various outcomes. The surgical management of anastomotic leakage after esophageal atresia repair can be challenging. We present a child with long-gap esophageal atresia and anastomotic leakage repair with pericardium. This article aimed to illustrate that pericardium may be a substitute for esophageal leakage repair.
March 2017: Türk Pediatri Arşivi
https://www.readbyqxmd.com/read/28424985/postoperative-complications-and-functional-outcome-after-esophageal-atresia-repair-results-from-longitudinal-single-center-follow-up
#14
Florian Friedmacher, Birgit Kroneis, Andrea Huber-Zeyringer, Peter Schober, Holger Till, Hugo Sauer, Michael E Höllwarth
BACKGROUND: Esophageal atresia (EA) and tracheoesophageal fistula (TEF) represent major therapeutic challenges, frequently associated with serious morbidities following surgical repair. The aim of this longitudinal study was to assess temporal changes in morbidity and mortality of patients with EA/TEF treated in a tertiary-level center, focusing on postoperative complications and their impact on long-term gastroesophageal function. METHODS: One hundred nine consecutive patients with EA/TEF born between 1975 and 2011 were followed for a median of 9...
April 19, 2017: Journal of Gastrointestinal Surgery: Official Journal of the Society for Surgery of the Alimentary Tract
https://www.readbyqxmd.com/read/28421172/aspiration-risk-and-respiratory-complications-in-patients-with-esophageal-atresia
#15
REVIEW
Thomas Kovesi
Chronic, long-term respiratory morbidity (CRM) is common in patients with a history of repaired congenital esophageal atresia, typically associated with tracheoesophageal fistula (EA/TEF). EA/TEF patients are at high risk of having aspiration, and retrospective studies have associated CRM with both recurrent aspiration and atopy. However, studies evaluating the association between CRM in this population and either aspiration or atopy have reported conflicting results. Furthermore, CRM in this population may be due to other related conditions as well, such as tracheomalacia and/or recurrent infections...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28417152/review-of-surgical-and-anesthetic-management-for-esophageal-atresia-with-tracheoesophageal-fistula-unilateral-pulmonary-agenesis-and-dextrocardia
#16
Rebecca M Rentea, Tolulope A Oyetunji, John Erkmann, Joshua Q Knowlton, Richard J Hendrickson
Association of unilateral severe pulmonary atresia or agenesis and esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) and dextrocardia is a rare and highly lethal combination. We report a case of a full-term female infant who had EA with TEF, right lung agenesis, and dextrocardia. Repair of the fistula took place on day of life 3. We describe anesthetic and surgical concerns of this patient's case which are keys to a good operative outcome as well as follow-up for the patient.
April 17, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28413304/simultaneous-single-staged-repair-of-anorectal-malformation-with-tracheoesophageal-fistula-lessons-learned
#17
Ajay Narayan Gangopadhyay, Vaibhav Pandey
INTRODUCTION: Anorectal malformation (ARM) associated esophageal atresia (EA) with tracheoesophageal fistula (TEF) spawns special therapeutic propositions. The outcome of these patients banks on numerous factors. We performed this study with an aim to compare the outcome of single-staged simultaneous primary repair of both anomalies versus staged repair of these disorders. MATERIALS AND METHODS: Retrospective review of cases with ARM and associated EA with TEF managed over a period of 5 years from July 2010 to June 2015 after ethical approval was undertaken...
April 2017: Journal of Indian Association of Pediatric Surgeons
https://www.readbyqxmd.com/read/28412031/alterations-in-hyolaryngeal-elevation-after-esophageal-anastomosis-a-possible-mechanism-for-airway-aspiration
#18
Numan Demir, Selen Serel Arslan, Sule Yalcin, Ayşe Karaduman, Feridun Cahit Tanyel, Tutku Soyer
AIM: A prospective study was performed to evaluate anatomical alterations and hyolaryngeal elevation (HE) by videofluoroscopic swallowing study (VFSS) in patients with esophageal atresia-tracheoesophageal fistula (EA-TEF). METHODS: Patients operated for EA-TEF were evaluated for age, sex, type of atresia and time to esophageal anastomosis. All patients were evaluated by videofluoroscopic swallowing study (VFSS). Penetration-Aspiration scale (PAS≥7 is considered as aspiration), distance between upper esophageal sphincter and 2nd cervical vertebrae (UES-C2) and hyolaryngeal elevation (HE) were evaluated by the same deglutitionist who was blind to the study...
April 5, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28410264/esophageal-atresia-tracheoesophageal-fistula-repair-complicated-by-tracheomalacia-a-case-report-of-successful-management-of-respiratory-distress-using-caudal-morphine
#19
Joanna M Dion, Anthony M-H Ho, Andrea Winthrop, Michael P Flavin
We report a case of severe respiratory distress in a neonate who was not endotracheally intubated soon after esophageal atresia/tracheoesophageal fistula (EA/TEF) repair. In this serious situation, any form of emergency respiratory support or definitive airway management may compromise the esophageal anastomosis and fistula repair. The cause of respiratory distress in the early postoperative period after EA/TEF is multifactorial, and in this case, included symptomatic tracheomalacia, which is commonly associated with EA/TEF...
April 13, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28409148/position-paper-of-inoea-working-group-on-long-gap-esophageal-atresia-for-better-care
#20
David C van der Zee, Pietro Bagolan, Christophe Faure, Frederic Gottrand, Russell Jennings, Jean-Martin Laberge, Marcela Hernan Martinez Ferro, Benoît Parmentier, Rony Sfeir, Warwick Teague
INoEA is the International Network of Esophageal Atresia and consists of a broad spectrum of pediatric specialties and patient societies. The working group on long-gap esophageal atresia (LGEA) set out to develop guidelines regarding the definition of LGEA, the best diagnostic and treatment strategies, and highlight the necessity of experience and communication in the management of these challenging patients. Review of the literature and expert discussion concluded that LGEA should be defined as any esophageal atresia (EA) that has no intra-abdominal air, realizing that this defines EA with no distal tracheoesophageal fistula (TEF)...
2017: Frontiers in Pediatrics
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