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Esophagic atresia

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https://www.readbyqxmd.com/read/29445485/esophageal-atresia-and-beckwith-wiedemann-syndrome-in-one-of-the-naturally-conceived-discordant-newborn-twins-first-report
#1
Gregorio Serra, Vincenzo Antona, Mandy Schierz, Davide Vecchio, Ettore Piro, Giovanni Corsello
Recent studies report a high incidence of monozygotic twinning in Beckwith-Wiedemann syndrome. A phenotypical discordance in monozygotic twins is rare. Twinning and Beckwith-Wiedemann syndrome show higher incidence in children born after assisted reproductive techniques. We report on the first observation of esophageal atresia and Beckwith-Wiedemann syndrome in one of the naturally conceived discordant monozygotic twins.
February 2018: Clinical Case Reports
https://www.readbyqxmd.com/read/29429770/structural-airway-abnormalities-contribute-to-dysphagia-in-children-with-esophageal-atresia-and-tracheoesophageal-fistula
#2
Katherine J Baxter, Lauren M Baxter, April M Landry, Mark L Wulkan, Amina M Bhatia
BACKGROUND: Long-term dysphagia occurs in up to 50% of repaired esophageal atresia and tracheoesophageal fistula (EA/TEF) patients. The underlying factors are unclear and may include stricture, esophageal dysmotility, or associated anomalies. Our purpose was to determine whether structural airway abnormalities (SAA) are associated with dysphagia in EA/TEF. METHODS: We conducted a retrospective chart review of children who underwent EA/TEF repair in our hospital system from 2007 to 2016...
January 31, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29429769/neurodevelopmental-outcomes-of-infants-with-esophageal-atresia-and-tracheoesophageal-fistula
#3
Wegdan Mawlana, Paul Zamiara, Hilary Lane, Margaret Marcon, Eveline Lapidus-Krol, Priscilla Pl Chiu, Aideen M Moore
BACKGROUND: Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) is a complex disorder, and most outcome data are confined to mortality and feeding-related morbidities. Our objective was to examine mortality, growth and neurodevelopmental outcomes in a large recent cohort of infants with EA/TEF. METHODS: Single center study of EA/TEF infants referred from January 2000 to December 2015. Data collected included associated defects, neonatal morbidity and mortality and growth and neurodevelopmental outcomes at age 12-36months...
January 31, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29399473/pre-and-post-operative-visualization-of-neonatal-esophageal-atresia-tracheoesophageal-fistula-via-magnetic-resonance-imaging
#4
Nara S Higano, Alister J Bates, Jean A Tkach, Robert J Fleck, Foong Y Lim, Jason C Woods, Paul S Kingma
Esophageal atresia (EA) is a relatively uncommon congenital anomaly, often observed in conjunction with tracheoesophageal fistula (TEF). Surgical repair in neonates typically takes place with little information about the pre-existing EA/TEF structure because there are currently no acceptable tools for evaluating EA/TEF anatomy prior to repair; chest x-ray radiograph does not identify malformation sub-type or gap length, while x-ray computed tomography (CT) demonstrate an unacceptably high exposure to ionizing radiation...
February 2018: Journal of Pediatric Surgery Case Reports
https://www.readbyqxmd.com/read/29395153/elongation-of-esophageal-segments-by-bougienage-stretching-technique-for-long-gap-esophageal-atresia-to-achieve-delayed-primary-anastomosis-by-thoracotomy-or-thoracoscopic-repair-a-first-experience-from-china
#5
Suna Sun, Weihua Pan, Wenjie Wu, Yiming Gong, Jia Shi, Jun Wang
OBJECTIVES: The treatment of long gap esophageal atresia (LGEA) is one of the most challenging congenital malformations in neonatal surgery. A preoperative bougienage stretching technique for elongation of the two segments of esophagus is applied to achieve utilizing the native esophagus to establish esophageal continuity by open or thoracoscopic approach. METHODS: From January 2015 to May 2017, 12 neonates who suffered from LGEA were admitted to our department...
December 27, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29381487/mandibulofacial-dysostosis-guion-almeida-type-caused-by-novel-eftud2-splice-site-variants-in-two-asian-children
#6
Kris P T Yu, Ho-Ming Luk, Christopher T Gordon, Genevieve Fung, Myriam Oufadem, Maria M Garcia-Barcelo, Jeanne Amiel, Brian H Y Chung, Ivan F M Lo, Yang Tan Tiong
Mandibulofacial dysostosis type Guion-Almeida (MFDGA) is a rare disease entity that results in congenital craniofacial anomalies that are caused by abnormal development of the first and second pharyngeal arches. MFDGA is characterized by malar and mandibular hypoplasia, microcephaly, developmental delay, dysplastic ears, and a distinctive facial appearance. Extracraniofacial malformations include esophageal atresia, congenital heart disease, and radial ray abnormalities. Heterozygous mutations in the elongation factor Tu GTP-binding domain containing 2 (EFTUD2) gene have been shown to result in MFDGA...
January 29, 2018: Clinical Dysmorphology
https://www.readbyqxmd.com/read/29373986/etiological-heterogeneity-and-clinical-variability-in-newborns-with-esophageal-atresia
#7
Ettore Piro, Ingrid Anne Mandy Schierz, Mario Giuffrè, Giovanni Cuffaro, Simona La Placa, Vincenzo Antona, Federico Matina, Giuseppe Puccio, Marcello Cimador, Giovanni Corsello
BACKGROUND: The aim of this study was to define different characteristics of infants with esophageal atresia and correlations with neonatal level of care, morbidity and mortality occurring during hospital stay. METHODS: Charts of all newborns with esophageal atresia (EA) admitted to our University NICU between January 2003 and November 2016 were reviewed and subdivided in four groups related to different clinical presentations; EA as an isolated form (A), with a concomitant single malformation (B), as VACTERL association (C), and in the context of a syndrome or an entity of multiple congenital anomalies (D)...
January 26, 2018: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/29373345/basic-knowledge-of-tracheoesophageal-fistula-and-esophageal-atresia
#8
Sura Lee
BACKGROUND: Tracheoesophageal fistula (TEF) and esophageal atresia (EA) are rare anomalies in neonates. Up to 50% of neonates with TEF/EA will have Vertebral anomalies (V), Anal atresia (A), Cardiac anomalies (C), Tracheoesophageal fistula (T), Esophageal atresia (E), Renal anomalies (R), and Limb anomalies (L) (VACTERL) association, which has the potential to cause serious morbidity. PURPOSE: Timely management of the neonate can greatly impact the infant's overall outcome...
February 2018: Advances in Neonatal Care: Official Journal of the National Association of Neonatal Nurses
https://www.readbyqxmd.com/read/29333824/eosinophilic-esophagitis-and-esophageal-atresia-coincidence-or-causality
#9
Karen V Stave Salgado, Ana M Rocca
Eosinophilic esophagitis is an immune-mediated chronic disease of the esophagus characterized by symptoms related to esophageal dysfunction and tissue eosinophilia. In the endoscopy, the esophageal mucosa may appear normal or show exudates, rings, edema, furrows, and strictures. Its management is based on elimination diet, topical corticosteroids and/or esophageal dilation. Atresia is the most common congenital alteration of the esophagus; it requires surgical repair and poses potential complications, such as gastroesophageal reflux, strictures, and esophageal dysmotility...
February 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29325785/characteristics-and-outcomes-of-children-with-ductal-dependent-congenital-heart-disease-and-esophageal-atresia-tracheoesophageal-fistula-a-multi-institutional-analysis
#10
Kriti Puri, Shaine A Morris, Carlos M Mery, Yunfei Wang, Brady S Moffett, Jeffrey S Heinle, J Ruben Rodriguez, Lara S Shekerdemian, Antonio G Cabrera
BACKGROUND: Extracardiac birth defects are associated with worse outcomes in congenital heart disease (CHD). The impact of esophageal atresia/trachea-esophageal fistula (EA/TEF) on outcomes after surgery for ductal-dependent CHD is unknown. METHODS: Retrospective matched cohort study using the Pediatric Health Information System database from 07/2004 to 06/2015. Hospitalizations with ductal-dependent CHD and EA/TEF, undergoing CHD surgery were included as cases...
January 8, 2018: Surgery
https://www.readbyqxmd.com/read/29299746/laryngotracheal-anomalies-associated-with-esophageal-atresia-importance-of-early-diagnosis
#11
Pierre Fayoux, Martin Morisse, Rony Sfeir, Laurent Michaud, Sam Daniel
OBJECTIVE: Esophageal atresia (EA) is the most common congenital esophageal malformation. Airway pathology, in particular, tracheomalacia and laryngotracheal anomalies is a major cause of morbidity and mortalilty in patients with EA. The aim of this study was to report the incidence and type of laryngotracheal anomalies seen in a large series of patients with EA, and to evaluate their impact on the management of children with EA. STUDY DESIGN: Retrospective study...
January 4, 2018: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/29273218/clinical-predictors-and-prevalence-of-receiving-special-preschool-school-support-in-children-with-repaired-esophageal-atresia
#12
Michaela Dellenmark-Blom, Linus Jönsson, Vladimir Gatzinsky, Kate Abrahamsson
BACKGROUND/PURPOSE: In a sparsely investigated field, we aimed to evaluate the use of special preschool/school support among children with repaired esophageal atresia (EA) and/or tracheoesophageal fistula (TEF), the predicting clinical factors for this support, and level of school absence. METHODS: Data on 119 EA/TEF children 2-17years old were collected through medical records and questionnaires (response rate 95%). Logistical regression analysis identified clinical predictors of special preschool/school support in the population without genetic disorders (n=105)...
November 28, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29250692/esophageal-atresia-with-distal-fistula-unusual-case-series-considerations-related-to-epidemiological-aspects-malformative-associations-and-prenatal-diagnosis
#13
Maria Livia Ognean, Laura Corina Zgârcea, Laura Bălănescu, Oana Boantă, Raluca Elena Dumitra, Florin Grosu, Dan Georgian Bratu, Adrian Gheorghe Boicean, Liliana Coldea, Radu Chicea
BACKGROUND: Esophageal atresia (EA) is the most frequent and severe congenital anomaly of the esophagus, occurring in 1:2500-1:4500 live births. Five types of EA have been described, EA with tracheoesophageal fistula (TEF) being the most frequent. AIM: The aim of this paper is to evaluate epidemiological aspects, malformative associations, and prenatal diagnosis in an unusual case series of EA with distal TEF. CASE PRESENTATIONS: The authors are analyzing a series of seven cases of EA with distal TEF...
2017: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/29241961/management-of-neonates-with-right-sided-aortic-arch-and-esophageal-atresia-international-survey-on-ipeg-and-espes-members%C3%A2-experience
#14
REVIEW
Monserrat Aguilera-Pujabet, Jose Andres Molino Gahete, Gabriela Guillén, Sergio López-Fernández, Marta Patricia Martin-Giménez, Josep Lloret, Manuel López
AIM: The optimum surgical approach of neonates with right-sided aortic arch (RAA) and esophageal atresia (EA)/tracheoesophageal fistula (TEF) is still an unsolved question. In order to propose an operative algorithm in the era of endoscopic surgery, we performed an international survey to know the current practice between pediatric endoscopic surgeons. Two of the most important societies in endoscopic pediatric surgery were queried: the International Pediatric Endosurgery Group (IPEG) and the European Society of Paediatric Endoscopic Surgeons (ESPES)...
November 23, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29232005/x-linked-vacterl-h-caused-by-deletion-of-exon-3-in-fancb-a-case-report
#15
Norikazu Watanabe, Seiji Tsutsumi, Yuki Miyano, Hidenori Sato, Satoru Nagase
VACTERL is a congenital malformation characterized by vertebral defects (V), anal atresia (A), cardiac malformation (C), tracheoesophageal fistula (T), esophageal atresia (E), radial or renal dysplasia (R), and limb abnormalities (L) (McCauley et al. 2011). An association of VACTERL with ventriculomegaly or clinical hydrocephalus, known as VACTERL-H, was reported to have poor prognosis. Here, we report a case of VACTERL-H with an X-linked family history and a deleted exon 3 in FANCB.
December 12, 2017: Congenital Anomalies
https://www.readbyqxmd.com/read/29224798/slide-esophagoplasty-vs-end-to-end-anastomosis-for-recalcitrant-esophageal-stricture-after-esophageal-atresia-repair
#16
Ali Kamran, Charles J Smithers, Michael A Manfredi, Thomas E Hamilton, Peter D Ngo, David Zurakowski, Russell W Jennings
BACKGROUND: Anastomotic stricture is a common complication following esophageal atresia (EA) repair. Patients with a recalcitrant stricture may require surgical intervention. The technique of re-anastomosis after stricture resection can affect patient outcomes. STUDY DESIGN: EA patients who underwent anastomotic stricture resection from July 2010 to February 2017 were reviewed. Patients after stricture resection in which slide esophagoplasty was performed were compared to the conventional approach of end-to-end anastomosis...
December 7, 2017: Journal of the American College of Surgeons
https://www.readbyqxmd.com/read/29224771/revision-repair-of-type-iv-laryngotracheoesophageal-cleft-using-multiple-long-tapered-engaging-grafts
#17
Evan J Propst
An 8 year-old female with esophageal atresia and a type IV laryngotracheoesophageal cleft underwent tracheostomy, gastric pull-up and fundoplication with G-tube insertion at birth. She remained nil per os due to aspiration. The trachealis was separated from the esophagus that was reapproximated and clavicular periosteum was placed. A long posterior costal cartilage graft that engaged behind the cricoid plate and tapered inferiorly was inserted. A second thin cartilage graft was sutured to it distally to extend the length of the repair...
December 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29216021/long-term-results-and-quality-of-life-assessment-in-biliary-atresia-patients-a-35-year-experience-in-a-tertiary-hospital
#18
Carol Wing Yan Wong, Patrick Ho Yu Chung, Paul Kwong Hang Tam, Kenneth Kak Yuen Wong
OBJECTIVES: To review long-term transplant-free survival and quality of life of patients with biliary atresia. METHODS: A retrospective study reviewing all patients with Kasai operation between 1 January 1980 and 31 December 2015 was performed to evaluate the transplant-free survival. Subgroup analysis of patients over 20 years old was carried out to assess the quality of life using the Short Form-36 Health Survey and incidences of disease-related complications...
December 5, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29212093/chewing-function-in-children-with-repaired-esophageal-atresia-tracheoesophageal-fistula
#19
Selen Serel Arslan, Numan Demir, Aynur Ayşe Karaduman, Feridun Cahit Tanyel, Tutku Soyer
No abstract text is available yet for this article.
December 6, 2017: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29201998/treatment-and-outcome-for-children-with-esophageal-atresia-from-a-gender-perspective
#20
Julia Ekselius, Martin Salö, Einar Arnbjörnsson, Pernilla Stenström
Background: Besides the incidence of esophageal atresia (EA) being higher in males, no other gender-specific differences in EA have been reported. The aim of this study was to search for gender-specific differences in EA. Methods: A retrospective study was conducted at a tertiary center for pediatric surgery. The medical charts of infants born with EA were reviewed. 20 girls were identified, and 20 boys were selected as matched controls with respect to concomitant malformations...
2017: Surgery Research and Practice
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