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Esophagic atresia

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https://www.readbyqxmd.com/read/29048729/beyond-down-syndrome-phenotype-paternally-derived-isodicentric-chromosome-21-with-partial-monosomy-21q22-3
#1
Manesha Putra, Urvashi Surti, Jie Hu, Deana Steele, Michele Clemens, Devereux N Saller, Svetlana A Yatsenko, Aleksandar Rajkovic
Inverted isodicentric chromosome 21 is a rare form of chromosomal rearrangement that may result in trisomy 21; sometimes this rearrangement may also lead to segmental monosomy of the terminal long arm of chromosome 21. In this report, we describe the prenatal diagnosis and neonatal follow-up of a child with a paternally derived, de novo isodicentric chromosome 21 and a concurrent ∼1.2 Mb deletion of the 21q22.3 region [46,XX,idic(21)(q22.3)]. This child presented with unusual phenotype of Down syndrome and additional defects including esophageal atresia and tethered cord syndrome...
October 19, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29034656/guidelines-of-the-italian-society-of-videosurgery-in-infancy-for-the-minimally-invasive-treatment-of-the-esophageal-atresia
#2
Salvatore Fabio Chiarenza, Maria Luisa Conighi, Andrea Conforti, Ciro Esposito, Maria Escolino, Fabio Beretta, Maurizio Cheli, Vincenzo Di Benedetto, Maria Grazia Scuderi, Giovanni Casadio, Maurizio Marzaro, Leon Francesco Fascetti, Claudio Vella, Cosimo Bleve, Daniela Codric, Paolo Caione, Pietro Bagolan
Not available.
October 4, 2017: La Pediatria Medica e Chirurgica: Medical and Surgical Pediatrics
https://www.readbyqxmd.com/read/29022107/preoperative-laryngotracheobronchoscopy-in-infants-with-esophageal-atresia-why-is-it-not-routine
#3
REVIEW
Kiarash Taghavi, Mark D Stringer
The value of laryngotracheobronchoscopy (LTB) immediately prior to repair of esophageal atresia with or without tracheo-esophageal fistula is contentious. Currently, there is a wide range of opinion on the utility of this investigation which is reflected by huge variation in clinical practice. This review is a critical analysis of the arguments for and against performing routine LTB prior to esophageal atresia repair. Reserving LTB for selected cases only is potentially disadvantageous since it limits the surgeon's and anesthetist's familiarity with the procedure, equipment, and range of potential findings...
October 11, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28974882/kluth-type-iv3-membranous-esophageal-atresia-at-middle-one-third-of-esophagus-an-extremely-rare-entity
#4
Rahul Gupta, Pramila Sharma, Arvind Kumar Shukla, Sunil Mehra
We herein present an extremely rare case of an isolated membranous atresia causing near-complete obstruction of the esophagus. The neonate presented with drooling of saliva and frothing from the mouth. A red rubber catheter met with an obstruction at 12 cm from the gum margins. Radiograph showed paucity of gas in the abdomen. Thoracotomy revealed external continuity of esophagus; dilated and elongated proximal segment constituting upper half of esophagus, and a membrane with tiny opening in the center, at its junction with narrow distal segment...
October 2017: Journal of Indian Association of Pediatric Surgeons
https://www.readbyqxmd.com/read/28974873/the-modified-posterior-thoracotomy-for-esophageal-atresia
#5
Mohamed Oulad Saiad
AIMS: Right dorsolateral thoracotomy with splitting or sparing the latissimus dorsi is the standard approach to the esophageal atresia. The thoracoscopic approach to the treatment of esophageal atresia is a demanding procedure used only by few surgeons in few centers. The purpose of this study is to present the modified posterior thoracotomy for neonates with esophageal atresia. PATIENTS AND METHODS: Between January 2007 and May 2016, the modified posterior thoracotomy was performed in 56 neonates with esophageal atresia...
October 2017: Journal of Indian Association of Pediatric Surgeons
https://www.readbyqxmd.com/read/28958717/musculoskeletal-deformities-following-neonatal-thoracotomy-long-term-follow-up-of-an-esophageal-atresia-cohort
#6
Sunny Wei, Neil Saran, Sherif Emil
BACKGROUND: Musculoskeletal deformities (MD), including scoliosis and chest wall anomalies, are potential long-term complications of neonatal thoracotomies. METHODS: We studied the incidence of MD in patients who underwent open repair of esophageal atresia between 1997 and 2012, had no other predisposition to MD, and subsequently received longitudinal follow-up in a multidisciplinary esophageal atresia clinic. Detailed chest wall and musculoskeletal exams were performed at each visit...
September 5, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28958264/substernal-colon-volvulus-with-ischemia-43-years-after-reconstruction-for-esophageal-atresia
#7
Christopher L Henry, J Matthew Reinerssman, Subrato J Deb
No abstract text is available yet for this article.
September 1, 2017: American Surgeon
https://www.readbyqxmd.com/read/28953251/respiratory-morbidity-in-children-with-repaired-congenital-esophageal-atresia-with-or-without-tracheoesophageal-fistula
#8
REVIEW
Maria Francesca Patria, Stefano Ghislanzoni, Francesco Macchini, Mara Lelii, Alessandro Mori, Ernesto Leva, Nicola Principi, Susanna Esposito
Congenital esophageal atresia with or without tracheoesophageal fistula (CEA ± TEF) is a relatively common malformation that occurs in 1 of 2500-4500 live births. Despite the refinement of surgical techniques, a considerable proportion of children experience short- and long-term respiratory complications, which can significantly affect their health through adulthood. This review focuses on the underlying mechanisms and clinical presentation of respiratory morbidity in children with repaired CEA ± TEF. The reasons for the short-term pulmonary impairments are multifactorial and related to the surgical complications, such as anastomotic leaks, stenosis, and recurrence of fistula...
September 27, 2017: International Journal of Environmental Research and Public Health
https://www.readbyqxmd.com/read/28920017/esophageal-atresia-with-tracheo-esophageal-fistula-presenting-beyond-7-days
#9
Nilesh Nagdeve, Mohini Sukhdeve, Tushar Thakre, Suresh Morey
AIM: To describe our experience of neonates with esophageal atresia with tracheo-esophageal fistula (EA with TEF) who presented after a week. DESIGN: Retrospective study of the patients of EA with TEF who presented after a week. STUDY SETTING: Department of Pediatric Surgery, Government Medical College Nagpur. Study Duration: Eight years. MATERIALS AND METHODS: Demographic information, hematological, biochemical and radiological data were obtained from the patients' medical records...
July 2017: Journal of Neonatal Surgery
https://www.readbyqxmd.com/read/28914345/comparison-of-clinical-outcomes-between-open-and-thoracoscopic-repair-for-esophageal-atresia-with-tracheoesophageal-fistula-a-systematic-review-and-meta-analysis
#10
Yuhao Wu, Hongyu Kuang, Tiewei Lv, Chun Wu
OBJECTIVE: A meta-analysis was performed for a comparison of outcomes between open repair (OR) and thoracoscopic repair (TR) for esophageal atresia with tracheoesophageal fistula (EA with TEF). METHODS: Electronic databases, including PubMed, Cochrane Library, and Medline, were searched systematically for the literatures aimed mainly at comparing the therapeutic effects for EA with TEF administrated by OR and TR. Corresponding data sets were extracted and two reviewers independently assessed the methodological quality...
September 15, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28878607/genetic-testing-in-a-cohort-of-complex-esophageal-atresia
#11
Eliane Beauregard-Lacroix, Jessica Tardif, Emmanuelle Lemyre, Zoha Kibar, Christophe Faure, Philippe M Campeau
The objective of the present study is to describe a cohort of complex esophageal atresia and the yield of genetic tests performed for such patients. We selected 45 patients with complex esophageal atresia (EA), namely those having at least one associated anomaly. We reviewed their medical records to assess clinical features, other diagnoses, and genetic investigations. Most of the patients had a diagnosis of VACTERL association (56%) with no genetic variant identified. Interestingly, 5 patients in the cohort (11%) had a right pulmonary hypoplasia or agenesis...
August 2017: Molecular Syndromology
https://www.readbyqxmd.com/read/28877681/fibronectin-glomerulopathy-complicated-with-persistent-cloaca-and-congenital-esophageal-atresia-a-case-report-and-literature-review
#12
Misaki Takii, Takaichi Suehiro, Aya Shima, Hideki Yotsueda, Satoshi Hisano, Ritsuko Katafuchi
BACKGROUND: Fibronectin glomerulopathy is a rare, inherited, autosomal dominant, glomerular disease characterized by proteinuria, microscopic hematuria, hypertension, massive glomerular deposits of fibronectin, and slow progression to end-stage renal failure. Because the incident of fibronectin glomerulopathy is extremely low, the pathophysiology, genetic abnormalities, epidemiology, and mechanisms remain to be elucidated. CASE PRESENTATION: We report a 21-year-old woman with fibronectin glomerulopathy, who had been diagnosed with persistent cloaca and congenital esophageal atresia at birth...
September 6, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28877007/creation-of-an-esophageal-atresia-animal-model-using-a-bifurcated-esophagus-to-maintain-digestive-tract-continuity
#13
Ian C Glenn, Nicholas E Bruns, Steve J Schomisch, Todd A Ponsky
INTRODUCTION: We previously developed a porcine model of long gap pure esophageal atresia (EA) to aid in the creation of novel devices and techniques for treatment of EA. Shortcomings of the model included difficulty decompressing the proximal esophageal pouch (leading to recurrent aspiration) and a requirement for gastrostomy tube (G-tube) feeds. Therefore, we sought to create a porcine model with a bifurcated esophagus wherein one portion of the esophageal lumen retained patency and the other part mimicked EA...
October 2017: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
https://www.readbyqxmd.com/read/28873491/no-tissue-expression-of-kras-or-braf-mutations-in-61-adult-patients-treated-for-esophageal-atresia-in-early-childhood
#14
Kien Xuan Dang, Tho Ho, Saara Sistonen, Antti Koivusalo, Mikko Pakarinen, Risto Rintala, Ulf-Hakan Stenman, Arto Orpana, Jakob Stenman
No abstract text is available yet for this article.
September 5, 2017: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28870218/respiratory-problems-in-children-with-esophageal-atresia-and-tracheoesophageal-fistula
#15
Federica Porcaro, Laura Valfré, Lelia Rotondi Aufiero, Luigi Dall'Oglio, Paola De Angelis, Alberto Villani, Pietro Bagolan, Sergio Bottero, Renato Cutrera
BACKGROUND: Children with congenital esophageal atresia (EA) and tracheoesophageal fistula (TEF) have chronic respiratory symptoms including recurrent pneumonia, wheezing and persistent cough. The aim of this study is to describe the clinical findings of a large group of children with EA and TEF surgically corrected and the instrumental investigation to which they have undergone in order to better understand the patient's needs and harmonize the care. METHODS: A retrospective data collection was performed on 105 children with EA and TEF followed at Department of Pediatric Medicine of Bambino Gesù Children's Hospital (Rome, Italy) between 2010 and 2015...
September 5, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28859370/management-of-recurrent-tracheoesophageal-fistula-after-esophageal-atresia-and-follow-up
#16
J Wang, M Zhang, W Pan, W Wu, W Yan, W Cai
Recurrent tracheoesophageal fistula (rTEF) is a complex complication after the repair of esophageal atresia (EA) and remains a challenge because of difficulties in preoperative management and the substantial rates of mortality and morbidity after reoperation. By reviewing a single institution's experience in the management of rTEF and assessing the outcome, we aimed to provide an optimal approach for managing rTEF and to evaluate growth and feeding problems after reoperations. The medical records of 35 patients with rTEF treated at a single institution from June 2012 to December 2015 were reviewed, and follow-up data were collected from all survivors...
September 1, 2017: Diseases of the Esophagus: Official Journal of the International Society for Diseases of the Esophagus
https://www.readbyqxmd.com/read/28831606/changes-in-quality-of-life-from-infancy-to-school-age-after-esophagoesophagostomy-for-tracheoesophageal-fistula-thoracotomy-versus-thoracoscopy
#17
Go Miyano, Shogo Seo, Hiroki Nakamura, Ryo Sueyoshi, Manabu Okawada, Takashi Doi, Hiroyuki Koga, Geoffrey J Lane, Atsuyuki Yamataka
BACKGROUND: We assessed the quality of life (QOL) of postoperative esophageal atresia (EA) with tracheoesophageal fistula (TEF) cases, comparing open with thoracoscopic repair. METHODS: A retrospective review of consecutive EA/TEF repairs (2001-2014) was performed, excluding cases with birth weight less than 2000 g and severe cardiac/chromosomal anomalies. Of 37 cases, 13 had thoracoscopic repair (TR) and 24 had open repair (OR) according to the operating surgeon's preference...
August 22, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28807203/utilizing-stricture-indices-to-predict-dilation-of-strictures-after-esophageal-atresia-repair
#18
Rachel M Landisch, Sheila Foster, David Gregg, Thomas Chelius, Laura D Cassidy, Diana Lerner, Dave R Lal
BACKGROUND: Anastomotic stricture is the most common postoperative complication in infants undergoing repair of esophageal atresia with or without tracheoesophageal fistula (EA/TEF). Stricture indices (SIs) are used to predict infants at risk for stricture requiring dilation. We sought to determine the most accurate SI and optimal timing for predicting anastomotic dilation. MATERIALS AND METHODS: A retrospective study of infants undergoing repair of EA/TEF between 2008 and 2013 was performed...
August 2017: Journal of Surgical Research
https://www.readbyqxmd.com/read/28803355/evaluation-of-developmental-prognosis-for-esophageal-atresia-with-tracheoesophageal-fistula
#19
Akiyoshi Nomura, Masaya Yamoto, Koji Fukumoto, Toshiaki Takahashi, Kei Ohyama, Akinori Sekioka, Yutaka Yamada, Naoto Urushihara
PURPOSE: This study investigated risk factors for developmental disorders after the repair of esophageal atresia with tracheoesophageal fistula (EA/TEF). METHODS: The clinical charts of all infants with EA/TEF (gross type C) treated at our institution between 1993 and 2013 were analyzed. Patients with chromosomal aberrations were excluded. Forty-seven patients were divided into groups according to the presence (D) or absence (N) of developmental disorders. Patients were assessed with appropriate developmental examinations at age >3 years...
August 12, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28801834/current-characteristics-and-management-of-congenital-esophageal-stenosis-40-consecutive-cases-from-a-multicenter-study-in-the-kyushu-area-of-japan
#20
Masaya Suzuhigashi, Tatsuru Kaji, Hiroyuki Noguchi, Mitsuru Muto, Michiko Goto, Motoi Mukai, Kazuhiko Nakame, Takafumi Kawano, Waka Yamada, Koji Yamada, Shun Onishi, Satoshi Ieiri
PURPOSE: Congenital esophageal stenosis (CES) is rare, and the available clinical data are limited. We explored the current diagnosis, treatment and outcomes of CES. METHODS: A questionnaire survey was performed using medical records at pediatric surgical centers in the Kyushu area. RESULTS: Over 10 years, 40 patients (24 males) had CES. The incidence of associated anomalies was 52.5% (21/40), and that of esophageal atresia was 20.0% (8/40)...
August 11, 2017: Pediatric Surgery International
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