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Tracheal atresia

Dave R Lal, Samir K Gadepalli, Cynthia D Downard, Daniel J Ostlie, Peter C Minneci, Ruth M Swedler, Thomas Chelius, Laura Cassidy, Cooper T Rapp, Katherine J Deans, Mary E Fallat, S Maria E Finnell, Michael A Helmrath, Ronald B Hirschl, Rashmi S Kabre, Charles M Leys, Grace Mak, Jessica Raque, Frederick J Rescorla, Jacqueline M Saito, Shawn D St Peter, Daniel von Allmen, Brad W Warner, Thomas T Sato
BACKGROUND/PURPOSE: Esophageal atresia/tracheoesophageal fistula (EA/TEF) is a rare congenital anomaly lacking contemporary data detailing patient demographics, medical/surgical management and outcomes. Substantial variation in the care of infants with EA/TEF may affect both short- and long-term outcomes. The purpose of this study was to characterize the demographics, management strategies and outcomes in a contemporary multi-institutional cohort of infants diagnosed with EA/TEF to identify potential areas for standardization of care...
December 5, 2016: Journal of Pediatric Surgery
Hae Soo Joung, Alexandra Leon Guerrero, Sandra Tomita, Keith A Kuenzler
Anorectal malformations (ARMs) commonly co-occur with other congenital anomalies, particularly VACTERL (vertebral, anorectal, cardiac, tracheal, esophageal, renal, limb, and duodenal) associations. However, this collection of associations is not comprehensive, and other concurrent anomalies may exist that can be missed during the standard work-up of patients with ARMs. We present a rare case of a neonate with a low ARM with concurrent jejuno-ileal atresia that was diagnosed after the correction of the ARM when the patient developed segmental volvulus...
October 2016: Journal of Neonatal Surgery
C Jason Smithers, Thomas E Hamilton, Michael A Manfredi, Lawrence Rhein, Peter Ngo, Dorothy Gallagher, John E Foker, Russell W Jennings
PURPOSE: Recurrent trachea-esophageal fistula (recTEF) is a frequent (5%-10%) complication of congenital TEF (conTEF) and esophageal atresia (EA) repair. In addition, postoperative acquired TEF (acqTEF) can occur in addition to or even in the absence of prior conTEF in the setting of esophageal anastomotic complications. Reliable repair often proves difficult by endoluminal or standard surgical techniques. We present the results of an approach that reliably identifies the TEF and facilitates airway closure as well as repair of associated tracheal and esophageal problems...
August 31, 2016: Journal of Pediatric Surgery
Jose Carlos Fraga, Russell W Jennings, Peter C W Kim
Tracheomalacia (TM) is defined as an increased collapsibility of the trachea due to structural anomalies of the tracheal cartilage and/or posterior membrane. Tracheomalacia has a wide range of etiologies but is most commonly present in children born with esophageal atresia and tracheal esophageal fistula. Clinical symptoms can range from minor expiratory stridor with typical barking cough to severe respiratory distress episodes to acute life-threatening events (ALTE). Although the majority of children have mild-to-moderate symptoms and will not need surgical intervention, some will need life-changing surgical treatment...
June 2016: Seminars in Pediatric Surgery
Aman Gupta, Chandrajeet Yadav, Sandeep Dhruw, Divyangi Mishra, Abhijeet Taori
INTRODUCTION: Congenital high airway obstruction syndrome (CHAOS) is a rare, usually lethal abnormality characterized by complete or near-complete intrinsic obstruction of the fetal airway. Laryngeal atresia is the most frequent cause, but other etiologies include laryngeal or tracheal webs, laryngeal cyst, subglottic stenosis or atresia, tracheal atresia and laryngeal or tracheal agenesis. When antenatal diagnosis of possible upper airway obstruction is made, specific type of obstruction is rarely determined making the term CHAOS introduced by Hedrick et al in 1994 more appropriate...
June 2016: Journal of Obstetrics and Gynaecology of India
Luv Javia, Matthew A Harris, Stephanie Fuller
Tracheal and bronchial pathologies in the neonate can be rapidly fatal if prenatal or quick postnatal diagnosis and intervention is not performed. Close multidisciplinary collaboration between multiple medical and surgical specialties is vital to the effective diagnosis and treatment of these pathologies. The fetal and neonatal airway may be affected or compromised by more prevalent pathologies such as tracheomalacia and tracheo-esophageal fistula with esophageal atresia. However, it is imperative that we also consider other potential sources that may perhaps be less familiar such as congenital cardiovascular abnormalities, tracheal stenosis, complete tracheal rings, tracheal sleeve, and foregut duplication cysts...
August 2016: Seminars in Fetal & Neonatal Medicine
Kristen M Rappazzo, Joshua L Warren, Robert E Meyer, Amy H Herring, Alison P Sanders, Naomi C Brownstein, Thomas J Luben
BACKGROUND: Birth defects are responsible for a large proportion of disability and infant mortality. Exposure to a variety of pesticides have been linked to increased risk of birth defects. METHODS: We conducted a case-control study to estimate the associations between a residence-based metric of agricultural pesticide exposure and birth defects. We linked singleton live birth records for 2003 to 2005 from the North Carolina (NC) State Center for Health Statistics to data from the NC Birth Defects Monitoring Program...
April 2016: Birth Defects Research. Part A, Clinical and Molecular Teratology
Carol Xie, Sujata De, Andrew Selby
The management of the airway in Apert syndrome is complex and multidisciplinary. This rare syndrome, occurring in up to approximately 1 in 65,000 live births, results in airway compromise at various anatomic levels, in addition to abnormal central respiratory drive. Obstructive apneas arise because of decreased airway caliber, which may occur in the form of congenital bony nasal stenosis, choanal atresia, a deviated nasal septum, a narrowed nasopharynx, a thick long soft palate, lateral palatal swellings, and a tracheal cartilage sleeve...
January 2016: Journal of Craniofacial Surgery
Danielle Mc Laughlin, Paula Murphy, Prem Puri
BACKGROUND: Tracheo-oesophageal malformations result from disturbed foregut separation during early development. The notochord, a specialised embryonic structure, forms immediately adjacent to the dividing foregut. In the Adriamycin mouse model of oesophageal atresia, foregut and notochord abnormalities co-exist, and the site and severity of foregut malformations closely correlate to the position and extent of the notochord defects. Notochord and foregut abnormalities also co-exist in the Noggin Knockout mouse as well in a small number of human cases...
January 2016: Pediatric Surgery International
Kirsten Gaerty, Joseph T Thomas, Scott Petersen, Edwin Tan, Sailesh Kumar, Glenn Gardener, Jane Armes
An unusual anatomic configuration of segmental tracheal agenesis/atresia with esophageal duplication on autopsy in a fetus that demised in utero at 29 weeks is reported. The mother was scanned initially for a cardiac anomaly at 20 weeks and on follow-up scan at 27 weeks had polyhydramnios and underwent amnioreduction. The final autopsy diagnosis was vertebral, ano-rectal, cardiac, tracheoesophageal, renal, and limb malformations (VACTERL). We discuss the autopsy findings along with the embryological mechanisms and compare the configuration with Floyd's classification for tracheal agenesis...
March 2016: Pediatric and Developmental Pathology
Alina C Hilger, Jan Halbritter, Tracie Pennimpede, Amelie van der Ven, Georgia Sarma, Daniela A Braun, Jonathan D Porath, Stefan Kohl, Daw-Yang Hwang, Gabriel C Dworschak, Bernhard G Hermann, Anna Pavlova, Osman El-Maarri, Markus M Nöthen, Michael Ludwig, Heiko Reutter, Friedhelm Hildebrandt
The VATER/VACTERL association describes the combination of congenital anomalies including vertebral defects, anorectal malformations, cardiac defects, tracheoesophageal fistula with or without esophageal atresia, renal malformations, and limb defects. As mutations in ciliary genes were observed in diseases related to VATER/VACTERL, we performed targeted resequencing of 25 ciliary candidate genes as well as disease-associated genes (FOXF1, HOXD13, PTEN, ZIC3) in 123 patients with VATER/VACTERL or VATER/VACTERL-like phenotype...
December 2015: Human Mutation
Deborah Snijders, Angelo Barbato
Congenital tracheomalacia is the most common congenital tracheal abnormality and occurs in approximately 1:2,100 children. Tracheomalacia can be isolated or associated with other airway anomalies such as laryngomalacia, bronchomalacia, and large laryngeal clefts. Also, an abnormal division of the embryonic foregut is frequently associated with congenital tracheomalacia; mostly in the form of proximal esophageal atresia with distal tracheoesophageal fistula. In such cases, the ratio between the cartilage ring and the posterior membranous wall drops from the normal ratio of 4 to 5:1 to 2 to 3:1...
August 2015: European Journal of Pediatric Surgery
Sigrid Bairdain, Charles Jason Smithers, Thomas E Hamilton, David Zurakowski, Lawrence Rhein, John E Foker, Christopher Baird, Russell W Jennings
PURPOSE: Tracheobronchomalacia (TBM) is associated with esophageal atresia, tracheoesophageal fistulas, and congenital heart disease. TBM results in chronic cough, poor mucous clearance, and recurrent pneumonias. Apparent life-threatening events or recurrent pneumonias may require surgery. TBM is commonly treated with an aortopexy, which indirectly elevates trachea's anterior wall. However, malformed tracheal cartilage and posterior tracheal membrane intrusion may limit its effectiveness...
June 2015: Journal of Pediatric Surgery
Eiji Nishijima
Surgical strategies and treatment for pediatric airway and lung diseases have advanced significantly, especially in the fields of subglottic stenosis, congenital tracheal stenosis, congenital cystic lung lesions, and endoscopic surgery, during the past 50 years. Pediatricians, pathologists, and pediatric surgeons have engaged in continuous discussions at scientific meetings to establish standard terminology and operative indications and to refine surgical techniques such as laryngoplasty, sliding tracheoplasty, lobectomy under thoracotomy and thoracoscopy...
November 2014: Nihon Geka Gakkai Zasshi
Cameron Stock, Meena Nathan, Ryan Murray, Reza Rahbar, Francis Fynn-Thompson
An infant with a ventricular septal defect; Vertebral anomalies, Anal atresia, Cardiac anomalies, Tracho Esophageal fistula (TEF), Renal anomalies, Limb anomalies syndrome; and tracheal stenosis with a bridging bronchus underwent repair of the ventricular septal defect and trachea-bronchial reconstruction at age 11 months. Herein we describe our surgical approach to resection of the bridging bronchus and a technique using a modified end-to-end tracheal anastomosis for the correction of this complex anomaly.
January 2015: Annals of Thoracic Surgery
Jetanachai Pravit
BACKGROUND: Down syndrome (DS) can affect the upper and lower respiratory tract in a number of ways and disorders of other systems can impact upon respiratory function, giving rise to a wide variety of respiratory manifestations. OBJECTIVE: To investigate the frequency, associated conditions, and type of airway anomalies in DS children with respiratory problems. MATERIAL AND METHOD: Twenty-nine children with DS were evaluated for airway anomalies, with indications of atelectasis, stridor, and recurrent or persistent pneumonia, using flexible fiberoptic bronchoscopy (FB)...
June 2014: Journal of the Medical Association of Thailand, Chotmaihet Thangphaet
Candace Wooten, Caius Doros, Gratian D Miclaus, Petru Matusz, Marios Loukas
Bilateral tracheal bronchus is a rare variation of the tracheobronchial tree. We present a 1-year 7-month-old male patient who presented with sepsis following endotracheal intubation. Upon review of multidetector computed tomography images, the patient was diagnosed with displaced bilateral tracheal bronchus. Imaging showed a right-sided anomalous bronchus arising 0.9 cm proximal to the carina. The left-sided anomalous bronchus arose 0.7 cm proximal to the carina, mimicking a tracheal trifurcation. When viewed together, the close proximity of both the right and left tracheal bronchi to the carina created a distinct tracheal quadrifurcation...
August 2015: Surgical and Radiologic Anatomy: SRA
Hiroyuki Koga, Masaya Yamoto, Tadaharu Okazaki, Manabu Okawada, Takashi Doi, Go Miyano, Koji Fukumoto, Geoffrey J Lane, Naoto Urushihara, Atsuyuki Yamataka
AIM: We assessed the outcome of 72 cases of type-C esophageal atresia (EAc) treated by open (OR) or thoracoscopic (TR) repair from 2000 to 2013. During OR, direct lung retraction (DLR) is required but during TR, CO2 insufflation causes lung collapse so DLR is unnecessary. METHODS: Patient demographics, operative time, respiratory tract impact (RTI: incidence of atelectasis, timing of extubation, need for reintubation, and duration of chest tube insertion), narcotic usage, commencement of oral feeding, and length of hospitalization (LOH) were compared...
December 2014: Pediatric Surgery International
V Hochart, P Verpillat, C Langlois, C Garabedian, J Bigot, V Houfflin Debarge, R Sfeir, F E Avni
OBJECTIVE: Present challenges are to improve the diagnosis rate of oesophageal atresia (OA) and evaluate as completely as possible a fetus affected by OA, specifically the type of OA and the length of the gap. Our aim was to evaluate the accuracy of fetal MR imaging (fMRI) for diagnosis of OA. METHODS: We reviewed fMRI performed because of sonographic suspicion of an OA. The signs reviewed included stomach size, "pouch sign", bowing of the trachea and visualization of the lower oesophageal lumen...
February 2015: European Radiology
BangDe Xue, BeiBei Liang, Shunmin Wang, LiMin Zhu, ZhaoHui Lu, Zhiwei Xu
OBJECTIVES: Although the outcome of surgical treatment of congenital tracheal stenosis (CTS) has improved, surgical intervention for these patients, especially for those with associated congenital heart disease (CHD) remains challenging. In this report, we summarized our experience with these defects. METHODS: Clinical data were collected for children undergoing tracheoplasty and CHD surgery from January 2001 to March 2013. Bivariate and multiple regression analyses were used to determine the correlation between the variables...
January 2015: Journal of Cardiac Surgery
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