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Journal of Cardiothoracic Surgery Mar 2019Congenital tracheal malformations are less common than congenital cardiac diseases and surgical repair of these anomalies is complex. We sought to examine the surgical...
BACKGROUND
Congenital tracheal malformations are less common than congenital cardiac diseases and surgical repair of these anomalies is complex. We sought to examine the surgical treatment and outcomes in cases of tracheal anomalies presenting with or without associated congenital malformations.
METHODS
We retrospectively reviewed the demographic, clinical, and imaging data of 49 children who underwent surgery for congenital tracheal malformations between August 2013 and September 2017. Data were collected from the hospital records.
RESULTS
In all, 49 patients (male, 30; female, 19) underwent surgeries at our center. The children were of ages between 3 and 36 months (average: 9.7 months). Associated congenital lesions included sling in31/49 (63%), vascularring: in 2/49; ventriculoseptaldefectin5/49; Fallot's tetraology in 2/49 (4.1%), and imperforate anus in 3/49 (6.1%). The outcomes of surgery were excellent in 42(85.7%) cases, good in 3 cases, while mortality occurred in 4(8.1%) cases. All cases of tracheal stenosis without any change in tracheobronchial arborization, 10/12 cases of bridge carina, and all cases of tripod carina were reconstructed using the slide tracheoplasty technique. Antetracheal translocation was performed for correction of associated pulmonary sling, without reimplantation of the pulmonary artery.
CONCLUSIONS
Reconstructive surgery is a feasible treatment option for congenital tracheal malformations. Slide tracheoplasty can be safely applied in all cases for the correction of tracheal stenosis. Segment resection was not required for any portion of the trachea. Pulmonary artery translocation is safe and effective for patients with pulmonary artery sling, rather than reimplantation. Mortality was associated with severe cardiac complications.
Topics: Child, Preschool; Female; Heart Defects, Congenital; Humans; Infant; Male; Pulmonary Artery; Plastic Surgery Procedures; Retrospective Studies; Trachea; Tracheal Stenosis; Treatment Outcome; Vascular Malformations; Vascular Surgical Procedures
PubMed: 30823928
DOI: 10.1186/s13019-019-0858-2 -
Italian Journal of Pediatrics Jan 2018The aim of this study was to define different characteristics of infants with esophageal atresia and correlations with neonatal level of care, morbidity and mortality... (Comparative Study)
Comparative Study
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).
RESULTS
We recruited 67 infants with EA (with or without tracheoesophageal fistula), distributed in groups as follows: A 31.3%, B 16.4%, C 26.8% and D 25.3%. Type of atresia was not statistically different among different groups. Mortality was higher in groups C and D, especially if associated with congenital heart defects. In survivors, we found different auxological evolution and prognostic profiles considering duration in days of invasive mechanical ventilation and total parenteral nutrition, as well as length of stay and corrected gestational age at discharge.
CONCLUSIONS
In the context of genetic and syndromic entities, subjects with VACTERL association showed a lower mortality rate although a higher and more complex level of intensive care was noted in comparison to infants without VACTERL genetic and syndromic entities.
Topics: Abnormalities, Multiple; Anal Canal; Cohort Studies; Databases, Factual; Esophageal Atresia; Esophagus; Female; Genetic Predisposition to Disease; Gestational Age; Heart Defects, Congenital; Hospital Mortality; Humans; Infant, Newborn; Intensive Care Units, Neonatal; Kidney; Length of Stay; Limb Deformities, Congenital; Male; Prognosis; Retrospective Studies; Risk Assessment; Spine; Survival Analysis; Trachea
PubMed: 29373986
DOI: 10.1186/s13052-018-0445-5 -
Orphanet Journal of Rare Diseases Mar 2024In this study we aimed to describe the morphological and pathogenetic differences between tracheal agenesis and tracheal atresia, which are not clearly distinguished...
BACKGROUND
In this study we aimed to describe the morphological and pathogenetic differences between tracheal agenesis and tracheal atresia, which are not clearly distinguished from each other in the literature, and to contribute thereby to the understanding and management of these conditions. Both tracheal agenesis and tracheal atresia represent rare disorders of still unknown aetiology that cannot be detected by prenatal ultrasound. If the affected foetuses survive until birth these conditions result in respiratory failure and in futile attempts to rescue the infant's life.
RESULTS
Autopsies and genetic analyses, including singleton or trio exome sequencing, were performed on five neonates/foetuses with tracheal agenesis and three foetuses with tracheal atresia. Tracheal agenesis was characterized by absence of the sublaryngeal trachea and presence of a bronchooesophageal fistula and by pulmonary isomerism and occurred as an isolated malformation complex or as part of a VACTERL association. Special findings were an additional so-called 'pig bronchus' and a first case of tracheal agenesis with sirenomelia. Tracheal atresia presenting with partial obliteration of its lumen and persistence of a fibromuscular streak resulted in CHAOS. This condition was associated with normal lung lobulation and single, non-VACTERL type malformations. Trio ES revealed a novel variant of MAPK11 in one tracheal agenesis case. Its involvement in tracheooesophageal malformation is herein discussed, but remains hypothetical.
CONCLUSION
Tracheal agenesis and tracheal atresia represent different disease entities in terms of morphology, pathogenesis and accompanying anomalies due to a primary developmental and secondary disruptive possibly vascular disturbance, respectively.
Topics: Infant, Newborn; Pregnancy; Female; Humans; Trachea; Constriction, Pathologic; Esophagus; Limb Deformities, Congenital
PubMed: 38475835
DOI: 10.1186/s13023-024-03106-z -
American Journal of Respiratory and... Nov 2019Complete tracheal ring deformity (CTRD) is a rare congenital abnormality of unknown etiology characterized by circumferentially continuous or nearly continuous...
Complete tracheal ring deformity (CTRD) is a rare congenital abnormality of unknown etiology characterized by circumferentially continuous or nearly continuous cartilaginous tracheal rings, variable degrees of tracheal stenosis and/or shortening, and/or pulmonary arterial sling anomaly. To test the hypothesis that CTRD is caused by inherited or mutations in genes required for normal tracheal development. CTRD and normal tracheal tissues were examined microscopically to define the tracheal abnormalities present in CTRD. Whole-exome sequencing was performed in children with CTRD and their biological parents ("trio analysis") to identify gene variants in patients with CTRD. Mutations were confirmed by Sanger sequencing, and their potential impact on structure and/or function of encoded proteins was examined using human gene mutation databases. Relevance was further examined by comparison with the effects of targeted deletion of murine homologs important to tracheal development in mice. The trachealis muscle was absent in all of five patients with CTRD. Exome analysis identified six , three recessive, and multiple compound-heterozygous or rare hemizygous variants in children with CTRD. variants were identified in (Sonic Hedgehog), and inherited variants were identified in (perlecan), (receptor tyrosine kinase-like orphan receptor 2), and (Wntless), genes involved in morphogenetic pathways known to mediate tracheoesophageal development in mice. The results of the present study demonstrate that absence of the trachealis muscle is associated with CTRD. Variants predicted to cause disease were identified in genes encoding Hedgehog and Wnt signaling pathway molecules, which are critical to cartilage formation and normal upper airway development in mice.
Topics: Animals; Cohort Studies; Disease Models, Animal; Humans; Mice; Mutation; Respiratory System Abnormalities; Trachea
PubMed: 31215789
DOI: 10.1164/rccm.201809-1626OC -
Developmental Biology Sep 2012Separation of the single anterior foregut tube into the esophagus and trachea involves cell proliferation and differentiation, as well as dynamic changes in cell-cell... (Review)
Review
Separation of the single anterior foregut tube into the esophagus and trachea involves cell proliferation and differentiation, as well as dynamic changes in cell-cell adhesion and migration. These biological processes are regulated and coordinated at multiple levels through the interplay of the epithelium and mesenchyme. Genetic studies and in vitro modeling have shed light on relevant regulatory networks that include a number of transcription factors and signaling pathways. These signaling molecules exhibit unique expression patterns and play specific functions in their respective territories before the separation process occurs. Disruption of regulatory networks inevitably leads to defective separation and malformation of the trachea and esophagus and results in the formation of a relatively common birth defect, esophageal atresia with or without tracheoesophageal fistula (EA/TEF). Significantly, some of the signaling pathways and transcription factors involved in anterior foregut separation continue to play important roles in the morphogenesis of the individual organs. In this review, we will focus on new findings related to these different developmental processes and discuss them in the context of developmental disorders or birth defects commonly seen in clinics.
Topics: Animals; Body Patterning; Esophagus; Gene Expression Regulation, Developmental; Humans; Signal Transduction; Trachea; Transcription Factors
PubMed: 22750256
DOI: 10.1016/j.ydbio.2012.06.016 -
Nicotine & Tobacco Research : Official... Dec 2020Electronic cigarettes (e-cigs) are currently used by millions of adults and adolescents worldwide. Major respiratory symptoms, such as coughing reported by e-cig users,...
INTRODUCTION
Electronic cigarettes (e-cigs) are currently used by millions of adults and adolescents worldwide. Major respiratory symptoms, such as coughing reported by e-cig users, including patients with e-cig, or vaping, product use-associated lung injury (EVALI), indicate e-cig constituent-induced sensory irritation. However, e-cig constituent-induced nociceptive activity in nasal and tracheal respiratory epithelia (RE) and neuronal activation in the trigeminal ganglia and brainstem nuclei, which receive airway chemosensory inputs have not been examined and compared. Comparisons of physiological responses between freebase nicotine and nicotine salts are also missing.
AIMS AND METHODS
Event-related potential (ERP) was recorded electrophysiologically to assess mouse nasal and tracheal RE chemosensory responses to various flavorings, nicotine, including freebase and nicotine salts, e-liquid mixtures, and tussigenic stimuli. Also, mice were subjected to inhalation exposure to aerosol of a vanilla-flavored e-liquid or air (control), and the activated-trigeminal nociceptive neurons and brainstem neurons were examined using immunohistochemistry.
RESULTS
Individual constituents and mixtures of e-liquids, capsaicin, and citric and acetic acids evoked significantly larger ERP in the nose than in the trachea with the exception of menthol. ERP responses to freebase nicotine were significantly larger than protonated nicotine. Four nicotine salts (benzoate, lactate, levulinate, and salicylate) induced similar responses. Compared with air-exposed mice, e-liquid aerosol-exposed mice showed a significant increase in numbers of activated trigeminal nociceptive neurons and brainstem neurons in the spinal trigeminal nucleus, paratrigeminal nucleus, and nucleus tractus solitarius.
CONCLUSIONS
E-liquid constituents region-dependently stimulate airway nociceptive chemosensory systems, and freebase nicotine is more potent than protonated nicotine.
IMPLICATIONS
Neural abnormalities have been implicated in the development of nasal and respiratory illnesses. The higher sensitivity of the nasal nociceptive chemosensory system to nicotine and flavorings may indicate a health risk for e-liquid aerosol-induced upper airway illnesses via neurogenic alteration and warrants further investigation.
Topics: Animals; Electronic Nicotine Delivery Systems; Female; Flavoring Agents; Irritants; Male; Mice; Mice, Inbred C57BL; Nasal Mucosa; Nicotine; Sensory Thresholds; Trachea
PubMed: 33320249
DOI: 10.1093/ntr/ntaa174 -
Cardiovascular Ultrasound Jan 2021The purpose of this study was to explore echocardiographic views and methods of aortic arch anomalies in infants, so as to improve the screening sensitivity and...
BACKGROUND
The purpose of this study was to explore echocardiographic views and methods of aortic arch anomalies in infants, so as to improve the screening sensitivity and diagnostic accuracy.
METHODS
140 children with abnormal aortic arch diagnosed by ultrasound in Children's Hospital of Hebei Province from January 2014 to December 2019 were selected for retrospective analysis. All were confirmed by surgery or/and computerized tomography angiography. Series of views for aortic arch (the three-vessel and tracheal view, aortic arch short axis view, left aortic arch long axis view, aortic arch long axis continuous scan views) were performed in all cases on the basis of the routine views of echocardiography. The screening sensitivity and diagnostic coincidence rate of different echocardiographic views for aortic arch anomalies were analyzed.
RESULTS
Among the 140 infants, right aortic arch were 21 cases (6/21 were accompanied by mirror branch and 15/21 were with aberrant left subclavian artery). Left aortic arch with aberrant right subclavian artery were 2 cases, and double aortic arch with both arches open were 20 cases. Double aortic arch with left arch atresia were 2 cases, and atresia of the proximal aorta with aortic arch dysplasia was 1 case. Coarctation of the aorta were 67 cases, and interruption of aortic arch were 27 cases. All the patients were correctly diagnosed except that 2 infants with interruption of aortic arch were incorrectly diagnosed as coarctation of the aorta, and 1 infant with coarctation of the aorta was misdiagnosed as interruption of aortic arch by echocardiography. The screening sensitivities of four views and four-view combination for abnormal aortic arch were 99.3, 73.6, 87.1, 99.3, and 100%; the diagnostic coincidence rates were 85.7, 27.1,66.4, 95.0%, and 97.9% respectively. On the basis of traditional left aortic long axis view, other three views had their own advantages. The screening sensitivity and diagnostic coincidence rate of four-view combination were significantly improved.
CONCLUSIONS
The three-vessel trachea view is simple and feasible, which is suitable for screening abnormal aortic arch. The combination of four views conduces to improving screening sensitivity and diagnostic accuracy of aortic arch abnormalities.
Topics: Aorta, Thoracic; Echocardiography; Female; Follow-Up Studies; Humans; Infant; Infant, Newborn; Male; Reproducibility of Results; Retrospective Studies; Vascular Malformations
PubMed: 33446185
DOI: 10.1186/s12947-021-00237-2 -
Translational Pediatrics Apr 2022Pulmonary artery sling (PAS) is associated with tracheal stenosis and left pulmonary artery (LPA) dysplasia in infants, both developmental abnormalities that may lead to...
BACKGROUND
Pulmonary artery sling (PAS) is associated with tracheal stenosis and left pulmonary artery (LPA) dysplasia in infants, both developmental abnormalities that may lead to pulmonary hypoplasia and lung volume changes. As such, we aimed to monitor the effects of tracheal stenosis and pulmonary vascular malformation on lung volumes in infants with PAS and their correlation with lung volumes in infants with PAS using dual-source computed tomography (DSCT).
METHODS
A case-control study was performed. From May 2009 to June 2017, we retrospectively enrolled patients with surgically confirmed PAS and compared them to matched normal controls (A healthy control group comprising age- and gender-matched patients with adequate imaging data was used for the comparisons.). All the patients underwent DSCT examinations. We measured and compared the diameters of the trachea, main bronchus, and main pulmonary artery (MPA) and its branches, and both lung volumes on the axial, and reconstructed CT images.
RESULTS
There were no statistical differences in the diameters of the MPA or right pulmonary artery (RPA) between patients (N=15) and controls (N=28). The diameter of the main bronchus, the bilateral trachea and the left pulmonary artery were all smaller in the PAS group than in the control group, and significant differences were evident in the left lung volume the right lung volume, and the right-to-left lung volume ratio between the 2 groups. Pearson's correlation and linear regression analyses between the diameters of the trachea and MPA, total lung volume, ipsilateral bronchial and pulmonary artery branches, and ipsilateral lung volume ranged from 0.71 to 0.87 and 0.57 to 0.77 for the control and PAS groups, respectively.
CONCLUSIONS
Tracheal stenosis and LPA dysplasia in infants with PAS cause alterations in lung tissue morphology and physiological development, resulting in reduced bilateral lung volumes.
PubMed: 35558972
DOI: 10.21037/tp-22-87 -
Respiratory Care Sep 2004
Topics: Aged; Bronchi; Bronchography; Bronchoscopy; Congenital Abnormalities; Female; Fiber Optic Technology; Follow-Up Studies; Hemoptysis; Humans; Risk Assessment; Severity of Illness Index; Tomography, X-Ray Computed; Trachea
PubMed: 15329178
DOI: No ID Found -
Canadian Family Physician Medecin de... Oct 2006To propose a diagnostic and therapeutic approach to feeding problems in early childhood. (Review)
Review
OBJECTIVE
To propose a diagnostic and therapeutic approach to feeding problems in early childhood.
QUALITY OF EVIDENCE
Articles were retrieved through a MEDLINE search from January 1990 to December 2005 using the MeSH terms eating disorders, infant, and child. Recommended practice is based mainly on levels II and III evidence.
MAIN MESSAGE
Feeding problems are classified under structural abnormalities, neurodevelopmental disabilities, and behavioural disorders, with overlap between categories. A medical approach also needs an evaluation of diet and an assessment of the interaction between parent and child. Treating medical or surgical conditions, increasing caloric intake, and counseling about general nutrition can alleviate mild to moderate problems. More complicated cases should be referred to multidisciplinary teams. Behavioural therapy aims to foster appropriate behaviour and discourage maladaptive behaviour.
CONCLUSION
Feeding problems in early childhood often have multifactorial causes and a substantial behavioural component. Family physicians have a key role in detecting problems, offering advice, managing mildly to moderately severe cases, and referring more complicated cases to multidisciplinary teams.
Topics: Behavior Therapy; Child Behavior Disorders; Child, Preschool; Developmental Disabilities; Eating; Esophagus; Feeding and Eating Disorders; Humans; Infant; Larynx; Medical History Taking; Mouth Abnormalities; Physical Examination; Trachea
PubMed: 17279184
DOI: No ID Found