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1.
Am J Emerg Med ; 85: 52-58, 2024 Aug 14.
Artículo en Inglés | MEDLINE | ID: mdl-39232455

RESUMEN

BACKGROUND: Chest X-ray, the established standard of confirming endotracheal tube (ETT) position, has important drawbacks including radiation exposure. Point-of-care airway ultrasound, which has been insufficiently studied in children, can overcome these problems. MATERIALS AND METHODS: This was a prospective cross-sectional study done on children aged 2 months to 17 years undergoing intubation with cuffed ETT in the PICU. The ETT cuff was filled with saline and three ultrasonographic techniques were used- 1) Suprasternal (SS) method 2) Cricoid (CC) metho and 3) Tracheal ring (TR) method. Position of the ETT as determined by ultrasound and X-ray were compared. The main outcomes were sensitivity, specificity, and area under curve (AUC) for ultrasound-based methods vs. X-ray. For the TR method, concordance between the X-ray and ultrasound categories were taken. RESULTS: Total 62 patients were enrolled. The sensitivity and specificity of SS method were 71% (95% CI: 57-83%) and 100% (40-100%). The CC distance method had an AUC of 0.94 (95% CI: 0.86, 1.0). In the TR method, 98% of correct position on X-ray were correctly classified by USG. The agreement between X-ray and ultrasound categories with the cuff between the first and third tracheal rings, was very good [kappa (95% CI): 0.87 (0.70, 1.00), p ≤0.001)]. CONCLUSION: Bedside ultrasound is a good method to confirm ETT depth in children. The tracheal ring method had the best diagnostic accuracy and is easy to perform. The new method using cricoid cuff distance needs further validation in different ICU settings.

2.
Paediatr Anaesth ; 31(12): 1310-1315, 2021 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-34608715

RESUMEN

BACKGROUND: Anatomically, the subglottic area and the cricoid ring are the narrowest portions of the larynx. To limit the potential for damage related to mucosal pressure injuries from the presence of an endotracheal tube, the cuff should be placed below the cricoid in children. Previously, no clinical or imaging method has been used in real time to determine the exact location of the endotracheal tube cuff after endotracheal intubation. Point-of-care ultrasound may provide an option as a safe and rapid means of visualizing the endotracheal tube cuff and its relationship to the cricoid ring thereby achieving ideal endotracheal tube cuff positioning-below the cricoid. METHODS: In this prospective, nonrandomized trial, point-of-care ultrasound was used following endotracheal intubation in children to evaluate the position of the endotracheal tube cuff in relationship to the cricoid and tracheal rings. After anesthesia was induced and the trachea was intubated, the endotracheal tube cuff and its position in relation to the cricoid and tracheal rings were identified in the longitudinal plane using point-of-care ultrasound. With the patient's neck in a neutral position, the level of the proximal (cephalad) margin of the saline-filled cuff of the endotracheal tube was identified and recorded in relationship to the cricoid and tracheal rings. The ideal position is defined as the cephalad margin of the endotracheal tube cuff below the level of the cricoid. RESULTS: The study cohort included 80 patients, ranging in age from 1 to 78 months. In all patients, the cuff of the ETT, cricoid, and tracheal rings were identified. The cephalad end of the endotracheal tube cuff was found at the level of the cricoid in 16.3% of patients, at the first tracheal ring in 27.5% of patients, at the second tracheal ring in 23.8% of patients, at the third tracheal ring in 17.5% of patients, and at below the fourth tracheal ring in 15% of patients. Initial endotracheal tube cuff position had no significant association with age, height, weight, endotracheal tube size, and endotracheal tube type. CONCLUSION: Point-of-care ultrasound provides a rapid and effective means of identifying the position of the endotracheal tube cuff in relationship to the cricoid ring. The technique may have applications in the perioperative arena, emergency departments, and intensive care units.


Asunto(s)
Intubación Intratraqueal , Sistemas de Atención de Punto , Niño , Preescolar , Humanos , Lactante , Estudios Prospectivos , Tráquea/diagnóstico por imagen , Ultrasonografía
3.
Proc Natl Acad Sci U S A ; 115(41): 10209-10217, 2018 10 09.
Artículo en Inglés | MEDLINE | ID: mdl-30249637

RESUMEN

In its most basic conception, a novelty is simply something new. However, when many previously proposed evolutionary novelties have been illuminated by genetic, developmental, and fossil data, they have refined and narrowed our concept of biological "newness." For example, they show that these novelties can occur at one or multiple levels of biological organization. Here, we review the identity of structures in the avian vocal organ, the syrinx, and bring together developmental data on airway patterning, structural data from across tetrapods, and mathematical modeling to assess what is novel. In contrast with laryngeal cartilages that support vocal folds in other vertebrates, we find no evidence that individual cartilage rings anchoring vocal folds in the syrinx have homology with any specific elements in outgroups. Further, unlike all other vertebrate vocal organs, the syrinx is not derived from a known valve precursor, and its origin involves a transition from an evolutionary "spandrel" in the respiratory tract, the site where the trachea meets the bronchi, to a target for novel selective regimes. We find that the syrinx falls into an unusual category of novel structures: those having significant functional overlap with the structures they replace. The syrinx, along with other evolutionary novelties in sensory and signaling modalities, may more commonly involve structural changes that contribute to or modify an existing function rather than those that enable new functions.


Asunto(s)
Evolución Biológica , Aves/anatomía & histología , Aves/fisiología , Tráquea/anatomía & histología , Animales , Fósiles , Laringe/anatomía & histología , Laringe/fisiología , Filogenia , Sistema Respiratorio/anatomía & histología , Tráquea/fisiología , Pliegues Vocales , Vocalización Animal
4.
Am J Respir Crit Care Med ; 200(10): 1267-1281, 2019 11 15.
Artículo en Inglés | MEDLINE | ID: mdl-31215789

RESUMEN

Rationale: 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.Objectives: To test the hypothesis that CTRD is caused by inherited or de novo mutations in genes required for normal tracheal development.Methods: 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.Measurements and Main Results: The trachealis muscle was absent in all of five patients with CTRD. Exome analysis identified six de novo, three recessive, and multiple compound-heterozygous or rare hemizygous variants in children with CTRD. De novo variants were identified in SHH (Sonic Hedgehog), and inherited variants were identified in HSPG2 (perlecan), ROR2 (receptor tyrosine kinase-like orphan receptor 2), and WLS (Wntless), genes involved in morphogenetic pathways known to mediate tracheoesophageal development in mice.Conclusions: 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.


Asunto(s)
Mutación/genética , Anomalías del Sistema Respiratorio/genética , Tráquea/anomalías , Animales , Estudios de Cohortes , Modelos Animales de Enfermedad , Humanos , Ratones , Anomalías del Sistema Respiratorio/diagnóstico , Anomalías del Sistema Respiratorio/cirugía
5.
Molecules ; 25(4)2020 Feb 13.
Artículo en Inglés | MEDLINE | ID: mdl-32069801

RESUMEN

Inhalation of vapors from a hot tea of Eucalyptus camaldulensis Dehnh. leaves is considered by Iraqi-Kurdistan people an effective spasmolytic and antipyretic remedy for the treatment of respiratory diseases. The constituents of volatile fractions isolated by hydrodistillation from dried leaves of the plant collected in Kurdistan were determined by GC-FID and GC-MS analyses. More than 90% components were identified. The most abundant constituents were 1,8-cineole, p-cymene, α-pinene, terpinen-4-ol, aromadendrene, and α-terpineol. The different volatile fractions induced relaxation on rat isolated aortic and tracheal rings in concentration-dependent manner. These effects appeared to be due to a complex interaction between various terpenoid components rather than being only due to the main oil constituent, 1,8-cineole. The KCa channel and the NO pathway were not significantly involved in the relaxation mechanism, while Ca2+ channels played a major role in the spasmolytic effects.


Asunto(s)
Eucalyptus/química , Extractos Vegetales/química , Hojas de la Planta/química , Animales , Aorta/efectos de los fármacos , Monoterpenos Bicíclicos/análisis , Dióxido de Carbono/química , Cromatografía de Gases , Monoterpenos Ciclohexánicos/análisis , Cimenos/análisis , Eucaliptol/análisis , Cromatografía de Gases y Espectrometría de Masas , Irak , Masculino , Extractos Vegetales/farmacología , Ratas , Terpenos/análisis , Tráquea/efectos de los fármacos
6.
Cardiol Young ; 29(3): 445-447, 2019 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-30698130

RESUMEN

Congenital complete tracheal rings are usually associated with pulmonary slings. We report a rare association of congenital complete tracheal rings with hypoplastic left heart variant. A term infant with diagnosis of a mildly hypoplastic mitral valve, unicuspid aortic valve, and moderately hypoplastic aortic arch with severe coarctation underwent a hybrid procedure initially. Upon failing extubation attempts, complete tracheal rings were seen on direct laryngoscopy. The combination of the lesions resulted in a poor outcome. In patients with failure of extubation post-cardiac surgery, a diagnosis of complete tracheal rings should be included in the differential and a direct laryngoscopy should be considered.


Asunto(s)
Anomalías Múltiples , Síndrome del Corazón Izquierdo Hipoplásico/diagnóstico , Tráquea/anomalías , Enfermedades de la Tráquea/congénito , Broncoscopía , Procedimientos Quirúrgicos Cardíacos/métodos , Ecocardiografía , Resultado Fatal , Femenino , Humanos , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Imagenología Tridimensional , Recién Nacido , Laringoscopía , Enfermedades Raras , Tomografía Computarizada por Rayos X , Enfermedades de la Tráquea/diagnóstico
7.
Cardiol Young ; 28(5): 783-785, 2018 May.
Artículo en Inglés | MEDLINE | ID: mdl-29490714

RESUMEN

Pulmonary artery slings and vascular rings are very rare congenital anomalies. It is even rarer to have both anomalies in the same setting. We present a case of a toddler who was diagnosed with a left pulmonary artery sling and a vascular ring as part of the screening process for the VACTERL association - co-occurrence of vertebral, anorectal, cardiac, tracheoesophageal, renal, and limb malformations. He underwent a successful surgical repair via median sternotomy and on cardiopulmonary bypass with an uneventful postoperative course.


Asunto(s)
Arteria Pulmonar/anomalías , Estenosis Traqueal/etiología , Anillo Vascular/diagnóstico , Broncoscopía , Angiografía por Tomografía Computarizada , Diagnóstico Diferencial , Humanos , Lactante , Laringoscopía , Masculino , Arteria Pulmonar/diagnóstico por imagen , Arteria Pulmonar/cirugía , Estenosis Traqueal/diagnóstico , Estenosis Traqueal/cirugía , Anillo Vascular/complicaciones , Anillo Vascular/cirugía
8.
Int J Pediatr Otorhinolaryngol ; 182: 111993, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-38885545

RESUMEN

INTRODUCTION: Slide tracheoplasty has become the gold standard surgery for congenital tracheal stenosis (CTS). This condition is rare and the surgery can be challenging and is performed by experienced surgeons in tertiary centers. A few reports involving relatively small cohorts have been published. The aim of this review is to evaluate the post-operative mortality and morbidity of pediatric slide tracheoplasty for CTS. METHODS: A systematic literature review was performed according to PRISMA guidelines. The Medline and EMBASE databases were screened using a search strategy defined in collaboration with a librarian. We included articles reporting the post-operative mortality rate of slide tracheoplasties for treatment of CTS in children, when at least 10 patients were included. RESULTS: A total of 932 articles were reviewed, and 15 studies were eligible with a total of 845 patients. The overall post-operative mortality rate was 9.3 %, and most deaths were airway related. The open revision surgery rate after surgery was 2.8 % and the endoscopic revision rate was 27.6 %. DISCUSSION: This study highlights key factors to consider before the surgery and helps anticipate post-operative follow-up considerations for children with CTS. Several factors were identified as predictors of mortality including young age, weight at the time of surgery and association with lung hypoplasia or aplasia. CONCLUSION: Although slide tracheoplasty has gained popularity in recent years due to better outcomes, it remains a major surgery with mortality risk and the need for multidisciplinary management.


Asunto(s)
Procedimientos de Cirugía Plástica , Tráquea , Estenosis Traqueal , Humanos , Estenosis Traqueal/cirugía , Estenosis Traqueal/congénito , Procedimientos de Cirugía Plástica/métodos , Tráquea/cirugía , Tráquea/anomalías , Resultado del Tratamiento , Complicaciones Posoperatorias/epidemiología , Niño , Reoperación/estadística & datos numéricos
9.
Ear Nose Throat J ; : 1455613241272640, 2024 Sep 04.
Artículo en Inglés | MEDLINE | ID: mdl-39230166

RESUMEN

This is a case report on the treatment of tracheal glomus tumor, which is a rare tumor originating from the glomus bodies in the trachea and form spherical or nodular masses. These tumors can lead to obstruction and narrowing of the tracheal lumen, causing symptoms such as dyspnea. The standard treatment for tracheal glomus tumors is typically surgical resection. In this case, the patient underwent a procedure involving low tracheostomy with closure of the airway and mechanical ventilation through the stoma, followed by endoscopic-assisted subglottic tracheal glomus tumor resection using plasma-assisted radiofrequency.

10.
Am J Physiol Lung Cell Mol Physiol ; 305(12): L1002-10, 2013 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-24163140

RESUMEN

Our previous studies have shown that the anti-asthma traditional Chinese medicine herbal formula ASHMI (anti-asthma simplified herbal medicine intervention) inhibits acetylcholine-induced contractions of tracheal rings from ovalbumin-sensitized and naive mice in a ß-adrenoceptor-independent manner. We sought to determine whether acute in vivo ASHMI administration inhibits airway hyperreactivity (AHR) in a murine model of allergic asthma and acetylcholine-induced tracheal ring constriction ex vivo and to elucidate the cellular mechanisms underlying these effects. Ovalbumin-sensitized mice received a single oral ASHMI dose 2 h before intravenous acetylcholine challenge. AHR was determined by invasive airway measurements. Myography was used to determine the effects of ASHMI on acetylcholine-induced constriction of tracheal rings from asthmatic mice with or without epithelial denudation. The effect of cyclooxygenase inhibition and EP2/EP4 receptor blockade on ASHMI attenuation of acetylcholine contractions was evaluated. Tracheal cAMP and PGE2 levels were measured by ELISA. A single acute oral dose of ASHMI dramatically reduced AHR in response to acetylcholine provocation in ovalbumin-sensitized mice (P < 0.001). In ex vivo experiments, ASHMI significantly and dose-dependently reduced tracheal ring constriction to acetylcholine (P < 0.05-0.001), which was epithelium independent and associated with elevated cAMP levels. This effect was abrogated by cyclooxygenase inhibition or EP2/EP4 receptor blockade. ASHMI also inhibited contraction to high K(+) (P < 0.001). ASHMI increased tracheal ring PGE2 release in response to acetylcholine or high K(+) (P < 0.05 for both). ASHMI produced direct and acute inhibition of AHR in vivo and blocked acetylcholine-induced tracheal ring constriction via the EP2/EP4 receptor pathway, identifying the mechanism by which ASHMI is an orally active bronchoprotective agent.


Asunto(s)
Antiasmáticos/uso terapéutico , Dinoprostona/metabolismo , Medicamentos Herbarios Chinos/uso terapéutico , Contracción Muscular/efectos de los fármacos , Músculo Liso/metabolismo , Receptores de Prostaglandina E/metabolismo , Animales , Asma/tratamiento farmacológico , Medicamentos Herbarios Chinos/administración & dosificación , Medicina de Hierbas/métodos , Ratones , Músculo Liso/efectos de los fármacos , Ovalbúmina/farmacología , Tráquea/efectos de los fármacos
11.
Ann Otol Rhinol Laryngol ; 132(5): 558-565, 2023 May.
Artículo en Inglés | MEDLINE | ID: mdl-35723210

RESUMEN

OBJECTIVE: To identify factors predicting success in slide tracheoplasty surgery at a regional children's hospital and compare with available published literature. MEASURES: Retrospective chart review comparing demographics (age, weight) and clinical (operative and hospital course, need for additional airway intervention) factors experienced with slide tracheoplasty. Findings were compared with a systematic review of published literature. RESULTS: Of the 16 tracheal stenosis patients in our cohort, 13 (81.3%) presented with an additional congenital or cardiovascular anomaly. When adjusted for cardiovascular anomalies, congenital tracheal stenosis patients had a mean age of 5.2 months (range 6 days-17 months), mean weight of 5.04 kg, and average ICU and hospital length of stay of 31.5 and 36.0 days, respectively. Tracheostomy was required for 4 patients and no early deaths were recorded. Of the 391 children in the grouped cohort, mean age and weight was older at 7.67 months and larger at 5.70 kg. Length of stay in both ICU and overall hospital course was 31.6 and 43.5 days, respectively. Mortality etiology for 44 patients was reported: 17 (38.6%) cardiac-related and 28 (63.6%) late mortalities. Our overall calculated mortality risk of 1.26 (P < .05) was lower than reported ratios of 2.0+. CONCLUSION: Despite the numerous institutional studies involving tracheal stenosis, mortality and surgical challenges remain high. Future studies with the inclusion of specific perioperative data can prove to further evaluate correlations between presentation characteristics and mortality.


Asunto(s)
Estenosis Traqueal , Niño , Humanos , Lactante , Recién Nacido , Estenosis Traqueal/cirugía , Estudios Retrospectivos , Resultado del Tratamiento , Tráquea/cirugía
12.
J Pediatr Surg ; 58(4): 624-628, 2023 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-36653203

RESUMEN

INTRODUCTION: Complete tracheal rings are a rare malformation that occurs in 1 out of 100,000 live births. It is rare, isolated tracheal or tracheobronchial anomaly developed due to abnormal cartilage growth with formation of complete ring and often resulting in airway stenosis. Slide tracheoplasty, as it was originally described by Tsang et al. and popularized by Grillo et al., overlaps stenotic segments of trachea, shortening trachea itself, thus, doubling the circumference and diameter of the stenotic area. MATERIALS AND METHODS: We have performed slide tracheoplasty in 12 children during the period of 2019-2021 in thoracic surgery department of our center. Median age was 15 ± 21,1 months (2 months-6 years),median weight - 8,04 ± 4,75 kg (3-20,7 kg),tracheal lumen varied from 2.5 to 3.0 mm, stenosis length - from 40 to 70% of the trachea length. RESULTS: Slide tracheoplasty was performed using central veno-arterial extracorporeal membrane oxygenation in 7 cases and using cardiopulmonary bypass in 5 cases. Concomitant heart disease was revealed in 5 children (pulmonary artery sling in 3 cases, ventricular septal defects - 1, aberrant subclavian artery -1). 5 children underwent one-stage correction of VSD: plastic VSD -1; left pulmonary artery reimplantation - 3; subclavian artery reimplantation - 1. All patients were on mechanical ventilation for 4,3 ± 2,78 days at postoperative period. Patients were discharged 16,3 ± 5,14 days after surgery. Satisfactory result of treatment in the form of respiratory failure relief was achieved in 10 patients. It was possible to increase the trachea lumen from 1.5 to 2 times in all cases. There were 2 (16,6%) fatal cases due to sepsis and multi-organ failure development. CONCLUSIONS: Children with complete tracheal rings are very complicated patients with various comorbidities. Despite the advances in medicine, sometimes it is impossible to save lives of these children. The use of extracorporeal circulation (ECMO and bypass) allows us to safely perform reconstructive surgery on the trachea and save the child from respiratory failure manifestations. If needed, simultaneous correction of heart and tracheal defects is possible. Slide tracheoplasty allows to increase trachea lumen at least in 1.5-2 times. Mechanical ventilation is an unfavorable predictive factor for the outcomes of congenital tracheal stenosis management. LEVEL OF EVIDENCE: III.


Asunto(s)
Cardiopatías Congénitas , Insuficiencia Respiratoria , Niño , Humanos , Lactante , Constricción Patológica , Resultado del Tratamiento , Tráquea/cirugía , Tráquea/anomalías , Cardiopatías Congénitas/cirugía , Estudios Retrospectivos
13.
Cureus ; 15(5): e39765, 2023 May.
Artículo en Inglés | MEDLINE | ID: mdl-37398814

RESUMEN

The flow-volume loop (FV-loop) provides a graphical representation of the inspiratory and expiratory flow of both mechanically provided breaths and patient-triggered breaths during invasive mechanical ventilation. The FV-loop on the ventilator-delivered breath displays the active inspiratory flow reflective of lung compliance and the passive expiratory flow reflective of airway resistance. Our case report highlights the importance of the FV-loop in determining a fixed airway obstruction. A five-month-old male presented to the emergency department with worsening respiratory distress in the setting of rhino-enterovirus. He was admitted to the pediatric intensive care unit (PICU) and intubated for acute hypoxic respiratory failure. The findings on his ventilator FV-loop graphics denoted a fixed airway obstruction, as seen by the truncation of inspiratory and expiratory flow. The patient was subsequently found to have a left pulmonary artery (LPA) sling with a vascular ring and several complete tracheal rings. He was transferred to a referral institution for operative management, returned to our PICU, and discharged home after 47 days of hospital management. During mechanical ventilation, FV-loops can be effectively utilized to assist in the diagnosis of fixed intra- or extra-thoracic airway obstructions.

14.
Laryngoscope ; 133(12): 3334-3340, 2023 12.
Artículo en Inglés | MEDLINE | ID: mdl-37159210

RESUMEN

OBJECTIVES: Bronchial anomalies are rare but challenging conditions to treat in children, encompassing a variety of structural abnormalities that could compromise airway patency. This includes complete rings, absent cartilage, traumatic avulsions, bronchoesophageal fistulas, and cartilaginous sleeves. The objective of this study is to describe the characteristics and outcomes of a series of pediatric cases of bronchial anomalies that were treated by slide tracheobronchoplasty. METHODS: This is a single-institution retrospective case series of pediatric patients with bronchial anomalies who underwent surgical treatment between February 2004 and April 2020. Data extracted from electronic medical records included patient demographics, comorbidities, and surgical outcomes. RESULTS: There were a total of 29 patients included in the study, of which 14 had complete bronchial rings, 8 had absent bronchial rings, 4 had traumatic bronchial avulsions, 2 had bronchoesophageal fistulas, and one had a cartilaginous sleeve. Median follow-up time was 13 months (with a range of 0.5-213 months). The overall mortality rate was 17.2% (5 patients), all of whom had complete bronchial rings. Patients with complete bronchial rings also had a higher rate of not only cardiac (85.7%) and pulmonary comorbidities (85.7%) but also secondary airway lesions (78.6%). CONCLUSION: This is the largest series to date describing surgical treatment for bronchial anomalies. Complete bronchial rings were the most common anomaly treated, followed by absent rings and trauma. Surgical treatment can be successful but mortality rates are higher in patients with complete bronchial rings, possibly due to higher rates of pulmonary and cardiac comorbidities. LEVEL OF EVIDENCE: 4 Laryngoscope, 133:3334-3340, 2023.


Asunto(s)
Fístula , Procedimientos de Cirugía Plástica , Estenosis Traqueal , Niño , Humanos , Lactante , Tráquea/cirugía , Tráquea/anomalías , Estenosis Traqueal/cirugía , Estudios Retrospectivos , Fístula/cirugía , Resultado del Tratamiento
15.
AJP Rep ; 13(4): e53-e60, 2023 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-37937269

RESUMEN

Down syndrome (DS, trisomy 21) with an extra copy of chromosome 21 is one of the most common aneuploidies in humans. Jacobs syndrome or XYY syndrome (trisomy XYY) with an extra copy of sex chromosome Y is a rare sex chromosome trisomy in males. Double aneuploidy (DA) with an extra copy of chromosome 21 and sex chromosome Y is an extremely rare occurrence. Most trisomy 21 results from nondisjunction during maternal oocyte meiosis-I, whereas trisomy XYY is results from nondisjunction during paternal spermatocyte meiosis-I. We present a case of natural conception premature newborn of 30.4 weeks gestational age who had a DS facial phenotype with extensive syndactyly on both hands and feet. Other multisystem congenital anomalies were discovered, including mal-aligned perimembranous ventricular septal defect, bicuspid aortic valve, Dandy-Walker malformation's tetra-ventriculomegaly, and a rare complete tracheal rings deformity (CTRD) with trachea stenosis. Prenatal amniocentesis and postnatal chromosomal karyotyping analysis detected 48, XYY, + 21 nontranslocation trisomy 21, and free-lying Y chromosome without translocation. The existence of DA is rarely reported in literature reviews. In this review, we will discuss the characteristics of DS and Jacobs syndrome as well as the associated multiorgan malformation including the rare lethal CTRD.

16.
Otolaryngol Clin North Am ; 55(6): 1253-1270, 2022 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-36371139

RESUMEN

Congenital tracheal stenosis is a rare but potentially life-threatening condition that is most commonly caused by complete tracheal rings. Slide tracheoplasty was initially introduced as a surgical treatment for congenital tracheal stenosis in 1989 and has significantly improved outcomes and overall survival rates for these patients. It has subsequently been adapted to treat other conditions such as laryngotracheal stenosis, tracheoesophageal fistula, and bronchial stenosis. This article reviews the history, the variety of applications, perioperative management, surgical techniques, potential complications, and new frontiers in slide tracheoplasty surgery.


Asunto(s)
Procedimientos de Cirugía Plástica , Estenosis Traqueal , Humanos , Lactante , Constricción Patológica , Resultado del Tratamiento , Estenosis Traqueal/cirugía , Tráquea/cirugía , Procedimientos de Cirugía Plástica/métodos , Estudios Retrospectivos
17.
Biol Trace Elem Res ; 200(3): 1212-1219, 2022 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-33939131

RESUMEN

Airway smooth muscle contraction is one of the primary factors involved in the initiation and progression of asthma which in turn is regulated by increased cytosolic Ca2+ concentration from intracellular stores and through transmembrane ion channels. Calcium-independent factors such as reactive oxygen species (ROS) generation, nitric oxide (NO) depletion and cyclooxygenase (COX) pathways also contribute to tracheal smooth muscle contraction. Studies on copper toxicity suggest significance of this essential micronutrient overdose in acute respiratory disorders, allergic asthma and ciliary motion in tracheal explants. However, the mechanism of copper caused hypercontraction upon direct exposure to tracheal smooth muscle is largely unknown. In this study we investigate the effect of copper exposure on isolated tracheal rings and relative contributions of various factors in acetylcholine-induced contractions. Results obtained suggest that rise in intracellular calcium concentration via voltage-operated Ca2+ channel (VOCC), store-operated Ca2+ channel (SOCC), stretch-activated channels (SAC) and TRP channel (transient receptor potential channel) activation is the major factor in copper-mediated hypercontraction. ROS generation or COX-dependent pathways do not appear to significantly contribute to Cu2+ caused hypercontraction.


Asunto(s)
Contracción Muscular , Músculo Liso , Acetilcolina , Animales , Calcio/farmacología , Bloqueadores de los Canales de Calcio/farmacología , Ratas
18.
J Laparoendosc Adv Surg Tech A ; 31(12): 1511-1515, 2021 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-34847727

RESUMEN

Introduction: Complete tracheal rings are a rare pathology occurring in 1 out of 100,000 live births. It is rare isolated tracheal or tracheobronchial anomaly resulting from abnormal cartilage growth, forming a complete ring, and leading to airway stenosis. A sliding tracheoplasty, primarily described by Tsang et al. and later widely presented by Grillo et al., overlaps the tracheal stenosing segments and shortens the trachea itself, and thus increases the diameter and circumference of the stenosing area double up. Materials and Methods: We have performed four slide tracheoplasties in the period between February 2019 and December 2020 in children who underwent medical treatment in the department of thoracic surgery in our center. Median age was 10 ± 5.5 months (2 months-1 year 6 months). Median weight was 6.9 ± 1.9 kg (4.5-9 kg). Slide tracheoplasty was performed using central venoarterial extracorporeal membrane oxygenation in 3 cases and using cardiopulmonary bypass in 1 case. Results: Patients were on artificial lung ventilation for 2-6 days in the postoperative period. Patients were discharged 14-18 days after the surgery. There were no lethal outcomes in our study. Discussion: Long segment congenital tracheal stenosis is an often and serious life-threatening anatomical malformation that bounded the length of trachea >50%. There is no unique treatment strategy for patients with such pathology. Sliding tracheoplasty can be recommended for all, but not for the shortest segments of stenosis, as it creates permissible voltage fluctuations during tracheal anastomosis. Excellent results in management of such severe patients can be achieved only through the collaboration of multidisciplinary team of specialists sharing organized and consistent patient-oriented approach.


Asunto(s)
Procedimientos de Cirugía Plástica , Estenosis Traqueal , Niño , Constricción Patológica , Humanos , Lactante , Estudios Retrospectivos , Tráquea/cirugía , Estenosis Traqueal/congénito , Estenosis Traqueal/cirugía , Resultado del Tratamiento
19.
Semin Pediatr Surg ; 30(3): 151059, 2021 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-34172205

RESUMEN

Tracheal resection is an uncommon procedure in children. It was described many years ago and many surgeons still rely on historical reports when performing the procedure, despite the technological and clinical advancements achieved during the last decades. We consider complex tracheal resections those resections of 30 to 50% of tracheal length, corresponding to at least 6-7 rings. With proper intra and post-operative management, this surgery is safe in children. In this review, we focused on the experience with this procedure of 3 reference centers, with 49 patients operated. We present a management algorithm for these patients, describing pre-operative evaluations, decision on surgical approach, need of tracheotomy, type of intra-operative oxygenation, surgical steps, and post-operative care.


Asunto(s)
Procedimientos de Cirugía Plástica , Estenosis Traqueal , Niño , Humanos , Lactante , Tráquea/cirugía , Estenosis Traqueal/cirugía , Traqueostomía , Traqueotomía , Resultado del Tratamiento
20.
World J Pediatr Congenit Heart Surg ; 12(3): 414-417, 2021 05.
Artículo en Inglés | MEDLINE | ID: mdl-33942690

RESUMEN

Substantial improvements in techniques of tracheal surgery for children have occurred in the past 20 years. Precise preoperative imaging with computed tomography clearly defines the anatomy for surgical planning and is assisted by on-the-table needle localization. The use of cardiopulmonary bypass greatly facilitates creation of an airtight, widely patent trachea. The use of Ciprodex as a postoperative nebulizer has significantly decreased granulation tissue along the suture line. Most important has been the adoption of slide tracheoplasty as the procedure of choice.


Asunto(s)
Procedimientos de Cirugía Plástica , Estenosis Traqueal , Puente Cardiopulmonar , Niño , Humanos , Lactante , Estudios Retrospectivos , Tráquea/diagnóstico por imagen , Tráquea/cirugía , Estenosis Traqueal/cirugía , Resultado del Tratamiento
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