Neonates With Tracheomalacia Generate Auto-Positive End-Expiratory Pressure via Glottis Closure.

BACKGROUND: In pediatrics, tracheomalacia is an airway condition that causes tracheal lumen collapse during breathing and may lead to the patient requiring respiratory support. Adult patients can narrow their glottis to self-generate positive end-expiratory pressure (PEEP) to raise the pressure in the trachea and prevent collapse. However, auto-PEEP has not been studied in newborns with tracheomalacia. The objective of this study was to measure the glottis cross-sectional area throughout the breathing cycle and to quantify total pressure difference through the glottis in patients with and without tracheomalacia. RESEARCH QUESTION: Do neonates with tracheomalacia narrow their glottises? How does the glottis narrowing affect the total pressure along the airway? STUDY DESIGN AND METHODS: Ultrashort echo time MRI was performed in 21 neonatal ICU patients (11 with tracheomalacia, 10 without tracheomalacia). MRI scans were reconstructed at four different phases of breathing. All patients were breathing room air or using noninvasive respiratory support at the time of MRI. Computational fluid dynamics simulations were performed on patient-specific virtual airway models with airway anatomic features and motion derived via MRI to quantify the total pressure difference through the glottis and trachea. RESULTS: The mean glottis cross-sectional area at peak expiration in the patients with tracheomalacia was less than half that in patients without tracheomalacia (4.0 ± 1.1 mm2 vs 10.3 ± 4.4 mm2; P = .002). The mean total pressure difference through the glottis at peak expiration was more than 10 times higher in patients with tracheomalacia compared with patients without tracheomalacia (2.88 ± 2.29 cm H2O vs 0.26 ± 0.16 cm H2O; P = .005). INTERPRETATION: Neonates with tracheomalacia narrow their glottises, which raises pressure in the trachea during expiration, thereby acting as auto-PEEP.

SMARCE1-related Coffin-Siris Syndrome: Case report and otolaryngologic manifestations of the syndrome.

Coffin-Siris Syndrome (CSS) is a genetic syndrome associated with multiple congenital anomalies due to mutations in the BAF-complex or SOX gene. Although well characterized overall, the subunits of the BAF-complex or SOX gene affected demonstrate phenotypic differences which are continuing to be defined. Among the variants is the SMARCE1 mutation, the least common identified genotype. This case report presents a pediatric patient with SMARCE1-related CSS, the seventh case reported in the literature. The congenital anomalies are discussed and compared to the reported cases of SMARCE1-related CSS and CSS overall with an emphasis on otolaryngologic manifestations.

The use of expandable metallic airway stent in a baby with tracheomalacia: A case report.

Tracheobronchomalacia is a rare condition in the pediatric age group which may be life-threatening when it occurs. The common form of tracheomalacia is congenital, presenting with wheezing and cough. We report a case of a 65-day-old baby who was treated with non-invasive mechanical ventilation due to respiratory distress since the day of birth. Tracheomalacia was diagnosed based on the physical examination and the thorax computerized tomography (CT) findings. Patient was initially treated with noninvasive positive pressure ventilation and thereafter, fitted with a tracheobronchial conical fully-covered self-expandable nitinol stent. After stent insertion and the respiratory situation of the patient improved, ventilatory weaning and extubation were possible. A careful selection of suitable patients, appropriate stent type and the site, where it has to be placed is mandatory for successful airway stenting. Also, children must be adequately followed-up to prevent the possible life-threatening complications after stent insertion.

Congenital tracheal malformations.

Congenital malformations of the trachea include a variety of conditions that cause respiratory distress in neonates and infants. A number of anomalies are self-limiting while others are life-threatening and require immediate therapy. The prevalence of congenital airway malformations has been estimated to range between 0.2 and 1 in 10,000 live births. The most frequent congenital tracheal malformations are: tracheomalacia, congenital tracheal stenosis, laryngotracheal cleft and tracheal agenesis. The management of congenital tracheal malformations is complex and requires an individualized approach delivered by a multidiscipilinary team within centralized units with the necessary expertise.

Upper Airway Anomalies in Congenital Tracheoesophageal Fistula and Esophageal Atresia Patients.

OBJECTIVE: To examine the prevalence of upper airway anomalies in patients diagnosed with congenital tracheoesophageal fistula and esophageal atresia (TEF/EA). METHODS: A retrospective review was conducted of all TEF/EA patients seen at a tertiary pediatric hospital between January 2008 and December 2013. Inclusion criteria included evaluation by the otolaryngology service. Exclusion criteria included age>18 years, acquired TEF/EA, subsequent rule out of TEF/EA, and otolaryngology evaluation for reasons not pertaining to the airway. Data collected and analyzed included demographics, comorbidities, presenting symptoms, surgical interventions, laryngoscopic and bronchoscopic examinations, and subsequent medical and surgical management. RESULTS: Four hundred and thirty patients were diagnosed with TEF/EA at our institution. In all, 32.3%, or 139 children, were included in the analysis; 56.1% (n=78) male, 43.9% (n=61) female. Of the analyzed patients, 4.3% (n=6) were diagnosed with laryngomalacia. Eighteen patients (12.9%) were diagnosed with subglottic stenosis. Thirty (21.6%) had vocal fold paresis or immobility. Laryngeal cleft was diagnosed in 25.9% (n=36). Tracheomalacia was the most common airway finding, diagnosed in 37.4% (n=52) patients. CONCLUSION: Patients diagnosed with congenital TEF/EA have a high rate of secondary upper airway anomalies. Consideration should be given to perform a complete airway evaluation in all of these patients.

Flexible bronchoscopy as a valuable tool in the evaluation of infants with stridor.

The aim is to determine clinical characteristics, flexible bronchoscopy (FB) findings including associated airway abnormalities and other conditions, treatment modalities and long term follow-up of children with congenital stridor. Medical records of children, who underwent FB for the evaluation of stridor between 1 January 2004 and 31 December 2009 were retrospectively reviewed. Demographic characteristics, symptoms and physical examination findings at presentation, FB findings, follow-up data including the time to resolution of symptoms and treatment modalities, presence of associated conditions were assessed. 109 children were enrolled to the study. Laryngomalacia was the most common etiology for stridor. Laryngomalacia was isolated in 37 patients and 54 patients had secondary airway lesions (SALs). Diagnoses other than laryngomalacia such as subglottic hemangioma, subglottic web, isolated tracheomalacia were found in 18 patients. In 90 % of patients, stridor resolved before 3 years of age without any surgical intervention and there was no significant difference in terms of the persistence of stridor between patients with isolated laryngomalacia and associated SALs. Duration of stridor was significantly longer in both patients with neurological abnormalities and reflux symptoms. Surgical procedure was performed in 19 of the patients. There is a high incidence of SALs in patients with laryngomalacia. FB is helpful for identifying anomalies requiring surgical treatment.

Laringotraqueomalacia: diagnóstico fibroendoscópico videoasistido: Presentación de un caso / Laryngotracheomalacia: Video-assisted fybroendoscopic diagnosis: Presentation of a case

Fundamento: La laringomalacia es la más común de las anomalías congénitas de la laringe y muchos niños que la presentan tienen anomalías sincrónicas de la vía aérea, entre ellas la traqueomalacia. La evaluación de la dinámica de la vía aérea mediante la endoscopia flexible es el método que permite el diagnóstico de confirmación de estas entidades. Presentación de caso: un niño de 4 meses de edad con estridor laríngeo congénito y empeoramiento progresivo asociado a deformidad torácica y distress respiratorio, en el que la fibroendoscopia videoasistida demostró que se trataba de una laringotraqueomalacia(AU)

Background: Laryngomalacia is the most common congenital anomaly of the larynx, and many children who suffer from it have synchronous anomalies of the airway, including tracheomalacia. The evaluation of the airways dynamics by means of flexible endoscopy is the diagnostic method for confirmation of these entities. Case presentation: a 4-month-old with congenital stridor and progressive worsening associated with respiratory distress and chest deformity, in which video-assisted fybroendoscopy showed that it was a laryngotracheomalacia(AU)

Early and long term outcome in children with esophageal atresia treated over the last 22 years.

BACKGROUND: The survival of infants born with esophageal atresia (EA) is > 90% at present. The purpose of this study was to evaluate early complications and long term outcome in children with EA treated at our institution. METHODS AND PATIENTS: Retrospective analysis of 111 children with EA undergoing repair of EA or tracheoesophageal fistula (TEF). Assessment of early and intermediate complications as well as long term morbidity and mortality. RESULTS: Primary anastomosis was performed in 90 (81%) and secondary anastomosis in 7 patients (6%). Gastric transposition was carried out in 14 children (13%). The postoperative mortality was 14/111 (12.6%) and could be estimated by the Spitz classification. At the age of 10 years, 33 patients (72%) were swallowing without problems, 39 children (85%) were eating at least most of the time with pleasure but 19 children (41%) had a body weight less than the 25 (th) percentile. Staged repair by gastric transposition resulted in the least amount of motility dysfunction. Long-term respiratory morbidity was high. CONCLUSION: The survival of children with EA has improved in the last two decades. For risk assessment the Spitz' classification is valid. Long term gastrointestinal and respiratory morbidity remains high. In children with long-gap EA gastric transposition performed as a staged procedure has satisfactory results and seems superior to techniques preserving the native esophagus.

Long-term outcome after external tracheal stabilization due to congenital tracheal instability.

BACKGROUND: Long-segment tracheobronchial malacia may cause life-threatening dysfunction of the airway system at different levels. This study presents the long-term follow-up (1992 through 2008) of patients who received surgical treatment with external tracheal stabilization in our institution. METHODS: Eleven patients fulfilled the inclusion criteria. In surviving patients who presented for reexamination, pulmonary function testing, ergometry, and magnetic resonance imaging (MRI) were performed. RESULTS: All patients could be weaned from the ventilator and discharged. Patients were aged a median 11 months (range, 3 to 48 months) at operation for tracheal compression. Age at follow-up was 9.1 years (range, 0.5 to 16.3 years). Median follow-up was 7.3 years (range, 0.1 to 15.1 years). Postoperatively, 1 patient was lost to follow-up, and 4 died at 2.6 years (range, 0.5 to 6.6 years) of comorbidities. Pulmonary function testing showed a moderate residual airflow restriction, with maximal vital capacity at 75% of normal (range, 45% to 92%). Treadmill exercise testing demonstrated 70% to 89% of the expected normal values for age. Magnetic resonance imaging examination confirmed tracheal patency, but the lumen of the left main bronchus in 2 patients was 50% smaller than on the right. Diaphragmatic motion was normal in all patients. CONCLUSIONS: Children with congenital tracheal stenosis benefit from external tracheal stabilization. Survival in patients after external tracheal stabilization is significantly influenced by concomitant conditions.

State-of-the-art CT imaging techniques for congenital heart disease.

CT is increasingly being used for evaluating the cardiovascular structures and airways in the patients with congenital heart disease. Multi-slice CT has traditionally been used for the evaluation of the extracardiac vascular and airway abnormalities because of its inherent high spatial resolution and excellent air-tissue contrast. Recent developments in CT technology primarily by reducing the cardiac motion and the radiation dose usage in congenital heart disease evaluation have helped expand the indications for CT usage. Tracheobronchomalacia associated with congenital heart disease can be evaluated with cine CT. Intravenous contrast injection should be tailored to unequivocally demonstrate cardiovascular abnormalities. Knowledge of the state-of-the-art CT imaging techniques that are used for evaluating congenital heart disease is helpful not only for planning and performing CT examinations, but also for interpreting and presenting the CT image findings that consequently guide the proper medical and surgical management.

Vocal fold paralysis in infants with tracheoesophageal fistula.

OBJECTIVES: We describe the clinical characteristics and management of vocal fold paralysis in infants who were born with a tracheoesophageal fistula (TEF). METHODS: This retrospective case series included all infants born with TEFs who presented to our pediatric otolaryngology unit and intensive care unit because of dyspnea or aphonia in the years 2005 and 2006, and who were found to have vocal fold paralysis. RESULTS: Five boys and 1 girl were studied. One infant had stridor before TEF repair, and 5 after it. All children underwent flexible laryngotracheobronchoscopy and were treated in the pediatric intensive care unit before diagnosis of the vocal fold paralysis (5 bilaterally and 1 unilaterally) was made. The ages at diagnosis of paralysis ranged between 14 days and 14 months. Five infants required tracheostomy. CONCLUSIONS: Vocal fold paresis in infants is difficult to diagnose. The risk for recurrent laryngeal nerve injury associated with TEF and TEF repair should be emphasized in these children. We recommend that all newborns with TEF should be examined by an otolaryngologist before operation to confirm the mobility of the vocal folds and to rule out other associated airway malformations, and examined after operation if respiratory difficulties develop.

Utility of virtual bronchoscopy in congenital tracheomalacia.

Utility of virtual bronchoscopy was evaluated in a case of congenital tracheomalacia by comparing virtual bronchoscopic images with those of fiberoptic bronchoscopy. Results indicate that virtual bronchoscopy is useful in diagnosing stationary lesions. However, its diagnostic value is inferior to that of fiberoptic bronchoscopy for mobile lesions, in that virtual bronchoscopy does not permit dynamic images. We recommend that the institutions, where fiberoptic bronchoscopy is available, should perform virtual bronchoscopy in parallel to explore its utility.