Video-assisted Thoracoscopic Resection of a Congenital Tracheobiliary Fistula in a Neonate: Technical Report and Review of the Literature.

BACKGROUND: Congenital tracheobiliary or bronchobiliary fistulae are rare developmental malformations associated with high morbidity and mortality primarily because of concomitant anomalies as well as delays in diagnosis. TECHNICAL REPORT: We report a case of a neonate who presented with bilious emesis and progressive respiratory failure requiring bronchoscopy and fluoroscopic bronchography for diagnosis with successful management through video-assisted thoracoscopic surgery. We also review the published literature on tracheobiliary fistula. CONCLUSION: Use of video-assisted thoracoscopic surgery for fistula resection has been reported in only one other neonate and provides a clinically less invasive option compared with open surgical ligation and resection. To our knowledge, this is the first English language report of thoracoscopic resection in a neonate with congenital tracheobiliary fistula.

Complete tracheal rings and hypoplastic left heart variant: a rare and fatal association.

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.

Fetal Head and Neck Masses: MRI Prediction of Significant Morbidity.

OBJECTIVE: The purpose of this study is to determine which MRI parameters of fetal head and neck masses predict high-morbidity neonatal outcomes, including ex utero intrapartum treatment (EXIT) procedure. MATERIALS AND METHODS: This retrospective study (2004-2016) included parameters of polyhydramnios (based on largest vertical pocket), mass effect on the trachea, mass midline extension, and morphologic grade and size of masses. The morbid cohort included those requiring an EXIT procedure, difficult intubation at delivery, or lethal outcome. Predictive modeling with a multivariable logistic regression and ROC analysis was then performed. RESULTS: Of 36 fetuses, five were delivered by EXIT procedures, there was one neonatal death within 12 hours after delivery, and another neonate required multiple intubation attempts. The remaining 29 fetuses were delivered at outside institutions with no interventions or neonatal morbidity. The largest vertical pocket and mass effect on the trachea were selected as independent predictors by the logistic regression. The cross-validated ROC AUC was 0.951 (95% CI, 0.8795-1). CONCLUSION: The largest vertical pocket measurement and mass effect on the trachea were the most contributory MRI parameters that predicted significant morbidity in fetuses with masses of the face and neck, along with other significant parameters. These parameters predict significant morbid neonatal outcomes, including the need for EXIT procedures.

Isolated Congenital Tracheobiliary Fistula.

BACKGROUND: Congenital tracheobiliary fistula is a rare developmental anomaly with a persistent communication between the biliary system and the trachea. CHARACTERISTICS: A 7-day-old baby with severe respiratory distress and aspiration pneumonia. OUTCOME: Tracheobilliary fistula identified on bronchoscopy. Open surgical excision of fistula was followed by improvement. MESSAGE: This condition should be considered in the differential diagnosis of intractable aspiration pneumonia.

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.

Tracheal surgery in children: outcome of a 12-year survey.

OBJECTIVES: Despite the fact that team management has improved the results in recent years, perioperative deaths and complications remain high in paediatric tracheal surgery. We reviewed our institutional experience by comparing our results with those in the literature. METHODS: Between 2005 and 2017, 30 children underwent surgery for tracheal disease. Fifteen were boys and fifteen were girls (50% vs 50%). The median age at operation was 7 months (15 days-9.6 years), and the median weight was 5.2 kg (2.8-34 kg). Congenital tracheal stenosis was diagnosed in 25 children (83.3%), and 5 (16.7%) had acquired lesions. The mean internal diameter in congenital tracheal stenosis was 1.5 mm, with complete tracheal rings present in all patients. Associated malformations were bronchopulmonary in 11 cases (36.7%) and cardiovascular in 16 (53.3%). RESULTS: No in-hospital deaths occurred in our data set. Overall mortality was 4 of 30 cases (13.3%). Twenty-four endoscopic reinterventions were required in 19 children (63%) and consisted of stent positioning in 13 (43.3%), balloon dilatation in 5 (16.7%), granulation removal in 4 (13.3%) and tracheostomy in 2 (6.7%). Of the survivors (26 of 30, 86.7%), 11 children (42.3%) did not require further examination on adequate tracheal diameter for age and absence of symptoms after a median follow-up period of 3.5 years. CONCLUSIONS: The result of paediatric tracheal surgery depends on several factors. The number of cases treated at a particular centre is an important one, but our experience, although limited, can be compared with that at centres with a higher volume of cases. We emphasize the need for applying a multidisciplinary approach to master the surgical command of different reconstructive tracheal procedures, to manage associated defects, particularly cardiovascular defects, and to manage complications under endoscopic guidance. These can be considered the mainstays of building a successful tracheal programme.

Tracheal Cartilaginous Sleeve in Syndromic Craniosynostosis: An Underrecognized Source of Significant Morbidity and Mortality.

Tracheal cartilaginous sleeve (TCS) is a rare and previously unrecognized source of morbidity and mortality in patients with certain craniosynostosis syndromes. There is a paucity of reporting on this airway anomaly, and the true incidence of TCS is largely unknown. The purpose of this study was to investigate the incidence of TCS among patients with syndromic craniosynostosis at our institution. Patients with syndromic craniosynostosis who underwent direct bronchoscopy and laryngoscopy were evaluated retrospectively by pediatric otolaryngologists for the presence of TCS and associated anomalies. Among patients with a diagnosis of syndromic craniosynostosis in our craniofacial database, 10 (37%) were found to have previous direct bronchoscopy and laryngoscopy reports. Of these 10 patients, 2 had Crouzon syndrome, 3 had Pfeiffer syndrome, 3 had Apert syndrome, 1 had Muenke syndrome, and 1 had Antley-Bixler syndrome. Eighty percent (8/10) of these patients were found to have some evidence of TCS. The most commonly observed associated findings included the following: tracheostomy dependency (7/10; 70%), hearing loss (6/10; 60%), obstructive sleep apnea (5/10; 50%), cervical spine anomalies (5/10; 50%), developmental delay (5/10; 50%), and enlarged cerebral ventricles (4/10; 40%). Larger multicenter studies are required to further characterize this airway anomaly and its impact on this patient population. Our results confirm the importance of thorough airway evaluation at initial presentation and the need for validated screening protocols.

Airway tissue engineering for congenital laryngotracheal disease.

Regenerative medicine offers hope of a sustainable solution for severe airway disease by the creation of functional, immunocompatible organ replacements. When considering fetuses and newborns, there is a specific spectrum of airway pathologies that could benefit from cell therapy and tissue engineering applications. While hypoplastic lungs associated with congenital diaphragmatic hernia (CDH) could benefit from cellular based treatments aimed at ameliorating lung function, patients with upper airway obstruction could take advantage from a de novo tissue engineering approach. Moreover, the international acceptance of the EXIT procedure as a means of securing the precarious neonatal airway, together with the advent of fetal surgery as a method of heading off postnatal co-morbidities, offers the revolutionary possibility of extending the clinical indication for tissue-engineered airway transplantation to infants affected by diverse severe congenital laryngotracheal malformations. This article outlines the necessary basic components for regenerative medicine solutions in this potential clinical niche.

Congenital Tracheal Web Malformation in a Wild Brown Bear ( Ursus arctos ), Sweden, 2010.

We describe a congenital tracheal web malformation in a wild female brown bear (Ursus arctos) yearling that was euthanized after being hit by a train in Norrbotten County, Sweden, December 2010. A 3-cm-long, abnormal, longitudinal mucosal fold divided the trachea into two halves, without obviously blocking the airflow.

Asociación de bronquio traqueal y drenaje venoso pulmonar anómalo parcial en paciente con neurofibromatosis tipo 1 y neurofibroma del nervio vago ipsilateral / Combination of tracheal bronchus and partial anomalous pulmonary venous return in a patient with type 1 neurofibromatosis and ipsilateral vagal nerve neurofibroma

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Congenital tracheobiliary fistula in an adolescent patient.

Congenital tracheobiliary fistula is a rare malformation that allows communication between the respiratory system and hepatobiliary tract. We describe a male adolescent patient who was admitted with a destroyed lung caused by repetitive bile pneumonitis with a congenital tracheobiliary fistula. Left pneumonectomy was performed, and the fistula tract was successfully divided.

[Lung perfusion scintigraphy in pediatric patients with congenital malformations]. / La scintigrafia polmonare in età neonatale e pediatrica per patologie congenite.

Congenital malformations are an heterogeneous group of disorders that can lead changes in pulmonary perfusion and then can be evaluated with lung perfusion scintigraphy. We selected five patients in which the role of lung perfusion scintigraphy emerges as a reliable and non-invasive imaging technique. Lung perfusion scintigraphy is a useful tool in pediatric patients with congenital malformations allowing an accurate evaluation of the best therapeutic strategy and its results.

The quantitative lung index (QLI): a gestational age-independent sonographic predictor of fetal lung growth.

OBJECTIVE: We sought to develop a gestational age-independent sonographic parameter to characterize lung growth. STUDY DESIGN: Reported descriptors of lung growth, including lung-to-head circumference (HC) ratio (LHR) and observed/expected LHR, were examined. A new index, the quantitative lung index (QLI) was derived using published data on HC and the area of the base of the right lung. RESULTS: Neither the LHR nor the observed/expected LHR proved to be gestational age independent. Right lung growth can be expressed using the following formula: QLI = lung area/(HC/10)^2. The 50th percentile of the QLI remained constant at approximately 1.0 for the gestational age between 16-32 weeks. A small lung (<1st percentile) was defined as a QLI <0.6. CONCLUSION: Fetal right lung growth can be adequately described using the QLI, independent of gestational age. Further studies are needed to assess the clinical accuracy of the QLI in characterizing fetal right lung growth.

Traqueobroncomegalia y bronquiectasias en el síndrome de Mouniere-Kuhn. Colonización por Pseudomonas aeruginosa y antibioterapia inhalada / Tracheobronchomalacia and bronchiectasis in the Mouniere-Kuhn syndrome. Pseudomonas aeruginosa colonization and inhaled antibiotic therapy

El síndrome de Mouniere-Kuhn se caracteriza por bronquiectasias (BQ) congénitas asociadas a traqueobroncomegalia. Es una enfermedad poco prevalente en la población general (1-4,5%), siendo infrecuente el diagnóstico en la edad adulta. Las infecciones respiratorias constituyen una complicación frecuente en su evolución y entre ellas, la infección y colonización por Pseudomona aeruginosa. La terapia antibiótica inhalada con colistina, tobramicina, etc. ha demostrado su eficacia en pacientes con BQ asociadas a fibrosis quística. La instauración de este tratamiento en nuestro caso se asoció con una mejoría en la sintomatología clínica y reducción de las exacerbaciones (AU)

Mounier-Kuhn syndrome is characterized by congenital bronchiectasis (BC) associated to tracheobronchomegaly. It is a disease of little prevalence in the general population (1-4.5%), its diagnosis being uncommon in the adult age. Respiratory infections are a frequent complication in its course and, among them, infection and Pseudomona aeruginosa colonization. Inhaled antibiotic therapy with colistin, tobramycin, etc. has demonstrated its efficacy in patients with cystic fibrosis bronchiectasis. The establishment of this treatment in our case was associated with an improvement in the clinical symptoms and reduction of the deteriorations (AU)