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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., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

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.

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.

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 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.

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 (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.

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., (Copyright © 2016. Published by Elsevier Inc.)

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.

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., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

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.

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., (Copyright © 2011 Mosby, Inc. All rights reserved.)

Acquired tracheal pouch known also as tracheocele is a rare air-filled diverticulum of tracheal pars membranacea. This disease may be due to esophageal or tracheal surgery, orotracheal intubation, or increased intralunimal pressure through a weak area of tracheal wall. When symptomatic and medical therapy is insufficient, this disorder must be treated surgically. Several surgical methods ranging from open neck or thoracic surgery to endoscopic managements have been reported. We report the case history of a 7-year-old boy affected by recurrent pneumonia due to tracheal pouch. The patient underwent successful brushing of tracheocele plus fibrin glue application by rigid tracheobroncoscopy. Furthermore, particular emphasis on endoscopic treatments of tracheal diverticulum is also presented., (Copyright 2010 Elsevier Inc. All rights reserved.)

Congenital tracheobiliary fistula is a rare malformation that can present with a variety of respiratory symptoms. We present a case of a newborn patient with a tracheobiliary fistula and severe respiratory insufficiency needing extracorporal membrane oxygenation to recover., (Copyright 2010 Elsevier Inc. All rights reserved.)

Objective: To present the indications, techniques, results, and limitations of endoscopic surgical treatment of laryngotracheal cleft., Design: Retrospective case note study (2005-2009)., Setting: Department of Otolaryngology-Head and Neck Surgery, Armand Trousseau Children's Hospital, Paris, France., Patients: Eleven patients who underwent endoscopic cleft closure as a primary (n = 8) or secondary (n = 3) procedure among 22 patients treated for laryngotracheal clefts. We report patients' demographics, symptoms leading to the diagnosis, endoscopic evaluation method, medical examination results, and surgical techniques., Intervention: Endoscopic closure of the cleft under spontaneous ventilation via 2-layer interrupted sutures after excision of the mucosal edge using a carbon dioxide laser in 10 patients and a thulium laser in 1., Main Outcome Measures: Analysis of postoperative complications, revision surgery, need for intensive care unit admission, closure of the cleft, and long-term symptom results., Results: Successful closure of the 11 clefts (with revision surgery in 3 patients) without the need for intubation or intensive care unit admission enabled the elimination of aspiration in 10 patients and significant improvement in 1 patient with bilateral vocal cord paralysis. There were no significant postoperative complications., Conclusions: Endoscopic closure of laryngotracheal clefts is a reliable technique that significantly reduces perioperative and postoperative morbidity. The results of this technique are entirely satisfactory, and we suggest that it is suitable as a primary procedure for the treatment of type I, II, and III clefts extending to the cervical trachea, including in neonates.

Tracheal atresia is an uncommon congenital malformation with a high mortality rate. Clinical symptoms occur suddenly after birth. The diagnosis is suspected in any infant in whom improved ventilation is obtained despite aggressive attempts at resuscitation. We describe a small gestational week 34 male newborn affected by tracheal atresia without esophageal fistula with associated fetal growth restriction, ascites and polyhydramnios. Post mortem examination revealed a diffuse cyanotic status, abdominal ascites and a low birth weight. A 3 cm tract of trachea was documented that distantly ended in a blind pouch and without tracheoesophageal fistulae and enlarged bulky lungs connected to each other by a common thin-walled bronchus. Histological examination showed a normal conformed larynx and scratchily cartilaginous disks in the proximal tract of the short trachea. Vascular space referred to small arteries and veins, thin bands of fibrous tissue and adipose tissue were detected under the blind pouch. Lung distal airspaces were lined by premature cubic epithelium separated by a broad poorly vascularized interstitium. A striking interstitial and alveolar edema was remarkable.

Congenital high airway obstruction syndrome (CHAOS) is a life-threatening condition characterized by complete blockage of the fetal airways associated with hydrops. We present a case of CHAOS due to the rare cause of complete tracheal agenesis. The ex utero intrapartum therapy (EXIT) strategy was employed to allow for neck and mediastinal exploration. Thymectomy allowed dissection to the level of the carina demonstrating the confluence of dilated mainstem bronchi but no trachea and no connection to the esophagus. A 2.5 endotracheal tube was inserted into the right mainstem bronchus and secured to the left clavicle. At 3 months of age, the infant succumbed to sepsis from Enterobacter mediastinitis due to friction between the tracheostomy tube and the nasogastric tube resulting in erosion of the esophagus. Complete tracheal agenesis, as seen in this case, is consistent with the failure of normal tracheal elongation as suggested by newer theories of foregut development. This case illustrates the most severe form of tracheal atresia causing CHAOS ever salvaged by the EXIT procedure at birth. The subsequent postnatal course highlights the need for early tracheal replacement in this particularly challenging form of CHAOS.

Background: We previously demonstrated that tracheobronchial innervation, originated from the vagus nerve and hence of neural crest origin, is deficient in rats with experimental congenital diaphragmatic hernia (CDH). The present study examines the development of this innervation during fetal life in an attempt to understand the nature of these deficiencies., Materials and Methods: Pregnant rats were given either 100 mg nitrofen or vehicle on E 9.5. Embryos were recovered on E15 and E18. Control and nitrofen/CDH pups (n = 10 each) were studied on each of these days and compared with our previous results on E21. Whole mount preparations of tracheas stained for anti-protein gene product 9.5 (PGP 9.5) and smooth muscle contractile alpha-actin were examined under confocal microscopy for the morphology of intrinsic neural network. Sections of tracheas were immunostained with anti-low-affinity neurotrophin receptor (p75(NTR)), neural cell marker PGP 9.5, and anti-glial cell marker S100 antibodies. The proportions of sectional areas occupied by neural and glial structures were measured in the proximal and distal trachea. PGP 9.5 protein, and mRNA expressions were determined. Mann-Whitney tests with a threshold of significance of P < 0.05 were used for comparison., Results: Positive staining for p75(NTR) confirmed the neural crest origin of tracheal neural cells. The neural network appeared less organized on E15, and it was less dense on E18 in nitrofen-exposed embryos than in controls. The proportions of section surface occupied by neural elements were similar in both groups on E15, but that of glial tissue was significantly increased in nitrofen-exposed embryos. On E18, the relative neural surface was significantly reduced in CDH embryos in contrast with increased glial tissue surface. On E21 the proportion of neural tissue was reduced only in the distal trachea. The expression of PGP 9.5 protein was decreased in CDH fetuses on E18 and E21. In contrast, PGP 9.5 mRNA levels were increased in CDH fetuses on E18 and E21., Conclusions: The development of intrinsic innervation of the trachea in rats with CDH is abnormal with reduction of neural tissue accompanied by increase of glial tissue that could represent a response to neural damage. The significance of increased PGP 9.5 mRNA levels is unclear.

Tracheal agenesis/atresia (TA) is a rare but fatal congenital disease in which the breathing tube fails to grow. The etiology of this serious condition remains largely unknown. We found that Bmp signaling is prominently present in the anterior foregut where the tracheal primordium originates and targeted ablation of Bmp4 (Bmp4(cko)) resulted in a loss-of-trachea phenotype that closely resembles the Floyd type II pathology, the most common form of TA in humans. In Bmp4(cko) embryos, tracheal specification was not affected; however, its outgrowth was severely impaired due to reduced epithelial and mesenchymal proliferation. In agreement, we also observed significant reduction in the expression of Cyclin D1, a key cell cycle regulator associated with cellular proliferation. However, the proliferative effect of Bmp signaling appears to be independent of Wnt signaling. Interestingly, we found significantly reduced expression of activated extracellular signal-regulated kinase (Erk) in the Bmp4(cko) ventral foregut, suggesting that Bmp signaling promotes Erk phosphorylation which has been associated with cellular proliferation. This study provides the first evidence linking Bmp signaling to tracheal formation by regulating the proliferative response of the anterior ventral foregut. Our finding sheds light on human tracheal malformations by providing a novel mouse model implicating Bmp signaling, non-canonical Erk activation and cellular proliferation.

Congenital tracheobiliary fistula (CTBF) is a rare malformation. So far 24 cases have been reported in the English language literature. The imaging techniques used in diagnosis have been bronchography, fistulography, cholangiography, hepatobiliary nuclear imaging and MRI. We report a newborn patient who presented with right lung consolidation and biliptysis. The radiographic diagnosis of tracheobiliary fistula was made on multidetector CT scan of the chest and abdomen. Multiple 3-D volume-rendered reformations were performed. An abnormal air-filled tract was seen connecting the posteroinferior aspect of the carina and left biliary system, which was successfully treated surgically.

Tracheal agenesis (TA) is an extremely rare, typically fatal congenital tracheal malformation. Lack of prenatal symptoms and emergent presentation usually lead to a failure to arrive at the correct diagnosis and manage the airway properly before the onset of irreversible cerebral anoxia. Esophageal atresia (EA) encompasses a group of congenital anomalies comprising an interruption of the continuity of the esophagus with or without a persistent communication with the trachea. In 86% of cases, there is a distal tracheoesophageal fistula (TEF); in 7%, there is no fistulous connection, whereas in 4%, there is a TEF without atresia. We report the case of an infant born with TA and EA with proximal and distal bronchoesophageal fistulas. During 3 consecutive antenatal ultrasound examinations, there had been polyhydramniosis, difficulty visualizing the stomach, and dilatation of proximal esophagus, leading to a presumptive diagnosis of EA. The clinical presentation, embryology, classification, and surgical management are discussed.

In cystic fibrosis (CF) patients, the major alteration in pulmonary function is due to peripheral airway obstruction. In the present study, we investigated the possibility that alterations in the extrathoracic airways, particularly in the trachea that expresses high levels of CFTR (CF transmembrane conductance regulator), may contribute to respiratory dysfunction. We performed morphological analyses of the trachea and airway functional studies in adult Cftr knockout (Cftr(-/-)) and F508del-CFTR mice and their controls. Macroscopic and histological examination of the trachea showed the presence of one to seven disrupted or incomplete cartilage rings in Cftr(-/-) mice (23/25) while only a few Cftr(+/+) mice (6/25) had one abnormal ring. Tracheal defects were mainly localized in the proximal trachea. In 14 Cftr(-/-) mice, frontal disruption of the first three to six rings below the cricoid cartilage were associated with upper tracheal constriction. Similar tracheal abnormalities were detected in adult F508del-CFTR and in newborn Cftr(-/-) and F508del-CFTR mice. Tracheal and ventilatory function analyses showed in Cftr(-/-) mice a decreased contractile response of the proximal trachea and a reduced breathing rate due to an increase in the inspiratory and expiratory times. In F508del-CFTR mice, the expiratory time was longer than in controls. Therefore, these structural and functional abnormalities detected in adult and newborn CF mouse models may represent congenital malformations related to CFTR dysfunction. These results raise important questions concerning the mechanisms governing tracheal development within the context of CFTR protein dysfunction and the implication of such abnormalities in the pathogenesis of airway disease in CF.

Purpose of Review: Pathologic airway conditions in pediatric patients include congenital or acquired subglottic stenosis, glottic stenosis, laryngotracheal stenosis, laryngeal webs or atresia, and tracheal lesions. Acute airway management via tracheotomy is often required with later surgical intervention for reconstruction and expansion of the airway. The effect of the surgical interventions used to expand the airway may impact upon the laryngeal functions of phonation and airway protection during swallowing. Overall, the specific outcomes of airway surgery have focused on airway restoration. Outcomes in regard to voice and swallowing parameters have been largely unexplored, though recent reports have begun to emerge that provide some research data specific to both voice and airway protection/swallowing postoperatively., Recent Findings: Research regarding outcomes following laryngotracheal reconstruction over the last year reveals the use of more systematic approaches to collection of voice data and recognition of postoperative dysphagia. There continues to be a dearth of available research regarding longer term swallowing outcomes or treatment of voice disorders in this population., Summary: Research findings that contribute to the base of knowledge regarding the effectiveness of surgical methodology for establishment of airway patency with preservation of laryngeal functions of voice and swallowing are beginning to accumulate. Increased evidence regarding types of reconstructive approaches and effect on laryngeal functions will assist surgeons and clinicians in designing specific treatment approaches.

This report describes a case of tracheal collapse in a four month old Uckermaerker heifer. The animal was born spontaneous without signs of dystocia. It was kept outside on the pasture and died in the summer at high temperatures under signs of acute dyspnea and respiratory failure. Clinical symptoms were not observed by the owner until a few hours before death. At necropsy, the middle and caudal third of the trachea was found to be collapsed due to an inward bending of the dorsal ends of the cartilage rings. The most severe luminal reduction was located in the thoracic part of the trachea approximately 5 cm cranial the tracheal bronchus. Additional pathological lesions were absent except for signs of circulatory failure (lung edema, congestion of the shock organs). Based on clinical history and pathological findings, a traumatic cause of the tracheal collapse is unlikely. However, a hereditary influence seems possible since analysis of the breeding scheme revealed inbreeding. The mother of the affected animal was mated with her grandson (inbreeding coefficient 0,125).

Objective: Traditionally vascular rings in infants and children are treated through an open thoracotomy. Recently, thoracoscopic surgery has been used for these complex procedures. This study reports our early experience with thoracoscopic division of vascular rings and evaluates the efficacy and safety of this approach., Material and Methods: Patients who underwent thoracoscopic division of vascular rings at King Khalid University Hospital, Riyadh, Saudi Arabia, from December 2004 to January 2006 are included. Their data were carefully analyzed looking at demographics, clinical presentation, diagnostic modality, type of the anomaly, operative details, complications, and outcome., Results: A total of 9 patients underwent thoracoscopic division of vascular rings. Age at surgery ranged between 2 and 108 months (mean, 24 months). Weight varied between 5.3 and 32 kg (mean, 10.3 kg). All patients were symptomatic. Computed tomographic scan was diagnostic and accurately defined the type of anomaly in all the patients. Four patients had a right aortic arch with an aberrant left subclavian artery and left ductus/ligamentum arteriosum, 2 had double aortic arches, and 3 had a right aberrant subclavian artery. One patient developed right-sided pneumothorax on the contralateral site, and another one developed apnea 12 hours after surgery, requiring mechanical ventilation. There was no mortality. Operative time ranged between 50 and 145 minutes, the mean being 107 minutes. The average hospital stay was 4 days. Five patients had their preoperative symptoms completely resolved, and the rest are showing steady improvement. The average follow-up period is 6 months., Conclusion: Our early experience indicates that thoracoscopic division of vascular rings is safe and effective. Because it takes away the need for thoracotomy, it is likely that it can result in less postoperative pain and rapid convalescence. It also prevents the ill effects of thoracotomy and gives good cosmetic results.

Tracheal agenesis is an extremely rare, typically fatal congenital tracheal malformation. Lack of prenatal symptoms and emergent presentation usually lead to a failure to arrive at the correct diagnosis and manage the airway properly before the onset of irreversible cerebral anoxia. We report the case history of an infant born with immediate respiratory failure who was diagnosed with tracheal agenesis. The clinical presentation, embryology, classification, and surgical management are discussed.

Unlabelled: Noninvasive treatment of bronchomalacia. Successful ventilation of a severely ill infant., Aim: To describe an effective treatment of a boy with bronchomalacia by noninvasive mechanical ventilation support., Methods: We describe a case of a severely ill patient with bronchomalacia from the time he was born and until the age of five. Bi-level positive airway pressure given through a specially adapted full face mask was used to treat his respiratory condition., Result: Our patient responded well to the noninvasive treatment of bronchomalacia., Conclusion: We found that noninvasive mechanical ventilation support is a low risk and highly effective treatment of infants and children with respiratory distress caused by bronchomalacia.

A 6-month-old, spayed female, mixed breed dog was referred for respiratory difficulty. Radiographic and bronchoscopic evaluations showed tracheal narrowing from the thoracic inlet to its bifurcation. Under anesthesia, the trachea ruptured and the dog died. Microscopic evaluation revealed underlying segmental tracheal and bronchial cartilaginous malformation (dysplasia).

We herein report the case of a newborn girl with oesophageal atresia associated with cardiac and gastrointestinal anomalies, including patent ductus arteriosus, tracheomalacia, and gastro-oesophageal reflux with hiatus hernia. In addition, she had a terminal deletion of the long arm of chromosome 2, with a breakpoint of 2q37.1. The patient died following a cardiac arrest at 90 days of age. No cause of death was identified at autopsy.

Two girls developed symptoms of wheezing which started shortly after birth. The symptoms did not respond to bronchodilators. At the age of 5 months, the first infant developed severe respiratory distress with decreased left-sided breathing sounds on auscultation. The chest X-ray showed left-sided hyperinflation. Bronchoscopy revealed isolated malacia of the left main stem bronchus. The second patient, who had a history ofcor vitium, was referred to a paediatric pulmonologist in an academic hospital for chronic coughing and wheezing. Bronchoscopy and CT angiogram, performed at the age of 14 months, revealed tracheal malacia due to compression from a right descending aortic arch. Broncho- and tracheomalacia are disorders which may rarely result in severe respiratory distress. These disorders should be considered when unexplained symptoms of wheezing or coughing are present in young infants, especially if the symptoms start shortly after birth and persist without signs of viral infection.

Introduction: Congenital tracheomalacia (CTM) is a rare disease causing tracheal wall collapse when breathing. Herein, we show our experience in the management of this type of airway anomaly, settling the indications for surgical or endoscopic treatment., Patients and Methods: We have performed a retrospective study, from 1991 to 2003, of patients with a bronchoscopic diagnosis of CTM or bronchomalacia (BM). We have analyzed the following facts: sex, age, indication of the initial bronchoscopy, ethiology, clinical group, anatomic type, associated malformations, treatment modality, complications, results, and time of follow-up., Results: 46 patients have been included in this study: 25 boys (54%) and 21 girls (45%). Mean age at diagnosis has been 11 months. The indications for diagnostic bronchoscopy have been: respiratory distress (24%), lung athelectasia (24%), stridor (21%), congenital tracheoesophageal fistula (11%), extubation failure (11%), apneic spells (6%), and recurrent pneumonia (2%). Secondary CTM has been much more frequent (82%) than the primary type (17%). Patients have been classified into 3 groups according to the severity of symptoms: group I--mild symptoms (7 patients); group II---moderate (22); and group III, severe (17). Tracheomalacia was diagnosed in 26 cases (56%), bronchomalacia in 12 (26%) and tracheobronchomalacia in 8 patients (17%). Almost all the patients (95%) have showed other associated malformations. Medical treatment has been instituted in 29 patients (63%), 15 cases (32%) have been managed surgically or endoscopically, and in 2 cases no treatment was tried because of their critical clinical status. In addition, in 17 patients (37%) an antireflux surgical procedure was performed. Satisfactory results have been achieved in 72% of treated patients, fair results were obtained in 4 (9%), and a poor outcome occurred in 2 (4,5%). Another 8 patients have died during follow-up due to unrelated causes. 36 patients (78%) are alive with a mean follow-up period of 5,3 years., Conclusions: Most patients with CTM can he treated conservatively though spontaneous resolution may he expected after the first year of life. Surgical or endoscopical procedures are indicated in those patients with severe respiratory symptoms.

Tracheal agenesis is a rare congenital malformation, which is usually fatal in the newborn period. Its incidence is approximately 1 in 50,000 births. Presentation is with respiratory insufficiency and no audible cry. Other anomalies are found in most cases. Six cases of tracheal agenesis were seen in our hospital since 1988. Their medical records were reviewed. Three of our cases classify as Floyd's type III, two as Floyd's type II and one as Floyd's type I. Associated anomalies were found in five cases. The classification of tracheal agenesis, associated anomalies and potential therapeutic options are discussed.

Tracheomalacia is a rare tracheal problem that leads to collapse of the airway and expiratory flow obstruction. Expiratory CT scans are the diagnostic test of choice in adults. Chronic obstructive pulmonary disease is the most common cause of adult tracheomalacia. Plication of the membranous wall to polypropylene mesh to recreate the normal airway shape (membranous wall tracheoplasty) is an effective treatment in adults.

Background: A left thoracotomy is the standard access for aortosternopexy in severe tracheomalacia. We report a modified technique for thoracoscopic aortopericardiosternopexy., Methods: The thymus is mobilized, and the needle is passed through the sternum and back. In extensive or recurrent tracheomalacia, not only the ascending aorta but also the innominate artery and pericardial base are fixed to the sternum. The effect is monitored bronchoscopically., Results: This technique showed dramatic success in two children, one 4-year-old and a 2-year-old. In the younger child, the thoracoscopy was a redo procedure after a previous open aortosternopexy., Conclusions: Thoracoscopic aortopericardiosternopexy is an effective procedure that does not impair postoperative respiration. It should therefore be considered for severe tracheomalacia or even redo operations.

Objective: To outline two cases of congenital tracheocutaneous fistula and discuss the potential pathogenesis of this previously unreported developmental abnormality., Methods: Two cases of tracheocutaneous fistula evaluated at John Peter Smith Hospital (Fort Worth, TX) from May to October 2001 were reviewed. The surgical treatment of one infant is described., Results: Two infants were evaluated with a congenital fistula extending from the suprasternal region of the neck dorsally to the trachea in the midline. The infants were otherwise developmentally normal with unremarkable prenatal histories. Primary surgical closure of a fistula was accomplished without complication., Conclusions: Congenital tracheocutaneous fistula appears to be an isolated developmental abnormality not associated with the same degree of morbidity as acquired tracheocutaneous fistula. The development of a congenital tracheocutaneous fistula may be the result of abnormal epidermal migration secondary to a localized midline mesodermal defect. Congenital tracheocutaneous fistula may be successfully treated with primary closure. Observation and close follow-up of asymptomatic fistulas may be reasonable.