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Congenital airway malformations can present with respiratory distress, cyanosis, and difficulty feeding in the neonate or infant. Clinical presentation may vary from asymptomatic to fatal airway obstruction. They may exist in isolation or in association with vascular rings and slings, bronchopulmonary malformations, and/or syndromes. We present an unusual case of bridging bronchus, complete bronchial rings, and left pulmonary artery sling presenting with recurrent croup, highlighting the importance of bronchoscopy and CT imaging to achieve an accurate diagnosis in patients with recurrent croup and/or respiratory failure not responding to usual treatment measures and a multidisciplinary treatment approach., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Introduction: Lung agenesis is a rare disorder with a variable, but potentially very bad clinical course. It necessitates complex clinical management, especially in life-threatening situations., Case Report: We describe a case of a 6-month-old girl with right lung agenesis who required venovenous extracorporeal membrane oxygenation (VV-ECMO) due to pneumonia complicated by exacerbated previously diagnosed left main bronchus stenosis. The stenosis was resolved by endobronchial intervention and X-ray-guided stent insertion, which enabled weaning from ECMO and was aimed at preventing such a life-threatening respiratory failure in the future. Unfortunately, even with the functional stent, the baby died 2 months post-procedure due to unresolvable bronchial spasms., Discussion: Despite high endobronchial stenting-related mortality in children, in cases where no effective pharmacological or surgical alternatives exist, stenting may be safely performed during VV-ECMO support and be a viable option to overcome critical respiratory failure caused by bronchial stenosis.

A newborn infant was diagnosed with an imperforate anus, and colostomy was performed one day after birth. He exhibited repeated episodes of poor oxygenation during intubation, and a CT scan revealed a bridging bronchus. He was scheduled to undergo sacroperineal repair in the prone position 8 months later. A tracheal tube, with its tip modified to widen the opening, was inserted through the nose. This led to normal oxygenation, even while in the prone position. The surgery was completed without any complications., (© 2018 John Wiley & Sons Ltd.)

Background: Pediatric surgeons frequently offer prenatal consultation for congenital pulmonary airway malformation (CPAM) and congenital diaphragmatic hernia (CDH); however, there is no evidence-based consensus to guide prenatal decision making and counseling for these conditions. Eliciting feedback from experts is integral to defining best practice regarding prenatal counseling and intervention., Methods: A Delphi consensus process was undertaken using a panel of pediatric surgeons identified as experts in fetal therapy to address current limitations. Areas of discrepancy in the literature on CPAM and CDH were identified and used to generate a list of content and intervention questions. Experts were invited to participate in an online Delphi survey. Items that did not reach first-round consensus were broken down into additional questions, and consensus was achieved in the second round., Results: Fifty-four surgeons (69%) responded to at least one of the two survey rounds. During round one, consensus was reached on 54 of 89 survey questions (61%), and 45 new questions were developed. During round two, consensus was reached on 53 of 60 survey questions (88%)., Conclusions: We determined expert consensus to establish guidelines regarding perinatal management of CPAM and CDH. Our results can help educate pediatric surgeons participating in perinatal care of these patients., Level of Evidence: V., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Congenital bronchial stenosis is a rare entity [1,2]. While there are some reports of congenital bronchial anomalies and their repair in the thoracic literature this is first report in the literature of a bronchial take off just below the larynx managed with surgical success. Here we present a case of a former 33-weeker born with a tracheoesophageal fistula, Tetralogy of Fallot, and a high bifurcation of the right mainstem bronchus with a concomitant long segment bronchial stenosis. Preoperative planning included printing 3-D reconstructed models of the airway to analyze various treatment options. At 3 months of age the patient was taken to the operating room for surgical repair of the Tetralogy of Fallot and side-to-side tracheobroncheoplasty. The patient was extubated on postoperative day 3 and was discharged home without need for any ventilatory support., (Copyright © 2018. Published by Elsevier B.V.)

Background: It is advocated that heterotaxy should be segregated into right or left isomerism according to atrial appendage morphology. However, atrial situs determination is often based on the pattern of associated findings rather than on atrial morphology itself, which can be difficult to define. The objective was to assess how often concordant patterns of isomerism classified by atrial appendage morphology, bronchopulmonary pattern, and splenic status are breached using cardiac magnetic resonance or computed tomography. The secondary objective was to determine the feasibility of defining atrial appendage morphology using cardiac magnetic resonance or computed tomography., Methods and Results: Retrospective review of 114 pediatric patients (median, 2.4 years; range, 1 day-17.9 years) with heterotaxy who underwent cardiac magnetic resonance or computed tomography was performed to evaluate atrial appendage, bronchopulmonary, and visceral organ arrangements. Atrial appendage and splenic anatomy were not definable in 17 of 114 (15%) and 4 of 114 (3.5%) patients, respectively. In the remaining 93 patients, 39% had classic right isomerism (bilateral right atrial appendages, right bronchopulmonary pattern, and asplenia) and 40% had classic left isomerism (bilateral left atrial appendages, left bronchopulmonary pattern, and polysplenia). Classic pattern of isomerism was breached in 20 of 93 (21.5%) patients: 13 (65%) displayed incongruent abdominal situs, 5 (25%) incongruent bronchial situs, 1 (5%) had discrepant appendage morphology, and 1 (5%) incongruent situs at all levels., Conclusions: Atrial appendage morphology is difficult to assess and not always indicative of bronchopulmonary or abdominal situs. Discordance between bronchopulmonary branching, atrial appendage arrangement, and splenic status was identified in >20% patients with heterotaxy. Independent description of each organ system is required when arrangements are disharmonious among different organ systems., (© 2018 American Heart Association, Inc.)

Background: Bodily isomerism, also referred to as heterotaxy, involves predominantly the thoracic organs, although other organs are usually abnormally positioned. Previously assessed on the basis of splenic anatomy, it is now understood that isomerism is better segregated on the basis of atrial appendage morphology. This allows for anticipation of associated findings. We aimed to assess the accuracy of segregation based on the morphology of the atrial appendages and other structures more easily identified by echocardiography., Methods: We reviewed postmortem specimens of hearts from the archives at four institutions categorised as obtained from patients with "heterotaxy". The cardiac structures were analysed using sequential segmental analysis. Non-cardiac structures were also examined if available. Statistical analyses were performed to compare differences in the settings of right as opposed to left isomerism., Results: Specimens were available from 188 patients. Of these, 57 had left isomerism, and 131 had right isomerism. Atrial appendages were isomeric in all patients. A coronary sinus was found only in left isomerism, whereas a terminal crest, or a Eustachian valve, was found only in right isomerism. Interruption of the inferior caval vein was associated with left isomerism, whereas totally anomalous pulmonary venous connection was associated with right isomerism., Conclusion: Isomerism is uniformly segregated on the basis of the morphology of the atrial appendages, itself defined by the extent of the pectinate muscles. Other features such as the presence of a coronary sinus and systemic venous return can further help with such segregation of isomerism.

Objectives: To determine the frequency of histologic features of impaired lung vascular and alveolar development and to identify the presence of intrapulmonary bronchopulmonary anastomoses (IBA) in infants and children who died with Down syndrome., Study Design: A retrospective review of autopsy reports and lung histology from 13 children with Down syndrome (ages: 0-8 years) was performed. Histologic features of abnormal lung development were identified and semiquantified, including the presence of IBA. Three-dimensional reconstructions of IBA were also performed. Comparisons were made with 4 age-matched patients without Down syndrome with congenital heart defects who underwent autopsies during this time period., Results: Of the 13 subjects with Down syndrome, 69% died from cardiac events, 77% had a congenital heart defect, and 46% had a clinical diagnosis of pulmonary hypertension. Lung histology from all subjects with Down syndrome demonstrated alveolar simplification, and 92% had signs of persistence of a double capillary network in the distal lung. The lungs from the subjects with Down syndrome frequently had features of pulmonary arterial hypertensive remodeling (85%), and prominent bronchial vessels and IBA were observed in all subjects with Down syndrome. These features were more frequent in subjects with Down syndrome compared with control subjects., Conclusions: Children with Down syndrome who died of cardiopulmonary diseases often have histologic evidence of impaired lung alveolar and vascular development, including the presence of prominent IBA and pulmonary hypertension. We speculate that children with Down syndrome are at risk for reduced lung surface area and recruitment of IBA, which may worsen gas exchange in subjects with Down syndrome., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Here we report a neonatal case of total anomalous pulmonary venous connection with left lung agenesis. Diagnostic imaging demonstrated that the left pulmonary veins were totally absent and the right pulmonary veins connected with the common pulmonary chamber. Drainage from the common pulmonary venous chamber entered the persistent left suerior vena cava. In addition, it revealed complete absence of the left main bronchus and left lung vessels. The neonate successfully underwent surgical repair 18 days after birth., (© The Author(s) 2014.)

Bilateral nephroblastomatosis (NB) is an uncommon renal anomaly characterized by multiple confluent nephrogenic rests scattered through both kidneys, with only a limited number of cases reported in the medical literature. Some of these children may have associated either Perlman or Beckwith-Wiedemann syndrome and others do not demonstrate syndromic features. We report a full-term boy with anteverted nose, bilateral bronchial stenosis due to lack of cartilage, bilateral obstructive renal dysplasia and NB with glomeruloid features. The infant had visceromegaly, but neither gigantism nor hemihypertrophy. Immunohistochemistry for PAX2 (Paired box gene-2) and WT-1 (Wilms Tumor 1) were strongly positive in the areas of NB. GLEPP-1 (Glomerular Epithelial Protein) did not stain the areas of NB with a glomeruloid appearance, but was positive in the renal glomeruli as expected. We found neither associated bronchial stenosis nor the histology of NB resembling giant glomeruli in any of the reported cases of NB.

An infant with a ventricular septal defect; Vertebral anomalies, Anal atresia, Cardiac anomalies, Tracho Esophageal fistula (TEF), Renal anomalies, Limb anomalies syndrome; and tracheal stenosis with a bridging bronchus underwent repair of the ventricular septal defect and trachea-bronchial reconstruction at age 11 months. Herein we describe our surgical approach to resection of the bridging bronchus and a technique using a modified end-to-end tracheal anastomosis for the correction of this complex anomaly., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

We report a case of a 14-year-old female with abnormally high takeoff of the right coronary artery (RCA) that was associated with a ventricular septal defect, right aortic arch, and bridging bronchus. During surgery, an exceptionally high takeoff of the RCA was discovered. Postoperative computed tomography confirmed the presence of the associated right aortic arch with anomalous branching pattern, and bridging bronchus., (© 2014 Wiley Periodicals, Inc.)

We present a 3-month-old boy with a type IIA sling left pulmonary artery associated with imperforate anus and rectourethral fistula. Tracheobronchial abnormalities are demonstrated using multidetector CT with 3-D volume rendering of the airways. This case represents a novel variant of an already rare entity with an unusually high right upper lobe bronchus and no evidence of associated tracheobronchial stenosis., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Congenital bronchial stenosis is rarely described and is difficult to manage. Here we report two cases of right intermediate bronchial stenosis (stenotic orifice with complete cartilage rings). Both cases were associated with ventricular septal defects, and a "trifurcation" pattern was found in both carinas. Both patients underwent surgical repair of the ventricular septal defects but could not be separated from ventilator support despite successful cardiac operations. Slide tracheobronchial plasty was applied to the right intermediate bronchus and lower trachea. After correction of the bronchial stenosis, both patients could be extubated and live without supplementary ventilation support., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

A 35-year-old woman with a high fever had a cystic lesion of the right lung including fluid and air. She was diagnosed with an infected bronchial cyst caused by congenital bronchial atresia with partial anomalous pulmonary venous return. She underwent a right upper lobectomy successfully. Patients with congenital bronchial atresia often have recurrent pulmonary infections with various imaging findings. The aberrant vein may relate to interruption of the bronchus in a patient with partial anomalous pulmonary venous return. Although segmental resection is recommended for this benign disease, lobar resection may be unavoidable because of adhesions or destruction of adjacent segments.

Pulmonary artery slings are rare congenital anomalies seen only on the left side. The presence of right pulmonary artery (RPA) sling is extremely rare. We report a case of a RPA sling with a single lung associated with tracheal stenosis and its management., (© 2013 Wiley Periodicals, Inc.)

We describe the case of a 2-month-old child with complex tracheal hypoplasia with bilateral bronchial hypoplasia and left pulmonary hypoplasia. Tracheal hypoplasia is complex when it is associated with critical stenosis, cricoid stenosis, bronchial hypoplasia, tracheal bronchus, or esophageal atresia with severe tracheomalacia. Slide tracheoplasty is the gold standard treatment for the complex tracheal hypoplasia., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Lesions of the thoracic inlet present a significant challenge to the surgeon due to the difficulty of access and proximity to important neurovascular structures within the region. We describe two cases of benign disease of the thoracic inlet in children, one bronchogenic cyst and an esophageal duplication, and report the cervical approach used to manage them. Both lesions extended from the neck through the thoracic inlet, but demonstrate how benign lesions in this area can be delivered up into a cervical incision, negating the need for the more invasive modified thoracotomies. A cervical approach can be safely and successfully used to approach benign pathology, such as bronchogenic cysts and oesophageal duplications of the thoracic inlet. Careful multidisciplinary planning is required for such procedures., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Communicating bronchopulmonary foregut malformations are extremely rare congenital malformations, characterized by a communicating fistula between an isolated part of the respiratory system and the esophagus or the stomach. In this article, we present a case of esophageal atresia type C, later diagnosed as a rare form of a communicating bronchopulmonary foregut malformation, an esophageal atresia combined with right main bronchus originating from the lower esophagus. Therapeutic resection of the right lung was complicated by postpneumonectomy syndrome., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Communicating bronchopulmonary foregut malformation (CBPFM) and congenital tracheal stenosis (CTS) are difficult developmental disorders especially when they are presented simultaneously in a patient. The authors report a case of a newborn boy born at 37 weeks of gestation weighing 2356 g with CBPFM (right esophageal lung) and long segment CTS. Staged surgical repair (by-force endotracheal intubation for securing the airway followed by bronchotracheal anastomosis for CBPFM, tracheostomy with handmade, length-adjustable tracheostomy tube, and slide tracheoplasty) was performed. He has been healthy without tracheostomy for 25 months after slide tracheoplasty. This is the first report of a successful tracheobronchial reconstruction for a patient with a long segment CTS and CBPFM preserving the affected lung function., (Copyright © 2012 Elsevier Inc. All rights reserved.)

We present a 28 year old man with repaired Tetralogy of Fallot who underwent CT imaging prior to pulmonary valve replacement. CT demonstrated a partial anomalous LPA with associated bronchial anomalies and normal variant pulmonary venous drainage. The case demonstrates the utility of multislice CT in delineating 3-dimensional vascular and bronchial anatomy in these complex anomalies., (Copyright © 2012 Society of Cardiovascular Computed Tomography. Published by Elsevier Inc. All rights reserved.)

To determine the most appropriate treatment strategy for neonates with coexisting congenital heart diseases (CHD) requiring surgery, 408 neonates treated in our department between 2002 and 2009 were retrospectively reviewed. Of the 408 neonates, 37 (9.1%) had coexisting CHD. Among the pediatric diseases requiring surgery, esophageal atresia (46.7%), anorectal malformation (24.1%), and tracheobronchial anomalies (75.0%) were frequently associated with CHD, and the survival rate among patients with coexisting CDH was 24.3%, about 2-fold higher than among those without coexisting CHD. Clinical observations in the present series suggested that fatal conditions such as tracheoesophageal fistula should be repaired immediately after birth, followed by palliative cardiac surgery to stabilize the circulatory status if necessary. Thereafter, radical repair of cardiac and pediatric anomalies should be performed, taking into account the balance between surgical stress and cardiac reserve. For this purpose, proper assessment of surgical stress and cardiac function and collaboration among specialists in relevant fields are essential during the perinatal period to determine the treatment strategy for patients with multiple-organ anomalies.

Tracheal bronchus is an aberrant bronchus usually originating from the right lateral wall of the trachea, with an incidence ranging from 0.1% to 5% and usually within 2.0cm above the carina. The incidence of lung cancer with bronchial anomaly is very rare. There are only nine cases of lung cancer developing from the tracheal bronchus reported in the literature. Histological examination showed squamous cell carcinoma in only three of them, and we present a fourth case who was a 57-year-old man. Interestingly our patient's anomaly included both an absence of the normal right upper bronchus as well as right upper lobe ventilated from the true tracheal bronchus. This is the first documented case in the world of a squamous carcinoma arising from the true tracheal bronchus. Post-surgical histological stage was T2aN0M0 (stage IB). The patient is well 48 months after the operation and has no evidence of recurrence., (2009 SEPAR. Published by Elsevier Espana. All rights reserved.)

A 34-year-old female with WPW syndrome has been under surgical treatment in our clinic with left basal chronic pleural empyema. During the surgical intervention multiple anatomical anomalies were detected, like unique pleural cavity, monoblock lung left-right, hypoplasic left lung, no scissures on the left side, no left pulmonary arteria with an aberrant arteria for the left lung derived from the right pulmonary arteria, the same for the bronchis. The pleural-pulmonary shelling proceeded without incidents and it has been finalized with the drainage of the whole pleural cavity.

Neonates with congenital diaphragmatic hernia (CDH) often have other major malformations. Anatomic airway anomalies associated with CDH include congenital stenosis, pulmonary hypoplasia, and abnormal bronchial branching. We describe an infant with CDH who, after developing recurrent upper-lobe atelectasis, was found to have a right tracheal bronchus. This clinically significant anomaly can have important and potentially preventable adverse consequences., (Thieme Medical Publishers.)

Horseshoe lung, a rare congenital anomaly, is almost always associated with unilateral (usually right-sided) lung hypoplasia, and, in most cases, in conjunction with the scimitar syndrome. We present an 8-month-old boy with horseshoe lung associated with left-lung hypoplasia, left pulmonary artery sling and bilateral agenesis of the upper lobe bronchi, diagnosed by multidetector CT (MDCT) imaging. The study also revealed an anomalous origin of the left vertebral artery as the last branch of the aortic arch, distal to the left subclavian artery, and an anomalous origin of the left common carotid artery from the brachiocephalic trunk. A hemivertebral anomaly of the seventh cervical vertebra was incidentally detected. MDCT with high-quality multiplanar and three-dimensional reconstructions is a noninvasive and rapid technique for detecting the complex combination of vascular, tracheobronchial and parenchymal anomalies, and any potential bone anomalies, in one imaging study.

A full-term newborn male infant presented with dyspnea and cleft lip and palate. He was thought to have esophageal atresia with tracheoesophageal fistula. He underwent bronchoscopy before operation that showed a laryngotracheoesophageal cleft (LTEC) type III. The left main bronchus originated from the lower esophagus. His diagnosis was communicating bronchopulmonary foregut malformation (CBPFM) type IA associated with LTEC type III. Enhanced chest computed tomographic scan showed the left pulmonary artery originated from the descending aorta. Staged operations were indicated. At first, reconstruction of the left pulmonary artery was done at 3 months of age. Then at 6 months of age, operations for LTEC (tracheoplasty and esophagostomy) and CBPFM left bronchoplasty were performed. Reconstruction of esophagus was performed at age of 1 year. He is now 3 years old and doing well with a mild degree of bronchomalacia. This is the first report of total reconstruction of CBPFM type IA associated with LTEC.

VACTERL association is an acronym made of associated defects including vertebral anomalies, anal atresia, cardiac, tracheal-esophageal fistula, and renal/radial limb anomalies. Tracheal bronchus is a condition characterized by ectopic location of the right upper lobe bronchus at the mid to distal trachea. This condition is associated with congenital anomalies and has been reported in one previous case of VACTERL. We report another infant with VACTERL presenting with respiratory complications due to presence of tracheal bronchus. She also had a narrowed segment of her right main stem bronchus., ((c) 2008 Wiley-Liss, Inc.)

Summary: Congenital oesophageal stenosis due to tracheobronchial remnants is a very rare condition characterized by the presence of tracheobronchial tissue in the oesophageal wall. The most common symptoms are dysphagia, regurgitation and hypersalivation. These usually appear in early infancy when solid food is introduced into the diet. Clinical diagnosis is difficult. Conventional radiology shows a dilated oesophagus without peristalsis, incomplete expansion of the gastro-oesophageal transition zone and delayed oesophageal emptying. Lesions suggesting oesophagitis may be present at endoscopy but pH-metry may be normal. The combination of stenosis with or without oesophagitis with normal pH-metry suggests that the patient's symptoms may be due to factors other than intraluminal and that further investigation is needed. The treatment of this condition requires surgical resection of the stenosis with little morbidity or mortality. The presence of tracheobronchial remnants is confirmed upon histopathological examination of the resected bowel segment.

We describe a case of "topsy-turvy heart" that necessitated simultaneous left tracheobronchial repair, aortic arch reconstruction, and bronchial stenting in an 11-month-old girl. This case highlights the significant therapeutic dilemmas clinicians are faced with when dealing with conditions having an unknown natural history and unpredictable surgical outcomes, and reinforces the need for these patients to be managed by a multidisciplinary team approach.

Bridging bronchus (BB) is a rare, congenital bronchial anomaly that is frequently associated with congenital cardiac malformations, especially left pulmonary artery sling. It represents an anomalous bronchus to the right originating from the left main bronchus. Discrimination from other bronchial anomalies is important, since BB is frequently associated with bronchial stenoses due to abnormal cartilage rings. This case study describes the findings of bronchoscopy, bronchography and multidetector computed tomography (MDCT) in three patients. Bronchoscopy was helpful in the description of the severity and length of bronchial stenoses. However, it was not possible to establish a diagnosis of BB based on this method in two patients, since it is difficult or even impossible to differentiate the bifurcation from the pseudocarina. It was not possible to establish the correct diagnosis in all patients based on bronchography or MDCT. MDCT was able to depict the relationship of bronchial and vascular structures, which is particularly important in patients with pulmonary artery sling. Multidetector computed tomography is preferable to bronchography as it is less invasive and due to its short acquisition time it can be performed in children with severe respiratory disease. In the current authors' experience, detection of cartilage rings still requires flexible bronchoscopy.