Management Strategies for Congenital Heart Disease Comorbid with Airway Anomalies in Children.

OBJECTIVE: To assess management strategies for pediatric patients with the challenging combination of congenital heart diseases (CHDs) and airway anomalies. STUDY DESIGN: Patients diagnosed with CHD and airway anomalies in the Pediatric Cardiac Surgery Centre of Fuwai Hospital from January 2016 to December 2020 were included in this retrospective study. Patients were divided into three groups based on different management, including the conservative group, the slide group (slide tracheoplasty), and the suspension group (suspension with external stenting). Patients' data and computed tomography measurements from medical records were reviewed. RESULTS: A total of 139 patients were included in the cohort; 107 had conservative airway treatment (conservative group), 15 had slide tracheoplasty (slide group), and 17 had tracheal suspension operation (suspension group). The top three associated intracardiac anomalies were ventricular septal defect (n = 34, 24%), pulmonary artery sling (n = 22, 16%), and tetralogy of Fallot (n = 15, 11%). Compared with patients with conservative airway management (100 minutes [median], 62-152 [IQR]), the extra airway procedure prolonged cardiopulmonary bypass duration, with 202 minutes (IQR, 119-220) for the slide group and 150 minutes (IQR, 125-161) for the suspension group. Patients who underwent slide tracheoplasty required prolonged mechanical ventilation (129 minutes [median], 56-328 [IQR]). Of the total cohort, 6 in-hospital deaths, all in the conservative group, and 8 mid-to long-term deaths, with 6 in the conservative group, occurred. CONCLUSIONS: Both conservative and surgical management of CHD patients with airway anomalies have promising outcomes. Extra tracheobronchial procedures, especially the slide tracheoplasty, significantly prolonged cardiopulmonary bypass duration. Based on multidisciplinary team assessment, individualized management strategies should be developed for these patients.

Type 2 bridging bronchus with left pulmonary artery sling.

Bridging bronchi are the rarest of the major airway anomalies reported in the literature. In this brief report, we present a case of a symptomatic adult male patient presenting with a type 2 bridging bronchus associated with left pulmonary artery sling.

Flexible Fiber-optic Bronchoscopy-directed Interventions in Children with Congenital Heart Diseases.

OBJECTIVE: In children, pulmonary and cardiac diseases are closely associated, and their integrated evaluation is important. Flexible fiber-optic bronchoscopy (FFB) can be used for both diagnostic and therapeutic purposes in pediatric cardiac intensive care units (PCICU). The objective of this study was to evaluate the utility of FFB in children with congenital heart disease (CHD). MATERIALS AND METHODS: A retrospective, descriptive study was conducted at a tertiary care center in pediatric patients who underwent FFB in PCICU over a period of 6 years (2012-2017). RESULTS: Total 71 bronchoscopies were done in 58 patients with CHD with median age and weight of 2.5 months and 3.4 kg, respectively. Total of 20 different cardiac lesions were present among patients who underwent FFB. While 38 (53.5%) and 30 (42.3%) procedures were performed in pre-op and postoperative patients, respectively, 3 intraoperative bronchoscopies were also performed. The main indications for FFB were persistent atelectasis (42/71), prolonged oxygen requirement (13/71), stridor (8/71), and suspected airway anomaly (6/71). Tracheobronchitis was the commonest bronchoscopy finding (51/71, 71.8%) followed by tracheobronchomalacia (27/71, 38.3%). Cause of stridor detected in 7/8 cases. Associated preoperative and postoperative respiratory complications were detected and necessary interventions were done. These included slide tracheoplasty (5/58), tracheostomy (5/58), antibiotic change based on bronchoalveolar lavage (BAL) cultures (11/71), and continued positive pressure ventilation (4/71). Nonconsequential complications were transient hypoxemia (10/71), bleeding (2/71), and transient bradycardia (1/71). CONCLUSION: Bedside FFB is a safe and a valuable diagnostic tool that also helps in guiding interventions in children with cardiac diseases. HOW TO CITE THIS ARTICLE: Sachdev A, Chhawchharia R, Gupta D, Gupta N, Joshi R, Agarwal N. Flexible Fiber-optic Bronchoscopy-directed Interventions in Children with Congenital Heart Diseases. Indian J Crit Care Med 2020;24(5):340-343.

Respiratory syncytial virus prophylaxis in infants with congenital airway anomalies compared to standard indications and complex medical disorders.

An observational study was conducted of children < 2 years who received ≥ 1 dose of palivizumab in 32 Canadian institutions from 2005 to 2017. We compared respiratory illness (RIH) and respiratory syncytial virus-related hospitalization (RSVH) hazards in children with a congenital airway anomaly (CAA) versus those prophylaxed for standard indications (SI) and serious medical disorders (SMD). Data were assembled on neonatal course, demographics, palivizumab utilization and adherence, and respiratory illness events, and analyzed using ANOVA, chi-square tests and Cox proportional hazards. Twenty-five thousand three children (1219 CAA, 3538 SMD, and 20,246 SI) were enrolled. Palivizumab adherence was 74.8% overall and similar across groups. For 2054 respiratory-related events, 1724 children were hospitalized. RIH rates were 13.6% (CAA), 9.6% (SMD), and 6.0% (SI). RSVH rates were 2.4% (CAA), 1.6% (SMD), and 1.5% (SI). After adjustment for demographic and neonatal differences, children with a CAA had a significantly increased RIH and RSVH hazard relative to SI (RIH, HR = 1.6, 95% CI 1.2-2.2, p = 0.002; RSVH, HR = 2.1, 95% CI 1.0-4.4, p = 0.037) but similar to SMD (RIH, HR = 1.3, 95% CI 0.9-1.9, p = 0.190; RSVH, HR = 1.7, 95% CI 0.7-4.1, p = 0.277).Conclusion: Children with a CAA experience higher RIH risk. RSVH hazard was similar between CAA and SMD but higher for CAA compared to SI, implying that this population requires surveillance for RSV prophylaxis. What is Known: • Children with congenital airway anomalies (CAA) are at risk for respiratory tract illness and respiratory syncytial virus-related hospitalization (RSVH) with accompanying morbidity and mortality • RSV prophylaxis may be useful in children with a CAA, but is not routinely recommended What is New: • Children with a CAA had a 1.6-2.3 fold greater risk of respiratory-related hospitalization and RSVH compared to those prophylaxed for standard, approved indications and serious medical disorders. • RSVH risk in children aged < 2 years with either upper or lower airway anomalies is similar. Children with a CAA require careful surveillance during the RSV season and prophylaxis may be appropriate.

Nasal high-flow oxygen delivery in children with abnormal airways.

BACKGROUND: Transnasal Humidified Rapid-Insufflation Ventilatory Exchange has been shown to safely prolong the safe apnea time in well children post induction of anesthesia and is rapidly becoming a new standard for apneic oxygenation in adults. The same oxygenation technique is described as nasal high flow and can be used in infants and children at risk of apnea during anesthesia. AIM: We investigated the use of nasal high flow oxygen delivery during anesthesia in children with abnormal airways requiring tubeless airway assessment or surgery. METHODS: Data and outcomes of pediatric patients receiving nasal high flow for upper airway procedures were analyzed. Four categories were defined: (i) tubeless airway surgery, (ii) flexible bronchoscopy, (iii) expected difficult airway, and (iv) comorbidity related risk of apnea. Anesthesia was induced intravenously or with sevoflurane (4-8%) and then converted to total intravenous anesthesia aiming for spontaneous ventilation. Age appropriate nasal high flow cannulae were secured with 100% oxygen delivery at weight-related flow rates. Topicalization of the airway was achieved with lignocaine. Complication rates of desaturation requiring interruption of procedure for rescue oxygenation were recorded. RESULTS: Twenty children were analyzed with age range of 5 days to 11 years, ASA 1-4, and weight range 3-57 kg. Fifteen were induced with sevoflurane and 100% oxygen, five received total intravenous anesthesia only. All children received Optiflow™ nasal high flow and intravenous anesthesia during their procedure. Average SpO2 recorded was 96% with lowest SpO2 77%. One required rescue oxygenation. Median length of procedure was 32 min, (range 3-61). Most common indication was tubeless airway surgery but seven children had more than one indication. CONCLUSION: Nasal high flow can be used in spontaneously breathing children with abnormal airways for maintenance of oxygenation during anesthesia for tubeless airway procedures.

The implications of airway anomalies on children with congenital heart disease and bronchiolitis.

BACKGROUND: Bronchiolitis is a common airway infection in young children. Hemodynamically significant congenital heart disease (CHD) predicts a more complicated course. However, the role of airway anomalies remains unknown. METHODS: We retrospectively reviewed the records of patients under 2 years old, diagnosed with CHD, and admitted between January 2011 and December 2013, before the palivizumab era. Records of bronchiolitis admissions were also extracted. Patients were grouped according to CHD condition and airway anomalies. RESULTS: A total of 230 patients with CHD were enrolled. A total of 180 (78%) and 71 (31%) patients had hemodynamically significant CHD and airway anomalies, respectively. A total of 52 (22.6%) patients were admitted for bronchiolitis 78 times. Among them, 33 (63.5%) had hemodynamically significant CHD, and 28 (53.8%) had airway anomalies. In patients with bronchiolitis admissions, the mean ventilator use, intensive care unit stay, and hospital stay were 1.08, 4.08, and 15.19 days, respectively. When compared, the mean hospital stay for bronchiolitis patients with airway anomalies was significantly longer than that of those without airway anomalies (19.8 vs. 9.9 days, p = 0.008). When further divided the patients by the presence hemodynamic significance, patients with hemodynamically significant CHD and airway anomaly had longer hospital stay than those who had neither. (21.7 vs. 8.3 days, p = 0.004) Airway anomaly was a significant risk factor for longer hospital stay in linear regression model (p = 0.007). CONCLUSIONS: Airway anomalies are common in children with CHD and are associated with longer hospital stays on bronchiolitis admission. An active survey for airway anomalies and adequate prophylaxis for bronchiolitis infection might be important in the care of children with CHD associated with airway anomalies.

Tracheoesophageal Fistula: Airway Management and Temporization in a Community Hospital Setting.

A tracheoesophageal fistula (TEF) is a rare anatomical abnormality that can present significant challenges for the anesthesia provider. TEFs, depending on location and size, can result in aspiration, hypoxia, and difficulty with ventilation in the intensive-care unit (ICU) and operating room (OR) settings. Though usually seen and most commonly described as a congenital abnormality, it can also be an acquired condition in adults. Early recognition and diagnosis of TEF are of paramount importance to avoid respiratory complications. The rapid isolation of the TEF is key to management and different methods can be used to temporize the clinical situation until definitive surgical or endoscopic procedures can be accomplished. We discuss methods of temporization of the clinical situation, especially in a community hospital setting with limited access to immediate and sophisticated treatment.

Airway anomalies in patients with 22q11.2 deletion syndrome: A scoping review.

OBJECTIVE: People with 22q11.2 deletion syndrome (22q11DS) can present with a wide variety of findings. Various airway anomalies have been described intermittently within this syndrome, but this feature has not been extensively investigated. Increased provider awareness of these findings may help guide clinical decision-making and improve overall patient outcomes. The objectives of this review are to identify the types of airway anomalies in 22q11DS and the prevalence of airway anomalies within symptomatic individuals. METHODS: PubMed/MEDLINE, Cochrane Library, and EMBASE databases were searched in February 2022 for all available articles. Search terms included those that described 22q11DS or one of its synonymous conditions AND those that described airway anatomy and anomalies. The term airway anomaly was defined as any structural aberration in the conductive airway from the oral or nasal vestibule region to the mainstem bronchus. Studies were screened by two authors. A review of references was conducted. Eligible manuscripts underwent full-text review for quality appraisal and data extraction. RESULTS: From a total of 909 unique manuscripts, 58 studies were selected, describing 328 people. The prevalence of airway anomaly diagnosis within symptomatic individuals ranged from 14% to 74%. Twenty-two unique airway anomalies were described. Laryngeal web was the most frequently described anomaly, followed by airway malacia and subglottic stenosis. Laryngeal web was 40% sensitive for suggesting a diagnosis of 22q11DS. Among affected individuals, as many as 46% had multiple concomitant airway anomalies. Aside from respiratory symptoms, other features that prompted airway evaluation included difficult intubation or failed extubation. CONCLUSION: The findings within this review support the notion that a wide variety of airway anomalies may be seen in people with 22q11DS and that these findings have been discovered frequently in those with airway symptoms. Providers should maintain a low threshold to perform an airway examination in those with 22q11DS, especially when airway symptoms are present.

Recurrence of Laryngeal Hemangioma in an Adult: A Case Report.

Laryngeal hemangiomas in adults are uncommon. Laryngeal hemangiomas are more common in children, and if treatment is required, it is treated with propranolol. An eighteen-year-old female with rare glottic hemangioma extending to a supraglottic region presented with shortness of breath and hoarseness of voice. Although complete resection of the hemangioma was impossible due to its proximity to the vocal cord, it was treated with Microlaryngoscopy ablation with a CO2 laser. She has been followed up for over 10 years. The patient has had three recurrences, which have also been described. The cause of recurrence after treatment and the trigger for the increase in the size of the hemangioma is yet to be identified. The patient is now stable after her last Microlaryngoscopy and CO2 ablation. In conclusion, a near complete resection of hemangioma is required to prevent regrowing of hemangioma.

Tracheal Bronchus and Associated Anomaly Prevalence Among Children.

Background and objectives Tracheal bronchus (TB) is a rare congenital airway anomaly originating from the trachea, with a reported prevalence of 0.9%-3% in children. Although TB was studied in the literature, this anomaly was not evaluated in Qatar. Our study aimed to identify the prevalence and congenital anomalies associated with TB in children in Qatar. Design In this descriptive study, we identified patients who underwent flexible bronchoscopy (FB) at two large tertiary centers in Qatar from July 2007 to November 2020. The patients' demographic, bronchoscopic, and radiologic data were collected. The prevalence of TB and associated congenital anomalies were determined. Results Of 1786 patients who underwent FB, 20 (1.12%) were diagnosed with TB. The median age at the time of diagnosis was 31 months (range, 2-154 months). The associated congenital anomalies were identified in 16 cases (80%; p = 0.007). Cardiac defects represent the most common associated anomaly (8/20, 40%). Conclusion This study revealed that TB is an uncommon airway anomaly and emphasizes its significant association with other congenital malformations. Our findings should alert physicians to other associated TB anomalies and provide timely management when needed.

The prevalence of lower airway anomalies in children with Down syndrome compared to controls.

INTRODUCTION: Children with Down syndrome (DS) often present with chronic respiratory symptoms. Congenital airway anomalies have been described but data about prevalence is scarce and a comparison to controls is lacking. We aim to compare the endoscopic and clinical data of children with DS to controls without significant medical history. METHODS: All endoscopic procedures under general anesthesia (broncho- and/or direct laryngoscopy) in patients with DS were reviewed. We compared clinical and endoscopic data to a cohort of children with respiratory symptoms but without any other relevant medical history. RESULTS: Endoscopic data were available for 65 patients with DS. The median age was 2.9 years (range: 0.2-17), 63% were boys. The most common clinical presentation was recurrent respiratory infections (37%). Other major symptoms were chronic cough and/or noisy breathing (23%) and stridor (20%). Endoscopy was normal in 29% of patients. The largest group of patients (44%) had some form of airway malacia. Tracheal bronchus and subglottic stenosis were each isolated findings in 3.1% of patients. Twenty percent presented with combined airway anomalies. The control group consisted of 150 children (matched for age and sex) without significant underlying disease. The most common presentations were chronic cough and/or noisy breathing (29%), persistent radiographic abnormalities (20%), and suspicion of aspiration of a foreign body (15%). In the majority of controls (68%), no airway anomaly was found. Other findings were malacia (22%), tracheal bronchus (1%), and subglottic stenosis (1%). A combined anomaly was found in 5%. CONCLUSION: Congenital airway anomalies were seen in 71% of patients with DS, compared with 32% of controls. Combined anomalies are more frequent in DS. Complete lower airway endoscopy is recommended in patients with DS as it may influence therapeutic decision-making.

The bridging bronchus: A comprehensive review of a rare, potentially life-threatening congenital airway anomaly associated with cardiovascular defects.

The bridging bronchus is a rare congenital airway anomaly in which the right upper lobe of the lung is supplied by the right main bronchus while the right lower lobe, and often the right middle lobe is supplied by an aberrant bronchus arising from the left main bronchus. The aberrant bronchus crosses (bridges) the lower part of the mediastinum, hence the term bridging bronchus (BB). This potentially life-threatening condition, usually accompanied by diffuse or focal airway stenosis, commonly presents with signs and symptoms related to large airway obstruction, such as respiratory distress, apnea, wheezing, stridor, and recurrent respiratory tract infections. Diagnosis of the BB is often challenging because the associated signs and symptoms mimic those of common conditions such as bacterial and viral bronchiolitis, bronchial asthma, cystic fibrosis, and foreign body aspiration. The BB is also often accompanied by congenital cardiovascular anomalies, including left pulmonary artery sling, atrial, and ventricular septal defects, tetralogy of Fallot, patent ductus arteriosus, and coarctation of the aorta. Patients presenting with the above signs and symptoms who are not responsive to standard treatment modalities, and have accompanying cardiovascular congenital anomalies should, therefore, be investigated for the BB. Herein, we review the anatomy, embryology, clinical presentation, differential diagnosis, imaging techniques and surgical management of the BB.

Airway Anomalies.

This article reviews congenital anomalies involving the larynx and trachea, including congenital subglottic stenosis, laryngeal webs, laryngeal cleft, and tracheal stenosis. Presenting signs and symptoms, prevailing surgical repair techniques, and postoperative care are discussed.

Esophageal lung with rare associated vascular and anorectal malformations.

Esophageal lung is a rare communicating bronchopulmonary foregut malformation in which the main bronchus arising from the trachea is absent. The affected lung is usually hypoplastic and aerated via an anomalous airway originating from the esophagus. Other anomalies such as esophageal atresia with tracheoesophageal fistula or VACTERL (vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, and limb abnormalities) association can co-exist. The initial radiographic findings may be normal, but subsequent imaging usually shows progressive and recurrent lung collapse, probably because of recurrent aspiration through the anomalous airway and poor compliance of the affected lung during breathing. In this report, we describe a neonate with esophageal lung and rare associated anomalies, including anorectal malformation, pulmonary artery sling, and inferior vena cava interruption with azygous continuation. To our knowledge, this is the first report of esophageal lung with such associations.

Association of airway abnormalities with 22q11.2 deletion syndrome.

INTRODUCTION: 22q11.2 deletion syndrome (22q11.2DS) presents with complex but variable symptoms, including cardiac, immune, palatal, endocrine, cognitive, and psychiatric issues. However, an association of 22q11.2DS with structural airway abnormalities has not been formally described. The aim of this study was to document the frequency of this association. METHODS: We retrospectively reviewed medical records of patients with 22q11.2DS evaluated in the 22q and You Center at the Children's Hospital of Philadelphia between 1999 and 2015 referred to otolaryngology for an airway assessment. Type of airway abnormality and presence of comorbidities, such as congenital heart disease, tracheostomy, and association with prenatal symptomatology such as polyhydramnios, were noted. RESULTS: Of the 104 patients who underwent an otolaryngology procedure (microlaryngoscopy or bronchoscopy), 71% (n = 74) had airway abnormalities. Patients with airway abnormalities ranged in age from 5 months to 37 years, with similar prevalence among males and females. Observed airway abnormalities included tracheomalacia (36%), subglottic stenosis (28%), laryngomalacia (26%), glottic web (21%), and bronchomalacia (16%). Most patients with airway abnormalities (91%) had an associated congenital heart defect, with ventricular septal defect and Tetralogy of Fallot being the most prevalent. Importantly, 30% of patients required a tracheostomy, and overall polyhydramnios was noted in 16% of pregnancies. CONCLUSION: Airway abnormalities are a common feature of 22q11.2DS, leading to substantial morbidity, particularly when combined with complex cardiac disease. Polyhydramnios may be an important prenatal clue to both the diagnosis of 22q11.2DS and airway anomalies. Postnatal assessment of airway structure and function among patients with 22q11.2DS is an important component of overall evaluation and will help guide long-term management.

The Tools of the Trade - Uses of Flexible Bronchoscopy.

Bronchoscopes have markedly improved the diagnosis as well as therapy in pediatric pulmonary disorders. Two types of bronchoscopes are available; flexible and rigid, with their own advantages and disadvantages. Depending on the clinical need and availability of skills, choice is made between the two. Typically, rigid scopes are largely used by the surgeons (pediatric or otolayngologists) while flexible bronchoscope stays in the domain of the pediatric pulmonologist and intensivists. Rigid scopes may be more versatile than flexible bronchoscopes in removing the foreign bodies from the airway. Flexible bronchoscopes on the other hand can even be introduced through an endotracheal tube. At times, use of both scopes may be required in a given patient for optimal results. Bronchoscopes give us a means to visualize the inside of the airway, which can be very informative for assessing various pathologies affecting the airways. Apart from the visualization of the parts of the airway tree and their structure as well as patency, it can also be used to take tissue biopsy specimens, collect secretions from the airways and bronchoalveolar lavage which can also get cellular elements from the distal alveoli. In the past few decades, more and more instruments are being used for expanding the utility of flexible bronchoscope for interventions ranging from bronchial toilet, foreign body removal, airway stenting and lasers or cryotherapy for airway lesions. The perinatologists have opened up more vistas and thrown newer challenges for using fiberoptic bronchoscopy (FB) for in utero tracheal occlusion in cases with diaphragmatic hernia. The vast applications of this tool makes it very relevant to pulmonary investigations and therapeutics.

Bronquio traqueal / Tracheal bronchio

Tracheal bronchus is considered an anatomical variant or bronchial malformation. Its clinical manifestations are rare, and are due to a failure to drain bronchial secretions. Anatomically it may be a supernumerary or ectopic bronchus. His diagnosis is made by direct visualization of images and airway. Generally its treatment is expectant.

El bronquio traqueal es considerado como una variante anatómica o malformación bronquial. Sus manifestaciones clínicas son infrecuentes, y se deben a una falla para el adecuado drenaje de secreciones bronquiales. Anatómicamente puede tratarse de un bronquio supernumerario o ectópico. Su diagnóstico se realiza mediante imágenes y visualización directa de la vía aérea. En general su tratamiento es expectante.