Clinical characteristics of persistent or recurrent pneumonia combined with airway malacia in children.

OBJECTIVE: The aim of this study is to investigate the clinical characteristics and pathogens involved in persistent or recurrent pneumonia combined with airway malacia in children. METHODS: We retrospectively reviewed the information of children hospitalised with persistent or recurrent pneumonia, including clinical presentations, laboratory examination results and pathogens. RESULTS: A total of 554 patients were admitted, 285 (51.44%) of whom were found to have airway malacia. There were 78 (27.37%), 166 (58.25%) and 41 (14.39%) patients with mild, moderate and severe malacia, respectively. Patients with airway malacia were younger than those without malacia (6.0 vs. 12.0 months, p < 0.01) and were more likely to present with wheezing (75.07%), fever (34.39%), dyspnoea (28.77%), cyanosis (13.68%) and wheezing in the lungs (78.95%). The incidence of preterm delivery, oxygen therapy, paediatric intensive care unit (PICU) admission and mechanical ventilation was higher, and the hospital stay (11.0 vs. 10.0 days, p = 0.04) was longer in these patients than in those without malacia. Patients with severe airway malacia were more likely to undergo oxygen therapy, PICU admission, mechanical ventilation and have multiple malacia than were those with mild or moderate malacia. Mycoplasma pneumoniae (30.18%) was the most common pathogen. CONCLUSION: Severe airway malacia likely aggravates conditions combined with pneumonia. The proportion of multisite malacia was greater in severe airway malacia patients.

Terapia con cánula nasal de alto flujo en pacientes pediátricos en domicilio / High flow nasal cannula therapy in pediatric patients at home

Se realiza comentario de estudio de Israel en el cual analizan 75 pacientes pediátricos que utilizaron cánula nasal de alto flujo (CNAF) en domicilio, evaluando la seguridad, las indicaciones, los parámetros de utilización, la duración del tratamiento, los resultados clínicos y la satisfacción de los padres. Se acompaña de una revisión de la literatura del tema.

A comment is made on a study conducted in Israel analyzing 75 pediatric patients who used high-flow nasal cannula at home, evaluating safety, indications, utilization parameters, treatment duration, clinical outcomes, and parental satisfaction. It is accompanied by a literature review on the topic.

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.

Perioperative Management of an Adolescent Fontan Patient with Narrowing of the Aortopulmonary Space for Posterior Scoliosis Surgery.

Congenital cardiac patients who received neonatal reconstructive aortic arch surgery are at risk of aortopulmonary space narrowing with compression of the left pulmonary artery and left main bronchus (LMB) later in life. We discuss a challenging adolescent single ventricle patient who presented for surgical treatment of a non-idiopathic thoracic scoliosis (posterior spinal fusion) with severe stenosis of the LMB and left pulmonary artery due to a narrow aortopulmonary space. Careful preoperative imaging, evaluation, and decision making resulted in successful surgical treatment and uneventful perioperative course.

Airway malacia in premature infant twins with bronchopulmonary dysplasia: Two case reports.

Premature infants who require surgery for retinopathy of prematurity often exhibit bronchopulmonary dysplasia. Reactive airway is a clinical manifestation of bronchopulmonary dysplasia. We describe premature infant twins who had difficulty with positive pressure ventilation during anesthesia. Both cases occurred during induction of anesthesia for binocular anterior chamber puncture and vitreous cavity injection. The most likely cause in each case was airway malacia. We recommend that endotracheal intubation is performed in infant patients with low body weight; the possibility of airway malacia occurrence should be considered, especially for infants with comorbid bronchopulmonary dysplasia.

Clinical characteristics of children with airway malacia complicated by pneumonia.

BACKGROUND: Airway malacia is an important cause of noisy breathing, recurrent wheezing and respiratory infections, chronic coughing, and episodes of respiratory distress in young children. As the clinical manifestations of airway malacia are not common, many clinicians have insufficient understanding of this disease. So the purpose of this study is to summarize the pathogenic bacteria and clinical manifestations of airway softening complicated with pneumonia in children. METHODS: Children hospitalized with airway malacia complicated by pneumonia were eligible for enrollment from January 1, 2013 to December 31, 2019. Medical records of patients were reviewed for etiology, clinical characteristics, and laboratory examination results. RESULTS: A total of 164 pneumonia patients with airway malacia were admitted. The male-to-female ratio was 3:1. The age of patients ranged from 1 month to 4 years old. The median age was 6 (3-10) months. The most commonly detected pathogen were Mycoplasma pneumoniae (25/164, 15.24%), Streptococcus pneumoniae (18/164, 10.98%), and respiratory syncytial virus (16/164, 9.76%). Common signs among the 164 patients with confirmed airway malacia included cough (98.78%), wheezing (67.07%), fever (35.37%), intercostal retractions (23.17%), dyspnea (10.98%), cyanosis (11.11%), and crackles (50%). Compared with those without airway malacia, the incidence of premature delivery and mechanical ventilation was higher, and the duration of symptoms before admission (median, 13.5 d) and hospital stay (median 10.0 d) were longer. Of the children with pneumonia, 11.59% of those with airway malacia required supplemental oxygen compared with 4.88% of those without airway malacia (p < 0.05). CONCLUSION: The median age of children with airway malacia was 6 months. The most common pathogen in patients with airway malacia complicated by pneumonia was Mycoplasma pneumoniae. Patients with airway malacia complicated by pneumonia often presented with a longer disease course, more severe symptoms, and had delayed recovery.

Airway Malacia: Clinical Features and Surgical Related Issues, a Ten-Year Experience from a Tertiary Pediatric Hospital.

BACKGROUND: Few studies have been carried out with the aim of describing the clinical course and follow-up of patients with tracheomalacia. We aim to describe the symptoms at diagnosis and the post-treatment clinical course of patients affected by airway malacia. METHODS: We retrospectively analyzed characteristics of pediatric patients with a diagnosis of airway malacia. Patients were classified into three groups: bronchomalacia (BM), tracheomalacia (TM) and tracheo-bronchomalacia (TBM). Demographic and clinical data, diagnostic work-up and surgical treatment were recorded. RESULTS: 13/42 patients were affected by congenital syndromes (30.9%). Esophageal atresia with or without tracheal-esophageal fistula (EA/TEF) was detected in 7/42 patients (16.7%). Cardiovascular anomalies were found in 9/42 (21.4%) and idiopathic forms in 13/42 (30.9%). BM occurred in 7/42 (16.6%), TM in 23/42 (54.7%) and TBM in 12/42 (28.6%). At the diagnosis stage, a chronic cough was reported in 50% of cases with a higher prevalence in EA/TEF (p = 0.005). Surgery was performed in 16/42 (40%) of children. A chronic cough and acute respiratory failure were correlated to the need for surgery. During follow-up, there was no difference in persistence of symptoms between conservative vs surgical treatment (p = 0.47). CONCLUSION: the management of tracheomalacia remains a challenge for pediatricians. Clinical manifestations, such as a barking cough and acute respiratory failure may suggest the need for surgery. Follow-up is crucial, especially in those patients affected by comorbidities, so as to be able to manage effectively the possible persistence of symptoms, including those that may continue after surgical treatment.

Obstructive Sleep Disorders in Down Syndrome's Children with and without Lower Airway Anomalies.

(1) Background: Obstructive sleep apnea (OSA) and lower airway anomalies are both highly prevalent in children with Down syndrome (DS). However, little is known on the interaction between both. We aim to investigate the co-occurrence of OSA (defined as obstructive apnea/hypopnea index (oAHI) ≥ 2/h) and lower airway anomalies in children with DS and explore their impact on OSA severity and treatment outcome. (2) Methods: Retrospective analysis of data from airway endoscopy and polysomnography (PSG) in a cohort of children with DS. (3) Results: Data on both lower airway evaluation and PSG were available for 70 patients with DS. Our study population was relatively young (mean age 3.5 years), not obese and presented with severe OSA (mean oAHI 13.1/h). Airway anomalies were found in 49/70 children (70%), most frequently laryngomalacia, tracheomalacia or a combined airway malformation. In the remaining 21 cases (30%), endoscopy was normal. A comparison between both groups showed a similar distribution of gender, age and BMI z-scores. The prevalence of OSA was not significantly higher in DS patients with airway anomalies (89.6% vs 71.4%, p = 0.078). Additionally, OSA severity or treatment choice (conservative, upper airway surgery or CPAP) were not significantly different. Follow-up data (available for 49/70 patients) showed a significant improvement of OSA in both groups. There is a not significant tendency to more patients with persistent OSA among those with lower airway anomalies (34.3% vs 7.1%, p = 0.075). (4) Conclusions: We found no significant differences in OSA severity, treatment choice or outcome between children with DS with and without lower airway anomalies. Further studies should investigate the role of DISE-directed treatment and compare the outcome of different treatment modalities in larger patient groups.

Pediatric flexible bronchoscopy: A single-center report.

INTRODUCTION: Pediatric flexible laryngotracheal bronchoscopy (FB) is an integral part of diagnostics and treatment at tertiary pediatric respiratory centers. AIM: FBs performed between 2013 and 2018 at our Pediatric Allergy and Respiratory Medicine Unit of the Department of Women's and Children's Health at Padua University were examined in terms of the indications, findings, and adverse events. MATERIALS AND METHODS: The electronic medical records of pediatric patients who underwent FB at least once between 1 January 2013 and 31 December 2018 were considered. Patients' clinical data, indications for FB, anatomical findings, information derived from bronchoalveolar lavage (BAL) and bronchial brushing, and possible adverse events were analyzed. RESULTS: There were 447 pediatric FBs performed in 428 patients (aged from 1 month to 18 years) for diagnostic purposes (92.4%), to clear secretions (3.6%), or to monitor a known condition (4.0%). The main indications were recurrent lower respiratory tract infections (LRTI, 32.2%) and chronic wet cough (9.4%). Lower airway malacia was the most common abnormal finding in these two groups (36.1% and 28.6%, respectively). BAL bacterial culture was positive in 55 children (39.6%) with recurrent LRTI and in 25 (59.5%) with chronic wet cough, being Haemophilus influenzae, Streptococcus pneumoniae, and Moraxella catarrhalis the microorganisms most commonly isolated. FB proved a safe procedure and was well tolerated. CONCLUSIONS: Pediatric FB is an essential tool at our tertiary pediatric respiratory center. It helps establish the anatomical conditions underlying several chronic respiratory conditions and any correlated microbiological findings, with a significant impact on further patient management.

Pharyngomalacia in Neonates: The Missed Issue.

Background: Airway malacia (AM) is a weakness of the airway's frameworks making them collapsible during the respiratory phases. Although the larynx, trachea, and bronchus are the usual sites for malacia to occur, there is another important type of malacia that involves the pharynx. Pharyngomalacia (PM) or concentric pharyngeal wall inspiratory collapse (PWIC) is mostly missed during bronchoscopic evaluations in the neonates with noisy breathing because people are not aware of this condition. Methods: This study aimed to evaluate the nasopharyngeal investigation among neonates suffering from noisy breathing. The retrospective study was undertaken to assess the frequency of PM and to propose indications for intervention in 100 neonates with noisy breathing. A thin fiberoptic bronchoscope was used to evaluate the upper airways under conscious status without any sedation in the neonates. Results: A total of 100 neonates with noisy breathing from September 2015 to October 2018 were retrospectively analyzed. The most common presenting symptom was inspiratory stridor which was observed in 35 (92.1%) of cases. PM was diagnosed in 38 neonates (38%) including 27 (71%) males and 13 (29%) females. Seventeen (44.7%) cases had mild, 11 (28.9%) cases had moderate, and 10 (26.4%) cases had a severe type of PM. PM was more prominent at the velopharynx level in 15 (39.4%) cases, and it was accompanied by up to six synchronous airway abnormalities. The most frequent synchronous airway abnormality was laryngomalacia in 13 (34.3%). Conclusion: PM is one of the causes of noisy breathing in infants. Since PM can be accompanied by the presence of other types of airway malacia, the issue becomes more complicated. On the other hand, lack of experience and facilities are two main causes for the accurate diagnosis and effective management among neonates. This study indicates that the investigation of pharynx is a missed part of the many workups that are used to diagnose the site of involvement in neonates with noisy breathing.

Bronchopulmonary Dysplasia: Then, Now, and Next.

Bronchopulmonary dysplasia (BPD) has evolved considerably since its first description over 50 years ago. This review aims to provide a historical framework for conceptualizing BPD and a current understanding of the changing definition, epidemiology, pathophysiology, treatment, and outcomes of BPD. The transdisciplinary approach that led to the initial phenotypic description of BPD continues to hold promise today. Investigators are refining the definition of BPD in light of changes in clinical care and increasing survival rates of very preterm infants. Despite improvements in perinatal care the incidence of BPD continues to increase. There is growing recognition that antenatal risk factors play a key role in the development of BPD. Strategies designed to prevent or limit neonatal lung injury continue to evolve. Defining the phenotype of infants with BPD can meaningfully direct treatment. Infants with BPD benefit from an interdisciplinary approach to longitudinal care with a focus on growth and neurocognitive development. While the ultimate impact of BPD on long-term pulmonary morbidity remains an active area of investigation, current data indicate that most children and adolescents with a history of BPD have a quality of life comparable to that of other preterm infants.

Extraluminal biodegradable splint to treat upper airway anterior malacia: A preclinical proof of principle.

OBJECTIVE: Upper airway malacia highly complicates the treatment of benign laryngotracheal stenosis, and no ideal option is available to date. We here explore the use of extraluminal biodegradable splints in an animal model of long-segment anterior tracheomalacia (TM). We show the efficacy, as well as the tissue tolerance, of a custom-made biodegradable extraluminal device surgically inserted around the trachea. STUDY DESIGN: Preclinical animal study. METHODS: Anterior TM was induced in rabbits through an anterior neck approach by removing eight consecutive anterior tracheal rings without damaging the underlying mucosa. Malacia was corrected during the same surgery by pexy sutures, suspending the tracheal mucosa to an experimental biodegradable device. Symptoms, survival, and tissue reaction were compared to healthy and sham surgery controls. RESULTS: The model induced death by respiratory failure within minutes. Ten animals received the experimental treatment, and those who survived the perioperative period remained asymptomatic with a maximum follow-up of 221 days. Histological studies at programmed euthanasia showed complete degradation of the prosthesis, with significant remnant fibrosis around the trachea. However, the tracheal stiffness of test segments was comparatively less than that of control segments. CONCLUSION: Extraluminal biodegradable splints rescued animals with a condition otherwise incompatible with life. It was well tolerated, leaving peritracheal fibrosis that was not as stiff as normal trachea. The external tracheal stiffening was sufficient for the test animals to live through the phase of severe acute hypercollapsibility. This represents a valid option to help pediatric patients with laryngotracheal stenosis and associated cartilaginous airway malacia. LEVEL OF EVIDENCE: NA. Laryngoscope, 128:E53-E58, 2018.

Congenital respiratory tract disorders in 22q11.2 deletion syndrome.

OBJECTIVE: Respiratory tract disorders have been reported in patients with 22q11.2 deletion syndrome, however infrequently. This study describes the respiratory tract disorders encountered in a cohort of 278 patients with 22q11.2 deletion syndrome. METHODS: We conducted a retrospective, cross-sectional, study at a single tertiary referral center. We identified the patients with 22q11.2 deletion syndrome and with an upper and/or lower respiratory tract disorder at our otorhinolaryngologic department. The different disorders were described. RESULTS: Out of 278 patients referred to the otorhinolaryngologic department, we identified 14 patients with a laryngeal and/or tracheal disorder. Nine patients had more than one congenital disorder in this anatomical area. Disorders included a choanal stenosis (n = 1), laryngeal web (n = 5), laryngeal cleft (n = 2), subglottic stenosis (n = 3), pharyngo-, laryngo-, tracheo- and/or bronchomalacia (n = 11) and tracheal stenosis (n = 1). CONCLUSION: Different types of respiratory tract disorders can be present in patients with 22q11.2 deletion syndrome. Clinicians should be aware of this clinical association for timely and accurate diagnosis and treatment. In addition, the diagnosis 22q11.2 deletion syndrome should be considered in patients presenting with a congenital respiratory tract disorder.

Laryngotracheobronchial anomalies in infants and the related risk factors of in-hospital mortality.

BACKGROUND: Laryngotracheobronchial anomalies (LTBAs) may cause respiratory problems during early childhood, and increase the risk of hospitalization or mortality in diseased children. This study investigated the initial hospitalization age and risk factors for in-hospital mortality in infants diagnosed with LTBAs during their first 5 years of life. METHODS: Hospitalized infants diagnosed with LTBAs were retrieved from Taiwan's National Health Insurance Research Database from 2003 to 2005. Their medical claim data were traced up to 59 months of age. The age distribution of all LTBA cases was analyzed, and then the enrolled infants were grouped into two age groups. Hospitalization-related comorbidities and risk factors for in-hospital mortality were also analyzed. RESULTS: A total of 1272 LTBA cases were retrieved. Most of them (976, 76.7%) were initially hospitalized at an age of 0-3 months, and 47 infants (3.7%) died. These enrolled cases were grouped into early and late LTBA groups, with ages of 0-3 months and 4-11 months, respectively. Patients in the late LTBA group had significantly more acute airway infections/asthma and neurological diseases, more frequent hospitalizations, longer hospitalization stay, and higher in-hospital mortality than did the early LTBA group (p < 0.001). The adjusted odds ratios (aORs) for in-hospital mortality were significantly higher in the children aged 4-11 months [aOR = 2.50, 95% confidence intervals (CI): 1.36-4.60], or having perinatal disease (aOR = 2.00, 95% CI: 1.07-3.73), cardiovascular disease (aOR = 2.45, 95% CI: 1.30-4.60), other congenital anomalies (aOR = 2.42, 95% CI: 1.28-4.60), and neurological diseases (aOR = 2.32, 95% CI: 1.18-4.53). CONCLUSION: Most infants with LTBAs were initially diagnosed and hospitalized when they were aged 3 months or younger. The risk factors for in-hospital mortality of the children with LTBAs included being diagnosed and treated at an age of 4 months and older, and the presence of perinatal disease, cardiovascular anomalies, other congenital anomalies, neurological diseases, and an age of 4 months and older.

Clinical features of airway malacia in children: a retrospective analysis of 459 patients.

AIM: To investigate the clinical features of airway malacia in children. MATERIAL AND METHODS: A comprehensive analysis was done on information of 459 young patients with airway malacia. RESULTS: Number of children with tracheomalacia, tracheobronchomalacia, and bronchomalacia was 7 (1.5%), 17 (3.7%), and 435 (94.8%), respectively. Incidence of bronchomalacia in left lung was 11.0% (n=48), while that of right lung was 53.3% (n=232). Meanwhile, bronchomalacia of both lungs were noticed in 155 children (35.6%). With regards to the extent of malacia, number of children with slight, moderate and severe malacia was 226, 195, and 38, respectively. All the children enrolled in this study were diagnosed with pulmonary infection, among which 376 were diagnosed with ordinary pneumonia, 83 were diagnosed with severe pneumonia. 227 children showed a disease course of less than 1 month, while 201 children reported a disease course of 1~3 months, and 31 children reported a disease course of more than 3 months. Statistical difference was noticed in the disease condition of respiratory tract infection of patients with various malacia extent (P < 0.05). Re-check of fiberoptic bronchoscopy was performed in 19 patients, among which 14 patients (73.7%) showed improvement compared with the previous conditions. CONCLUSION: Airway malacia has been frequently noticed in male children aged ≤ 2 years old. Patients with severe airway malacia were apt to develop severe pneumonia compared with those with slight or moderate malacia. Improvements or even elimination of malacia were noticed with the aging of the children and the anti-infection therapy.

Anaesthetic management of a child with multiple congenital anomalies scheduled for cataract extraction.

SUMMARY: In infants & children variety of conditions and syndromes are associated with difficult Airway. Anaesthetic management becomes a challenge if it remains unrecognized until induction and sometimes results in disaster, leading to oropharyngeal trauma, laryngeal oedema, cardiovascular & neurological complications. A 4-month-old child with multiple congenital anomalies was posted for cataract extraction for early and better development of vision. He had history of post birth respiratory distress, difficulty in feeding, breath holding with delayed mile stones. He was treated as for Juvenile asthma. This child was induced with inhalation anaesthesia. There was difficulty in laryngoscopic intubation and could pass much smaller size of the tube than predicted. He developed post operative stridor and desaturation. The problems which we faced during the anaesthetic management and during postoperative period are discussed with this case.