Airway findings in trisomy 13 and trisomy 18: A 10-year retrospective review.

BACKGROUND AND OBJECTIVES: Trisomy 18 and trisomy 13 are the most common autosomal trisomies following trisomy 21, with overall incidence rising. Both diagnoses are characterized by multisystem involvement and were previously thought to be incompatible with life. New data suggest that prolonged survival is possible, and thus many families are opting for more aggressive medical interventions. This study aims to describe airway findings in trisomy 18 and trisomy 13, as these have not been comprehensively studied and can impact medical decision-making. We hypothesize that most children with trisomy 18 and trisomy 13 will have abnormal findings on airway endoscopy. METHODS: This a 10-year retrospective analysis of children with trisomy 13 or trisomy 18 who underwent endoscopic airway evaluation at a single center between 2011 and 2021. A total of 31 patients were evaluated. RESULTS: Thirty-one patients were included and underwent flexible bronchoscopy by a pediatric pulmonologist, often in conjunction with rigid bronchoscopy performed by pediatric otolaryngology. Findings were typically complimentary. All patients had at least one clinically significant finding on evaluation, and most patients had both upper and lower airway, as well as static and dynamic airway findings. The most common airway findings in children with trisomy 13 and 18 include tracheomalacia, bronchomalacia, laryngomalacia, hypopharyngeal collapse, glossoptosis, and bronchial compression. CONCLUSION: These findings can have significant implications for clinical care, and thus knowledge of trends has the potential to improve counseling on expected clinical course, presurgical planning, and informed consent before interventions.

Clinicopathologic features, comorbid diseases, and prevalence of pulmonary hypertension in dogs with bronchomalacia.

BACKGROUND: Reports of clinicopathologic features of bronchomalacia (BM) differ because of inconsistent definitions and frequent prevalence of comorbid cardiopulmonary disease. Pulmonary hypertension (PH) secondary to BM is poorly described. OBJECTIVES: Dogs with BM will be older but of any somatotype, and increased expiratory effort, ≥1 comorbid disease, and PH will be more common than in dogs without BM. ANIMALS: Client-owned dogs (n = 210) evaluated for respiratory signs. METHODS: Medical records of dogs with paired inspiratory: expiratory-breath-hold computed tomography, tracheobronchoscopy, or both between January 2016 and December 2019 were retrospectively reviewed. Comparisons between dogs with and without BM using Mann-Whitney rank sum or χ2 tests (P < .05 significant were made). Because of high numbers of variables, criteria with high prevalence (>25%) were identified (n = 10) for univariate analysis (P < .005 significant). Significant variables were submitted for multivariate analysis. RESULTS: Bronchomalacia was identified in 41% of dogs of all sizes/somatotypes; 38% were >10 kg. All dogs with BM had ≥1 comorbid cardiopulmonary disorder. Dogs with BM were significantly older (P < .001), smaller (P < .001), and were more likely diagnosed with tracheal or mainstem bronchial collapse (P < .001) or bronchiectasis (P < .001). Multivariate analysis confirmed associations with age, tracheal or mainstem bronchial collapse, and bronchiectasis. In dogs with BM, PH was more prevalent. CONCLUSIONS AND CLINICAL IMPORTANCE: Although significantly more common in older, smaller dogs, BM occurs in dogs of all sizes and in all instances with comorbidities. Echocardiography should be considered in dogs with BM to identify PH.

Left Main Bronchus Stenosis Lesion, Neutrophil Count, and Platelet Count Are Predictors of Post-Tuberculosis Bronchomalacia.

BACKGROUND Post-tuberculosis bronchomalacia (PTBM) is one of the main conditions occurring in patients after tracheobronchial tuberculosis (TBTB), and is also associated with the recurrence of symptoms. The present study aimed to investigate the predictors of PTBM in patients who had been undergoing appropriate TB treatment. MATERIAL AND METHODS Clinical data of 104 patients with symptomatic airway stenosis after TBTB between January 01, 2019 and June 31, 2020 were recorded and analyzed. The association between baseline clinical characteristics, laboratory results, and PTBM was calculated with logistical regression. The time from onset of bronchoscopic intervention was examined by Kaplan-Meier estimates; differences between the 2 groups were tested by the log-rank test. RESULTS Fifty-seven patients (54.81%) had PTBM. In the multivariate logistical analysis, the left main bronchus stenosis lesion (odds ratio [OR]=3.763), neutrophil (NEUT) count (OR=1.527), and platelet (PLT) (OR=1.010) count were predictors of PTBM. During follow-up, patients with BM had a significantly longer duration from onset of bronchoscopic intervention than patients without BM (hazard ratio=2.412, P<0.0001). Further, all patients needing long-term bronchoscopic intervention therapy were subsequently identified as having PTBM. Additionally, blood PLT counts were significantly decreased to normal levels in the non-BM group (P<0.05), but not in the BM group (P>0.05). CONCLUSIONS PTBM is most likely to occur in the left main bronchus. The inflammatory and immune responses associated with NEUT and PLT may represent therapeutic targets of PTBM. Our study is the first to report that decreased blood PLT count has the potential to monitor the treatment response.

Detecting respiratory viruses in children with protracted bacterial bronchitis.

OBJECTIVE: This study is to investigate the status and clinical significance of respiratory viruses in bronchoalveolar lavage fluid (BALF) of children with PBB. METHODS: Sixty-eight children with PBB aged from 3 months to 5 years were enrolled and retrospectively reviewed from January 2014 to December 2017. Thirty-five children with persistent pneumonia or chronic pneumonia were matched as controls. Bronchoalveolar lavage fluid samples were collected for respiratory virus detection and bacterial culture. RESULTS: The detection rate of bacteria in BALF of children with PBB was 61.8%, which was significantly higher than that of control group (20%) (P < 0.001). The detection rate of virus in BALF of children with PBB was 23.5%, including 6 (8.8%) of rhinovirus, 4 (5.9%) of parainfluenza virus type 3, 2(2.9%) of bocavirus, 2 (2.9%) of respiratory syncytial virus 1 (1.5%) of human metapneumonia virus and 1 (1.5%) of influenza virus A. 10 cases (28.6%) of virus were detected in the control group, including 3 (8.6%) respiratory syncytial virus, 3 (8.6%) rhinovirus and 2 (5.7%) bocavirus. There was no significant difference of viral detection rate between the two groups (P = 0.577). CONCLUSION: Respiratory viruses can be detected in BALF of children with PBB, However, there is no evidence that PBB is virus-induced.

Tracheal bronchus diagnosed in children undergoing flexible bronchoscopy.

This paper describes the clinical features of paediatric patients with tracheal bronchus (TB) identified with flexible bronchoscopy (FB) in a tertiary care hospital. A retrospective review of every FB with diagnosis of TB carried out in our centre since 1990 was performed which considered specifically: age at diagnosis, gender, semiology, somatic anomalies, tracheal bronchus type, other bronchoscopic findings and clinical progress. Out of 1665 FB in 1337 patients, TB was found in 26 (1.9%). The median age was 15 months (age range 1 month-13 years), with no gender differences. Of 26 patients, a total of 24 had associated congenital pathologies (92.3%) (heart disease 69%, chromosomal abnormality 35% and spinal fusion defect 11%). FB was performed for a number of reasons including: recurrent or persistent wheezing, pneumonia or atelectasis, persistent stridor and refractory cough. The type of TB identified was primarily ectopic lobar of the right upper lobe (73%) and the apical supernumerary (11.5%). In addition to TB, other defects were found by FB in 90% of cases, such as tracheomalacia, bronchomalacia or tracheal stenosis. The review confirmed a higher prevalence of TB than expected in previous series and highlighted its association with other airway or somatic malformations, notably congenital cardiac defects and spinal fusion defects. All patients were managed conservatively for the TB.

Types of laryngomalacia in children: interrelationship between clinical course and comorbid conditions.

The aim of this study was to: (1) find out whether laryngomalacia (LM) types are related to clinical course; (2) which patients with LM are at higher risk of other airway malacia [tracheomalacia (TM) and/or bronchomalacia (BM)]; and (3) evaluate the prevalence of LM in our region. Patients with established LM diagnosis and complete clinical and endoscopy records were enrolled. They were classified into different LM types according to classification based on the side of supraglottic obstruction. One hundred ten children were included. The most common LM appearance was type I-58 children, followed by combine types (I + II and I + III)-38. The other airway malacia were found in 47 patients: TM in 31, BM in 10, and TM with BM in 6. Other comorbidities (cardiac, neurological, and genetic disorders) were identified in 30 children. Patients with combine types of LM differ from those with single type of LM in terms of prematurity (13 vs 31 %, p = 0.04) and higher weight on the examination day (p = 0.006). Patients with other airway malacia differ from children with isolated LM in terms of prematurity (40 vs 13 %, p = 0.008), comorbidities (38 vs 19 %, p = 0.024), and lower weight on the examination day (p = 0.014). The prevalence of clinically relevant LM was one in 2600-3100 newborns. Clinical course of LM cannot be anticipated on the basis of solely endoscopic evaluation of the larynx. Comorbidities and prematurity increase the risk of other airway malacia. The prevalence of LM is relatively high in the middle-south part of Poland.

Respiratory Care of Infants and Children with Congenital Tracheo-Oesophageal Fistula and Oesophageal Atresia.

Despite acute respiratory and chronic respiratory and gastro-intestinal complications, most infants and children with a history of oesophageal atresia / trachea-oesophageal fistula [OA/TOF] can expect to live a fairly normal life. Close multidisciplinary medical and surgical follow-up can identify important co-morbidities whose treatment can improve symptoms and optimize pulmonary and nutritional outcomes. This article will discuss the aetiology, classification, diagnosis and treatment of congenital TOF, with an emphasis on post-surgical respiratory management, recognition of early and late onset complications, and long-term clinical outcomes.

Airway complications after single-stage unifocalization for pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries.

OBJECTIVE: We analyze the incidence of postoperative severe airflow limitation after single-stage unifocalization in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) and comment on the treatment performed. METHODS: From 1994 until 2014, 118 patients with diagnosis of PA, VSD, MAPCAs underwent surgical treatment. Four patients (3.4%) developed severe airflow complications postoperatively. Chromosome 22q11 deletion was present in three of them. Median age at the time of unifocalization was 6.2 months (range 21 days to 11 months). RESULTS: The first patient developed malacia and compression of the left bronchus from the distal RV-PA conduit and was treated with external bronchial stenting with two incomplete costal cartilage rings. The second patient developed recurrent esophagus-left bronchus fistula treated with multiple surgical esophageal and bronchus reconstructions. The third child presented with bilateral bronchial malacia treated with bilateral stenting followed by surgical elongation of the neo-left pulmonary artery to avoid external compression. The last patient developed bilateral bronchomalacia treated with bilateral bronchial stenting followed by RV-PA conduit replacement and endobronchial stenting calibration. CONCLUSION: Particular categories of patients with PA, VSD, MAPCAs (22q11 chromosome deletion, neonates/infants, patients with dominant/exclusive collaterals) may be more predisposed to develop airway compromise. The treatment of the lesion should be individualized according to the pathogenic mechanism. We suggest endoluminal treatment in absence of compression by vascular structures while surgery was used in case of extrinsic compression.

Lower airway anomalies in children with CATCH 22 syndrome and congenital heart disease.

OBJECTIVE: CATCH 22 syndrome is a medical acronym for multiple abnormalities, especially cardiac defect. The patients with congenital heart disease (CHD) usually have more complicated post-surgery course. METHODS: We reviewed 4-year experience in our hospital to define the lower airway anomalies and the clinical implications in patients with CATCH 22 syndrome and CHD. From 2004 to 2007, 18 patients who underwent computed tomography for planning of cardiovascular treatment were enrolled. Detection of airway anomalies were performed on computed tomography. RESULTS: Characteristic dysmorphic facial features were noted in 8 out of 18 patients (44%). Ten patients (55.6%) had mild to moderate tracheal or bronchial stenosis. Five patients (28%) had tracheomalacia or bronchomalacia. Comparing to control group, patients received the first major surgery with a median hospital stays of 42 days in study group had a significantly longer hospital stay (42 vs. 16, P = 0.005) and longer duration of ventilator usage (16 vs. 4, P = 0.014). The difference of the mean position of the carina on computed tomography image between study groups and control groups was about three-quarters of a vertebral body height (P < 0.05). CONCLUSIONS: Patients with CATCH 22 syndrome and CHD had a shorter length of trachea compared to that of the age-matched similar CHD. The incidence of associated lower airway anomalies in CHD children with CATCH 22 syndrome was higher. It may lead to longer time of ventilator support after surgery as well as total hospital stays than those of CHD without CATCH 22 syndrome.

[Tracheobronchomalacia]. / Traqueobroncomalacia.

Tracheobronchomalacia is a central airway disease characterised by weakness of the wall and dynamic decrease in the tracheal lumen and the large bronchi, particularly while exhaling. It is more common in middle age and the elderly with previous exposure to cigarettes. It causes chronic symptoms such as cough, dyspnea, increase in recurrent infections, and poor secretion management, but it can also progress to chronic respiratory failure and death. It is usually confused with other common diseases like chronic obstructive pulmonary disease (COPD) or asthma. Its causes can be congenital or acquired and its diagnosis involves the dynamic assessment of the airway with tomography and fibrobronchoscopy. It is classified as mild, moderate or severe depending on the degree of collapse of the airway when exhaling. Management consists of a primary phase, in which concomitant diseases must be controlled, such as COPD, asthma or gastro-oesophageal reflux. In diffuse moderate to severe symptomatic tracheobronchomalacia tracheobronchoplasty must be considered with strengthening of the posterior wall. Silicone and "Y" stents can be used to identify patients who could potentially benefit from surgical treatment as well as being used for the definitive symptomatic treatment with high surgical risk. More prospective studies need to be done in order to standardise certain common criteria for the management of this usually under-diagnosed disease.

Synchronous airway lesions and outcomes in infants with severe laryngomalacia requiring supraglottoplasty.

OBJECTIVE: To quantify the prevalence and the impact of synchronous airway lesions identified by endoscopy in infants undergoing supraglottoplasty for severe laryngomalacia (LM). DESIGN: Retrospective study. SETTING: Tertiary care pediatric hospital. PATIENTS: Sixty patients who underwent supraglottoplasty for severe LM from 2002 to 2006. Patients who underwent preoperative tracheotomy, had previous airway surgery, or did not have 6 months of follow-up were excluded. Fifty-two patients met inclusion criteria. INTERVENTION: Supraglottoplasty (with carbon dioxide laser). MAIN OUTCOME MEASURES: Presence of synchronous airway lesions and their contribution to upper airway obstruction (UAO) and their effect on the postoperative course after supraglottoplasty. RESULTS: Fifty-eight percent of patients had synchronous airway lesions (SALs), of whom 77% had subglottic stenosis (SGS) and 47% had tracheomalacia, bronchomalacia, or both. Sixty-three percent of all patients required postoperative nonsurgical airway support. Eight patients had residual UAO requiring additional surgical intervention, with 3 revision supraglottoplasties and 7 tracheotomies performed. Infants with neurological conditions had a high rate of surgical intervention (55%; P = .001). Patients with SGS exceeding 35% but without any neurological condition had a prolonged hospital stay (>3.6 days; P = .02) and an 83% incidence (P = .04) of postoperative UAO requiring intubation. Infants with LM with laryngeal edema (LE) alone had increased frequency of postoperative nonsurgical airway support (P = .02) and a prolonged hospital stay of 1 day (P = .01) compared with infants without edema. CONCLUSIONS: There is a high incidence of SALs in patients undergoing supraglottoplasty. Neurological conditions, hypoplastic mandible, SGS greater than 35%, and preexisting LE independently adversely affected the postoperative course.