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Journal of Thoracic Disease Nov 2020Tracheobronchomalacia (TBM) is an obstructive airway disease characterized by laxity and redundancy of the posterior membrane of the main airways leading to dynamic... (Review)
Review
Tracheobronchomalacia (TBM) is an obstructive airway disease characterized by laxity and redundancy of the posterior membrane of the main airways leading to dynamic airway collapse during exhalation. The gold standard for diagnosis is dynamic computed tomography (DCT) scan and dynamic flexible bronchoscopy (DFB). Patients with complete or near-complete collapse (>90% reduction in cross-sectional area) of the airway are possible candidates for surgical management. Central airway stabilization by tracheobronchoplasty (TBP) effectively corrects malacic airways and has demonstrated significant improvement in objective functional measures, which is often but not uniformly accompanied by equal improvement in health-related quality of life (HRQOL) metrics. This article reviews HRQOL instruments used to report outcomes after TBM surgery.
PubMed: 33282396
DOI: 10.21037/jtd.2020.03.08 -
Medicine Nov 2020Surgical closure of patent ductus arteriosus (PDA) has been considered the only way to rescue preterm neonates following nonsteroidal anti-inflammatory drugs closure...
OBJECTIVE
Surgical closure of patent ductus arteriosus (PDA) has been considered the only way to rescue preterm neonates following nonsteroidal anti-inflammatory drugs closure failure. However, PDA closure by catheter-based interventions has become another therapeutic choice. The aim of this report was to investigate the timing and treatment methods for hemodynamically significant PDA (hsPDA) in preterm neonates.
METHODS
We retrospectively studied 4 ventilator-dependent preterm neonate cases with hsPDA who had an urgent need of PDA closure and who attended our hospital between October 2016 and March 2018. We assessed the efficacy and safety of transcatheter closure of the hsPDA, and evaluated the dependence of the infants on mechanical ventilation.
RESULTS
The 4 infants with hsPDA underwent successful transcatheter closures. Two infants were weaned from the ventilatory support within 24 hours after the closure. Those 2 preterm neonates demonstrated normal growth and development during the postoperative follow-up. However, the other 2 infants still needed ventilatory support beyond 48 hours post procedure. One of them presented a bronchial stenosis, underwent a bronchial stent placement by bronchoscopy 10 days after the PDA closure, and was only then finally withdrawn from the ventilatory support. The other infant had a severe bronchomalacia and was only weaned from the ventilator 36 days post PDA closure.
CONCLUSION
Transcatheter closure could be an acceptable alternative to surgical ligation when medication treatment fails to close hsPDAs in ventilator-dependent preterm neonates. This intervention should be considered to minimize mechanical ventilation duration, reduce the incidence of bronchopulmonary dysplasia, and improve the prognoses of these infants.
Topics: Anti-Inflammatory Agents, Non-Steroidal; Cardiac Catheterization; Ductus Arteriosus, Patent; Female; Guideline Adherence; Humans; Infant, Newborn; Infant, Premature; Male; Respiration, Artificial; Retrospective Studies
PubMed: 33217791
DOI: 10.1097/MD.0000000000022528 -
Respirology Case Reports Dec 2020Tracheobronchial stenosis due to tuberculosis (TSTB) is a potentially debilitating complication of endobronchial tuberculosis (EBTB). Endobronchial interventions...
Tracheobronchial stenosis due to tuberculosis (TSTB) is a potentially debilitating complication of endobronchial tuberculosis (EBTB). Endobronchial interventions including silicone stent insertion is an acceptable approach to improve quality of life among patients with TSTB. However, little is known about the optimal management strategy for patients with bronchomalacia secondary to EBTB (B-EBTB) and whether stent-related complication rates are higher among this group of patients. Herein, we report two patients with B-EBTB who unfortunately developed bronchial tear related to silicone endobronchial stenting. Both patients were successfully managed conservatively without the need for emergency open surgery. We hypothesize that endobronchial intervention might be more beneficial for patients with pure TSTB and might be riskier in cases of bronchomalacia with reduced airway thickness and loss of airway cartilaginous support. More future studies are needed to bridge the current gap in knowledge regarding the optimal management and role of endobronchial interventions among patients with B-EBTB.
PubMed: 33194207
DOI: 10.1002/rcr2.684 -
Journal of Cystic Fibrosis : Official... Mar 2021We report the first case of biodegradable airway stent insertion for a patient with bronchomalacia and cystic fibrosis (CF). This female infant with antenatally...
We report the first case of biodegradable airway stent insertion for a patient with bronchomalacia and cystic fibrosis (CF). This female infant with antenatally diagnosed cystic fibrosis developed respiratory distress by three weeks of age. On lower airway examination she was found to have severe left main stem bronchomalacia causing left upper lobe hyperinflation and subsequent right upper lobe collapse. By eight weeks of age she developed life-threatening respiratory failure requiring high pressure invasive ventilation. A biodegradable bronchial stent was inserted to the left main bronchus at thirteen weeks of age with successful extubation from invasive respiratory support four days later. A second biodegradable stent was inserted nine weeks later, due to persistent bronchomalacia following stent degradation and redevelopment of oxygen requirement. She was discharged home off all respiratory support eight weeks later and has remained well, requiring no further surgical intervention for bronchomalacia to date, now age three years.
Topics: Biocompatible Materials; Bronchomalacia; Cystic Fibrosis; Female; Humans; Infant, Newborn; Stents
PubMed: 33168477
DOI: 10.1016/j.jcf.2020.10.004 -
ASAIO Journal (American Society For... Aug 2021We report a 6-year-old with single ventricle physiology secondary to tricuspid atresia who had cardiorespiratory failure who was not a candidate for further single...
We report a 6-year-old with single ventricle physiology secondary to tricuspid atresia who had cardiorespiratory failure who was not a candidate for further single ventricle palliation. The patient underwent planned staged left pneumonectomy for recurrent pneumonias secondary to bronchomalacia followed by orthotopic heart transplantation. This aggressive approach improved the patient candidacy for heart transplantation by removing the source of recurrent infection and respiratory failure (left lung).
Topics: Child; Heart Failure; Heart Transplantation; Heart Ventricles; Humans; Pneumonectomy; Univentricular Heart
PubMed: 33165000
DOI: 10.1097/MAT.0000000000001304 -
Medicine Oct 2020Expiratory central airway collapse is defined by excessive inward bulging of either tracheobronchial posterior membrane or cartilage. The former is called excessive...
INTRODUCTION
Expiratory central airway collapse is defined by excessive inward bulging of either tracheobronchial posterior membrane or cartilage. The former is called excessive dynamic airway collapse (EDAC), and the latter, depending on the site of collapse, tracheomalacia, bronchomalacia or tracheobronchomalacia. Due to their non-specific symptoms and lack of awareness amongst clinicians they tend to be mislabeled as common obstructive lung disorders, or complicate their course undetected. Particular controversies refer to EDAC sometimes considered just as a symptom of obstructive lung disease and not a separate entity. Nonetheless, a growing body of evidence indicates that EDAC might be present in patients without apparent obstructive lung disease or it might be an independent risk factor in chronic obstructive pulmonary disease or asthma patients.
PATIENT CONCERNS
Patient #1 was admitted because of idiopathic chronic cough whereas patient #2 was admitted for differential diagnosis of dyspnea of uncertain etiology. In both patients symptoms were unresponsive to bronchodilators and inhaled corticosteroids.
FINDINGS AND DIAGNOSIS
In both patients an excess collapse of tracheobronchial posterior membrane was detected during bronchoscopy; in patient #1, of right main bronchus and right upper lobe bronchus and in patient #2 of right upper lobe bronchus and both main bronchi. Excess central airway collapse in patient #2 was also visualized on expiratory chest CT. In patient #1 spirometry did not reveal obturation, whereas in patient #2 only mild, irreversible, obstruction was revealed, disproportionate to patients significant breathlessness.
INTERVENTIONS
Both patients were treated with N-acetylcysteine and adjustable positive expiratory pressure valves.
OUTCOMES
Due to aforementioned treatment chronic cough in patient #1 subsided almost completely whereas patient's #2 dyspnea improved significantly.
CONCLUSIONS
In presented cases EDAC was an unexpected finding, even though, it firmly corresponded with reported symptoms. Treatment modification led to improvement of patients quality of life.
Topics: Acetylcysteine; Adult; Aged; Bronchoscopy; Diagnosis, Differential; Female; Humans; Positive-Pressure Respiration; Spirometry; Tomography, X-Ray Computed; Tracheobronchomalacia
PubMed: 33080680
DOI: 10.1097/MD.0000000000022449 -
Respirology (Carlton, Vic.) Mar 2021Long-term data on children with PBB has been identified as a research priority. We describe the 5-year outcomes for children with PBB to ascertain the presence of... (Review)
Review
BACKGROUND AND OBJECTIVE
Long-term data on children with PBB has been identified as a research priority. We describe the 5-year outcomes for children with PBB to ascertain the presence of chronic respiratory disease (bronchiectasis, recurrent PBB and asthma) and identify the risk factors for these.
METHODS
Prospective cohort study was undertaken at the Queensland Children's Hospital, Brisbane, Australia, of 166 children with PBB and 28 controls (undergoing bronchoscopy for symptoms other than chronic wet cough). Monitoring was by monthly contact via research staff. Clinical review, spirometry and CT chest were performed as clinically indicated.
RESULTS
A total of 194 children were included in the analysis. Median duration of follow-up was 59 months (IQR: 50-71 months) post-index PBB episode, 67.5% had ongoing symptoms and 9.6% had bronchiectasis. Significant predictors of bronchiectasis were recurrent PBB in year 1 of follow-up (OR = 9.6, 95% CI: 1.8-50.1) and the presence of Haemophilus influenzae in the BAL (OR = 5.1, 95% CI: 1.4-19.1). Clinician-diagnosed asthma at final follow-up was present in 27.1% of children with PBB. A significant BDR (FEV improvement >12%) was obtained in 63.5% of the children who underwent reversibility testing. Positive allergen-specific IgE (OR = 14.8, 95% CI: 2.2-100.8) at baseline and bronchomalacia (OR = 5.9, 95% CI: 1.2-29.7) were significant predictors of asthma diagnosis. Spirometry parameters were in the normal range.
CONCLUSION
As a significant proportion of children with PBB have ongoing symptoms at 5 years, and outcomes include bronchiectasis and asthma, they should be carefully followed up clinically. Defining biomarkers, endotypes and mechanistic studies elucidating the different outcomes are now required.
Topics: Bacterial Infections; Bronchiectasis; Bronchitis; Bronchitis, Chronic; Child; Cough; Humans; Prospective Studies
PubMed: 33045125
DOI: 10.1111/resp.13950 -
Journal of Thoracic Disease Jul 2020Patients with tracheobronchial stenosis due to tuberculosis (TSTB) have a variable clinical course and response to treatment including airway intervention. There are no...
BACKGROUND
Patients with tracheobronchial stenosis due to tuberculosis (TSTB) have a variable clinical course and response to treatment including airway intervention. There are no clear guidelines on the best approach to manage such patients. This study examines long-term outcomes of patients with TSTB and factors associated with recurrent symptoms or need for repeat airway intervention following initial bronchoscopic intervention.
METHODS
This is a retrospective analysis of patients with TSTB over an 18-year period. Symptoms, radiological, bronchoscopic findings, airway interventions and complications were obtained. Multivariate logistic regression analysis was performed to identify factors predictive of recurrence of symptoms or need for repeat airway intervention.
RESULTS
A total of 131 patients with mean age 50±18 years and median follow-up 5 (interquartile range, 2-10) years were included. Nineteen (29.7%) patients underwent balloon dilatation alone, 22 (34.4%) had additional resection or stenting, and 19 (29.7%) underwent all 3 procedures. Among patients conservatively managed, a larger proportion (55.6%) of patients with no recurrence of symptoms had active TB. Patients who developed recurrent symptoms had longer symptom duration (12 3 months, P=0.025), and more had recurrent lower respiratory tract infections (50% 5.6%, P=0.003), lung collapse (90.0% 41.7%, P=0.011) or linear fibrosis (70.0% 30.6%, P=0.033) compared to those without recurrent symptoms. Bronchomalacia (OR =17.18; 95% CI, 3.43-86.18) and prior bronchoscopic airway resection (OR =4.97; 95% CI, 1.20-20.60) were strongly associated with the recurrence of symptoms or need for repeat airway intervention on multivariate logistic regression analysis.
CONCLUSIONS
Bronchomalacia and prior bronchoscopic airway resection are associated with the recurrence of symptoms despite airway intervention. Patients who are diagnosed with TSTB early in the course of active TB may be conservatively managed.
PubMed: 32802443
DOI: 10.21037/JTD-20-670 -
Medicina (Kaunas, Lithuania) Jul 2020Persistent wheezing (PW) is defined as prolonged or recurrent episodes of wheezing despite regular treatment. Flexible bronchoscopy (FB) is recommended to determine the...
Persistent wheezing (PW) is defined as prolonged or recurrent episodes of wheezing despite regular treatment. Flexible bronchoscopy (FB) is recommended to determine the etiology of PW in children. This study aimed to determine the etiology of PW based on FB findings in a national pediatric center. Children presenting with PW that underwent flexible bronchoscopy from April 2016 to August 2019 at the Mother and Child Health Institute of Serbia were included in this observational study. After endoscopic evaluation, bronchoalveolar lavage fluid (BALF) samples were taken and further analyzed. Quantitative microbiology, cytological analysis and oil-red staining of specimens were performed to determine cellular constituents and presence of lipid laden macrophages (LLM). Upper gastrointestinal series were performed to exclude gastroesophageal reflux disease, swallowing dysfunction and vascular ring. Pathological findings were revealed in 151 of 172 study participants, with bacterial lower airway infection (BLAI) (48.3%) and primary bronchomalacia (20.4%) as the most common. Younger participants were hospitalized for significantly longer periods (ρ = -0.366, < 0.001). Study participants with BLAI and associated mucus plugging were notably younger ( < 0.001). Presence of LLM in BALF was not associated with findings of upper gastrointestinal series. All patients with confirmed BLAI were treated with oral antibiotics. Although FB is considered to be invasive, there were no complications associated with the procedure. Flexible bronchoscopy has an exceptional diagnostic value in evaluation of PW. In younger patients with BLAI, presence of mucus plugs may complicate the clinical course, so significant benefits can be achieved with therapeutic lavage during bronchoscopy.
Topics: Adolescent; Bronchoalveolar Lavage; Bronchoscopes; Bronchoscopy; Child; Child, Preschool; Female; Humans; Infant; Male; Respiratory Sounds; Retrospective Studies; Serbia
PubMed: 32630648
DOI: 10.3390/medicina56070329 -
American Journal of Medical Genetics.... Sep 2020Hand hyperphalangism leading to shortened index fingers with ulnar deviation, hallux valgus, mild facial dysmorphism and respiratory compromise requiring assisted...
Hand hyperphalangism leading to shortened index fingers with ulnar deviation, hallux valgus, mild facial dysmorphism and respiratory compromise requiring assisted ventilation are the key features of Chitayat syndrome. This condition results from the recurrent heterozygous missense variant NM_006494.2:c.266A>G; p.(Tyr89Cys) in ERF on chromosome 19q13.2, encoding the ETS2 repressor factor (ERF) protein. The pathomechanism of Chitayat syndrome is unknown. To date, seven individuals with Chitayat syndrome and the recurrent pathogenic ERF variant have been reported in the literature. Here, we describe six additional individuals, among them only one presenting with a history of assisted ventilation, and the remaining presenting with variable pulmonary phenotypes, including one individual without any obvious pulmonary manifestations. Our findings widen the phenotype spectrum caused by the recurrent pathogenic variant in ERF, underline Chitayat syndrome as a cause of isolated skeletal malformations and therefore contribute to the improvement of diagnostic strategies in individuals with hand hyperphalangism.
Topics: Adolescent; Adult; Child; Child, Preschool; Facies; Female; Fingers; Genetic Predisposition to Disease; Hallux Valgus; Humans; Pierre Robin Syndrome; Repressor Proteins; Exome Sequencing; Young Adult
PubMed: 32592542
DOI: 10.1002/ajmg.a.61735