Variable Airway Obstruction Research Articles

Conservative Airway Management Successful in Majority of Infants With Pierre-Robin Sequence at Queensland Children's Hospital: A Retrospective Review.

Pierre-Robin Sequence (PRS) is a congenital abnormality characterized by micrognathia, glossoptosis, and variable severity upper airway obstruction. Clear management algorithms are lacking, particularly the indications for surgical versus nonsurgical intervention. The authors reviewed the management of these children in Queensland. All children diagnosed with PRS at Queensland Children's Hospital from April 2014 to October 2019 were identified (n=45), and their charts were retrospectively reviewed. Three management patterns emerged: prone/lateral positioning, nasopharyngeal airway (NPA) use, and surgery (tracheostomy or mandibular distraction). Most children (n=30; 67%) were managed successfully nonsurgically with an NPA (median age of insertion 0.25 mo, median duration 5.0 mo). Of these, 12 patients (40%) also required supplemental oxygen. The median age of NPA cessation was 5.5 months, with oxygen therapy ceasing at a median 8.25 months, upon which no further support was required. The remaining majority (n=13; 29%) of children were managed without an NPA, using positioning alone (10/13; 77%) or positioning combined with supplemental oxygen (1/13), CPAP (1/13), or both adjunct measures (1/13). Only 2 patients underwent surgical intervention. Feeding supplementation using nasogastric tube was necessary in 78% of patients for a median duration of 4 months. Cleft palate co-existed in all but one patient. Management of upper airway obstruction in PRS children is variable between units. Over a 5-year period, 96% of children with PRS were successfully managed without surgical intervention at the Queensland Children's Hospital. These findings contrast with some other literature and may suggest that more careful consideration of surgical intervention in PRS patients is prudent.

Bronchial reactivity and asthma at school age after early-life metapneumovirus infection.

The association between early-life lower respiratory tract infection (LRTI) and asthma is well established. Knowledge about bronchial hyperresponsiveness (BHR) and asthma after metapneumovirus (MPV) LRTI is scarce. The aim of this study was to assess BHR and current asthma in school-aged children after hospital admission for early-life LRTI with MPV, and to compare with more well-known viruses, rhinovirus (RV) and respiratory syncytial virus (RSV), and with controls. A cohort consisting of children admitted for LRTI and controls was followed-up at school age with a clinical research assessment and lung function tests, including a methacholine provocation test. Current asthma was defined based on objective variable airway obstruction and clinical symptoms. BHR and asthma were compared according to viral groups. 135 children (median age 9.3 years) were included (16 MPV, 34 RV, 51 RSV, 13 mixed infections and 21 controls). Compared with controls there was increased BHR after MPV and RV LRTI (provocative dose causing a 20% fall in forced expiratory volume in 1 s and dose-response slope; p<0.05). Using Kaplan-Meier statistics, BHR was increased for MPV compared with both controls and RSV (p=0.02 and p=0.01). The proportion of children with current asthma at follow-up was higher in the LRTI children compared with the controls (46% versus 24%; p=0.06). Among children who had undergone MPV and RV infection, 50% fulfilled the asthma criteria compared with 43% in the RSV group (p=0.37). We found increased BHR and a high prevalence of asthma in school-aged children after early-life MPV infection, and findings were similar to RV, and less to RSV, compared with controls.

Memo to a patient with bronchial asthma

Bronchial asthma (BA) is a heterogeneous disease characterized by chronic inflammation of the airways and the presence of respiratory symptoms such as wheezing, dyspnea, chest congestion, and cough that vary in time and intensity and manifest themselves with variable airway obstruction.

Type 2 cytokine genes as allergic asthma risk factors after viral bronchiolitis in early childhood.

Genome-wide association studies of asthma have identified associations with variants in type-2 related genes. Also, specific interactions between genetic variants and viral bronchiolitis in the development of asthma has been suggested. To conduct a gene-based analysis of genetic variants in type 2 cytokine related genes as risk factors for allergic asthma at school age, and further, to study their interaction with specific viral infections in early childhood. A prospectively investigated cohort of children with previous bronchiolitis and controls came for follow-up at school age. The research visit, blinded to viral exposure, included detailed lung function tests, laboratory investigation, and questionnaires. Allergic asthma was defined as typical symptoms plus objective variable airway obstruction, in addition to laboratory verified atopy (elevated eosinophil count or sensitization to an allergen). Targeted and complete sequencing was performed for nine type 2 cytokine candidate genes: IL4, 5, 13, 25, 33 and 37, IL17RB, CRLF2 and TSLP. At follow-up, there were 109 children with genetic data, 91 with a history of bronchiolitis (46% respiratory syncytial virus, 24% human rhinovirus, 15% human metapneumovirus and 14% mixed viral etiology) and 18 without. The median age was 9.4 years (range 6-13) and 41 (38%) had laboratory verified atopy. Twenty-one children (19%) met the definition of allergic asthma. After adjusting for age, sex and five viral categories, IL33 achieved nominal significance (p = 0.017) for a positive association with allergic asthma development. In the gene-virus interaction analysis, the variant set in IL17RB demonstrated a nominally significant positive interaction with human metapneumovirus infection (p=0.05). The results highlight the multifactorial nature of allergic asthma risk, with both viral infection and inherited genetic variants contributing to increasing risk. Results for IL33 and IL17RB were nominally significant and are potential candidate targets for designing therapeutics and early screening, but these results must be replicated in an independent study.

Respiratory support strategies in the management of severe, longstanding bronchopulmonary dysplasia.

Despite efforts to minimize ventilator-induced lung injury, some preterm infants require positive pressure support after 36 weeks' post-menstrual age. Infants with severe BPD typically experience progressive mismatch of ventilation and perfusion, which manifests as respiratory distress, hypoxemia in room air, hypercarbia, and growth failure. Lung compliance varies, but lung resistance generally increases with prolonged exposure to positive pressure ventilation and other sources of inflammation. Serial lung radiographs reveal a heterogeneous pattern, with areas of both hyperinflation and atelectasis; in extreme cases, macrocystic changes may be noted. Efforts to wean the respiratory support are often unsuccessful, and trials of high frequency ventilation, exogenous corticosteroids, and diuretics are common. The incidence of pulmonary hypertension increases with the severity of BPD, as does the mortality rate. Therefore, periodic screening and efforts to mitigate the risk of PH is fundamental to the management of longstanding BPD. Failure of conventional, lung-protective strategies (e.g., high rate/low tidal-volume and/or high frequency ventilation) warrants consideration of ventilatory strategies individualized to the disease physiology. Non-invasive modes of respiratory support may be successful in infants with mild to moderate BPD phenotypes. However, infants with moderate to severe BPD phenotypes often require invasive respiratory support, and pressure-limited or volume-targeted conventional ventilation may be better suited to the physiology than high-frequency ventilation. The consistent provision of adequate support is fundamental to the management of longstanding BPD and is best achieved with a stepwise increase in ventilator support until comfortable spontaneous respirations are achieved. Adequately supported infants typically experience improvements in both oxygenation and ventilation, which, if sustained, may arrest and generally reverses the course of a potentially lethal lung disease. Care should be individualized to address the most likely pulmonary mechanics, including variable lung compliance, elevated airway resistance, and variable airway obstruction.

Potential diagnostic role of galactomannan and β-D-glucan in occupational asthmatics in wheat mills

Occupational asthma is defined as variable airway obstruction due to exposure to a specific substance in the workplace environment. The aim of the study was to find out the frequency of occupational asthma in wheat mill workers and to explore the role of fungi biomarkers (galactomannan (GM) and β-D-glucan (βDG)) in occupational asthma. Subjects and methods: Assessment of fungi in air was done. Spirometric function of workers (n=120) were assessed during diurnal (before and 15 minutes after inhalation of salbutamol) and nocturnal time. Blood samples were analyzed to measure serum GM and βDG and allergen specific immunoglobulin E. The workers were asked about asthma symptoms. Results and conclusion: A significant proportion of workers had occupational asthma (25%) and only four workers (3.3%) had non-occupational asthma (excluded from the study). The rest were normal (86 workers). Penicillium and Aspergillus were the only species found in the air environment. Mean GM concentration (GM-c) was significantly higher among occupational asthmatics than normal workers. The estimated risk of occupational asthma (Odds ratio) was 13.3 times higher among workers with high GM-c (>1 pg/ml). Ln (GM) was the only significant predictor of PEFR variability. GM has a potential diagnostic role in occupational asthma due to fungal exposure among wheat mill workers. βDG did not have a role in occupational asthma. We recommend that wheat mill flour workers should do periodic spirometric functions and serum GM every 6 - 12 months for early diagnosis and control of occupational asthma.

Bronchial asthma in the work of a general practitioner

Bronchial asthma is a heterogeneous disease, characterized by chronic inflammation of the airways and certain respiratory symptoms such as shortness of breath, chest tightness, cough, and sibilant rale. All symptoms vary in intensity and duration, occur at any time of the day, but especially often at night or in the early morning, and present with variable airway obstruction. It is one of the most common diseases: there are about 348 million patients in the world, with 32 million patients in Europe. According to experts in the Russian Federation, the prevalence of bronchial asthma is 6.9 % in adult population and about 10 % in children and adolescents. The disease can occur at any age; approximately in half of patients, bronchial asthma develops before the age of 10, and in third — before the age of 40. Among children with asthma, there are 2 times more boys than girls; by the age of 30, this ratio levels off. The death rate from asthma in children is 2.5 per million (about 185 patients).

Evaluation of methacholine challenge test results in patients with chronic unexplained cough

Introduction: Asthma is one of the most common chronic diseases in adults characterized by variable airway obstruction. Asthma is diagnosed with wheezing and shortness of breath, however it sometimes manifests as a chronic cough that is difficult to diagnose and requires spirometry stimulation tests. Objectives: This study aimed to investigate the relationship between methacholine test results and respiratory symptoms in patients with chronic unexplained cough. Patients and Methods: In this cross-sectional study, 102 patients with chronic cough and normal basal spirometry were included. All patients underwent a methacholine challenge test, and their airway irritabilities were assessed. Then, the relationship between methacholine test results and clinical symptoms and also demographic characteristics were investigated. Results: Of 102 patients, 69 (67.6 %) and 33 (32.4%) were female and male, respectively. Most of patients were non-smoker (90.2%). Around 25 (24.5%) patients had a positive methacholine test. Rhinitis had a statistically significant difference between patients with positive and negative tests (positive test group: 48%, negative test group: 23.4%; P = 0.01). Postnasal discharge was reported in 60% of positive methacholine test patients, which was significant compared to the negative group (36.4%; P = 0.03). Most patients who had positive tests were housewives (76%). Logistic regression showed that female gender (OR: 7.75, 95% CI: 1.7-35.2), postnasal discharge (OR: 3.19, 95% CI: 1.25-8.2), rhinitis (OR: 3.02, 95% CI: 1.17-7.79), and allergy symptoms OR: 3.51, 95% CI: 1.35-9.18) were directly associated with a positive methacholine test. Conclusion: The presence of airway hypersensitivity, postnasal discharge, allergic rhinitis, female gender and the housewife’s job were strongly associated with a positive methacholine test.

Tracheobronchial Compression by Right-sided Aortic Arch in a Middle Aged Male

Exertional dyspnoea is a common symptom among middle-aged population. Diagnostic evaluation of such patients is often challenging and confusing. We report a patient presenting with exertional dyspnoea and an obstructive ventilatory defect on spirometry that was refractory to bronchodilator therapy. Careful review of the chest radiograph and spirometry pointed towards variable intra-thoracic airways obstruction as a cause of dyspnoea. Contrast enhanced computed tomography (CECT) of the thorax and bronchoscopy established the diagnosis of a right-sided aortic arch resulting in tracheobronchial compression and tracheomalacia.

0820 Sleep and Oculopharyngeal Muscular Dystrophy: disease progression affecting ventilatory needs and treatment of sleep-disordered breathing

Abstract Introduction Oculopharyngeal muscular dystrophy (OPMD) is an autosomal-dominant, late-onset, and progressive disease characterized by ptosis and dysphagia, sometimes proximal limb weakness and gait abnormalities. It often presents in patients in their 50s. The progressive functional decline of the pharyngeal muscles results in feeding difficulties and aspiration; however, patients may also have risk of nocturnal hypoventilation and sleep apnea, complicated by variable airway obstruction and compliance. Report of Cases: Using retrospective chart review, we identified patients with a known diagnosis of OPMD treated at the Raymond G. Murphy VA Sleep Center. We present a case where OPMD progression necessitated increased ventilatory support and affected positive airway pressure (PAP) compliance. A 58-year-old male with OPMD, DMT2, depression, and memory impairment underwent home sleep apnea testing showing severe OSA (REI 32.7, SpO2 nadir 72%). He started Auto-PAP 6-16 cwp and presented to discuss issues tolerating PAP. Pressures were lowered, but he continued to require maximum pressures without increased utilization. An in-lab CPAP titration showed treatment-emergent centrals but did not find optimal pressures due to limited sleep time. Having failed CPAP, he returned for an ASV titration which controlled his apnea in lateral position. Patient was switched to auto ASV to increase efficacy and comfort. Two months later he discontinued ASV due to frustration with disease progression and feeling unable to breathe deeply with the machine. Nocturnal oxygen at 1L was ordered while he awaited Neurology consult for OPMD. Later, concerned about progressive dyspnea, he resumed ASV, now with 3L O2 bleed. Given suspicion of hypoventilation (bicarbs 27-30), and that ASV could not adjust to his continually changing airway tone with his OPMD, he was switched to iVAPS. This resulted in good control of his sleep apnea, tidal volumes and minute ventilation. However, he reported pressures felt too high, returned to ASV for a period, then discontinued PAP altogether. Conclusion Patients with OPMD and sleep apnea require close follow-up as their disease progression may affect their ventilatory support needs. These patients may require more complex PAP modalities, such as AVAPs, and routine PFTs to help determine timing of ENT involvement for surgical airway planning. Support (If Any)

Adaptation to Asthma in Children: A Matter of Coping and Stress Control

ABSTRACT Asthma is the most common chronic lower respiratory disease in childhood throughout the world; it is characterized by bronchial hyperreactivity and variable airway obstruction. Asthma is a disorder involving autonomic nervous system, immunologic, and psychologic factors in individuals. This work sought to describe the relationship among stress, coping, and disease control in children with asthma between 6 and 12 years of age. This was a cross-sectional correlational quantitative study with the participation of 280 children with asthma admitted to outpatient consultation and to a hospitalization service at a tier IV health institution in the city of Bogotá. An inverse correlation was found between stress and coping (r = −0.581; p < .05); between stress and asthma control (r = −0.545¸ p < .05); and a direct correlation between coping and asthma control (r = 0.759; p < .05). The results show that children with low stress levels have greater control of their disease and assume functional coping against their health status. The theory of Adaptation to Chronic Health Conditions permitted analyzing children’s conduct and behaviors against a health situation. These findings provide evidence for promoting interventions directly to patients with asthma, focusing on patients, families, and schools. Thus, it is possible to improve the quality of health care and strengthen primary health care.

Clinical Relevance of Plasma Concentrations of MBL in Accordance with IgE Levels in Children Diagnosed with Bronchial Asthma.

Background and objectives: Bronchial asthma is a heterogeneous, multifactorial pulmonary disease characterized by variable airway obstruction caused by chronic inflammation. Our study investigates the clinical relevance of MBL plasma levels in accordance with IgE values in children who attended a pediatric consult for respiratory symptoms with bronchial asthma. Materials and Methods: The study population consists of patients <18-years-old and included 43 patients with bronchial asthma and 64 age-matched healthy subjects as a control group. We used the ELISA Human MBL Immunoassay kit and the electrochemiluminescence immunoassay (ECLIA) kit for IgE determination. Results: Our results show significantly different distributions of patients in the bronchial asthma group and control group. The measured values were within the normal range for most controls, while the bronchial asthma patients displayed higher values of plasma MBL and IgE levels. We observed a wider heterogeneity in MBL concentrations in bronchial asthma patients when compared to the healthy age-matched controls. Our results also suggest a potential clinical usefulness of plasma MBL concentrations in accordance with IgE and eosinophil cells levels in the diagnosis of bronchial asthma, and our results may suggest a prognostic role of MBL in the evolution of asthmatic disease; however, further studies are necessary to confirm these findings. Conclusions: We can say that plasma MBL concentrations present a relative diagnostic role for bronchial asthma in pediatric patients and may suggest a more severe disease progression; however, further studies are needed to elucidate the role played by MBL in the determination and evolution of this disease.

Correlations of forced oscillometric bronchodilator response with airway inflammation and disease duration in asthma.

Airway resistance and reactance, measured by forced oscillometry, are used to measure the airway obstruction in patients with asthma. This study aimed to investigate the oscillometric bronchodilator responses in treated and untreated asthma and evaluate its association with airway inflammation and disease duration. This study included 30 nonsmoking patients with mild to moderate treated asthma, 25 patients with newly diagnosed untreated asthma and 29 control subjects. Spirometric and oscillometric measurements were performed before and after inhalation of 400µg salbutamol. Disease duration was defined as the number of years since asthma diagnosis. At airway resistance of 5Hz (R5) and 20Hz (R20), bronchodilator responses in patients with untreated and treated asthma were greater than those in control subjects. In patients with untreated asthma, higher fractional exhaled nitric oxide concentration (FeNO) levels were strongly correlated with greater reversibility of R20 (rs =-0.621, P< 0.001). In patients with treated asthma, there was no significant association between FeNO and oscillometric reversibility, whereas longer disease duration was significantly associated with lesser bronchodilator response at R20 (rs =0.441, P< 0.05). Treated asthma patients with longer disease duration (≥10years) showed significantly higher post-bronchodilator R5 and R20 than the treated asthma patients with shorter disease duration (<10years). The present study provides further evidence of an association between airway inflammation and variable airway obstruction in asthma patients and indicates that long-term disease duration could be related to poorly reversible airway resistance in patients with mild to moderate asthma.

The Treatment of Mild and Moderate Asthma in Adults.

Asthma is a chronic inflammatory airway disease that usually causes variable airway obstruction. It affects 5-10% of the German population. This review is based on relevant publications retrieved by a selective search, as well as on national and international guidelines on the treatment of mild and moderate asthma in adults. The goal of treatment is to attain optimal asthma control with a minimal risk of exacerbations and mortality, loss of pulmonary function, and drug side effects. This can be achieved with a combination of pharmacotherapy and non-drug treatment including patient education, exercise, smoking cessation, and rehabilitation. Pharmacohterapy is based on inhaled corticosteroids (ICS) and bronchodilators. It is recommended that mild asthma should be treated only when needed, either with a fixed combination of ICS and formoterol or with short-acting bronchodilators. For moderate asthma, maintenance treatment is recommended, with an inhaled fixed combinations of ICS and long-acting beta-mimetics, possibly supplemented with longacting anticholinergic agents. Allergen immunotherapy, i.e., desensitization treatment, should be considered if the allergic component of asthma is well documented and the patient is not suffering from uncontrolled asthma. Asthma control should be monitored at regular intervals, and the treatment should be adapted accordingly. The treatment of asthma in adults should be individually tailored, with anti-inflammatory treatment as its main component.

Advances of nitric oxide and inducible nitric oxide synthase in asthma

Asthma is a chronic inflammatory respiratory disease characterized by variable airway obstruction, airway hyperresponsiveness, and airway remodeling.In the respiratory tract, nitric oxide(NO) is generated via oxidation of L-arginine that is catalyzed by inducible nitric oxide synthase(iNOS). In hypoxia-ischemia and inflammation processes, iNOS is induced to produce excessive NO, playing an important role in asthma, including the modulation of airway and vascular smooth muscle tone and the inflammation.This review focuses on recent research achievements regarding the relationship between NO and iNOS and asthma. Key words: iNOS; NO; Asthma

The Global Impact of Asthma in Adult Populations.

Asthma is a disease characterized by chronic airway inflammation, leading to intermittent symptoms of wheeze, dyspnea, cough and chest tightness in combination with variable expiratory airway obstruction. Clinical diagnosis is usually established based on the presence of symptoms and documented variability in expiratory airflow limitation as measured by pulmonary function testing. Presently, asthma is a major chronic disease affecting approximately 334 million people worldwide. The epidemic spares no age group, race or ethnicity; however ethnicity and socioeconomic status do influence the prevalence, morbidity and mortality of asthma in the United States and various countries throughout the world. Moreover, asthma places a huge burden at the societal, financial and health-care levels of multiple nations.

Biologics and the lung: TSLP and other epithelial cell-derived cytokines in asthma.

Asthma is a chronic airway inflammatory disorder with characteristic symptoms of dyspnea, wheeze, chest tightness and cough, and physiological abnormalities of variable airway obstruction, airway hyperresponsiveness, and in some patients with chronic long standing disease reduced lung function. The physiological abnormalities are due to chronic airway inflammation and underlying structural changes to the airway wall. The interaction between the airway epithelium and the environment is crucial to the pathobiology of asthma. Several recent discoveries have highlighted a crucial role of airway epithelial derived cytokines such as interleukin (IL)-25, IL-33 and thymic stromal lymphopoietin (TSLP). These cytokines are collectively known as epithelial "alarmins", which act solely or in concert to activate and potentiate the innate and humoral arms of the immune system in the presence of actual or perceive damage. Understanding the role of alarmins and how they are activated and released may allow the development of novel new therapeutics to treat asthma. This review describes the interactions between inhaled air, the pulmonary microbiome, airway epithelial cell layer and the alarmins, IL-25, IL-33 and TSLP. There is already compelling evidence for a role of TSLP in the airway responses to environmental allergens in allergic asthmatics, as well as in maintaining airway eosinophilic inflammation in these subjects. Further work is required to develop human monoclonal antibodies (hMabs) directed against IL-25 and IL-33 or their receptors, to help understand their role in the initiation and/or persistence of asthma.

Asthma: definitions and pathophysiology.

Asthma is a common condition due to chronic inflammation of the lower respiratory tract. Chronic lower airway inflammation is known to be more common in individuals that also have inflammatory disorders of the upper airway. The scientific understanding of asthma continues to improve and it is important for providers who treat upper or lower airway inflammation to be familiar with asthma's definition and pathophysiology. Articles were selected based on literature reviews through PubMed and personal knowledge of the author. The search selection was not standardized. Asthma is a heterogenic condition that is underdiagnosed and undertreated despite that the skills needed to diagnose it are readily attainable and effective treatments are available. Providers need a working understanding of asthma in order to be proficient at managing their patients with chronic nasal or sinus inflammation. This article provides a primer focusing on the current conception asthma in terms of definition, possible etiologies, inflammatory profile, pathophysiology, subtypes, and overlapping conditions. Asthma is a chronic inflammatory disorder arising from not fully understood heterogenic gene-environment interactions. It features variable airway obstruction and bronchial hyperresponsiveness. Clinically, asthmatics exhibit recurrent episodes of wheeze, cough, chest tightness, and shortness of breath.

What goes up must come down: biomarkers and novel biologicals in severe asthma.

Asthma is a heterogeneous airway disease characterized by typical symptoms in combination with variable airway obstruction. Most patients with asthma have well controlled symptoms and a low risk of asthma attacks with inhaled corticosteroid (ICS) treatment. However, a clinically important subgroup (~10%) remains symptomatic and/or at risk of asthma attacks despite maximum inhaled therapy. Patients with severe asthma are responsible for a significant proportion of healthcare costs attributable to asthma and have a large unmet need for better treatments. An important advance in recent years has been the recognition that severe asthma is heterogeneous with respect to clinical problems and the pattern of lower airway inflammation. Identification of eosinophilic inflammation in the airways has become an important priority as novel biologicals that target Th2 cytokines, such as anti-IL5, anti-IL-13 and combined anti-IL-4/13 are showing considerable promise as treatments for this subgroup. It has also become clear that anti-IgE (Omalizumab), the first monoclonal antibody registered for treatment of severe asthma, is only active in patients with active eosinophilic airway inflammation. The future will be identification of potentially responsive patients on the basis of raised biomarkers and, as suggested by the title of this review, targeted treatment with specific cytokine blockade that has a direct effect on the biomarkers. In this review, we outline an approach to the clinical assessment of patients potentially suitable for biological treatment and describe in detail the likely clinical impact of established and new biological treatments.

Not All That Wheezes Is Asthma

A 62-year-old female complained of exertional dyspnoea associated with occasional wheeze over the past 10 years. She could not identify any trigger factors such as exposure to cold air, exercise or respiratory tract infections. She was treated empirically for bronchial asthma by her family physician with inhaled Salbutamol and inhaled Fluticasone without much relief in symptoms. She also recently complained of mild dysphagia on swallowing large boluses of food. There was no associated chest pain, weight loss or dyspepsia. The patient worked as a seamstress and was a non-smoker. Physical exam was remarkable for expiratory wheeze, which was monophonic. Her voice was normal and there was no associated cervical adenopathy or goitre on neck examination. A fl ow-volume loop showed variable intrathoracic upper airway obstruction (Fig. 1A). Spirometry was normal. The chest radiograph is as shown in Figure 1B. What is the diagnosis causing her symptoms? A. Intrathoracic goitre B. Paratracheal lymadenopathy C. Aortic aneurysm D. Vascular ring E. Teratoma The computed tomography scan of the chest (Fig. 2) confi rmed a right-sided aorta associated with a diverticulum of Kommerell, an aberrant left subclavian artery and the left carotid artery forming a vascular ring. Bronchoscopy showed a mild stricture of the mid-trachea due to extrinsic compression. Therefore this patient has congenital vascular ring anomaly causing narrowing and displacement of the trachea and esophagus resulting in dyspnoea and dysphagia lusoria.