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Tracheostomy tubes act as foreign bodies, predisposing the surrounding airway to respiratory infections. Initial treatment for infections is topical - nebulized tobramycin - although guidelines for standardized treatment are lacking. To quantify tobramycin delivery to simulated, tracheostomized children to inform future administration guidelines. A breathing simulator was programmed for volume-controlled ventilation at 6 mL.kg -1 , for a 3 kg and a 16 kg child representing under or over 6-yrs, respectively. Nebulized tobramycin doses based on current guidelines for non-tracheostomized children (80 mg, or 300 mg, under and over 6-yrs, respectively) were delivered using standard hospital protocol, collected on filters, and assayed with chromatography to quantify average tobramycin delivered dose from six replicate measurements. The jet nebulizer delivered more tobramycin than the vibrating mesh nebulizer from an 80 mg (ages <6-yrs) dose for both a 3 kg child: 2.1 vs. 0.7 mg (3 mm, p = 0.047) and a 16 kg child: 8.7 vs. 3.5 mg (5 mm size, p = 0.022), 11.4 vs. 8.3 mg (4 mm size, p = 0.2). The jet nebulizer delivered more tobramycin than the vibrating mesh nebulizer for both a 3 kg child: 8.4 vs. 3.7 mg (3 mm, p = 0.00076) and a 16 kg child: 33.2 vs. 25 mg (5 mm, p = 0.2) but not for a 16 kg child: 39.4 vs. 46.5 mg (4 mm, p = 0.18) The low amount and poor distribution of drug delivered warrants consideration and review of dosing regimens for treatment. Future research should investigate improving the efficiency of drug delivery to tracheostomized children and the safety and efficacy of higher-dosage regimens., (Copyright © 2024. Published by Elsevier Masson SAS.)

Background: Current treatments for respiratory infections are severely limited. Ethanol's unique properties including antimicrobial, immunomodulatory, and surfactant-like activity make it a promising candidate treatment for respiratory infections if it can be delivered safely to the airway by inhalation. Here, we explore the safety, tolerability, and pharmacokinetics of inhaled ethanol in a phase I clinical trial., Methods: The study was conducted as a single-centre, open-label clinical trial in 18 healthy adult volunteers, six with no significant medical comorbidities, four with stable asthma, four with stable cystic fibrosis, and four active smokers. A dose-escalating design was used, with participants receiving three dosing cycles of 40, 60%, and then 80% ethanol v/v in water, 2 h apart, in a single visit. Ethanol was nebulised using a standard jet nebuliser, delivered through a novel closed-circuit reservoir system, and inhaled nasally for 10 min, then orally for 30 min. Safety assessments included adverse events and vital sign monitoring, blood alcohol concentrations, clinical examination, spirometry, electrocardiogram, and blood tests., Results: No serious adverse events were recorded. The maximum blood alcohol concentration observed was 0.011% immediately following 80% ethanol dosing. Breath alcohol concentrations were high (median 0.26%) following dosing suggesting high tissue levels were achieved. Small transient increases in heart rate, blood pressure, and blood neutrophil levels were observed, with these normalising after dosing, with no other significant safety concerns. Of 18 participants, 15 completed all dosing cycles with three not completing all cycles due to tolerability. The closed-circuit reservoir system significantly reduced fugitive aerosol loss during dosing., Conclusion: These data support the safety of inhaled ethanol at concentrations up to 80%, supporting its further investigation as a treatment for respiratory infections. Clinical trial registration: identifier ACTRN12621000067875., Competing Interests: BC is a director of Inspiring Pty Ltd. who are developing the reservoir spacer system for commercialisation. WD has shares in Inspiring and has been contracted by Inspiring to perform aerosol testing in the past. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Hancock, Ditcham, Ferguson, Karpievitch, Stick, Waterer and Clements.)

Background: Structural lung disease and neutrophil-dominated airway inflammation is present from 3 months of age in children diagnosed with cystic fibrosis after newborn screening. We hypothesised that azithromycin, given three times weekly to infants with cystic fibrosis from diagnosis until age 36 months, would reduce the extent of structural lung disease as captured on chest CT scans., Methods: A phase three, randomised, double-blind, placebo-controlled trial was done at eight paediatric cystic fibrosis centres in Australia and New Zealand. Infants (aged 3-6 months) diagnosed with cystic fibrosis following newborn screening were eligible. Exclusion criteria included prolonged mechanical ventilation in the first 3 months of life, clinically significant medical disease or comorbidities other than cystic fibrosis, or macrolide hypersensitivity. Participants were randomly assigned (1:1) to receive either azithromycin (10 mg/kg bodyweight orally three times per week) or matched placebo until age 36 months. Randomisation was done with a permuted block strategy and an interactive web-based response system, stratified by study site. Unblinding was done once all participants completed the trial. The two primary outcomes were the proportion of children with radiologically defined bronchiectasis, and the percentage of total lung volume affected by disease. Secondary outcomes included clinical outcomes and exploratory outcomes were inflammatory markers. Analyses were done with the intention-to-treat principle. This study is registered at ClinicalTrials.gov (NCT01270074)., Findings: Between June 15, 2012, and July 10, 2017, 281 patients were screened, of whom 130 were enrolled, randomly assigned, and received first study dose. 68 participants received azithromycin and 62 received placebo. At 36 months, 88% (n=50) of the azithromycin group and 94% (n=44) of the placebo group had bronchiectasis (odds ratio 0·49, 95% CI 0·12 to 2·00; p=0·32), and total airways disease did not differ between groups (median difference -0·02%, 95% CI -0·59 to 0·56; p=0·96). Secondary outcome results included fewer days in hospital for pulmonary exacerbations (mean difference -6·3, 95% CI -10·5 to -2·1; p=0·0037) and fewer courses of inhaled or oral antibiotics (incidence rate ratio 0·88, 95% CI 0·81 to 0·97; p=0·0088) for those in the azithromycin group. For the preplanned, exploratory analysis, concentrations of airway inflammation were lower for participants receiving azithromycin, including interleukin-8 (median difference -1·2 pg/mL, 95% CI -1·9 to -0·5; p=0·0012) and neutrophil elastase activity (-0·6 μg/mL, -1·1 to -0·2; p=0·0087) at age 36 months, although no difference was noted between the groups for interleukin-8 or neutrophil elastase activity at 12 months. There was no effect of azithromycin on body-mass index at age 36 months (mean difference 0·4, 95% CI -0·1 to 0·9; p=0·12), nor any evidence of pathogen emergence with the use of azithromycin. There were few adverse outcomes with no differences between the treatment groups., Interpretation: Azithromycin treatment from diagnosis of cystic fibrosis did not reduce the extent of structural lung disease at 36 months of age; however, it did reduce airway inflammation, morbidity including pulmonary exacerbations in the first year of life and hospitalisations, and improved some clinical outcomes associated with cystic fibrosis lung disease. Therefore we suggest thrice-weekly azithromycin is a strategy that could be considered for the routine early management of paediatric patients with cystic fibrosis., Funding: Cystic Fibrosis Foundation., Competing Interests: Declaration of interests During the conduct of the trial, SMS and HAWMT had a patent pending for the PRAGMA-CT scoring method used to analyse the CT scans in this trial (PCT/AU2016/000079). CW is on the International Advisory Board for Vertex Pharmaceuticals. HAWMT is Chief Medical Officer for Thirona. All other authors declare no competing interests., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

Background: Neutrophil elastase is a significant risk factor for structural lung disease in cystic fibrosis, and Pseudomonas aeruginosa airway infection is linked with neutrophilic inflammation and substantial respiratory morbidity. We aimed to evaluate how neutrophil elastase (NE) activity changes after P. aeruginosa eradication and influences early disease outcomes., Methods: We assessed participants in the AREST CF cohort between 2000 and 2018 who had P. aeruginosa cultured from their routine annual bronchoalveolar lavage (BAL) fluid and who underwent eradication treatment and a post eradication BAL. Factors associated with persistent P. aeruginosa infection, persistent neutrophilic inflammation following eradication and worse structural lung disease one year post-eradication were evaluated., Results: Eighty-eight episodes (3 months to 6 years old) of P. aeruginosa infection were studied. Eradication was successful in 84.1% of episodes. Median activity of NE was significantly reduced post-eradication from 9.15 to 3.4 nM (p = 0.008) but persisted in 33 subjects. High post-eradication NE levels were associated with an increased risk for P. aeruginosa infection in the next annual visit (odds ratio=1.7, 95% confidence interval 1.1-2.7, p = 0.014). Post-eradication NE levels (difference, 0.8; 95% confidence interval, 0.1-1.5) and baseline bronchiectasis computed tomography (CT) score (difference, 0.4; 95% confidence interval, 0.1-0.8) were the best predictors of bronchiectasis progression within 1 year (backward stepwise linear regression model, R 2 = 0.608, P<0.001), independent of eradication., Conclusion: In children with CF, NE activity may persist following successful P. aeruginosa eradication and is significantly associated with bronchiectasis progression. Evaluating strategies to diminish neutrophilic inflammation is essential for improving long-term outcomes., Competing Interests: Declaration of Competing Interest There are no conflicts of interest for the authors in regard to the work presented in this publication., (Copyright © 2020. Published by Elsevier B.V.)

Background: Anomalous systemic artery to the left lower lobe (ASALLL) is a rare congenital anomaly. The primary symptoms include hemoptysis and lung infection, though some patients may remain asymptomatic. Currently, there is no consensus on the indications for treatment or the optimal choice of therapy for this condition. This case presents a case of an asymptomatic adult who underwent coil embolization. Case presentation: A 48-year-old male was admitted to our hospital due to the discovery of a space-occupying lesion in the left hilum. The contrast-enhanced pulmonary computed tomography scan was used to diagnose the patient with an anomalous systemic artery to the left lower lobe. We performed coil embolization on this patient, who underwent a follow-up computed tomography angiography of the pulmonary and bronchial arteries one year later. Result demonstrated complete embolization of the abnormal systemic arteries and a slight reduction in the volume of the left lower lobe. Conclusion: Coil embolization is a safe and minimally invasive procedure for adult patients who have an anomalous systemic artery to the left lower lobe. [ABSTRACT FROM AUTHOR]

When do infants and young children with cystic fibrosis acquire infection with Pseudomonas aeruginosa? Can this be eradicated when first detected? Children <6 yrs of age participated in an annual bronchoalveolar lavage (BAL)-based microbiological surveillance programme in Perth, Australia. When P. aeruginosa was detected, an eradication programme using combination treatment with i.v., oral and nebulised antibiotics was undertaken. Repeat BAL was performed 3 months following treatment, to assess eradication success. P. aeruginosa was detected in 33 (28.4%) children; median (range) age at detection was 30.5 (3.3-71.4) months. P. aeruginosa was mucoid at detection in six (18.2%) out of 33 patients and associated with respiratory symptoms in 16 (48.5%) out of 33 children. In total, 26 children underwent eradication therapy, with P. aeruginosa eradicated in 20 (77%) out of 26 following one eradication cycle and in three (total 88%) additional children following a second cycle. Eradication was associated with a significant decrease in neutrophil elastase and interleukin-1beta in BAL fluid 12 months post eradication. Eradication of Pseudomonas aeruginosa infection is achievable in young children with cystic fibrosis for up to 5 yrs using combination i.v., oral and nebulised antibiotic therapy and is associated with reduced pulmonary inflammation 12 months post eradication.

Copyright of Magyar Radiológia Online is the property of Society of Hungarian Radiologists and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Key Clinical Message: Pleural effusion is rare during neonatal period with an estimated prevalence of 0.06%. It may sometimes uncommonly be secondary to pulmonary sequestration. Besides common conditions like hydrops fetalis, congenital heart disease, congenital chylothorax, chromosomal abnormalities; pulmonary sequestration should also be considered while evaluating the cause for neonatal pleural effusion. [ABSTRACT FROM AUTHOR]

This study used the instructional humor processing theory to test how different humor subtypes employed by teachers (course-related, course-unrelated, self-disparaging, other-disparaging) relate to students' well-being, sense of belonging, and engagement. The participants comprised 395 students (boys = 106; girls = 270; other = 8; NA = 11) (secondary school students = 291; primary school students = 97, NA = 7) from five public school boards located in rural areas, and one private secondary school situated in an urban area (Mage = 14.11) with a proportion of 93% speaking French at home. Correlational and structural equation modeling methods were used to analyze these relationships. Results showed that only humor related to course content (positive association) and other-disparaging humor (negative association) were significantly associated with the sense of belonging, which, in turn, was positively associated with a cognitive, affective, and behavioral engagement. Results also showed that only course-related humor (positive association) and unrelated humor (negative association) were significantly associated with students' emotional well-being, which, in turn, was positively associated with cognitive and affective engagement. As far as this study is concerned, humor in the classroom should be course-related when it comes to supporting students' emotional well-being, sense of belonging, and engagement. [ABSTRACT FROM AUTHOR]

Horseshoe lung (HL) is a rare congenital anomaly represented by the fusion of both lungs, posterior to the heart, and is typically associated with various bronchopulmonary and cardiovascular malformations. Multi-detector contrast enhanced CT is the imaging modality of choice to demonstrate the pathology and associated malformations. There has been inconsistency in the nomenclature used for such cases in literature. To resolve ambiguity, the authors emphasise that only two terms: HL and pseudo-HL be used on imaging to describe variants of this congenital malformation. Contribution: A description of the imaging features in four cases of HL, with their associated malformations and a review of the nomenclature. [ABSTRACT FROM AUTHOR]

Three cases of a congenitally misplaced segment of lung are described. In one case the abnormal segment was identified at thoracotomy; it extended from an origin in the right upper lobe across the midline into the left hemithorax. The remaining two patients, with identical radiographic features, were identified in a review of 24 cases of bronchovascular malformation. In all three patients the crossover segment was associated with a variant of the scimitar syndrome: right lung hypoplasia with dextrocardia, aberrant systemic arterial supply to the right lower lobe, and anomalous venous drainage of the whole of the right lung, and in two patients with eventration of the right diaphragm.

Congenital malformations of the bronchopulmonary airway and related arterial blood supply are a complex group of lesions in which abnormalities of venous drainage and lung parenchyma may coexist. Twenty five cases have been analysed, by a method whereby each anatomical component is separately considered. All 25 patients had abnormalities of the tracheobronchial tree, with no connection to the abnormal segment in nine cases. The aberrant arterial blood supply was single in 16 cases and multiple in nine cases, one patient from the latter group having a mixed pulmonary and systemic arterial supply to a part of the abnormal segment. Seventeen patients had anomalous venous drainage. In nine of these the vein or veins (they were multiple in four cases) drained the major part or the whole of the lung, whereas the aberrant arterial supply was limited to the right lower zone--that is, mismatched anomalous venous drainage. Abnormalities of lung parenchyma included changes within the lesion (for example, cysts, foregut inclusions) and associated abnormalities of surrounding lung (for example, hypoplasia, abnormal lobation). This information, together with the clinical features and haemodynamic data, was found to be essential for decisions on appropriate management. Patients presenting in infancy with haemodynamic disturbance continue to present major management problems (50% mortality), particularly if there are associated congenital heart defects. The role of aberrant systemic artery occlusion or ligation as a first stage procedure is well established in patients with haemodynamic abnormalities. There may be a place for this procedure in selected patients who have no haemodynamic disturbance at presentation.

The pulmonary sequestration spectrum and related congenital lung anomalies present an extremely complex and varied group of bronchopulmonary-vascular malformations. Previous attempts at nomenclature and classification have proved inadequate. In this article we present a classification of the newly named pulmonary malinosculation spectrum, which includes all congenital lung anomalies where there is abnormal connection (that is, malinosculation) of one or more of the four major components of lung tissue--namely, tracheobronchial airway, lung parenchyma, arterial supply, and venous drainage, which in various combinations make up these lesions. We feel that this simple descriptive approach will improve our understanding and management of these complicated lesions and this is supported by the clinical experience we report in the next article.

A 52‐year‐old female never‐smoker with an abnormal shadow in the right lung detected on radiography was referred to our institution. Contrast‐enhanced computed tomography revealed an irregular nodule in the upper lobe of the right lung, suggestive of a pulmonary vascular abnormality. Angiography revealed a direct communication between the right internal mammary artery (IMA) and the right upper lobe pulmonary artery branches, with dilated and tortuous vascular proliferation. As multiple branch arteries were seen flowing into the upper lobe from the IMA, transcatheter selective embolization of these vessels and right upper lobectomy by video‐assisted thoracoscopic surgery were performed. Contrary to the clinical diagnosis, the pathological finding was a pulmonary adenocarcinoma of the right upper lobe. Additional lymph node dissection was performed later. We report an extremely rare and unprecedented case of pulmonary adenocarcinoma fed by the right IMA, with a literature review. [ABSTRACT FROM AUTHOR]

Background: Cystic Fibrosis (CF) is a genetic condition characterized by neutrophilic inflammation and recurrent infection of the airways. How these processes are initiated and perpetuated in CF remains largely unknown. We have demonstrated a link between the intestinal microbiota-related metabolites bile acids (BA) and inflammation in the bronchoalveolar lavage fluid (BALF) from children with stable CF lung disease. To establish if BA indicate early pathological processes in CF lung disease, we combined targeted mass spectrometry and amplicon sequencing-based microbial characterization of 121 BALF specimens collected from 12-month old infants with CF enrolled in the COMBAT-CF study, a multicentre randomized placebo-controlled clinical trial comparing azithromycin versus placebo. We evaluated whether detection of BA in BALF is associated with the establishment of the inflammatory and microbial landscape of early CF lung disease, and whether azithromycin, a motilin agonist that has been demonstrated to reduce aspiration of gastric contents, alters the odds of detecting BA in BALF. We also explored how different prophylactic antibiotics regimens impact the early life BALF microbiota. Results: Detection of BA in BALF was strongly associated with biomarkers of airway inflammation, more exacerbation episodes during the first year of life, increased use of oral antibiotics with prolonged treatment periods, a higher degree of structural lung damage, and distinct microbial profiles. Treatment with azithromycin, a motilin agonist, which has been reported to reduce aspiration of gastric contents, did not reduce the odds of detecting BA in BALF. Culture and molecular methods showed that azithromycin does not alter bacterial load or diversity in BALF. Conversely, penicillin-type prophylaxis reduced the odds of detecting BAs in BALF, which was associated with elevated levels of circulating biomarkers of cholestasis. We also observed that environmental factors such as penicillin-type prophylaxis or BAs detection were linked to distinct early microbial communities of the CF airways, which were associated with different inflammatory landscapes but not with structural lung damage. Conclusions: Detection of BA in BALF portend early pathological events in CF lung disease. Benefits early in life associated with azithromycin are not linked to its antimicrobial properties. AFd3CvqLfCjKZ9n4H93NFT Video Abstract [ABSTRACT FROM AUTHOR]

Background: Pulmonary sequestration is a rare congenital lung anomaly, presenting mostly in childhood and adolescence. Case presentation: We report the case of a 26-year-old male patient presenting with pleuritic left sided chest pain and haemoptysis. Computed tomography of the chest showed features of intralobar pulmonary sequestration involving the left lower lobe, with arterial supply arising from the descending thoracic aorta above the diaphragm and normal venous drainage. Video assisted thoracic surgery was planned to perform a left lower lobectomy. Considering the risk of bleeding from the large artery supplying the sequestered segment, a posterolateral thoracotomy incision was made and left lower lobectomy was completed, with successful division of the arterial feeder. The patient was discharged home without complications. Pathologic examination of the specimen grossly revealed partial division of the lobe by two fissures with extensive adhesions into an upper and lower portion with no clear demarcation and a large vessel which enters the lower portion at the posterior inferior aspect, separate from the hilum with a diameter 10 mm. Microscopically, both portions of the lobe showed normally alveolated lung tissue with patchy recent intra-alveolar haemorrhage and evidence of chronic inflammation in the sequestered segment. There was no evidence of malignancy. Conclusion: This case highlights the rare presentation of pulmonary sequestration in adulthood and the importance of imaging to identify anomalous arterial supply to the sequestered segment in the left lower lobe of the lung. The use of safe surgical techniques to control the anomalous systemic arterial feeding vessel cannot be overemphasized. [ABSTRACT FROM AUTHOR]

Fetal therapy term has been described for any therapeutic intervention either invasive or noninvasive for the purpose of correcting or treating any fetal malformation or condition. Fetal therapy is a rapidly evolving specialty and has gained pace in last two decades and now fetal intervention is being tried in many malformations with rate of success varying with the type of different fetal conditions. The advances in imaging techniques have allowed fetal medicine persons to make earlier and accurate diagnosis of numerous fetal anomalies. Still many fetal anomalies are managed postnatally because the fetal outcomes have not changed significantly with the use of fetal therapy and this approach avoids unnecessary maternal risk secondary to inutero intervention. The short-term maternal risk associated with fetal surgery includes preterm labor, premature rupture of membranes, uterine wall bleeding, chorioamniotic separation, placental abruption, chorioamnionitis, and anesthesia risk. Whereas, maternal long-term complications include risk of infertility, uterine rupture, and need for cesarean section in future pregnancies. The decision for invasive fetal therapy should be taken after discussion with parents about the various aspects like postnatal fetal outcome without fetal intervention, possible outcome if the fetal intervention is done, available postnatal intervention for the fetal condition, and possible short-term and long-term maternal complications. The center where fetal intervention is done should have facility of multi-disciplinary team to manage both maternal and fetal complications. The major issues in the development of fetal surgery include selection of patient for intervention, crafting effective fetal surgical skills, requirement of regular fetal and uterine monitoring, effective tocolysis, and minimizing fetal and maternal fetal risks. This review will cover the surgical or invasive aspect of fetal therapy with available evidence and will highlight the progress made in the management of fetal malformations in last two decades. [ABSTRACT FROM AUTHOR]

Copyright of Fudan University Journal of Medical Sciences is the property of Fudan University Journal of Medical Sciences and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Chronic lung disease in children with systemic juvenile idiopathic arthritis (SJIA-LD) is an emerging and potentially life-threatening disease complication. Despite recent descriptions of its clinical spectrum, preliminary immunologic characterization, and proposed hypotheses regaarding etiology, optimal approaches to diagnosis and management remain unclear. Here, we review the current clinical understanding of SJIA-LD, including the potential role of biologic therapy in disease pathogenesis, as well as the possibility of drug reactions with eosinophilia and systemic symptoms (DRESS). We discuss approaches to evaluation of children with suspected SJIA-LD, including a proposed algorithm to risk-stratify all SJIA patients for screening to detect LD early. We review potential pharmacologic and non-pharmacologic treatment approaches that have been reported for SJIA-LD or utilized in interstitial lung diseases associated with other rheumatic diseases. This includes lymphocyte-targeting therapies, JAK inhibitors, and emerging therapies against IL-18 and IFNγ. Finally, we consider urgent unmet needs in this area including in basic discovery of disease mechanisms and clinical research to improve disease detection and patient outcomes., (© 2023. The Author(s), under exclusive licence to Springer Nature Switzerland AG.)

Background and Aim: We sought to address the varied anatomical details, the diagnostic challenges, associated cardiopulmonary anomalies, the techniques, and outcomes of management, including re‐interventions of scimitar syndrome. Methods: A total of 92 published investigations of scimitar syndrome were reviewed. Diagnostic information was provided by clinical presentations, radiographic findings, transthoracic and transesophageal echocardiography, computed‐tomographic angiography, magnetic resonance imaging, angiocardiography, and ventilation/perfusion scans. These investigations served to elucidate the origin, course, and termination of the scimitar vein, the intracardiac anatomy, the presence of associated defects, and the patterns of any accompanying pulmonary lesions prior to surgical intervention. Results: Of the patients described, up to four‐fifths presented during infancy, with cardiac failure, increased pulmonary flow, and pulmonary hypertension. Associated cardiac and extracardiac defects, particularly hypoplasia of the right lung, are present in up to three‐quarters of cases. Overall operative mortality has been cited between 4.8% and 5.9%. Mortality was highest in patients with preoperative pulmonary hypertension, and those undergoing surgery in infancy. Despite timely surgical intervention, post‐repair obstruction of the scimitar vein, intra‐atrial baffle obstruction, or stenosis of the inferior caval vein were reported in up to two‐thirds of cases. The venous obstruction could not be related to any particular surgical technique. On long term follow‐up, one sixth of patients reported persistent dyspnoea and recurrent respiratory infections. Conclusions: Any infants presenting with heart failure, right‐sided heart, and hypoplastic right lung should be evaluated to exclude the syndrome. An increased appreciation of variables will contribute to improved surgical management. [ABSTRACT FROM AUTHOR]

Objectives/Hypothesis: Adenotonsillectomy is first‐line treatment for pediatric obstructive sleep apnea syndrome (OSAS) when not otherwise contraindicated. There is concern severe OSAS increases risk of comorbid cardiopulmonary abnormalities, such as ventricular hypertrophy or pulmonary hypertension, which preoperative testing could detect. Our objective is to determine if there is a severity of pediatric OSAS where previously undetected cardiopulmonary comorbidities are likely. Study Design: Retrospective chart review. Methods: We performed a retrospective review of 358 patients ≤21 years with severe OSAS who underwent adenotonsillectomy at a tertiary hospital June 1, 2016 to June 1, 2018. We extracted demographics, comorbidities, polysomnography, and preoperative tests. Wilcoxon rank‐sum and logistic regression estimated associations of OSAS severity (based on obstructive apnea‐hypopnea index [OAHI], hypoxia, hypercarbia) with preoperative echocardiograms and chest X‐rays (CXRs). Results: Mean age was 5.9 (±3.6) years and 52% were male. Mean OAHI and oxygen saturation nadir were 30.3 (±23.8) and 80.7% (±9.2), respectively. OAHI ≥60 was associated with having a preoperative echocardiogram (OR, 3.8; 95% CI, 1.7–8.5) or CXR (OR, 3.0; 95% CI, 1.4–6.8) compared to OAHI 10–59. There were no significant associations between OSAS severity and test abnormalities. The presence of previously diagnosed cardiopulmonary comorbidities was associated with abnormalities on echocardiogram (OR, 36; 95% CI, 4.1–320.1) and CXR (OR, 4.1; 95% CI, 1.2–14.4). Conclusions: Although pediatric patients with very severe OSAS (OAHI ≥60) underwent more pre‐adenotonsillectomy cardiopulmonary tests, OSAS severity did not predict abnormal findings. Known cardiopulmonary comorbidities may be a better indication for cardiopulmonary testing than polysomnographic parameters, which could streamline pre‐adenotonsillectomy evaluation and reduce cost. Level of Evidence: 4 Laryngoscope, 131:2361–2368, 2021 [ABSTRACT FROM AUTHOR]

Objectives: Prader‐Willi syndrome (PWS) increases the risk of obstructive sleep apnea (OSA) due to obesity, hypotonia, and abnormal ventilatory responses. We evaluated post‐adenotonsillectomy complications, polysomnography changes, and quality of life in children with OSA and PWS. Study Design: Systematic review and meta‐analysis. Methods: We conducted a systematic review and meta‐analysis by searching PubMed, Embase, Cochrane, Web of Science, and Scopus. Two researchers independently reviewed studies about adenotonsillectomy for OSA in patients <21 years with PWS. We extracted study design, patient numbers, age, complications, polysomnography, and quality of life. We pooled postoperative changes in apnea hypopnea index (AHI) for meta‐analysis. We applied Methodological Index for Nonrandomized Studies (MINORS) criteria to assess study quality. Results: The initial search yielded 169 studies. We included 68 patients from eight studies with moderate to high risk of bias. Six studies reported on complications and 12 of 51 patients (24%) had at least one. Velopharyngeal insufficiency was the most commonly reported complication (7/51, 14%). We included seven studies in meta‐analysis. Mean postoperative improvement in AHI was 7.7 (95% CI: 4.9–10.5). Postoperatively 20% (95% CI: 3%–43%) had resolution of OSA with AHI < 1.5 while 67% (95% CI: 50%–82%) had improvement from severe/moderate OSA to mild/resolved (AHI < 5). Two studies evaluated quality of life and demonstrated improvement. Conclusions: Children with PWS undergoing adenotonsillectomy for OSA have a substantial risk of postoperative complications that may require additional interventions, especially velopharyngeal insufficiency. Despite improvements in polysomnography and quality of life, many patients had residual OSA. This information can be used to counsel families when considering OSA treatment options. Laryngoscope, 131:898–906, 2021 [ABSTRACT FROM AUTHOR]

Objective: Rare disease Background: Isolated systemic arterial supply to normal (unsequestered) lung (ISSNL) without associated pulmonary malformation is rare, and lies towards the milder end of the spectrum of congenital lung abnormalities. Aneurysmal dilatation of the anomalous artery is an infrequent complication, with only 5 published cases thus far. Case Report: We present the case of a 61-year-old man whose screening chest radiograph showed a retrocardiac mass. Further evaluation with axial imaging demonstrated an ISSNL, complicated by aneurysmal dilatation. The genesis of this condition has been postulated to be due to persistence of primitive aortic branches to the developing lung bud. Initially reported in 1777, this entity is now more accurately classified within the spectrum of pulmonary and bronchovascular abnormalities, with refinement of the latter. The origin of an aberrant artery from the aorta implies that a higher-pressure systemic circulation is being shunted into a lower-pressure pulmonary circulation. While these supplying arteries are known to be large, aneurysmal dilation is exceptionally rare. Here, we review the cases published in the literature and present a case of our own. We aim to describe its pathogenesis, and touch on the classification systems and management. Conclusions: ISSNL is usually first suspected on a screening chest radiograph, as many patients are asymptomatic. Based on contrast-enhanced axial imaging, the diagnosis can be established non-invasively. Definitive management includes surgical and endovascular techniques. [ABSTRACT FROM AUTHOR]

Aim: Oesophageal atresia is frequently associated with other malformations, and our aim was to use computed tomography (CT) to explore intrathoracic malformations in patients with this condition.Method: This was retrospective study of children aged 0-16 with oesophageal atresia who were born in 1996-2013 and followed up at the French reference centre for rare oesophageal diseases at the University of Lille. Computed tomography scans were available for 48 of the 234 patients during follow-up visits, and these were reviewed by a thoracic radiologist.Results: More than two-thirds of the scans were performed to explore persistent respiratory symptoms. We found that six patients had a pulmonary malformations: four lobar agenesis, one right pulmonary aplasia and one congenital cystic adenomatoid malformation. Computed tomography enabled us to diagnose unexpected thoracic malformations in 16 patients: four lobar agenesis, six arteria lusoria, five persistent left superior vena cava and one partial anomalous pulmonary venous return. It also confirmed the diagnoses of suspected malformations in five patients: one congenital cystic adenomatoid malformation, one pulmonary hypoplasia, two right-sided aortic arches and one communicating bronchopulmonary foregut malformation.Conclusion: Intrathoracic anomalies were frequently associated with oesophageal atresia, and contrast-enhanced chest CT scans should be performed on patients with persistent respiratory symptoms. [ABSTRACT FROM AUTHOR]

A foregut duplication cyst occurring together with both a congenital cystic pulmonary airway malformation and extralobar pulmonary sequestration is an unusual combination. Prenatal ultrasound, MRI, and postnatal CT are helpful for operative planning. Surgical resection is the definitive management for all three anomalies. [ABSTRACT FROM AUTHOR]

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Bronchopulmonary sequestration is a rare congenital pulmonary abnormality of the lower airways, which includes an abnormal and non-functioning lung tissue not communicating with the tracheobronchial tree and having aberrant blood supply from systemic circulation with variable venous drainage. The incidence of sequestration is around 0.15%–6.4% of all congenital lung malformations.Common presenting features are cough and expectoration. Misdiagnosed cases may present with recurrent infections and haemoptysis. CT of the chest with contrast is the imaging modality of choice.This is a case report of a 32-year- old woman who presented with cough and haemoptysis. CT of the chest showed a multiloculated mass-like lesion in the left lower lobe with a feeding artery from coeliac plexus and venous drainage via the normal left pulmonary vein.Based on CT chest findings, diagnosis of intralobar pulmonary sequestration was made. The patient was reviewed by cardiothoracic surgeons and underwent surgical resection of the sequestrated lung. Common presenting features are cough and expectoration. Misdiagnosed cases may present with recurrent infections and haemoptysis. CT of the chest with contrast is the imaging modality of choice. [ABSTRACT FROM AUTHOR]

Rationale: Disruption of normal pulmonary development is a leading cause of morbidity and mortality in infants. Congenital lung malformations are a unique model to study the molecular pathogenesis of isolated structural birth defects, as they are often surgically resected.Objectives: To provide insight into the molecular pathogenesis of congenital lung malformations through analysis of cell-type and gene expression changes in these lesions.Methods: Clinical data, and lung tissue for DNA, RNA, and histology, were obtained from 58 infants undergoing surgical resection of a congenital lung lesion. Transcriptome-wide gene expression analysis was performed on paired affected and unaffected samples from a subset of infants (n = 14). A three-dimensional organoid culture model was used to assess isolated congenital lung malformation epithelium (n = 3).Measurements and Main Results: Congenital lung lesions express higher levels of airway epithelial related genes, and dysregulated expression of genes related to the Ras and PI3K-AKT-mTOR (phosphatidylinositol 3-kinase-AKT-mammalian target of rapamycin) signaling pathways. Immunofluorescence confirmed differentiated airway epithelial cell types throughout all major subtypes of congenital lung lesions, and three-dimensional cell culture demonstrated a cell-autonomous defect in the epithelium of these lesions.Conclusions: This study provides the first comprehensive analysis of the congenital lung malformation transcriptome and suggests that disruptions in Ras or PI3K-AKT-mTOR signaling may contribute to the pathology through an epithelial cell-autonomous defect. [ABSTRACT FROM AUTHOR]

We present an unusual case of an 18-day-old term neonate with coexistent bilateral bronchopulmonary vascular malformations and right isomerism. This case highlights the importance of computed tomography angiography in depicting such complex anomalies and classifying them according to components involved providing a systematic approach for evaluation of the disease process., (© 2021 Wiley Periodicals LLC.)