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The term congenital lung malformation (CLM) is used to describe a wide range of pathological conditions with different imaging and clinical manifestations. These anomalies stem from abnormal embryological lung development, potentially occurring across various stages of prenatal life. Their natural history can be variable, presenting in a wide range of severity levels and encompassing asymptomatic individuals who remain so until adulthood, as well as those who experience respiratory distress in the neonatal period. Through the PubMed database, we performed an extensive review of the literature in the fields of congenital lung abnormalities, including their diagnostic approach and findings. From our RIS-PACS database, we have selected cases with a final diagnosis of congenital lung malformation. Different diagnostic approaches have been selected, including clinical cases studied using plain radiograph, CT scan, prenatal ultrasound, and MR images. The most encountered anomalies can be classified into three categories: bronchopulmonary anomalies (congenital pulmonary airway malformations (CPAMs), congenital lobar hyperinflation, bronchial atresia, and bronchogenic cysts), vascular anomalies (arteriovenous malformation), and combined lung and vascular anomalies (scimitar syndrome and bronchopulmonary sequestration). CLM causes significant morbidity and mortality; therefore, the recognition of these abnormalities is necessary for optimal prenatal counseling and early peri- and postnatal management. This pictorial review aims to report relevant imaging findings in order to offer some clues for differential diagnosis both for radiologists and pediatric consultants. [ABSTRACT FROM AUTHOR]

Holt-Oram syndrome comprises a rare spectrum of congenital cardiovascular and appendicular skeletal anomalies. However, only a few cases have reported lung involvement in Holt-Oram syndrome. We reported the rare case of a 1-year-old male child patient who presented with upper limb abnormalities and respiratory distress and was diagnosed with pulmonary agenesis and pulmonary arterial hypertension secondary to an atrial septal defect. [ABSTRACT FROM AUTHOR]

Background: Structured light plethysmography (SLP) is a novel light-based method that captures chest wall movements to evaluate tidal breathing. Methods: Thirty-two children who underwent lung surgery were enrolled. Their clinical history was collected along with spirometry and SLP. Results: Median age of surgery was 9 months (interquartile range 4–30). Most frequent diagnosis was congenital pulmonary airway malformation (14/32), then pulmonary sequestration (9/32), tumor (5/32), and bronchogenic cyst (4/32). The most frequent surgical approach was lobectomy (59%), segmentectomy (38%), and complete resection (3%). More than 80% had surgery when younger than 3 years of age. Eight patients had short-term complications (pleural effusion was the most frequent), while long-term effects were reported in 15 patients (19% recurrent cough, 13% thoracic deformities, 13% airway infections, 9% wheezing, 6% reduced exercise tolerance, and 3% columnar deformities). Spirometry was normal in 9/22 patients. Nine patients had a restrictive pattern, while 4 showed a mild bronco-reactivity. Ten patients did not perform spirometry because of young age. SLP revealed the presence of obstructive pattern in 10% of patients (IE50 > 1.88) and showed a significant difference between the two hemithorax in 29% of patients. Discussion: SLP may be a new method to evaluate lung function, without collaboration and radiation exposure, in children who underwent lung resection, also in preschool age. [ABSTRACT FROM AUTHOR]

Tracheal agenesis (TA) is a rare congenital anomaly with an incidence of 1 per 50,000 newborns. It appears at birth with severe respiratory distress, cyanosis, and inaudible crying. Prompt esophageal intubation and long-term management of the esophageal airway are essential to overcome this catastrophic condition. In the long-term management, external stenting of the esophageal airway has been reported as promising to support the fragile esophageal wall; this technique was taken from the surgery for tracheomalacia. We experienced a case of an infant with tracheal agenesis whose respiratory status was stabilized after external esophageal stenting. The stenting was performed based on a lesson learned in the extensive experience in the surgical treatment for tracheomalacia, and the surgical techniques for successful stenting are herein described. [ABSTRACT FROM AUTHOR]

Background: Most congenital pulmonary airway malformations (CPAMs) are detected antenatally. The majority of newborns are asymptomatic. Patients are prone to subsequent respiratory complications and to a lesser extent malignant transformation remains concerning. In Israel, until 2013, pediatric surgeries were performed by thoracotomy. To minimize its morbidity, we introduced thoracoscopy using a mentorship approach. We present our experience with thoracoscopic resections coordinated by the mentorship of a pediatric worldwide leader in his field and compare our results with resections performed by thoracotomy. Materials and Methods: A retrospective review of records of children operated between 2013 and 2020 was conducted. Data were compared using t-test for quantitative variables. Results: Fifty patients were operated by thoracoscopy with a median age of 4 years, a thoracoscopic lobectomy performed in 68%. There was no conversion with a median length of stay (LOS) of 3½ days. Thirty patients were operated by thoracotomy by a thoracic surgeon with a median age of 3.5 years. A lobectomy was performed in 87% with a median LOS of 7 days. Conclusions: Thoracoscopic lobectomy is a technically demanding procedure with a long learning curve, strongly related to the low volume of cases. The role of a mentorship program in acquiring those surgical skills is crucial through standardization of the technique applied and supervised by the mentor. Early thoracoscopy for congenital pulmonary lesions at an early age can be achieved with a low conversion rate and minimal complications creating a change in the paradigm of practice when considering surgery for CPAM in Israel. [ABSTRACT FROM AUTHOR]

Pulmonary sequestration (PS) is a rare congenital pulmonary dysplasia which presents with recurrent chest infections and haemoptysis. Its diagnosis is difficult and suspected on contrast-enhanced chest CT. The definitive treatment of pulmonary sequestration is surgical resection or endoluminal occlusion of the abnormal feeding vessel. Here, we report an innovative technique in which per-operative indocyanine green guided excision of intra-lobar sequestrated lung parenchyma and ligation of abnormal feeding vessel. The first patient was a 44-year-old gentleman with recurrent bouts of cough for 2 years. Courses of antibiotics failed to resolve it. Fibreoptic bronchoscopy and routine blood tests did not show any abnormality. CECT chest showed multiloculated cystic solid and low density mass in the right lower lung. The area of the lesion received its blood supply from anomalous systemic artery originating from coeliac trunk. Intraoperative, indocyanine green was injected into the atypical vessel, and pulmonary sequestration turned green. A sequential atypical lung resection could be completely removed with parenchyma preservation. The second patient was a 53-year-old lady presenting with chest pain, cough and expectoration for 2 years not responding to general management and antibiotics. CECT showed a low-density mass in the right lower lung lobe, with a feeding arterial vessel arising from the abdominal aorta. This was diagnosed as PS and resected using per-operative indocyanine green with parenchymal preservation and ligation of feeding vessel. Both patients have been followed up for at least 1 year and are symptom free. The intraoperative use of indocyanine green could greatly facilitate the presentation of the extent of a lung sequestration and safe resection. [ABSTRACT FROM AUTHOR]

Background: Cavitary lung lesions are frequent findings on imaging, with the most common sources being malignancies and infections. They have multiple etiologies and differential diagnoses, which can have overlapping imaging characteristics, posing a diagnostic difficulty. This article is an educational pictorial essay highlighting the pitfalls and differential diagnoses of lung cavities, and focusing on the typical imaging patterns, the clinical and biological contexts of each etiology, illustrated by images that were extracted from the images archiving system of our radiology department. Short conclusion: The radiologist should be aware of all etiologies of cavitary lung lesions, including the less frequent ones, and be familiar with their imaging patterns and characteristics, which aids in establishing the diagnosis or, at the very least, narrowing down the evoked diagnoses. [ABSTRACT FROM AUTHOR]

Background: A pulmonary sequestration (PS) is an area of bronchopulmonary tissue with aberrant arterial supply. Transcatheter occlusion of PSs is an appealing treatment option, but data on outcomes remain scarce. We aim to describe our experience with transcatheter management of PS in infants and children. Methods: Retrospective review of clinical data of all patients with suspected PS sent for diagnostic and/or interventional cardiac catheterization at our institution between January 1999 and May 2021. Procedural considerations, techniques, standard safety, and outcomes were assessed. Results: We identified 71 patients (52.1% males), with median age and weight of 4.9 months (IQR, 2.1–26.6) and 4.2 kg (IQR, 3.9–12.1), respectively. Sixty-one (86%) patients had associated congenital heart defects (CHDs). Forty-two (59%) patients had pulmonary arterial hypertension (PAH) at the time of diagnosis. Fifty-three (74.7%) patients underwent embolization of the PS feeding vessel using microcoils and/or vascular plugs, and eight (15.1%) of these were neonates who presented with severe PAH and cardiac failure. Two patients had large feeding vessels and were treated surgically. Sixteen (22.5%) patients with small feeding vessels received conservative management. At median follow-up of 36.4 months (IQR, 2.1–89.9), seven patients had died, 24 patients had CHD corrective surgeries, 26 patients had redo catheterizations, and five patients had persistent PAH. No PS surgical resection was needed, and no infection of the remaining lung tissue occurred. Conclusions: Transcatheter assessment and treatment of PSs is a safe and effective procedure. Neonates with large PSs are severely symptomatic and improve remarkably after PS closure. PS embolization and surgical repair of associated CHDs generally leads to the normalization of pulmonary pressures. [ABSTRACT FROM AUTHOR]

Aim: A variety of developmental abnormalities of the tracheobronchial tree and pulmonary parenchyma are found in the newborn. There is limited data available on their presentation and clinical course from third world countries. Materials and Methods: A retrospective review of the medical records of children with congenital lung malformations was conducted at our tertiary care hospital from June, 2014 to June, 2018. Results: This study included 30 patients with 12 males and 18 females. The mean age at diagnosis was 13 months (range 1 month to 4 years). Congenital pulmonary airway malformation was the most common malformation present in 18 (60%) patients, followed by congenital lobar emphysema in 4 (13.3%), bronchogenic cyst in 3 (10%), bronchopulmonary sequestration in 2 (6.7%), bronchial atresia in 2 (6.7%) and pleuropulmonary blastoma in 1 (3.3%) patient. The most common presentation was recurrent upper respiratory tract infection (53.3%). Lobectomy was the most common surgical procedure performed in 86.6% of the patients, followed by excision of cyst in 10% of the patients. The post-operative complication rate was 30% with an overall survival rate of 93.3%. The average follow-up duration was 9 months. Conclusion: The predominant lung malformation in this study was congenital pulmonary airway malformation. Recurrent respiratory tract infection with or without respiratory distress was the most common mode of presentation. Health education to raise awareness and emphasis on antenatal ultrasonography can avoid inordinate delays in diagnosis and treatment. Surgery is curative with good long-term outcomes. [ABSTRACT FROM AUTHOR]

Purpose: Developmental dysplasia of the hip (DDH) encompasses a wide range of abnormal hip development and is a common condition in the pediatric population. Congenital pulmonary abnormalities are typically mild in the pediatric population but can be associated with severe comorbid conditions. The purpose of this study was to analyze the effect of structural pulmonary/airway abnormalities on the incidence of postoperative complications following surgical management of DDH. Methods: From 2012 to 2019, the National Surgical Quality Improvement Program-Pediatric database was utilized to identify pediatric patients undergoing surgical treatment for hip dysplasia. Patients were stratified into two groups: patients with a structural pulmonary/airway abnormality and patients without a pulmonary abnormality. Patient demographics, comorbidities, and postoperative complications were compared between the two cohorts with the use of various statistical analyses, including bivariate and multivariate analyses. Results: Of the 10,853 patients who underwent surgical treatment for hip dysplasia, 10,157 patients (93.6%) did not have a structural pulmonary/airway abnormality whereas 696 (6.4%) had an airway abnormality. Following adjustment on multivariate analysis, patients with a structural pulmonary abnormality had an increased risk of cardiac arrest requiring cardiopulmonary resuscitation (OR 2.342; p = 0.045). Conclusion: The results indicated that patients with a structural pulmonary abnormality had an increased risk of cardiac arrest requiring cardiopulmonary resuscitation compared to those without a pulmonary abnormality. Ensuring appropriate preoperative evaluation with a multidisciplinary team and close monitoring postoperatively is important to prevent the risk of severe outcomes in this vulnerable patient population. [ABSTRACT FROM AUTHOR]

Background: Congenital abdominal wall defects might be associated with other anomalies, such as atresia in gastroschisis and cardiac anomalies in omphalocele patients. However, in the current literature, an overview of these additional anomalies and potential patient-specific risk factors is missing. Therefore, we aimed to assess the prevalence of associated anomalies and their patient-specific risk factors in patients with gastroschisis and omphalocele. Methods: A mono-center retrospective cohort study between 1997 and 2023 was performed. Outcomes were the presence of any additional anomalies. Risk factors were analyzed via logistic regression analysis. Results: In total, 122 patients were included, of whom 82 (67.2%) had gastroschisis, and 40 (32.8%) had omphalocele. Additional anomalies were identified in 26 gastroschisis patients (31.7%) and in 27 omphalocele patients (67.5%). In patients with gastroschisis, intestinal anomalies were most identified (n = 13, 15.9%), whereas, in patients with omphalocele, cardiac anomalies were most identified (n = 15, 37.5%). Logistic regression showed that cardiac anomalies were associated with complex gastroschisis (OR: 8.5; CI-95%: 1.4–49.5). Conclusions: In patients with gastroschisis and omphalocele, intestinal and cardiac anomalies were most identified, respectively. Cardiac anomalies were found to be a risk factor for patients with complex gastroschisis. Therefore, regardless of the type of gastroschisis and/or omphalocele, postnatal cardiac screening remains important. [ABSTRACT FROM AUTHOR]

Simple Summary: Interstitial lung disease (ILD) is a life-threatening toxicity caused by chemoradiotherapy and durvalumab; however, the risk factors for ILD during durvalumab treatment after chemoradiotherapy have not been established in non-small-cell lung cancer patients. We examined whether interstitial lung abnormalities (ILAs) could be risk factors for ≥grade-two ILD during durvalumab treatment. The prevalence of ILAs in 148 patients before durvalumab treatment was 37.8%, and the multivariate analysis revealed ILAs as independent risk factors for ≥grade-two ILD. Attention should be paid to the development of ILD during durvalumab treatment in patients with ILAs. Introduction/Background: Chemoradiotherapy (CRT) followed by durvalumab, an immune checkpoint inhibitor, is the standard treatment for locally advanced non-small-cell lung cancer (NSCLC). Interstitial lung disease (ILD) is a life-threatening toxicity caused by these treatments; however, risk factors for the ILD have not yet been established. Interstitial lung abnormalities (ILAs) are computed tomography (CT) findings which manifest as minor interstitial shadows. We aimed to investigate whether ILAs could be risk factors for grade-two or higher ILD during durvalumab therapy. Patients and Methods: Patients with NSCLC who received durvalumab after CRT from July 2018 to June 2021 were retrospectively enrolled. We obtained patient characteristics, laboratory data, radiotherapeutic parameters, and chest CT findings before durvalumab therapy. Results: A total of 148 patients were enrolled. The prevalence of ILAs before durvalumab treatment was 37.8%. Among 148 patients, 63.5% developed ILD during durvalumab therapy. The proportion of patients with grade-two or higher ILD was 33.8%. The univariate logistic regression analysis revealed that older age, high dose-volume histogram parameters, and the presence of ILAs were significant risk factors for grade-two or higher ILD. The multivariate analysis showed that ILAs were independent risk factors for grade-two or higher ILD (odds ratio, 3.70; 95% confidence interval, 1.69–7.72; p < 0.001). Conclusions: We showed that pre-existing ILAs are risk factors for ILD during durvalumab treatment after CRT. We should pay attention to the development of grade-two or higher ILD during durvalumab treatment in patients with ILAs. [ABSTRACT FROM AUTHOR]

Background: Bronchogenic cysts (BCs) refer to congenital lesions that result from primitive or abnormal foregut budding, and can be pulmonary or mediastinal. Their occurrence can take place at any point on the tracheobronchial tree, but they are usually localized in the lung parenchyma and mediastinum, and may be symptomatic or asymptomatic. Bronchogenic cyst symptoms can vary, depending on the size and location of the cyst. Methods: A retrospective review of the charts of five patients with a histopathological diagnosis of bronchogenic cysts was performed between 2014 and 2020. The patients reported in this study were diagnosed and managed at Abha Maternity and Children Hospital, Abha, southwest Saudi Arabia. In addition, demographic information, as well as diagnostic and therapeutic information, was provided for each patient, both at discharge and after discharge. All patients had confirmed congenital bronchogenic cysts with different clinical phenotypes and radiological findings. Results: All patients had histopathologically confirmed bronchogenic cysts with different clinical and radiological presentations. Two patients had mediastinal-located cysts; one had a laryngeal cyst; and the last two patients had infected intrapulmonary bronchogenic cysts. All patients underwent complete excision and did not experience recurrence or other postoperative complications during the follow-up period. The latter two patients required lobectomies of the right middle and upper lobes. Conclusions: Although bronchogenic cysts are considered a rare congenital pulmonary malformation, they should be considered in the differential diagnosis of pediatric patients with unusual airway and parenchymal lung manifestations, particularly, persistent stridor, feeding difficulty, and complicated pneumonia. Surgical excision of the cyst is the gold-standard therapy for symptomatic bronchogenic cysts and is highly recommended for asymptomatic ones. Long-term follow-up studies will be required to explore any long-term complications of BCs, particularly regarding the malignancy transformation. [ABSTRACT FROM AUTHOR]

Rationale: Primary ciliary dyskinesia (PCD), an inherited lung disease, is characterized by abnormal ciliary function leading to progressive bronchiectasis. There is wide variability in respiratory disease severity at birth and later in life. Objectives: To evaluate the association between neonatal hospital length of stay (neonatal-LOS) and supplemental oxygen duration (SuppO2) with lung function in pediatric PCD. We hypothesized that longer neonatal-LOS and SuppO2 are associated with worse lung function (i.e., forced expiratory volume in 1 second percent predicted [FEV1pp]). Methods: We performed a secondary analysis of the Genetic Disorders of Mucociliary Clearance Consortium prospective longitudinal multicenter cohort study. Participants enrolled, during 2006-2011, were <19 years old with a confirmed PCD diagnosis and followed annually for 5 years. The exposure variables were neonatal-LOS and SuppO2, counted in days since birth. The outcome, FEV1pp, was measured annually by spirometry. The associations of neonatal-LOS and SuppO2 with FEV1pp were evaluated with a linear mixed-effects model with repeated measures and random intercepts, adjusted for age and ciliary ultrastructural defects. Results: Included were 123 participants (male, 47%; mean enrollment age, 8.3 yr [range, 0 to 18 yr]) with 578 visits (median follow-up, 5 yr). The median neonatal-LOS was 9 d (range, 1 to 90 d), and median SuppO2 was 5 d (range, 0 to 180 d). Neonatal-LOS was associated with worse lung function (-0.27 FEV1pp/d [95% confidence interval, -0.53 to -0.01]; P = 0.04). SuppO2 was not associated with lung function. Conclusions: Neonatal-LOS is associated with worse lung function in pediatric PCD, independent of age and ultrastructural defects. Future research on the mechanisms of neonatal respiratory distress and its management may help us understand the variability of lung health outcomes in PCD. [ABSTRACT FROM AUTHOR]

Rationale: Pulmonary hypoplasia secondary to congenital diaphragmatic hernia is characterized by reduced branching morphogenesis, which is responsible for poor clinical outcomes. Administration of amniotic fluid stem cell extracellular vesicles (AFSC-EVs) rescues branching morphogenesis in rodent fetal models of pulmonary hypoplasia. Herein, we hypothesized that AFSC-EVs exert their regenerative potential by affecting autophagy, a process required for normal lung development. Objectives: To evaluate autophagy in hypoplastic lungs throughout gestation and establish whether AFSC-EV administration improves branching morphogenesis through autophagy-mediated mechanisms. Methods: EVs were isolated from c-kit+ AFSC-conditioned medium by ultracentrifugation and characterized for size, morphology, and EV markers. Branching morphogenesis was inhibited in rat fetuses by nitrofen administration to dams and in human fetal lung explants by blocking RAC1 activity with NSC23766. The expression of autophagy activators (BECN1 and ATG5) and adaptor (SQSTM1/p62) was analyzed in vitro (rat and human fetal lung explants) and in vivo (rat fetal lungs). Mechanistic studies on rat fetal primary lung epithelial cells were conducted using inhibitors for microRNA-17 and -20a contained in the AFSC-EV cargo and known to regulate autophagy. Measurements and Main Results: Rat and human models of fetal pulmonary hypoplasia showed reduced autophagy mainly at pseudoglandular and canalicular stages. AFSC-EV administration restored autophagy in both pulmonary hypoplasia models by transferring miR-17∼92 cluster members contained in the EV cargo. Conclusions: AFSC-EV treatment rescues branching morphogenesis partly by restoring autophagy through microRNA cargo transfer. This study enhances our understanding of pulmonary hypoplasia pathogenesis and creates new opportunities for fetal therapeutic intervention in congenital diaphragmatic hernia babies. [ABSTRACT FROM AUTHOR]

Rationale: Knowledge on biomarkers of interstitial lung disease is incomplete. Interstitial lung abnormalities (ILAs) are radiologic changes that may present in its early stages. Objectives: To uncover blood proteins associated with ILAs using large-scale proteomics methods. Methods: Data from two prospective cohort studies, the AGES-Reykjavik (Age, Gene/Environment Susceptibility-Reykjavik) study (N = 5,259) for biomarker discovery and the COPDGene (Genetic Epidemiology of COPD) study (N = 4,899) for replication, were used. Blood proteins were measured using DNA aptamers, targeting more than 4,700 protein analytes. The association of proteins with ILAs and ILA progression was assessed with regression modeling, as were associations with genetic risk factors. Adaptive Least Absolute Shrinkage and Selection Operator models were applied to bootstrap data samples to discover sets of proteins predictive of ILAs and their progression. Measurements and Main Results: Of 287 associations, SFTPB (surfactant protein B) (odds ratio [OR], 3.71 [95% confidence interval (CI), 3.20-4.30]; P = 4.28 × 10-67), SCGB3A1 (Secretoglobin family 3A member 1) (OR, 2.43 [95% CI, 2.13-2.77]; P = 8.01 × 10-40), and WFDC2 (WAP four-disulfide core domain protein 2) (OR, 2.42 [95% CI, 2.11-2.78]; P = 4.01 × 10-36) were most significantly associated with ILA in AGES-Reykjavik and were replicated in COPDGene. In AGES-Reykjavik, concentrations of SFTPB were associated with the rs35705950 MUC5B (mucin 5B) promoter polymorphism, and SFTPB and WFDC2 had the strongest associations with ILA progression. Multivariate models of ILAs in AGES-Reykjavik, ILAs in COPDGene, and ILA progression in AGES-Reykjavik had validated areas under the receiver operating characteristic curve of 0.880, 0.826, and 0.824, respectively. Conclusions: Novel, replicated associations of ILA, its progression, and genetic risk factors with numerous blood proteins are demonstrated as well as machine-learning-based models with favorable predictive potential. Several proteins are revealed as potential markers of early fibrotic lung disease. [ABSTRACT FROM AUTHOR]

Rationale: Primary ciliary dyskinesia (PCD) is a heterogeneous, multisystem disorder characterized by defective ciliary beating. Diagnostic guidelines of the American Thoracic Society and European Respiratory Society recommend measurement of nasal nitric oxide (nNO) for PCD diagnosis. Several studies demonstrated low nNO production rates in PCD individuals, but underlying causes remain elusive. Objectives: To determine nNO production rates in a well-characterized PCD cohort, including subgroup analyses with regard to ultrastructural and ciliary beating phenotypes. Methods: This study included 301 individuals assessed according to European Respiratory Society guidelines. Diagnostic cutoffs for nNO production rates for this study cohort and subgroups with normal and abnormal ultrastructure were determined. Diagnostic accuracy was also tested for the widely used 77 nl/min cutoff in this study cohort. The relationship between nNO production rates and ciliary beat frequencies (CBFs) was evaluated. Results: The study cohort comprised 180 individuals with definite PCD diagnosis, including 160 individuals with genetic diagnosis, 16 individuals with probable PCD diagnosis, and 105 disease controls. The 77 nl/min nNO cutoff showed a test sensitivity of 0.92 and specificity of 0.86. Test sensitivity was lower (0.85) in the subgroup of 47 PCD individuals with normal ultrastructure compared with 133 PCD individuals with abnormal ultrastructure (0.95). The optimal diagnostic cutoff for the nNO production rate for the whole study cohort was 69.8 nl/min (sensitivity, 0.92; specificity, 0.89); however, it was 107.8 nl/min (sensitivity, 0.89; specificity, 0.78) for the subgroup of PCD with normal ultrastructure. PCD individuals with normal ultrastructure compared with abnormal ultrastructure showed higher ciliary motility. Consistently, PCD individuals with higher CBFs showed higher nNO production rates. In addition, laterality defects occurred less frequently in PCD with normal ultrastructure. Conclusions: Measurements of nNO below the widely used 77 nl/min cutoff are less sensitive in detecting PCD individuals with normal ultrastructure. Our findings indicate that higher nNO production in this subgroup with a higher cutoff for the nNO production rate (107.8 nl/min) and higher residual ciliary motility is dependent on the underlying molecular PCD defect. Higher nNO production rates, higher residual CBFs, and the lower prevalence of laterality defects hamper diagnosis of PCD with normal ultrastructure. Adjusting the cutoff of nNO production rate to 107.8 nl/min might promote diagnosing PCD with normal ultrastructure. [ABSTRACT FROM AUTHOR]

Rationale: Interstitial lung abnormalities (ILAs) are being increasingly identified in clinical practice. In particular, for subpleural nonfibrotic ILAs, the risk of progression over time and the risk factors for progressive behavior are still largely unknown. Objectives: To determine the age band prevalence of ILAs and the risk of radiological progression of subpleural nonfibrotic ILAs over time in a large health checkup population and to identify how reticulation contributes to the risk of radiological progression. Methods: On the basis of the ILAs definition by the Fleischner Society, low-dose chest computed tomography images from the community-dwelling population who have undergone health checkups were evaluated for ILAs. Multivariable logistic regression was used to assess the risk of radiological progression. Measurements and Main Results: Among 155,539 individuals, 3,300 (2.1%) were confirmed to have ILAs: the vast majority (81.7%) were defined as subpleural nonfibrotic ILAs. The prevalence of ILAs increased linearly with age (P for trend < 0.0001). Of 454 individuals with subpleural nonfibrotic ILAs, 198 (43.6%) had radiological progression over 4 years. The presence of reticulation on initial imaging was an independent predictor of radiological progression (odds ratio, 1.9; 95% confidence interval, 1.2-3.0; P = 0.0040). No difference in radiological progression was identified between subpleural nonfibrotic ILAs with extensive reticulation and subpleural fibrotic ILAs (73.0% vs. 68.8%; P = 0.7626). Conclusions: The prevalence of ILAs increases linearly with age. Nearly half of subpleural nonfibrotic ILAs progress radiologically over 4 years. The presence of reticulation is a risk factor for radiological progression. Subpleural nonfibrotic ILAs with extensive reticulation are likely to be a feature of subpleural fibrotic ILAs. [ABSTRACT FROM AUTHOR]

(1) Background: Lung abscess is a lung infection that leads to the destruction of the lung parenchyma, resulting in a cavity formation and central necrosis filled with purulent fluids. It is an uncommon pediatric problem, and there is a paucity of literature reviews on this subject, especially for the pediatric age group. Lung abscess is commonly divided into those considered primary in previously well children or secondary in those with predisposing co-morbidities. The predominant pathogens isolated from primary lung abscesses are the aerobic organisms, including streptococcal species, Staphylococcus aureus, and Klebsiella pneumoniae, while anaerobic bacteria such as Bacteroides species are predominant in secondary groups. Children usually present with fever, cough, shortness of breath, chest pain, and sputum. While physical examination may reveal diffuse crackles on auscultation, the diagnosis is usually confirmed by chest X-ray. (2) Methods: We report four different cases with lung abscesses from both primary and secondary group with similar presentations and radiological findings, but the approach was different in each according to the underlining cause. (3) Conclusions: Conservative therapies with a prolonged course of antibiotics remain the cornerstone of therapy for both primary and secondary lung abscesses. The underlying cause should be considered when there is a suboptimal response. However, invasive intervention is becoming more popular with better yield, shorter duration of antibiotics and admission, and excellent prognosis. [ABSTRACT FROM AUTHOR]

Objective: To retrospectively profile the ED usage for a cohort of adults with cerebral palsy (CP). Methods: Five years of ED data from a Victorian hospital network was analysed to identify participants with CP using the Victorian Emergency Minimum Dataset supplemented with scrutiny of inpatient admission data to identify cases because of limited ED coding of CP. Presentation frequency, emergency diagnoses (International Classification of Diseases, 10th Revision codes) and presentation sequelae were calculated and described. An investigation into rates of low urgency presentations was conducted. Differences between adult and paediatric cohorts were described. Results: Participants with CP constituted 1586 ED presentations. Adults represented 43% (n = 689) of these. Thirty percent of adults presented more than five times over the study period, with respiratory (25%), gastrointestinal (17%) and epilepsy/convulsion diagnoses (11%) being the most common presentations. Rates of inpatient hospital admissions from the ED increased with age in adults (P < 0.001). Low urgency presentations made up 8.9% of total adult presentations. Conclusions: The high rates of respiratory diagnoses and epilepsy/convulsions, both ambulatory care‐sensitive conditions, may be indicative of transitional challenges between paediatric and adult healthcare, potentially highlighting difficulties in accessing primary care services. Relatively low rates of 'low urgency' presentations may suggest perceived medical fragility in this vulnerable population. People with CP who present to ED and were not admitted may be underrepresented in this data. National expansion of this research will aid the development of an evidence‐based model of care for CP in Australia. [ABSTRACT FROM AUTHOR]

Objective: Primary ciliary dyskinesia (PCD) is a rare hereditary disease. Most reports of PCD in Japan are case reports, and clinical analysis has not been performed. Differences in the causative genes might affect the clinical features in different ethnic groups. The purpose of this study was to clarify the clinical features of Japanese patients with PCD.Methods: We performed a retrospective chart review of PCD patients seen at Mie University Hospital and patients whose blood samples were sent to us for genetic analysis from 2011 to 2020. Data on the following items were collected and analyzed: age at first visit to the hospital, age at diagnosis of PCD, process of referral to our facility, chief complaint, situs status, PrImary CiliARy DyskinesiA Rule (PICADAR) score, nasal nitric oxide concentration, otoscopic findings, rhinoscopic findings, and paranasal computed tomography scan findings.Results: Sixty-seven patients (24 male, 43 female) were diagnosed with PCD during the study period. Age at diagnosis ranged from 2 months to 69 years (median, 17 years). Respiratory symptoms (77%) were the most common complaint, followed by nasal (15%) and aural (8%) symptoms. Situs inversus was present in 17 (25%) cases. Only 2 cases had congenital cardiac anomalies. The mean PICADAR score was 7.3 (range, 3-14) points. Approximately 50% of tympanic membranes showed retraction, suggesting otitis media with effusion. The mean Lund-Mackay score was 12.8 (range, 7-17) points, suggesting that the radiographic findings were not always severe. There was no significant difference in the total Lund-Mackay score between patients with and without situs inversus (12.7 vs. 12.6, respectively).Conclusion: Situs inversus was present in 25% of Japanese PCD patients, which is much lower than observed in other countries. This is a result of differences in the major disease-causing genes. The general rule that "situs inversus is observed in approximately 50% of PCD patients" cannot be applied, at least, in Japanese PCD patients. [ABSTRACT FROM AUTHOR]

Rationale: Higher blood monocyte counts are associated with worse survival in adults with clinically diagnosed pulmonary fibrosis. Their association with the development and progression of interstitial lung abnormalities (ILA) in humans is unknown. Objectives: We evaluated the associations of blood monocyte count, and other immune cell types, with ILA, high-attenuation areas, and FVC in four independent cohorts. Methods: We included participants with measured monocyte counts and computed tomographic (CT) imaging enrolled in MESA (Multi-Ethnic Study of Atherosclerosis, n = 484), AGES-Reykjavik (Age/Gene Environment Susceptibility Study, n = 3,547), COPDGene (Genetic Epidemiology of COPD, n = 2,719), and the ECLIPSE (Evaluation of COPD Longitudinally to Identify Predictive Surrogate End-points, n = 646). Measurements and Main Results: After adjustment for covariates, a 1-SD increment in blood monocyte count was associated with ILA in MESA (odds ratio [OR], 1.3; 95% confidence interval [CI], 1.0-1.8), AGES-Reykjavik (OR, 1.2; 95% CI, 1.1-1.3), COPDGene (OR, 1.3; 95% CI, 1.2-1.4), and ECLIPSE (OR, 1.2; 95% CI, 1.0-1.4). A higher monocyte count was associated with ILA progression over 5 years in AGES-Reykjavik (OR, 1.2; 95% CI, 1.0-1.3). Compared with participants without ILA, there was a higher percentage of activated monocytes among those with ILA in MESA. Higher monocyte count was associated with greater high-attenuation areas in MESA and lower FVC in MESA and COPDGene. Associations of other immune cell types were less consistent. Conclusions: Higher blood monocyte counts were associated with the presence and progression of interstitial lung abnormalities and lower FVC. [ABSTRACT FROM AUTHOR]

The article presents the case study of two cases of anomalous systemic arterial supply to the basal segment of the left lung in infants. It emphasizes the importance of balloon occlusion pulmonary angiography for accurate diagnosis and surgical planning. It is reported that the congenital anomaly involves abnormal systemic arterial branches supplying normal lung segments.

Congenital lung malformations are most often identified on prenatal US screening. Fetal MRI is often performed to further evaluate these lesions. Although some of these lesions might cause prenatal or early postnatal symptoms that require urgent management, the majority are asymptomatic at birth and might be subtle or invisible on chest radiographs. Postnatal imaging is frequently deferred until 3–6 months of age, when surgery or long-term conservative management is contemplated. High-quality imaging and interpretation is needed to assist with appropriate decision-making. Contrast-enhanced chest CT, typically with angiographic technique, has been the usual postnatal imaging choice. In this review, the author discusses and illustrates the indications and use of postnatal MR imaging for bronchopulmonary malformations as well as some differential diagnoses and the advantages and disadvantages of MR versus CT. [ABSTRACT FROM AUTHOR]

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Aim: The purpose of our study was to evaluate the diagnostic performance of fetal magnetic resonance imaging (MRI)-based texture analysis (TA) to differentiate the two most common lung malformations, congenital pulmonary airway malformation (CPAM) and pulmonary sequestration (PS). Materials and Methods: This retrospective single-center study included 24 patients with CPAM and 8 patients with PS who had a fetal MRI examination between January 2015 and December 2020. T2-weighted coronal images were used for TA. One reader designated the malformation borders and drew a region-of-interest for TA. The differences in values of the texture features between the groups were assessed and receiver operating characteristic curves were calculated for each statistically significant feature. P-value<0.05 was considered statistically significant. Results: Forty-eight texture features were calculated for each malformation. Twenty features on T2-weighted images were significantly different between the CPAMSs and PSs. Among these, short-run high gray-level emphasis and long-run emphasis, which are gray-level runlength matrix features parameters, had the largest area under the curves: 0.956 (sensitivity 87%, specificity 95%) and 0.943 (sensitivity 87%, specificity 85%), respectively. Conclusion: Our results suggest that fetal MRI-based TA may be used to distinguish CPAMs from PSs in fetuses with uncertain pulmonary findings prior to birth. [ABSTRACT FROM AUTHOR]

Background: Children with cystic fibrosis (CF) have recurrent lung infections and these exacerbation periods have conventionally been treated in hospital. Hospital in the Home (HITH) programs have recently been introduced but equivalence of care has not previously been established. Objectives: To determine if standardization of treatment (application and frequency) for children with CF during a pulmonary exacerbation would produce equivocal clinical outcomes (lung function and weight), regardless of whether treatment was received in hospital or HITH. Design and Participants: A retrospective audit was conducted on electronic medical records from 39 children with CF (6–17 years). Main Outcome measures: Forced expiratory volume in one second (FEV1), forced vital capacity (FVC), weight and length of stay (LOS) were compared between participants treated either in hospital or under the provision of HITH. Results: Care provided by HITH was found to be equivalent to hospital-based care (mean difference; 95% CI) for: FEV1 (0.067; −0.104, 0.238); FVC (0.051; −0.102, 0.204); weight (0.718; −0.251, 1.687); and LOS (−0.781, −2.505, 0.943). All investigated clinical measurements significantly improved (FEV1p =.001; FVC p <.001; and weight p <.001) from admission to discharge for both hospital and HITH participants. Conclusions: HITH appears comparable to hospital provision of care for children with CF during a pulmonary exacerbation in terms of post-treatment outcomes (FEV1, FVC, weight, and LOS). [ABSTRACT FROM AUTHOR]

Tracheobronchomegaly is a rare disease with congenital abnormal change in respiratory tract; its image features are also very special. In this case, we described a 57-year-old male with cough, expectoration, chest pain, and dyspnea. In our institution, the result of chest CT scan is highly extraordinary, which showed obvious dilation of the trachea and main bronchi, emphysema, and a number of pulmonary bullae, and there was a big bulla with air-fluid level on the lower lobe of the right lung. Fortunately, after wedge resection for the big bulla on the lower lobe of right lung under video-assisted thoracoscopic surgery, this patient's symptoms were significantly relieved. The clinical manifestations of tracheobronchomegaly lack specificity; this disease has freakish image features. At present, there are no effective treatments for tracheobronchomegaly, which just was an accidental discovery in this patient; we just mainly take surgical measures to treat the big bulla for relieving symptoms. [ABSTRACT FROM AUTHOR]

BACKGROUND AND OBJECTIVES: Respiratory diseases are common in children with esophageal atresia (EA), leading to increased morbidity and mortality in the first year. The primary study objective was to identify the factors associated with readmissions for respiratory causes in the first year in EA children. METHODS: A population-based study. We included all children born between 2008 and 2016 with available data and analyzed factors at birth and 1 year follow-up. Factors with a P value <.10 in univariate analyses were retained in logistic regression models. RESULTS: Among 1460 patients born with EA, 97 (7%) were deceased before the age of 1 year, and follow-up data were available for 1287 patients, who constituted our study population. EAs were Ladd classification type III or IV in 89%, preterm birth was observed in 38%, and associated malformations were observed in 52%. Collectively, 61% were readmitted after initial discharge in the first year, 31% for a respiratory cause. Among these, respiratory infections occurred in 64%, and 35% received a respiratory treatment. In logistic regression models, factors associated with readmission for a respiratory cause were recurrence of tracheoesophageal fistula, aortopexy, antireflux surgery, and tube feeding; factors associated with respiratory treatment were male sex and laryngeal cleft. CONCLUSIONS: Respiratory morbidity in the first year after EA repair is frequent, accounting for >50% of readmissions. Identifying high risk groups of EA patients (ie, those with chronic aspiration, anomalies of the respiratory tract, and need for tube feeding) may guide follow-up strategies. [ABSTRACT FROM AUTHOR]

Purpose: To examine the association between the MSCT quantitative measurements of congenital lung malformations (CLM) and the selection of surgical approaches (lobectomy vs. lung-sparing surgery).Methods: This retrospective study evaluated CLM surgical cases at our institution from 2016 to 2018. MSCT quantitative measurements were generated by a semi-automated approach: the volume of the lesion (Vlesion), the volume of the lesion-involved lobe (Vlobe), the volume of the lesion-involved lung (Vlung) and the volume of the total lung (Vtotal lung). The proportions of Vlesion to Vlobe (Plesion/lobe), Vlesion to Vlung (Plesion/lung), and Vlesion to V total lung (Plesion/total lung) were calculated. We used Logistics regression to examine whether quantitative measurements were associated with the selection of surgical approaches.Results: 151 patients were included (median age at surgery 6 months). 82 patients underwent lung-sparing surgery, and 69 patients underwent lobectomy. Vlesion (OR 1.51, 95% CI 1.09-2.07), Plesion/lobe (OR 1.78, 95% CI 1.16-2.72), Plesion/lung (OR 1.63, 95% CI 1.13-2.35), and Plesion/total lung (OR 1.58, 95% CI 1.12-2.22) were positively associated with the selection of lobectomy.Conclusion: The application of quantified MSCT analysis may provide insight into the quantitative characteristics of CLM, which could be potentially useful for surgical approach selection. [ABSTRACT FROM AUTHOR]

Background Long-term health sequelae of coronavirus disease 2019 (COVID-19) may be multiple but have thus far not been systematically studied. Methods All patients discharged after COVID-19 from the Radboud University Medical Center, Nijmegen, the Netherlands, were consecutively invited to a multidisciplinary outpatient facility. Also, nonadmitted patients with mild disease but with symptoms persisting >6 weeks could be referred by general practitioners. Patients underwent a standardized assessment including measurements of lung function, chest computed tomography (CT)/X-ray, 6-minute walking test, body composition, and questionnaires on mental, cognitive, health status, and quality of life (QoL). Results 124 patients (59 ± 14 years, 60% male) were included: 27 with mild, 51 with moderate, 26 with severe, and 20 with critical disease. Lung diffusion capacity was below the lower limit of normal in 42% of discharged patients. 99% of discharged patients had reduced ground-glass opacification on repeat CT imaging, and normal chest X-rays were found in 93% of patients with mild disease. Residual pulmonary parenchymal abnormalities were present in 91% of discharged patients and correlated with reduced lung diffusion capacity. Twenty-two percent had low exercise capacity, 19% low fat-free mass index, and problems in mental and/or cognitive function were found in 36% of patients. Health status was generally poor, particularly in the domains functional impairment (64%), fatigue (69%), and QoL (72%). Conclusions This comprehensive health assessment revealed severe problems in several health domains in a substantial number of ex–COVID-19 patients. Longer follow-up studies are warranted to elucidate natural trajectories and to find predictors of complicated long-term trajectories of recovery. [ABSTRACT FROM AUTHOR]

Primary ciliary dyskinesia (PCD) is an inherited cause of bronchiectasis. The estimated PCD prevalence in children with bronchiectasis is up to 26% and in adults with bronchiectasis is 1 to 13%. Due to dysfunction of the multiple motile cilia of the respiratory tract patients suffer from poor mucociliary clearance. Clinical manifestations are heterogeneous; however, a typical patient presents with chronic productive cough and rhinosinusitis from early life. Other symptoms reflect the multiple roles of motile cilia in other organs and can include otitis media and hearing loss, infertility, situs inversus, complex congenital heart disease, and more rarely other syndromic features such as hydrocephalus and retinitis pigmentosa. Awareness, identification, and diagnosis of a patient with PCD are important for multidisciplinary care and genetic counseling. Diagnosis can be pursued through a multitest pathway which includes the measurement of nasal nitric oxide, sampling the nasal epithelium to assess ciliary function and structure, and genotyping. Diagnosis is confirmed by the identification of a hallmark ultrastructural defect or pathogenic mutations in one of > 45 PCD causing genes. When a diagnosis is established management is centered around improving mucociliary clearance through physiotherapy and treatment of infection with antibiotics. The first international randomized controlled trial in PCD has recently been conducted showing azithromycin is effective in reducing exacerbations. It is likely that evidence-based PCD-specific management guidelines and therapies will be developed in the near future. This article examines prevalence, clinical features, diagnosis, and management of PCD highlighting recent advances in basic science and clinical care. [ABSTRACT FROM AUTHOR]

Background: Obesity is associated with restrictive ventilatory defects and a faster rate of decline in FVC. This association is not exclusively mediated by mechanical factors and may reflect direct pulmonary injury by adipose-derived mediators.Research Question: Is adipose tissue involved in the pathogenesis of interstitial lung disease (ILD)?Study Design and Methods: We evaluated the association of CT measures of pericardial, abdominal visceral, and abdominal subcutaneous adipose tissue with high-attenuation areas (HAAs) and interstitial lung abnormalities (ILAs) in a large multicenter cohort study of community-dwelling adults, using multivariable-adjusted models. We secondarily evaluated the association of adipose depot size with FVC and biomarkers of obesity and inflammation.Results: In fully adjusted models, every doubling in pericardial adipose tissue volume was associated with a 63.4-unit increase in HAA (95% CI, 55.5-71.3), 20% increased odds of ILA (95% CI, -2% to 50%), and a 5.5% decrease in percent predicted FVC (95% CI, -6.8% to -4.3%). IL-6 levels accounted for 8% of the association between pericardial adipose tissue and HAA. Every doubling in visceral adipose tissue area was associated with a 41.5-unit increase in HAA (95% CI, 28.3-54.7), 30% increased odds of ILA (95% CI, -10% to 80%), and a 5.4% decrease in percent predicted FVC (95% CI, -6.6% to -4.3%). IL-6 and leptin accounted for 17% and 18%, respectively, of the association between visceral adipose tissue and HAA.Interpretation: Greater amounts of pericardial and abdominal visceral adipose tissue were associated with CT measures of early lung injury and lower FVC in a cohort of community-dwelling adults. Adipose tissue may represent a modifiable risk factor for ILD. [ABSTRACT FROM AUTHOR]

Case Presentation: A 19-year-old woman presented to pulmonary clinic with recurrent episodes of fevers and productive cough over the last 2 years. She was diagnosed with several episodes of respiratory infection that required antibiotic therapy. Her symptoms improved transiently after antibiotic therapy. However, symptoms continued to recur every 1 to 2 months. She denied any close TB contacts or travel outside the United States. She was a nonsmoker and had no history of immunodeficiency. There was no history of cystic fibrosis or any foreign body aspiration. [ABSTRACT FROM AUTHOR]

Introduction: Nasal duplication is a rare congenital deformity with many subtypes including supernumerary nostril. The challenge of surgical correction is to achieve nasal symmetry and restore nasal airflow. However, there is no defined protocol for treatment, especially with regard to presurgical therapy. Methods: We performed a review of literature of studies reporting on patients with supernumerary nostril to complete this review. We then report on a patient with supernumerary nostril who was treated with nostril expansion therapy prior to surgical intervention. Results: We identified 59 cases of nostril duplication. Because of the rarity of the condition, treatment protocols varied greatly. For our patient, preoperative nasal appliance therapy was implemented for 3 months prior to surgical intervention. Patient was followed-up regularly for 1 year. Discussion: Although literature on nasal duplication is scarce, there is a general agreement that early intervention has psychological, anatomic, and functional benefits to the patient. In our case report, nostril expansion therapy was easy to implement and facilitated surgical reconstruction, resulting in aesthetic outcome and expanded airway 1 year postoperatively. [ABSTRACT FROM AUTHOR]

Kartagener syndrome (KS) is a rare autosomal recessive disease. The disease is characterized by three typical symptoms: chronic sinusitis, situs viscerum inversus (SVI), and bronchiectasis. The laparoscopic cholecystectomy (LC) is the standard procedure in most cases of cholelithiasis, but in SVI patients it can be difficult, especially for right-handed surgeons. We report the case of a 24-year-old female affected by KS, presenting with a history of symptomatic cholelithiasis. Ultrasound and magnetic resonance cholangiopancreatography confirmed SVI totalis and cholelithiasis. The patient underwent a laparoscopic cholecystectomy by a right-handed surgeon performed with a mixed approach without complications. Laparoscopic cholecystectomy in SVI patients can be a safe and reliable technique especially for a left-handed surgeon. The described technique is also easy for a right-handed surgeon. However, it is considered a technically challenging procedure and often requires technical modification. [ABSTRACT FROM AUTHOR]

Background: This study aimed to report a European multi-institutional experience about thoracoscopic management of children with congenital lung malformations. Methods: The records of 102 patients (49 girls and 53 boys) with median age at surgery of 1 year (range 6 months-1.5 years), who underwent thoracoscopic lobectomy in five European Pediatric Surgery units, were retrospectively collected. Indications for surgery included congenital pulmonary airway malformation (CPAM) (n = 47), intra- and extralobar pulmonary sequestration (n = 34), hybrid lesion (CPAM/intralobar sequestration) (n = 2), severe bronchiectasis (n = 9), congenital lobar emphysema (n = 8), and others (n = 2). The condition was asymptomatic in 77/102 (75.5%), whereas symptoms such as recurrent pneumonia and/or respiratory distress were present in 25/102 (24.5%). Results: Surgical procedures included 18 upper, 20 middle, and 64 lower lobe resections. No conversions to open were reported. A 3 mm sealing device and 5 mm stapler were adopted in the last 48/102 patients (47%). The median operative time was 92.2 minutes (range 74-141). The median operative time significantly decreased in patients in whom the vessel division and bronchial sealing were performed using sealing devices (75.5 minutes) compared with suture ligations (118.9 minutes) (P = .001). The median hospital stay was 3.7 days (range 2-6.2). Three/102 patients (2.9%) developed postoperative complications, including air leakage requiring pleural drainage (n = 1) (Clavien IIIb) and respiratory infection (n = 2) (Clavien II). A reoperation was required in one patient with residual pleuropulmonary blastoma (0.9%). All symptomatic patients reported resolution of symptoms postoperatively. Conclusions: Thoracoscopic lobectomy is a safe and effective procedure with excellent cosmetic outcome, in expert hands. Based upon our experience, we strongly recommend surgery in patients with congenital lung malformations by the first year of life, to reduce the risk of infection and make the procedure technically easier, despite the small patients' size. Surgeon's experience and use of miniaturized instruments and sealing devices remain key factors for successful outcome. [ABSTRACT FROM AUTHOR]

Objectives: To develop a clinical guideline for structured assessment and uniform reporting of congenital lung abnormalities (CLA) on Computed Tomography (CT)-scans.Materials and Methods: A systematic literature search was conducted for articles describing CT-scan abnormalities of congenital pulmonary airway malformation (CPAM), bronchopulmonary sequestration (BPS), congenital lobar emphysema (CLE) and bronchogenic cyst (BC). A structured report using objective features of CLA was developed after consensus between a pediatric pulmonologist, radiologist and surgeon.Results: Of 1581 articles identified, 158 remained after title-abstract screening by two independent reviewers. After assessing full-texts, we included 28 retrospective cohort-studies. Air-containing cysts and soft tissue masses are described in both CPAM and BPS while anomalous arterial blood supply is only found in BPS. Perilesional low-attenuation areas, atelectasis and mediastinal shift may be found in all aforementioned abnormalities and can also be seen in CLE as a cause of a hyperinflated lobe. We have developed a structured report, subdivided into five sections: Location & Extent, Airway, Lesion, Vascularization and Surrounding tissue.Conclusions: CT-imaging findings in CLA are broad and nomenclature is variable. Overlap is seen between and within abnormalities, possibly due to definitions often being based on pathological findings, which is an unsuitable approach for CT imaging. We propose a structured assessment of CLA using objective radiological features and uniform nomenclature to improve reporting. [ABSTRACT FROM AUTHOR]

Pulmonary sequestration (PS) is a rare congenital lung malformation. We present a case of newborn with antenatally diagnosed case of PS. The baby was delivered by cesarean section to primigravida mother at 38 + 3 weeks of gestation with birth weight of 2700 g. At 20 weeks of gestation, the antenatal ultrasound showed a triangular echogenic area in left lung supplied by feeding artery from descending aorta. The baby had respiratory distress soon after birth. Computerized tomographic pulmonary angiogram revealed abnormal blood supply of left lower lobe arising from descending aorta. The feeding vessel was abnormally large and was almost half of the diameter of the aorta. The baby underwent left lower lobectomy with double ligation of feeding artery at day 7 of life and was discharged on day 14 of life. Antenatal diagnosis of congenital lung malformation helps in careful planning of perinatal care and facilitates the early diagnosis and management. [ABSTRACT FROM AUTHOR]

Background: Congenital bronchial atresia is a rare pulmonary abnormality characterized by the disrupted communication between the central and the peripheral bronchus and is typically asymptomatic. Although it can be symptomatic especially when infections occur in the involved areas, fungal infections are rare complications in patients with bronchial atresia. We report a case of congenital bronchial atresia complicated by a fungal infection.Case Presentation: A 30-year-old man with no previous history of immune dysfunction was brought to a nearby hospital and diagnosed with a left lung abscess. Although antimicrobial treatment was administered, it was ineffective, and he was transferred to our hospital. Since diagnostic imaging findings and bronchoscopy suggested congenital bronchial atresia and a fungal infection, he was treated with voriconazole and surgical resection was subsequently performed. A tissue culture detected Aspergillus fumigatus and histopathological findings were compatible with bronchial atresia. After discharge, he remained well and voriconazole was discontinued 5 months after the initiation of therapy.Conclusion: Bronchial atresia is a rare disease that is seldom complicated by a fungal infection, which is also a rare complication; however, physicians should consider fungal infections in patients with bronchial atresia who present with infections resistant to antimicrobial treatment. [ABSTRACT FROM AUTHOR]

Objective: The objective of this study is to assess the effectiveness and safety of intrafetal vascular laser ablation (VLA) for fetuses with bronchopulmonary sequestration (BPS) with hydrops.Methods: First, we present 3 cases of fetuses with BPS and hydrops treated by VLA. Second, we aimed to conduct a narrative review to identify all reported cases of fetuses with BPS treated by intrafetal VLA.Results: The review of the literature identified 41 fetuses treated by VLA for BPS with hydrops. The median gestational age of the VLA was 27+0 weeks' gestation [25+0-31+0] with an associated procedure at the same time in 43% of the cases (pleuroamniotic shunt, thoracentesis, and amniodrainage). A second procedure was required in 25% of cases for residual flow in the feeding vessel. No stillbirth or neonatal death was reported. The complications reported were a fetal thoracic hematoma complicated by fetal anemia and 4 preterm deliveries with a rate of 9%.Conclusion: VLA of the feeding vessel can be an effective treatment but is not without complications. In cases demonstrating cardiac output failure, intrafetal VLA should be considered as a treatment for BPS. [ABSTRACT FROM AUTHOR]

BACKGROUND: Congenital diaphragmatic hernia is associated with a high risk of neonatal mortality and long-term morbidity due to lung hypoplasia, pulmonary hypertension, and prolonged exposure to positive-pressure ventilation. Ventilator-associated lung injury may be reduced by using approaches that facilitate the transition from invasive ventilation to noninvasive ventilation (NIV), such as with neurally-adjusted ventilatory assist (NAVA). We reported our use of NAVA in neonatal patients with congenital diaphragmatic hernia during the transition from invasive ventilation to NIV. METHODS: A retrospective analysis of neonatal subjects with congenital diaphragmatic hernia admitted to a tertiary care children's hospital between December 2015 and May 2018 was conducted. Subject data and factors that affected the use of NAVA were analyzed. RESULTS: Ten neonatal subjects with congenital diaphragmatic hernia were placed on NAVA, and 6 were successfully transitioned, after surgery, from pressure control synchronized intermittent mandatory ventilation to invasive ventilation with NAVA and then to NIV with NAVA without the need for re-intubation. The transition from pressure control synchronized intermittent mandatory ventilation to invasive ventilation with NAVA resulted in a decrease in peak inspiratory pressure, mean airway pressure, and FIO2. Barriers to the use of NAVA included symptomatic pleural effusion or chylothorax and pulmonary sequestration. CONCLUSIONS: Both invasive ventilation with NAVA and NIV with NAVA were used successfully in subjects with congenital diaphragmatic hernia during the transition from invasive ventilation to NIV. The transition to NAVA was associated with a decrease in peak inspiratory pressure, mean airway pressure, and the need for supplemental oxygen. A prospective trial is needed to determine the short- and long-term impacts of this mode of ventilation in neonates with congenital diaphragmatic hernia. [ABSTRACT FROM AUTHOR]

AUTH Why you should read this article: • To enhance your knowledge of the development of the respiratory system before and after birth • To understand the causes and management of various common respiratory conditions that infants and children may present with • To count towards revalidation as part of your 35 hours of CPD, or you may wish to write a reflective account (UK readers) • To contribute towards your professional development and local registration renewal requirements (non-UK readers) This article is the fifth in a series on the biological basis of child health. It describes the development of the respiratory system, which starts relatively late in the embryo and continues after birth until the age of seven to eight years. It explains what the developing anatomy of the respiratory system in infants and children means in terms of the conditions that may occur and the precautions required when assessing them. The article provides an overview of the elements of respiratory assessment in infants and children and describes some respiratory conditions seen in these patient groups. It also discusses some of the changes in the care of children with respiratory conditions, which has increasingly moved from hospital into the community and become nurse-led, multidisciplinary and holistic. [ABSTRACT FROM AUTHOR]

Background: The nomenclature of both intralobar pulmonary sequestration (ILS) and aortic origin of a pulmonary artery (AOPA) remains controversial. According to this review, both ILS and AOPA have an anomalous systemic arterial supply to all or part of the lung with venous drainage into the pulmonary veins, which leads to pulmonary hypertension, congestive heart failure, and fatal pulmonary haemorrhage. The purpose of this review was to consider whether these two rare congenital anomalies have similar anatomical, clinical and pathological characteristics.Methods: This review was conducted by researching relevant literature using PubMed and MEDLINE databases to January 2019. All researched literature was related to the anatomical, associated anomalies, pathophysiology and clinical features of the extralobar pulmonary sequestration (ELS), ILS, and AOPA, and the therapeutic method for ILS and AOPA.Results: Through research literature, it was found that ILS and AOPA may differ in terms of embryonic origin, but some of the anatomical, histopathological, physiological and clinical features of these two congenital malformations are similar. However, ELS and ILS have significant differences in their anatomical, histopathological, physiological, and clinical features.Conclusions: This study proposes that ILS and AOPA could be classified as one single condition - systemic arterialisation of the lung - and further divided into three subtypes, namely: types I, II and III. This new classification nomenclature permits the appropriate change of novel surgical techniques, which obviate the need for lobectomy or segmentectomy in specific cases, thereby minimising fatal postoperative complications. [ABSTRACT FROM AUTHOR]

The association between folic acid supplementation and birth defects other than neural tube defects (NTD) remains unclear. We used a log-binomial regression model to investigate if periconceptional folic acid and/or multivitamin use was associated with birth defects in Norway with prospectively collected data from the Medical Birth Registry of Norway (MBRN) during 1999–2013. We used the European Surveillance of Congenital Anomalies (EUROCAT) classification system to define eleven organ-specific major birth defect groups (nervous system, eye, ear–face–neck, cardiovascular system, respiratory system, oral clefts, digestive system, abdominal wall, urinary system, genital organs and limb), with additional subgroups. Fetuses or infants whose mothers used folic acid and/or multivitamin supplements before and during pregnancy were classified as exposed. During the years 1999–2013, 888 294 (99·0 %) live-born infants, 6633 (0·7 %) stillborn infants and 2135 (0·2 %) fetuses from terminated pregnancies due to fetal anomalies were registered in the MBRN. Among the live- and stillborn infants of women who used vitamin supplements compared with infants of non-users, the adjusted relative risk (aRR) was 0·94 (95 % CI 0·91, 0·98) for total birth defects (n 18 382). Supplement use was associated with reduced risk of abdominal wall defects (aRR 0·58; 95 % CI 0·42, 0·80, n 377), genital organ defects (aRR 0·81; 95 % CI 0·72, 0·91, n 2299) and limb defects (aRR 0·81; 95 % CI 0·74, 0·90, n 3409). Protective associations were also suggested for NTD, respiratory system defects and digestive system defects although CI included the null value of 1. During the full study period, statistically significant associations between supplement use and defects in the eye, ear–face–neck, heart or oral clefts were not observed. [ABSTRACT FROM AUTHOR]

The 'EX-utero Intrapartum Treatment' (EXIT) procedure is one option for the management of babies where there is a prenatal suspicion or diagnosis of airway anomaly which may compromise respiration and the ability to establish an effective airway in the first few minutes of life. The decision to proceed with an EXIT procedure, and the subsequent planning and execution, is an illustration of multi-disciplinary team working at its most complex and sophisticated. It is important to have clear assessment of all options, to balance the risks and benefits for both mother and baby of each of these, and to encourage close involvement of families in the decision-making process. There are two steps; the decision to proceed with a specialist delivery and the development of an individualised delivery plan. [ABSTRACT FROM AUTHOR]

Background: Uniportal video-assisted thoracic surgery (UVATS) technique has been increasingly used for many thoracic diseases. Whether UVATS has equivalent or better perioperative outcomes for pulmonary sequestration (PS) patients remains controversial. Our study aimed to evaluate the feasibility of UVATS in anatomical lung resection for pulmonary sequestration.Methods: A total of 24 patients with PS including fifteen males and nine females with the mean age of 40 (range, 18-65) years old, who had received completely UVATS anatomical lung resection for PS in Nanjing Chest Hospital between January 2016 and December 2018 were retrospectively reviewed. Related clinical data were retrieved from hospital records and analyzed.Results: All 24 patients had been treated with the UAVTS approach successfully without aberrant artery ruptured or massive hemorrhage, and no patients died during the perioperative period. Overall mean surgery time was 102 mins (range, 55-150 min), the mean blood loss was 94 ml (range, 10-300 ml), the mean days of chest tube maintained were 4 days (range,1-10 days), and the mean postoperative hospitalization days was 6 days (range,2-11 days). All patients were cured, without cough, fever, hemoptysis, and so on, associated with PS, occurring during the average follow-up of 17 months (range, 3-35 months).Conclusions: Our preliminary results revealed that anatomical lung resection by UVATS is a safe and feasible mini-invasive technique for PS patients, which might be associated with less postoperative pain, reduced paresthesia, better cosmetic results, and faster recovery. [ABSTRACT FROM AUTHOR]

Background: Kartagener syndrome, an autosomal recessive disorder with a triad of chronic sinusitis, bronchiectasis, and situs inversus, is characterized by recurrent respiratory tract infections and chronic inflammation of the lung. Information on comorbidities other than infections in patients with Kartagener syndrome is currently limited.Case Presentation: A 39-year-old, non-smoking female was diagnosed with Kartagener syndrome and admitted to Saitama Medical Center, Jichi Medical University, Japan. Computed tomography revealed an endobronchial massive shadow at the ostial site of the right upper lobe bronchus with atelectasis of the right upper lobe. The mass was surgically resected and pathologically diagnosed as mucoepidermoid carcinoma. The lesion had no vascular invasions and no metastases to the lungs or lymph nodes. The surgical margin was negative for carcinoma. Following surgery, the patient has been in good condition.Conclusions: The present case showed different clinicopathological characteristics from those previously reported in cases of Kartagener syndrome complicated by carcinoma. To the best of our knowledge, this is the first reported case of a young, non-smoking female with comorbid Kartagener syndrome and pulmonary mucoepidermoid carcinoma. This case report may provide a new perspective on the complications of Kartagener syndrome. [ABSTRACT FROM AUTHOR]

The article presents questions and answers related to neonatal and infant upper airway malformations including sound that an infant make when she inhales, best way to confirm a diagnosis of laryngomalacia, and information on subglottic stenosis.