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Introduction: Congenital lung malformations (CLMs) have a variable natural history. Larger lesions with CLM volume ratio (CVR) ≥ 1.6 are associated with hydrops and fetal mortality. The purpose of this study is to describe the management and outcomes of high-risk (CVR ≥ 1.6) CLM patients., Methods: A retrospective cohort study was performed for all fetuses evaluated between May 2015 and May 2022. Demographics, prenatal imaging factors, prenatal and postnatal treatment, and outcomes were collected. Descriptive statistics were used to compare the cohorts., Results: Of 149 fetal CLM patients referred to our fetal center, 21/149 (14%) had CVR ≥ 1.6. One CLM patient had intrauterine fetal demise, and 2 patients were lost to follow-up. Of the remaining 18 patients, 11/18 (67%) received maternal steroids. Seven out of 18 patients (39%) underwent resection at the time of delivery with 1/7 (14%) undergoing exutero intrapartum treatment (EXIT)-to-resection, 5/7 (71%) undergoing EXIT-to-exteriorization-to-resection, and 1/7 (14%) undergoing a coordinated delivery to resection; among those undergoing resection, there were 2 fatalities (28.5%). Seven out of 18 (39%) patients required urgent neonatal open lobectomies, and the remaining 4/18 (22%) patients underwent elective thoracoscopic lobectomies with no mortality., Conclusions: The natural history and outcomes of severe CLM patients remain highly variable. The EXIT-to-exteriorization-to-resection procedure may be a safe and effective approach for a subset of CLM patients with persistent symptoms of mass effect and severe mediastinal shift due to the observed decreased operative time requiring placental support observed in our study., (Copyright © 2023 Elsevier Inc. All rights reserved.)

A 6-month-old infant with recurrent respiratory infections, rapid breathing, and reduced air entry on the right side was diagnosed with congenital bronchopulmonary foregut malformation (CBPFM). Imaging revealed a collapsed and underdeveloped right lung, with the right bronchus appearing to originate from the lower part of the esophagus. An esophagogram confirmed the diagnosis by showing contrast flowing freely from the lower esophagus to the right bronchus., (© 2023 Wiley Periodicals LLC.)

Introduction:  Opinion remains divided on whether to resect an asymptomatic congenital lung malformation (CLM) and on optimal timing of resection. This study aimed to determine if age at resection of CLM correlates with the presence of histological inflammation and/or incidence of prior antibiotic administration for lower respiratory tract infection (LRTI)., Materials and Methods:  A retrospective review of all CLMs resected between 2009 and 2021 was carried out. Data on antenatal detection, incidence of preoperative antibiotic use for LRTI, operative details, and histological reports were analyzed. Fisher's exact test and logistic regression were used to look for correlation between age at resection and (1) histological inflammation and/or (2) preoperative LRTI., Results:  A total of 102 patients underwent resection at age 14 months (interquartile range: 6-23). Eighty percent of children were asymptomatic in the neonatal period and 22% of these went on to develop a respiratory symptom. In total, 59% of specimens had histological evidence of inflammation, with a significantly higher rate of inflammation after 10 months of age (71 vs. 35%; p  = 0.0012). Logistic regression showed there was a positive correlation between age at resection and treatment for previous LRTI ( p  = 0.020)., Conclusion:  Detection rates of inflammation in specimens resected after 10 months of age are double the rates of those resected prior to 10 months. Delaying resection of CLMs showed a higher frequency of treatment of LRTI. Earlier resection may therefore be advantageous for centers pursuing a resection strategy for asymptomatic lesions., Competing Interests: None declared., (Thieme. All rights reserved.)

Aim: To evaluate the impact of previous infection on perioperative outcomes in patients undergoing thoracoscopic lobectomy for congenital lung anomalies. Methods: This was a single-institution retrospective observational study for which patients who underwent thoracoscopic lobectomy for congenital lung disease between 2009 and 2021 were enrolled, and patients with extralobar sequestration were excluded. Patient background and data related to the surgery were compared between patients who had an infection before surgery (Group 1) and those who did not (Group 2). Results: This study included 34 patients, 13 in Group 1 and 21 in Group 2. The sex-based distribution and pathological diagnosis were similar between the two groups. Malformations were prenatally diagnosed in 1 patient in Group 1 (7.7%) and 18 patients in Group 2 (86%; P  < .001). The median age and weight at the time of the procedure and procedure duration were comparable between the two groups. The amount of blood loss was significantly higher in Group 1 (60 mL) than in Group 2 (20 mL; P  = .0042). Four patients in Group 2 required reoperation due to air leakage, pyothorax, and cardiac tamponade, whereas none of the Group 1 patients required reoperation ( P  = .12). No conversion to thoracotomy was required in either group. The duration of postoperative admission was similar between the two groups (Group 1: 6 days versus Group 2: 6 days; P  = .14). Conclusions: Preceding infection increased the amount of bleeding during thoracoscopic lobectomy but had little effect on other outcomes.

Objective: People with 22q11.2 deletion syndrome (22q11DS) can present with a wide variety of findings. Various airway anomalies have been described intermittently within this syndrome, but this feature has not been extensively investigated. Increased provider awareness of these findings may help guide clinical decision-making and improve overall patient outcomes. The objectives of this review are to identify the types of airway anomalies in 22q11DS and the prevalence of airway anomalies within symptomatic individuals., Methods: PubMed/MEDLINE, Cochrane Library, and EMBASE databases were searched in February 2022 for all available articles. Search terms included those that described 22q11DS or one of its synonymous conditions AND those that described airway anatomy and anomalies. The term airway anomaly was defined as any structural aberration in the conductive airway from the oral or nasal vestibule region to the mainstem bronchus. Studies were screened by two authors. A review of references was conducted. Eligible manuscripts underwent full-text review for quality appraisal and data extraction., Results: From a total of 909 unique manuscripts, 58 studies were selected, describing 328 people. The prevalence of airway anomaly diagnosis within symptomatic individuals ranged from 14% to 74%. Twenty-two unique airway anomalies were described. Laryngeal web was the most frequently described anomaly, followed by airway malacia and subglottic stenosis. Laryngeal web was 40% sensitive for suggesting a diagnosis of 22q11DS. Among affected individuals, as many as 46% had multiple concomitant airway anomalies. Aside from respiratory symptoms, other features that prompted airway evaluation included difficult intubation or failed extubation., Conclusion: The findings within this review support the notion that a wide variety of airway anomalies may be seen in people with 22q11DS and that these findings have been discovered frequently in those with airway symptoms. Providers should maintain a low threshold to perform an airway examination in those with 22q11DS, especially when airway symptoms are present., Competing Interests: Declaration of competing interest The authors have no conflicts of interest to report., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Background: Tracheal papillomatosis is a relatively rare condition with limited data on successful treatment modalities. To our knowledge, this is the first report to describe a papilloma arising from an accessory bronchus. Furthermore, this case report demonstrates successful treatment with clinical and patient-centered improvements after use of Spray Cryotherapy., Case Presentation: A 71-year-old woman presented with one year history of recurrent fevers and intermittent hemoptysis. Imaging and video bronchoscopy revealed an obstructing papilloma of an accessory tracheal bronchus to the right upper lobe. She was treated with debridement followed by multiple cryotherapy treatments resulting in complete clinical and radiographic resolution of her post-obstructive pneumonia., Conclusions: This case report not only supports existing literature on the use of cryotherapy for airway diseases but also presents a unique form of obstructing papilloma confined to an accessory bronchus, the only report of its kind based on extensive literature review., (© 2022. The Author(s).)

We performed a single-centre retrospective analysis using data from databases that were prospectively maintained in our centre between January 2019 and September 2021. Patients were divided into two groups based on the degree of pulmonary fissure completeness (PFC), using the fissure development scoring system. Patients with grades 2 or 3 PFC were considered to have incomplete pulmonary fissures and were included in Group A, and patients with grades 0 and 1 were considered to have complete pulmonary fissures and were included in Group B. The differences in demographics, perioperative characteristics and clinic outcomes between the two groups were evaluated. Multivariate logistic regression analysis was performed. A total of 213 patients with congenital lung malformation (CLM) underwent video-assisted thoracoscopic lobectomy. There were 30 patients in Group A and 183 patients in Group B. Our data showed that compared with Group B, Group A had a higher incidence of complications, especially Clavien-Dindo grade II and grade III complications. The degree of PFC was significantly correlated with the length of chest tube drainage and postoperative hospital stay. Multivariate logistic regression analysis showed that the degree of PFC could be used to predict the incidence of postoperative complications.ConclusionsThe degree of PFC is a predictor of the incidence of complications after thoracoscopic lobectomy in children with CLM., (© 2022. The Author(s).)

Tracheal bronchus, also known as bronchus suis , is a rare congenital anomaly of the airway where an accessory bronchus originates directly from the trachea. With an estimated incidence of 0.001%-2.0%, this condition is rarely reported in literature. It is usually discovered as an incidental finding in an otherwise asymptomatic individual. However, it can act as a focus of recurrent infection or present as persistent radiographic infiltrates. Multidetector CT imaging and bronchoscopy play a crucial role in the identification of this entity. We hereby report the case of a middle-aged man who presented with recurrent right upper lobe pneumonia, which was found to be due to an underlying tracheal bronchus., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

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.

Objectives: Nasal bone fracture is a frequent entity consulted to the otolaryngologists, approximately accounting for 39% of all facial bone fractures. The most frequent mechanisms of injury consist of assault, sport-related injuries, falls, and motor vehicle accidents. In this study, we examined the effects of the COVID-19 pandemic on pediatric nasal fractures., Methods: Children with nasal fracture who applied to Malatya Training and Research Hospital during the year before the first case and the following year were included in this study. Data of 172 patients for the pre-pandemic period and 79 patients for pandemic were available and included in the study. Demographic information, clinical features, nasal fracture etiology, nasal fracture type, type and time of intervention, and other accompanying pathologies were recorded., Results: While falls was the leading cause of fracture etiology before the pandemic (64 patients [37.21%]), assault seems to be the leading cause during the pandemic period (27 children [34.18%]). In the pre-pandemic period, the intervention for patients with nasal fractures was performed on an average of 5 days, while this period was calculated as an average of 6 days during the pandemic period. When the 2 groups are compared in terms of nasal fracture intervention time, it was seen that the intervention time was statistically significantly later in the pandemic period ( P < .001). According to the results of the analysis, the most cases in the pandemic period were seen in the fourth month, which indicated a-month period between 11 June and 11 July., Conclusions: In conclusion, our number of nasal fracture cases was decreased during the pandemic period compared to the 1-year period before the pandemic. We observed the most common type IIA nasal fracture. We gave outpatient treatment to most of the patients. Our most common cause of fracture was assault. We intervened in our cases in an average of 6 days and preferred closed reduction most frequently. We could not find any study on the same subject in the literature, and we aimed to contribute to the literature with this study.

Background: Cigarette smoking is a risk factor for interstitial lung abnormalities (ILAs) and interstitial lung diseases (ILDs). Investigation defining the relationships between ILAs/ILDs and clinical, radiographic, and pathologic findings in smokers have been incomplete. Employing a cohort undergoing surgical resection for lung nodules/masses, we (1) define the prevalence of ILAs/ILDs, (2) delineate their clinical, radiographic and pathologic predictors, and (3) determine their associations with mortality., Methods: Patients undergoing resection of lung nodules/masses between 2017 and 2020 at a rural Appalachian, tertiary medical center were retrospectively investigated. Predictors for ILAs/ILDs and mortality were assessed using multivariate logistic regression analysis., Results: In the total study cohort of 352 patients, radiographic ILAs and ILDs were observed in 35.2% and 17.6%, respectively. Among ILA patterns, subpleural reticular changes (14.8%), non-emphysematous cysts, centrilobular (CL) ground glass opacities (GGOs) (8% each), and mixed CL-GGO and subpleural reticular changes (7.4%) were common. ILD patterns included combined pulmonary fibrosis emphysema (CPFE) (3.1%), respiratory bronchiolitis (RB)-ILD (3.1%), organizing pneumonitis (2.8%) and unclassifiable (4.8%). The group with radiographic ILAs/ILDs had a significantly higher proportion of ever smokers (49% vs. 39.9%), pack years of smoking (44.57 ± 36.21 vs. 34.96 ± 26.22), clinical comorbidities of COPD (35% vs. 26.5%) and mildly reduced diffusion capacity (% predicated 66.29 ± 20.55 vs. 71.84 ± 23). Radiographic centrilobular and paraseptal emphysema (40% vs. 22.2% and 17.6% vs. 9.6%, respectively) and isolated traction bronchiectasis (10.2% vs. 4.2%) were associated with ILAs/ILDs. Pathological variables of emphysema (34.9% vs. 18.5%), any fibrosis (15.9% vs. 4.6%), peribronchiolar metaplasia (PBM, 8% vs. 1.1%), RB (10.3% vs. 2.5%), and anthracosis (21.6% vs. 14.5%) were associated with ILAs/ILDs. Histologic emphysema showed positive correlations with any fibrosis, RB, anthracosis and ≥ 30 pack year of smoking. The group with ILAs/ILDs had significantly higher mortality (9.1% vs. 2.2%, OR 4.13, [95% CI of 1.84-9.25])., Conclusions: In a rural cohort undergoing surgical resection, radiographic subclinical ILAs/ILDs patterns were highly prevalent and associated with ever smoking and intensity of smoking. The presence of radiographic ILA/ILD patterns and isolated honeycomb changes were associated with increased mortality. Subclinical ILAs/ILDs and histologic fibrosis correlated with clinical COPD as well as radiographic and pathologic emphysema emphasizing the co-existence of these pulmonary injuries in a heavily smoking population., (© 2022. The Author(s).)

The poor outcomes of babies with congenital diaphragmatic hernia (CDH) are directly related to pulmonary hypoplasia, a condition characterized by impaired lung development. Although the pathogenesis of pulmonary hypoplasia is not fully elucidated, there is now evidence that CDH patients have missing or dysregulated microRNAs (miRNAs) that regulate lung development. A prenatal therapy that supplements these missing/dysregulated miRNAs could be a strategy to rescue normal lung development. Extracellular vesicles (EVs), also known as exosomes when of small dimensions, are lipid-bound nanoparticles that can transfer their heterogeneous cargo (proteins, lipids, small RNAs) to target cells to induce biological responses. Herein, we review all studies that show evidence for stem cell-derived EVs as a regenerative therapy to rescue normal development in CDH fetal lungs. Particularly, we report studies showing that administration of EVs derived from amniotic fluid stem cells (AFSC-EVs) to models of pulmonary hypoplasia promotes fetal lung growth and maturation via transfer of miRNAs that are known to regulate lung developmental processes. We also describe that stem cell-derived EVs exert effects on vascular remodeling, thus possibly preventing postnatal pulmonary hypertension. Finally, we discuss future perspectives and challenges to translate this promising stem cell EV-based therapy to clinical practice., (© 2022 John Wiley & Sons Ltd.)

Tetralogy of Fallot with unilateral pulmonary anomalies such as the unilateral absence of pulmonary artery or unilateral pulmonary agenesis is an extremely rare complex congenital heart anomaly. There is no established surgical algorithm for tetralogy of Fallot with concomitant unilateral pulmonary anomalies. This condition is still challenging, especially in the surgical field. In this review we also present our experiences in our center, Dr Cipto Mangunkusumo General Hospital, Jakarta, Indonesia. This literature review aimed to discuss systematic treatment options and hoped to help the decision-making process when surgeons face these rare anomalies.

Pulmonary growth abnormality (PGA) is a common type of diffuse lung disease in infants. Although the histologic and radiographic features of PGA have been described in the literature in varying detail, the clinical spectrum of disease has not. The array of case series and case reports has led to a clinical picture that could be confusing to clinicians. We describe three subsets of PGA, including its association with the histologic marker of pulmonary interstitial glycogenosis, and its common association with pulmonary hypertension. We propose a new approach to what we consider an increasingly broad array of different disease entities., (© 2020 Wiley Periodicals LLC.)

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.

Background: Congenital lung malformations (CLMs) have a variable natural history: some patients require urgent perinatal surgical intervention (UPSI) and others remain asymptomatic. These lesions have potential growth until 26-28 wk gestation. CLM volume ratio (CVR) has been shown to predict the risk of hydrops in CLMs. However, no criteria exist to delineate lesions requiring urgent surgical intervention in the perinatal period. Our goal was to determine prenatal diagnostic features that predict the need for UPSI in patients diagnosed with CLM., Methods: Records and imaging features of all fetuses evaluated by our fetal center between May 2015 and December 2018 were retrospectively reviewed. Data included demographics, fetal ultrasound and magnetic resonance imaging, CVR, surgical treatment, and outcome. Features were analyzed for their ability to predict the need for UPSI., Results: Sixty-four patients were referred for CLM, with 48 patients serially followed. Nine (18.8%) patients were followed nonoperatively, 35 (72.9%) underwent resection, and four (8.3%) were lost to follow-up. Of the patients who underwent resection, 24 (68.5%) were electively resected and 11 were urgently resected. Five (14.3%) patients underwent ex utero intrapartum treatment resection, and six (17.1%) were urgently resected for symptomatic CLM. There were no cases of UPSI with final CVR <1.1. Of the patients with final CVR 1.1-1.7, 43% required urgent resection. CVR ≥1.1 has 100% sensitivity and 87.8% specificity to predict patients requiring UPSI (area under the curve of 0.98)., Conclusions: A final CVR ≥1.1 is highly predictive for UPSI. Patients with a final CVR ≥1.1 should be referred for delivery at centers with pediatric surgeons equipped for potential UPSI for CLM., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Competing Interests: Competing interests: None declared.

Congenital airway malformations can present with respiratory distress, cyanosis, and difficulty feeding in the neonate or infant. Clinical presentation may vary from asymptomatic to fatal airway obstruction. They may exist in isolation or in association with vascular rings and slings, bronchopulmonary malformations, and/or syndromes. We present an unusual case of bridging bronchus, complete bronchial rings, and left pulmonary artery sling presenting with recurrent croup, highlighting the importance of bronchoscopy and CT imaging to achieve an accurate diagnosis in patients with recurrent croup and/or respiratory failure not responding to usual treatment measures and a multidisciplinary treatment approach., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Objectives: Children admitted with stridor and respiratory distress comprise a complex patient group that requires the otolaryngologist to decide when to assess and intervene with direct laryngoscopy and bronchoscopy (DLB). Historically, the diagnosis of viral upper respiratory tract infection (URTI) can lead to postponement of surgery due to concerns of perioperative complications related to acute illness. Respiratory viral panels (RVP) are often used to confirm the presence of recent or active viral infection and can affect the differential diagnosis of upper airway obstruction. This study examined whether positive RVP testing is associated with perioperative complications and operative findings in pediatric patients undergoing inpatient DLB., Methods: A retrospective chart review of 132 pediatric patient encounters was performed. Viral testing results, DLB indication, DLB findings, and perioperative complications were compared., Results: Sixty encounters (45.5%) involved a positive RVP, and 72 (54.5%) involved a negative RVP. Those with positive RVP were less likely to have a preoperative structural airway diagnosis (P =.0250) and more likely to have a history of recurrent upper respiratory infections (P =.0464). The most common reason for DLB was the need to assess the airway due to concern for structural pathology. Anatomic abnormalities were seen in a majority of encounters (77.3%) Laryngospasm occurred in 1 (1.7%) RVP positive and 1 (1.4%) RVP negative encounter, and 2 (2.8%) RVP negative encounters required reintubation. No other major complications were observed. No association was noted between RVP results and incidence of major or minor complication., Conclusions: Major perioperative complications after surgical intervention with DLB for the management of complex, inpatient children with stridor and respiratory distress are rare. RVP positivity, specific pathogens identified on RVP, and presence of URI symptoms were not associated with perioperative complications., Competing Interests: Declaration of competing interest None., (Copyright © 2020. Published by Elsevier B.V.)

Background: To date there are limited data in the literature to guide the initial evaluation for etiologies of apnea in full-term infants born at greater than or equal to 37 weeks conceptional age (apnea of infancy [AOI]). Pediatricians and pediatric pulmonologists are left to pursue a broad, rather than targeted and a stepwise approach to begin diagnostic evaluation., Methods: We performed a retrospective chart review of 101 symptomatic full-term infants (age under 12 months) diagnosed with apnea with an inpatient multichannel pneumogram (six channels) or a fully attended overnight pediatric polysomnogram in our outpatient sleep center accredited by American Academy of Sleep Medicine (AASM), scored using the standards set forth by the AASM. The infant was diagnosed as having AOI if the apnea hypopnea index (AHI) was greater than 1 (AHI is defined as the number of apnea and hypopnea events per hour of sleep). The final diagnosis/etiology was determined based on physician clinical assessment and work up. We then determined the frequency for each diagnosis., Results: We found that the three most common etiologies were gastroesophageal reflux disease (GERD) (48/101), upper airway abnormalities/obstruction (37/101), and neurological diseases (19/101). There were significant numbers of infants with multiple etiologies for AOI., Conclusion: Based on the frequencies obtained, pediatric practitioners caring for full-term infants with apnea of unknown etiology are advised to begin with evaluation of more likely causes such as GERD and upper airway abnormalities/obstruction before evaluating for less common causes., (© 2020 Wiley Periodicals, Inc.)

Objectives: To illustrate the clinical and radiological presentation of a rare etiology of nasal obstruction in neonates, midnasal stenosis (MNS), including a comparison of nasal dimensions with those of normal infants., Methods: We retrospectively reviewed medical charts and computerized tomography (CT) imaging of neonates with nasal obstruction diagnosed as stenosis in the midnasal area in a tertiary pediatric medical center. MNS was defined clinically by inability to visualize the middle turbinate with an endoscope despite the absence of stenosis of the anterior aperture or any gross septal deviation. CT measurements of the midnasal width were taken by an experienced neuroradiologist. We compared widths between the bony inferior turbinate to the bony septum in the narrowest area of symptomatic patients, to widths in a control group of asymptomatic children., Results: Nine neonates from birth to three months old presenting with nasal obstruction, severe stertor, and blocked nasal passage at the midnasal level in endoscopic examination, were diagnosed with MNS. 6/9 had CT scans. Four had isolated unilateral stenosis, two unilateral MNS and contralateral choanal atresia, and three bilateral MNS. All patients were managed conservatively, initially with nasal saline irrigation and local steroids and topical antibiotics; Median time to resolution of symptoms was 14 days. When comparing the dimensions at the midnasal narrowest area of the stenotic group with a control group of 139 healthy children, the median bony width was 1.7 mm vs. 3.2 mm, respectively (p < 0.00001). Average dimensions according to age groups until the age of 12 months are given., Conclusion: In neonates with nasal obstruction, when choanal atresia and pyriform aperture stenosis are excluded, stenosis of the midnasal area should be considered. Most of these neonates can be managed conservatively., Level of Evidence: 4., Competing Interests: Declaration of competing interest The authors have no conflicts of interest to declare., (Copyright © 2020. Published by Elsevier B.V.)

Introduction: Congenital midnasal stenosis (MNS) is an extremely rare disease which may be life threatening, and shows difficulty in diagnosis and management. This case series summarizes superiority of using intranasal mometasone furoate spray (IMS) and continuous positive airway pressure (CPAP) to treat nasal obstruction in neonates with MNS., Methods: This study reviewed six consecutive cases of MNS., Results: Three patients were treated with IMS and CPAP. Two patients were treated with endoscopic balloon dilatation without stenting, followed by IMS and CPAP due to persisting nasal obstruction after the operation. One patient was treated with endoscopic balloon dilatation without stenting alone., Conclusion: This study is the first to review the use of CPAP and IMS as an effective therapy for some patients with MNS. It suggests that conservative management with CPAP and IMS may be an alternative therapeutic option to surgery., Competing Interests: Declaration of competing interest No potential conflict of interests relevant to this paper was reported., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Introduction: Children with Down syndrome (DS) often present with chronic respiratory symptoms. Congenital airway anomalies have been described but data about prevalence is scarce and a comparison to controls is lacking. We aim to compare the endoscopic and clinical data of children with DS to controls without significant medical history., Methods: All endoscopic procedures under general anesthesia (broncho- and/or direct laryngoscopy) in patients with DS were reviewed. We compared clinical and endoscopic data to a cohort of children with respiratory symptoms but without any other relevant medical history., Results: Endoscopic data were available for 65 patients with DS. The median age was 2.9 years (range: 0.2-17), 63% were boys. The most common clinical presentation was recurrent respiratory infections (37%). Other major symptoms were chronic cough and/or noisy breathing (23%) and stridor (20%). Endoscopy was normal in 29% of patients. The largest group of patients (44%) had some form of airway malacia. Tracheal bronchus and subglottic stenosis were each isolated findings in 3.1% of patients. Twenty percent presented with combined airway anomalies. The control group consisted of 150 children (matched for age and sex) without significant underlying disease. The most common presentations were chronic cough and/or noisy breathing (29%), persistent radiographic abnormalities (20%), and suspicion of aspiration of a foreign body (15%). In the majority of controls (68%), no airway anomaly was found. Other findings were malacia (22%), tracheal bronchus (1%), and subglottic stenosis (1%). A combined anomaly was found in 5%., Conclusion: Congenital airway anomalies were seen in 71% of patients with DS, compared with 32% of controls. Combined anomalies are more frequent in DS. Complete lower airway endoscopy is recommended in patients with DS as it may influence therapeutic decision-making., (© 2020 Wiley Periodicals, Inc.)

Esophageal lung is a rare variety of communicating bronchopulmonary foregut malformation with anomaolous communication between an isolated portion of respiratory tissue and esophagus. Children present in early life with recurrent cough and pneumonia. Majority of the reported cases are associated with other anomalies like tracheoesophageal fistula. We report a case of a 7-month-old girl with right sided esophageal lung who was misdiagnosed as dextrocardia with right sided pneumonitis.

Background: Meconium peritonitis (MP) is an infrequent prenatal complication. Association between MP and pulmonary hypoplasia has never been reported. Case Report: A female infant with antenatally diagnosed MP and ascites was delivered at 36 gestational weeks. She died shortly after birth due to pulmonary insufficiency. Autopsy confirmed the presence of MP and ascites, and additionally revealed intestinal malrotation, volvulus, necrosis and perforation, and pulmonary hypoplasia. Congenital cytomegalovirus (CMV) infection was also noted. This was an unexpected finding as the mother was tested negative for CMV IgM at 26 gestational weeks after sonographic detection of fetal ascites. Conclusions: This is the first reported case of lethal pulmonary hypoplasia in a neonate with MP-associated ascites complicated by congenital CMV infection. This case illustrates that a negative maternal CMV IgM might not be sufficient to rule out congenital CMV, and that a concomitant infectious etiology should always be considered even when a primary cause for fetal ascites (e.g., MP in this case) is identified.

Congenital airway malformations are most often identified in early childhood. The development of bronchiectasis in association with malformations of the lower airway has been described, particularly among adult patients. The coexistence in a pediatric population of these conditions is not well described. This study was conducted to identify whether younger patients with airway malformations commonly develop bronchiectasis. International Classification of Diseases, Ninth revision (ICD-9 codes) were defined for airway anomalies and bronchiectasis. The electronic medical records system of a children's hospital was then searched for the number of patients with upper airway anomalies with or without bronchiectasis. The airway database was then cross referenced with the ICD codes for bronchiectasis to identify patients with both conditions. There were 844 patients with airway anomalies and 117 with bronchiectasis in the electronic system during the time period of August 1, 2009 to September 30, 2014. There was only 3 patients identified with both bronchiectasis and airway anomalies. The coexistence of bronchiectasis is low among the pediatric population with upper airway anomalies studied. This would suggest that the children with airway anomalies have been treated with strategies that are effective in the prevention of recurrent lower respiratory tract infection. Further study may be done to define the effectiveness of various strategies in preventing aspiration and lower respiratory tract infection. In addition, this methodologic technique utilizing database integrative platforms is useful in the identification of patients for further study and to identify the coexistence of pediatric conditions.

Objective: To investigate the key issues in the diagnosis and treatment of foreign body aspiration in children with tracheobronchial variations. Methods: A retrospective study was performed for 11 pediatric patients who were treated in Department of Otorhinolaryngology and Head and Neck Surgery, Henan Province People's Hospital after a diagnosis of foreign body aspiration with tracheobronchial variations between January 2015 and December 2017. There were 7 males and 4 females among the 11 cases of foreign body aspiration with tracheobronchial variations, ranging between 9 months and 11 years of age. Results: Among 11 cases, the types of variationswere tracheal bronchus in 9 cases, bridging bronchus in 1 case and simple tracheal stenosis in 1 case. All of the pediatric patients were under general anesthesia, and the foreign bodies were removed by bronchoscopy successfully with no significant complications. Conclusions: The possibility of tracheobronchial variations should be considered in children with recurrent wheezing and poor efficacy of regular treatment before foreign body aspiration. Removal of foreign body via rigid bronchoscope under general anesthesia is a safe and effective treatment. These children are needed to combine the situation oftracheobronchial variations and the location of foreign bodies to guide the operation, and strengthened the perioperative treatment.

Introduction: The purpose of this study was to evaluate short-term respiratory outcomes in neonates with symptomatic congenital lung malformations (CLM)., Methods: Consecutive newborns who underwent surgical resection of a CLM were retrospectively reviewed. Demographic, prenatal, and outcomes data were analyzed as appropriate (p < 0.05)., Results: Twenty-one neonates were managed at a median gestational age of 36.2 weeks [interquartile range (IQR), 33.8-39.0]. Endotracheal intubation was required in 14 (66.7%) for a median of 7.5 days [interquartile range (IQR), 3.0-25.8]. Three (14.3%) children underwent ex utero intrapartum treatment-to-resection, and another 14 (66.7%) had neonatal lung resections performed at a median age of 2.0 days (IQR, 0.08-19.5 days). Excluding one patient who received comfort care at birth, all neonates survived to hospital discharge with a median length of hospitalization of 36.5 days (IQR, 23.8-56.5). More than one-quarter were discharged on supplemental oxygen by nasal cannula. Based on a median follow up of 35.5 months (IQR, 19.0-80.8), CLM-related morbidity was still evident in 55.0%., Conclusion: Our study suggests a high incidence of complications and chronic respiratory morbidity after neonatal lung resection for symptomatic CLMs. These data highlight the need to provide realistic expectations in perinatal counseling discussions with families and the importance of coordinating appropriate multidisciplinary follow up for these children., Level of Evidence: Level IV., (Copyright © 2019 Elsevier Inc. All rights reserved.)

We describe a case of H1N1 pneumonia with left upper lobe bronchial atresia. Although bronchial atresia as an isolated occurrence is an innocuous finding, but when it is superimposed by another major insult, it can amplify the disease effect and can have adverse implications leading to significant morbidity. This report highlights the fact that anatomical anomalies can be the cause of inordinately severe or prolonged course of acute respiratory infection in children., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Bardet-Biedl syndrome (BBS) is a rare autosomal-recessive disease characterized by rod-cone dystrophy, obesity, postaxial polydactyly, cognitive impairment, hypogonadism and renal abnormalities. Bifid epiglottis and anterior laryngeal web are rare congenital anomalies and are often constituent of polymalformation syndromes. We report a case of a 9-month-old patient initially referred in otolaryngology (ENT) for dysphonia and recurrent respiratory infections. Physical exam and fiberoptic nasopharyngolaryngoscopy showed bifid epiglottis and laryngeal web associated with BBS. Those laryngeals anomalies may be underdiagnosed in BBS and this case supports the importance of upper airway evaluation by an ENT team, especially with respiratory symptoms or dysphagia., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Objectives: Pediatric patients born with single functional lung can be associated with symptomatic airway abnormalities. Management of such patients is not only technically demanding but is also ethically challenging. This study reports our experience of managing symptomatic airway abnormalities in pediatric patients with congenital single functional lung., Methods: Observational retrospective cohort study performed at a tertiary children's hospital from January 2001 to September 2017. All children (0 to 18 years old) with congenital single functional lung (agenesis and hypoplasia) presenting with symptomatic airway abnormalities (long segment congenital tracheal stenosis and tracheomalacia) and requiring surgical interventions were included in the study. Children with single functional lung secondary to non-congenital causes were excluded., Results: A total of 16 patients with single functional lung (agenesis=8 and hypoplasia=8) and airway abnormalities (long segment congenital tracheal stenosis=12 and tracheomalacia =4) were eligible for the study. Lung abnormalities were common on the right side (n = 10, 62.5%). Associated abnormalities (cardiac and non-cardiac) were seen in 11 patients (68.8%). Surgical interventions for airway abnormalities, alone or in combination, included slide tracheoplasty (n=12), aortopexy with or without pericardiopexy (n=7), excision of rudimentary lung (n=4) and placement of intrathoracic tissue expanders to reposition the mediastinum (n=3). Nine patients (56.3%) underwent a one-stage repair while staged repairs (airway and cardiac) were performed in 7 (43.7%). Fourteen patients (87.5%) survived to hospital discharge. Of the survivors, 9 (64.2%) had stable airways not requiring respiratory support at home., Conclusion: Management of pediatric patients with airway abnormalities in the setting of congenital single functional lung is feasible with acceptable surgical outcomes. This is facilitated by staged repairs and repositioning of mediastinum before a definitive airway repair in patients with significant comorbidities. Treatment should not be deferred to these patients if there are no obvious contraindications., Type of Study: Retrospective Case Control Study LEVEL OF EVIDENCE: Level III., (Copyright © 2018 Elsevier Inc. All rights reserved.)

An observational study was conducted of children < 2 years who received ≥ 1 dose of palivizumab in 32 Canadian institutions from 2005 to 2017. We compared respiratory illness (RIH) and respiratory syncytial virus-related hospitalization (RSVH) hazards in children with a congenital airway anomaly (CAA) versus those prophylaxed for standard indications (SI) and serious medical disorders (SMD). Data were assembled on neonatal course, demographics, palivizumab utilization and adherence, and respiratory illness events, and analyzed using ANOVA, chi-square tests and Cox proportional hazards. Twenty-five thousand three children (1219 CAA, 3538 SMD, and 20,246 SI) were enrolled. Palivizumab adherence was 74.8% overall and similar across groups. For 2054 respiratory-related events, 1724 children were hospitalized. RIH rates were 13.6% (CAA), 9.6% (SMD), and 6.0% (SI). RSVH rates were 2.4% (CAA), 1.6% (SMD), and 1.5% (SI). After adjustment for demographic and neonatal differences, children with a CAA had a significantly increased RIH and RSVH hazard relative to SI (RIH, HR = 1.6, 95% CI 1.2-2.2, p = 0.002; RSVH, HR = 2.1, 95% CI 1.0-4.4, p = 0.037) but similar to SMD (RIH, HR = 1.3, 95% CI 0.9-1.9, p = 0.190; RSVH, HR = 1.7, 95% CI 0.7-4.1, p = 0.277).Conclusion: Children with a CAA experience higher RIH risk. RSVH hazard was similar between CAA and SMD but higher for CAA compared to SI, implying that this population requires surveillance for RSV prophylaxis. What is Known: • Children with congenital airway anomalies (CAA) are at risk for respiratory tract illness and respiratory syncytial virus-related hospitalization (RSVH) with accompanying morbidity and mortality • RSV prophylaxis may be useful in children with a CAA, but is not routinely recommended What is New: • Children with a CAA had a 1.6-2.3 fold greater risk of respiratory-related hospitalization and RSVH compared to those prophylaxed for standard, approved indications and serious medical disorders. • RSVH risk in children aged < 2 years with either upper or lower airway anomalies is similar. Children with a CAA require careful surveillance during the RSV season and prophylaxis may be appropriate.

Background: Long-term dysphagia occurs in up to 50% of repaired esophageal atresia and tracheoesophageal fistula (EA/TEF) patients. The underlying factors are unclear and may include stricture, esophageal dysmotility, or associated anomalies. Our purpose was to determine whether structural airway abnormalities (SAA) are associated with dysphagia in EA/TEF., Methods: We conducted a retrospective chart review of children who underwent EA/TEF repair in our hospital system from 2007 to 2016. Children with identified SAA (oropharyngeal abnormalities, laryngeal clefts, laryngomalacia, vocal cord paralysis, and tracheomalacia) were compared to those without airway abnormalities. Dysphagia outcomes were determined by the need for tube feeding and the modified pediatric Functional Oral Intake Scale (FOIS) at 1 year., Results: SAA was diagnosed in 55/145 (37.9%) patients with EA/TEF. Oropharyngeal aspiration was more common in children with SAA (58.3% vs. 36.4%, p=0.028). Children with SAA were more likely to require tube feeding both at discharge (79.6% vs. 48.3%, p<0.001) and at 1 year (52.7% vs. 13.6%, p<0.001) and had lower mean FOIS (4.18 vs. 6.21, p<0.001). In the logistic regression model adjusting for gestational age, long gap EA, and esophageal stricture, the presence of SAA remained a significant risk factor for dysphagia (OR 4.17 (95% CI 1.58-11.03))., Conclusion: SAA are common in children with EA/TEF and are associated with dysphagia, even after accounting for gestational age, esophageal gap and stricture. This study highlights the need for a multidisciplinary approach, including early laryngoscopy and bronchoscopy, in the evaluation of the EA/TEF child with dysphagia., Level of Evidence: Level II retrospective prognostic study., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: A displaced bronchus is a rare disorder of the left upper lobe. Displaced bronchi are often accompanied by an anomaly of a pulmonary artery, but rarely of a pulmonary vein., Case Presentation: We here present a patient with primary lung cancer and simultaneous migration abnormalities of the pulmonary artery and vein in a displaced bronchus of the left upper lobe. Previous reports and our findings indicate that anomalies of the pulmonary artery and vein combined with a displaced bronchus of the left upper lobe have the following characteristics: (1) the left main pulmonary artery does not cross the dorsal side of the displaced bronchus; (2) V 1 + 2 returns to the inferior pulmonary vein; and (3) there is an accessory fissure (aberrant fissure) in the segments dominated by the displaced bronchus., Conclusions: Prevention of intraoperative damage during procedures for a displaced bronchus and pulmonary arteriovenous anomalies requires careful preoperative evaluation and surgical technique with particular attention to the above-listed characteristics.

Aim: Management of congenital pulmonary airway malformations (CPAM) is controversial, especially for asymptomatic patients. We aim to describe the clinical manifestations and management of CPAM at a tertiary paediatric hospital using a retrospective audit., Methods: Infants with CPAM were identified on the Fetal Diagnostic Unit database from 2007 to 2014. Information on antenatal and post-natal management was collected from medical record., Results: Thirty-five infants with antenatally diagnosed CPAM were included. Fetal CPAM volume ratio (CVR) was calculated from antenatal ultrasound measurement and used to categorise the infants into three groups of large (CVR ≥ 1.6, n = 8), medium (CVR of 0.5-1.6, n = 12) and small CPAM (CVR of ≤0.5, n = 15), respectively. Ten infants (10/35 = 29%) were symptomatic in the neonatal period. Overall, nine infants (26%) had surgical resection, among whom eight had large or medium-sized CPAM lesions as defined by the antenatal CVR. Three infants had neonatal emergency surgery and the remaining six had late elective surgery. Histology of eight cases showed CPAM, but one case showed congenital lobar emphysema. Criteria for surgery varied and included persistent symptoms after birth, complications during childhood and persistently abnormal chest X-ray. Most asymptomatic infants with CPAM were safely managed using a conservative approach, with no significant increase in late symptoms or complications., Conclusions: Conservative management of CPAM may be considered for infants/children who remain asymptomatic, especially those with a small lesion. For large and medium-sized CPAM, delineation using computed tomography is required, and surgery may be beneficial to prevent late symptoms and the risk of emergency surgery., (© 2017 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

Objectives: This study aims to describe a congenital laryngeal structural variant, hemilaryngeal microsomia (HLM), and to correlate identification on physical examination with computerized tomography scan (CT) and laryngoscopy findings., Methods: The study was conducted at a tertiary care center. Six patients presenting with hoarseness were admitted to a tertiary care otolaryngology office. These patients had asymmetrical thyroid cartilage prominence on palpation during physical examination. A diagnosis of HLM was made. All patients underwent laryngostroboscopy and CT scan. Four control patients with normal thyroid cartilage anatomy on physical examination, CT, and stroboscopy results were included for comparison., Results: Disparities in thyroid cartilage angles correlated with documented physical examination findings for six out of six HLM patients. On CT scan, the average difference in left and right thyroid laminar angles was 30.2° ± 18.3° in HLM patients vs 4.00° ± 1.63° in control patients (P = 0.023). Strobosocopic findings also correlated with HLM. The arytenoid cartilage was anteriorly or medially displaced on the microsomic side in all six HLM patients. Three patients had anterior placement of the vocal process resulting in shortening of the vocal fold on the microsomic side of the larynx., Conclusions: HLM is a congenital structural anomaly of the larynx that may be palpated on physical examination. HLM found on physical examination can be correlated with asymmetries found on CT scan and endoscopy. There is no evidence that the structural features of HLM were causally related to voice symptoms, but the findings on HLM may lead to misdiagnosis. A larger study is indicated to confirm laryngeal structural differences between patients with HLM on physical examination and the general population. Whether or not HLM affects clinical or surgical outcomes remains to be studied., (Copyright © 2017. Published by Elsevier Inc.)

Background and Aims: Situs inversus is a rare congenital abnormality involving partial or complete transposition of the thoracic or abdominal viscera. In situs inversus totalis, both the thoracic and abdominal viscera are transposed. The incidence of this condition is 0.01% to 0.02%. Bronchopulmonary sequestration (BPS) is a rare congenital abnormality of the respiratory tract with an incidence of 0.15% to 1.80%. Intralobar sequestration is uncommonly associated with congenital anomalies., Methods: A routine chest X-ray of a 41-year-old asymptomatic man showed dextrocardia, a left-sided liver, right-sided stomach, and left paracardiac opacity., Results: Computed tomography (CT) and CT pulmonary angiography revealed dextrocardia with situs inversus totalis and left paracardiac intralobar BPS in the lingular segment of the left upper lobe., Conclusion: We present a rare case of combined situs inversus totalis and intralobar BPS with an atypical location and feeder artery., (© 2015 John Wiley & Sons Ltd.)

Bronchial atresia is a rare congenital malformation that is often complicated by infectious disease; however, mycobacterial infection is rare. Here we report a case of Mycobacterium avium infection complicating congenital bronchial atresia. A 50-year-old woman consulted our institution for further investigation of a nodular lesion in an emphysematous lung due to congenital bronchial atresia. Because we were unable to rule out malignant disease, we performed video-assisted thoracoscopic surgery. After left S8 segmentectomy, the nodular lesion were diagnosed as Mycobacterial avium infection. Three years after surgery, there has been no recurrence of mycobacterium infection in the absence of medication., (© The Author(s) 2016.)

Background: Wheezing is a common symptom in early childhood. However, refractory wheezing is difficult to treat, and it may thus account for extensive use of medical resources. It is therefore important to improve our understanding of the pathophysiology of refractory childhood wheezing., Methods: In this descriptive study, we studied 156 children with refractory wheezing using fiberoptic bronchoscopy and bronchoalveolar lavage (BAL), and compared the results with a control group of 46 children with various pulmonary diseases but no wheezing. Etiology and cell classification were analyzed for each BAL sample., Results: Overall, 21.8 % of children with refractory wheezing had airway malformations including tracheomalacia, airway stenosis, and tracheal bronchus. The incidence of airway malformations increased to 31 % in infants under 12 months of age. A significant increase in neutrophil ratio and decrease in macrophage ratio were observed in BAL from children with refractory wheezing compared with controls. Pathogen infection led to a higher ratio of neutrophils in the wheezing group compared with controls. However, there were no significant differences in neutrophil ratios among children with various pathogen infections. Furthermore, children with refractory wheezing had a high rate of Mycoplasma pneumoniae infection., Conclusions: Airway malformations might play an important role in children under 3 years of age with refractory wheezing, especially in infants under 12 months of age. Neutrophil-mediated airway inflammation was characteristic of refractory wheezing in children under 3 years of age. In addition, infections such as M. pneumoniae may aggravate airway inflammation and affect refractory wheezing.

Objective: Abnormal cardiac axis and/or malposition prompts evaluation of congenital heart disease; however, etiology may be difficult to clarify using obstetrical ultrasound or fetal echocardiography (echo) alone. We aimed to use fetal magnetic resonance imaging (MRI) as a complementary tool to identify causes of cardiac malposition., Methods: Review of fetuses diagnosed with cardiac malposition by fetal ultrasound and echo was performed. Etiology was classified as either because of heterotaxy syndrome or extracardiac masses. Reclassification was then performed with fetal MRI findings. Results were compared with postnatal diagnoses., Results: Forty-two fetuses were identified as having abnormal cardiac axis and/or malposition. Twenty three of 42 cases (55%) had extracardiac anomalies, while 19 (45%) were because of heterotaxy. Twelve of 42 (29%) cases were reassigned by fetal MRI (five in heterotaxy group and seven in the lung anomaly group). Four cases (33%) had both cardiac disease and extracardiac masses, not previously recognized. Fetal MRI clarified heterotaxy subtype or removed heterotaxy diagnosis in five (26%) patients. Fetal MRI findings were confirmed in 8 of these 12 cases postnatally., Conclusion: Fetal MRI is a useful complementary tool to define etiology of cardiac malposition in complex cases for informative prenatal counseling and planning. © 2016 John Wiley & Sons, Ltd., (© 2016 John Wiley & Sons, Ltd.)

Antenatally, congenital pulmonary airway malformation (CPAM) causing fetal hydrops can be palliated with thoracoamniotic shunts, which may become displaced in utero. We report a case of an infant born at 34 weeks gestational age with an antenatally diagnosed macrocystic lung lesion, fetal hydrops and an internally displaced thoracoamniotic shunt. The infant suffered refractory pneumothoraces despite multiple chest drains, and stabilised only after surgical resection of the lesion. Intraoperatively, the shunt was noted to form a connection between a type I CPAM and the pleural space. As the shunt was displaced internally, this complication was not immediately obvious during the initial resuscitation. In infants with large cystic lung lesions, clinicians should be aware that internally displaced thoracoamniotic shunts could contribute to refractory tension pneumothoraces and anticipate the need for advanced neonatal resuscitation, including early thoracocentesis or chest drain insertion. Furthermore, displaced shunts may require early surgical intervention., (2016 BMJ Publishing Group Ltd.)

Costello syndrome (CS) is a multisystem disorder caused by heterozygous germline mutations in the HRAS proto-oncogene. Respiratory system complications have been reported in individuals with CS, but a comprehensive description of the full spectrum and incidence of respiratory symptoms in these patients is not available. Here, we report the clinical course of four CS patients with respiratory complications as a major cause of morbidity. Review of the literature identified 56 CS patients with descriptions of their neonatal course and 17 patients in childhood/adulthood. We found that in the neonatal period, respiratory complications are seen in approximately 78% of patients with transient respiratory distress reported in 45% of neonates. Other more specific respiratory diagnoses were reported in 62% of patients, the majority of which comprised disorders of the upper and lower respiratory tract. Symptoms of upper airway obstruction were reported in CS neonates but were more commonly diagnosed in childhood/adulthood (71%). Analysis of HRAS mutations and their respiratory phenotype revealed that the common p.Gly12Ser mutation is more often associated with transient respiratory distress and other respiratory diagnoses. Respiratory failure and dependence on mechanical ventilation occurs almost exclusively with rare mutations. In cases of prenatally diagnosed CS, the high incidence of respiratory complications in the neonatal period should prompt anticipatory guidance and development of a postnatal management plan. This may be important in cases involving rarer mutations. Furthermore, the high frequency of airway obstruction in CS patients suggests that otorhinolaryngological evaluation and sleep studies should be considered. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)

Tracheal bronchus is a rarely seen congenital anomaly generally originating from the right lateral wall of the trachea and approximately 2 cm above the carina. It was firstly defined by Sandifort in 1785 and its frequency of incidence in normal population changes between 0.1% and 2%. There are two types called ''Supernumerary'' and ''Displaced''. It is a rarely seen kind of tracheal anomaly although fairly well defined. The cases accompanied by lung cancer are seen more rarely. Nine cases of this association were reported in literature and tracheal bronchus-lung cancer association whose pathological result is undifferentiated large-cell carcinoma has not been stated so far. We present a 75 years old male patient as possibly the first case having tracheal bronchus and large-cell carcinoma association in literature.

A five day old patient with mild VACTERL syndrome had repair of a type IV congenital laryngeal web with successful decannulation 76 days later. Voice and respiratory outcome is good with follow up 15 years later. This case presents a rare clinical finding of a type IV laryngeal web successfully repaired with a keel and subsequent long term follow up during an era when it was suggested that repair be delayed until 18 months of age at the earliest., (Copyright © 2016 Elsevier Inc. All rights reserved.)