Management and Outcomes of Patients With High-Risk (Congenital Lung Malformation Volume Ratio≥ 1.6) Congenital Lung Malformations.

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.

Esophageal lung complicated by recurrent pneumonia.

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.

Does Timing of Resection Influence the Presence of Inflammation within Congenital Lung Malformations?

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.

Impact of Previous Infection on Perioperative Outcomes of Thoracoscopic Lobectomy for Congenital Lung Malformation.

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.

Airway anomalies in patients with 22q11.2 deletion syndrome: A scoping review.

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.

Use of cryotherapy to treat obstructing papilloma of an accessory tracheal bronchus: case report.

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.

Effects of pulmonary fissure completeness on major outcomes in children after video-assisted thoracoscopic congenital lung malformation lobectomy.

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.

Tracheal bronchus: a rare cause of recurrent pneumonia in adults.

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.

Interstitial lung abnormalities and interstitial lung diseases associated with cigarette smoking in a rural cohort undergoing surgical resection.

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.

Reticulation Is a Risk Factor of Progressive Subpleural Nonfibrotic Interstitial Lung Abnormalities.

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.

Fetal lung regeneration using stem cell-derived extracellular vesicles: A new frontier for pulmonary hypoplasia secondary to congenital diaphragmatic hernia.

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.

Effects of the COVID-19 Pandemic on Pediatric Nasal Fractures.

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.

Surgical management in tetralogy of Fallot with rare unilateral pulmonary anomalies: A literature review.

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.

Congenital bronchial atresia complicated by a lung abscess due to Aspergillus fumigatus: a case report.

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.

Pulmonary growth abnormalities as etiologies for pediatric pulmonary hypertension.

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.

Prenatal Imaging to Predict Need for Urgent Perinatal Surgery in Congenital Lung Lesions.

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.

Bridging bronchus (pseudocarina) and left pulmonary artery sling: A case report and literature review.

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.

Respiratory viral panels and pediatric airway evaluation: The role of testing for upper respiratory infections to stratify perioperative risk.

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.