Congenital Lung Malformations Research Articles

Current approaches to prenatal imaging of lung malformations: a review

INTRODUCTION: Congenital lung malformations are result of developmental disorders at any stage of organ formation. Manifestations of these changes vary significantly: from minimal in size to large space-occupying lesion of the lung, which can cause fetal death or severe postnatal respiratory disorders. In proper time and accurate prenatal diagnosis of lung development abnormalities is one of the fundamental factors determining management of pregnancy and prognosis.OBJECTIVE: To demonstrate the most typical features of a wide range of congenital lung anomalies by ultrasound and MRI.MATERIALS AND METHODS: The literature search was performed in open Russian and English databases Medline, PubMed, Web of Science, RSCI, eLIBRARY using keywords and phrases: «prenatal diagnosis», «fetal MRI», «ultrasound diagnostics», «сongenital lung malformations».RESULTS: Based on literature data and our own experience in the use of ultrasound and MRI in diagnosis of сongenital lung malformations, the main signs of normal development and the most specific types of lung anomalies are described. Algorithm for diagnostic search for congenital lung malformations is presented.CONCLUSION: Ultrasound is the main method for diagnosing congenital lung anomalies. Fetal MRI is used to clarify the nature and extent of changes, a more detailed assessment of adjacent organs, also to determine Multiple congenital anomalies. Understanding the characteristics of semiotics of lung malformations and the main signs of these anomalies is a fundamental criterion for prenatal counseling, as well as peri- and postnatal management of this category of patients.

Thoracoscopic Lobectomy for Congenital Lung Malformation in Children: Evolving Management Strategies and Their Impact in Outcomes

BackgroundThe aim of this study was to determine the incidence of complications after thoracoscopic lobectomy in children according to a verified classification. Secondary aim was to evaluate the modifications made in our management protocol in order to decrease the rate of complications. MethodsRetrospective study of pediatric patients in whom a lobectomy was performed for congenital lung malformation (CLM) in our institution between 2003 and 2021. The following data were collected: demographics, preoperative clinical symptoms, surgical technical details, and postoperative complications following the Clavien-Dindo (CD) classification. Main outcome measure was the presence of complications in the first month after lung lobectomy. Results90 lobectomies were performed in 89 patients. There were 46 girls (51.6%) and 43 boys with a median age of 12.4 months. Most patients (86.6%) remained asymptomatic until the surgical procedure. Postsurgical complications were detected in 26 cases (28.8%) and distributed into their respective category according to CD classification. In our experience, closure of the bronchial stump with interrupted suture or placement of an endoloop showed a statistically significant association with complications (p = 0.022 and p = 0.006 respectively). Moreover, patients in which a device combining sealing and section was used showed significantly fewer complications (p = 0.006). ConclusionsThoracoscopic lobectomy continues to be a challenging procedure. The CD grading system for postoperative complications has proved to be useful in this setting. Evolving surgical strategies and new miniaturized endosurgical devices have enabled a safer and quicker procedure with a positive impact in the development of complications. Level of EvidenceIII.

Simultaneous unilateral thoracoscopic resection of bilateral pulmonary sequestration

BackgroundIntra-lobar (ILS) and extra-lobar lung (ELS) sequestrations represent rare congenital lung malformations. Despite their benign nature, the lesions pose risks such as recurrent pulmonary infections, hemoptysis, congestive heart failure, and tumor development. Pulmonary sequestration (PS) typically manifests in two forms, ILS and ELS, with bilateral occurrence being exceptionally rare and mostly requiring bilateral thoracic surgery.Case presentationA 9-year-old child, who initially presented with bilateral lung lesions without respiratory symptoms, was diagnosed with PS following a chest computed tomography scan. The surgical approach was determined based on the absence of inflammation and the clear demarcation of the lesions from normal lung tissue, highlighted by a unique tissue connection between the ILS and ELS across the chest cavities. We used a novel method wherein the left ELS was successfully pulled into the right chest cavity and both sequestrations were concurrently resected. Postoperative recovery was smooth, with no complications or residual lesions.ConclusionsOur findings highlight the importance of thorough preoperative planning with enhanced computed tomography. Simultaneous unilateral thoracoscopic surgery can be a viable, less invasive option for treating bilateral PS, offering benefits such as reduced recovery time and better cosmetic outcomes.

Association of Prenatal Congenital Lung Malformation Volume Ratio to Postnatal Computed Tomography Characteristics

IntroductionCongenital lung malformations (CLMs) are diverse and readily diagnosed on prenatal ultrasound. Postnatal computed tomography (CT) characteristics, including volume, are used in centers for the clinical decision-making of asymptomatic CLM. We aim to evaluate the relationship of prenatal CLM volume ratio (CVR) to postnatal CT characteristics by suspected prenatal diagnosis and postnatal radiological diagnosis. MethodsWe performed a single-center retrospective review of all patients evaluated with prenatally diagnosed CLM (May 2015-December 2022). Demographics, prenatal imaging findings at initial evaluation, and postnatal radiological diagnosis/imaging findings were analyzed with descriptive statistics. Pairwise correlation coefficient tests were performed to analyze the correlation between prenatal CVR and postnatal CT lesion size stratified by diagnosis. ResultsOf 123 patients referred and evaluated, suspected prenatal diagnoses consisted of 68 bronchial atresia (BA), 20 intralobar bronchopulmonary sequestration (iBPS), 20 extralobar bronchopulmonary sequestration (eBPS), and nine congenital pulmonary airway malformation (CPAM). Postnatal radiological diagnoses consisted of 53 BA, 22 iBPS, 14 eBPS, and 20 CPAM. Overall correlation coefficient of prenatal CVR to postnatal CT lesion size volume was 0.56. By suspected prenatal diagnosis, correlation coefficients were 0.61 (BA), 0.59 (iBPS), 0.29 (eBPS), and 0.51 (CPAM). For postnatal radiological diagnosis, correlation coefficients were 0.58 (BA), 0.56 (iBPS), 0.33 (eBPS), and 0.62 (CPAM). ConclusionsOur results indicate that initial CVR is overall consistent with the postnatal CT lesion size. This correlation is present in suspected prenatal diagnoses of BA and iBPS and postnatal radiological diagnoses of BA, iBPS, and CPAM. Additional studies analyzing long-term follow-up should be conducted to specify the safety of patients who undergo observation rather than surgical intervention.

Postnatal chest X-ray in children with asymptomatic congenital lung malformations.

The clinical implications of a postnatal chest X-ray (CXR) in asymptomatic children with a prenatally diagnosed congenital lung malformation (CLM) are uncertain. We assessed the justification for the postnatal use of CXR in these children. We included patients with CLM confirmed through chest computed tomography angiography or histopathological analysis who were asymptomatic at birth, underwent routine postnatal CXR, and participated in our standard of care prospective structured longitudinal follow-up program. Children with major associated morbidities were excluded. Primary outcomes were the positive and negative predictive values (PPV and NPV) of CXR findings for symptom development at 4 weeks and 6 months of age. Secondarily, we sought to establish whether CXR findings were associated with undergoing additional diagnostics during the initial observational hospital stay or prolonged postnatal hospital admission. Among 121 included patients, CXR showed no abnormalities in 35 (29%), nonspecific abnormalities in 23 (19%), and probable CLM in 63 (52%). The PPV of CXR in relation to symptom development at 4 weeks and 6 months was 0.05 and 0.25, respectively. Corresponding NPVs were 0.96 and 0.91. An association was identified between CXR findings and undergoing further diagnostics during the initial observational hospital stay (p = .047). Additional diagnostic findings did not influence clinical management. CXR findings were not associated with prolonged initial hospital stay (p = .40). The routine practice of postnatal CXR in asymptomatic patients with prenatally diagnosed CLM can be omitted, as CXR findings do not influence subsequent clinical management.

Clinical and histopathological spectrum of congenital pulmonary airway malformations: A case series

BackgroundCongenital pulmonary airway malformations (CPAM) are a spectrum of cystic and non-cystic anomalies arising from abnormal airway development in utero, with an incidence of 1 in 25,000 to 35,000 births. CPAM can present prenatally or postnatally with respiratory distress, recurrent infections, or occasionally as an incidental finding. This case series aims to highlight the clinical, radiological, and histopathological characteristics of CPAM through three pediatric cases, which include types 1, 2, and 3 CPAM. Case presentationCase 1: A four-month-old male presented with cough, cold, and respiratory distress. Imaging revealed hypoattenuation and overinflation of the left upper lobe with mediastinal shift. Left upper lobectomy confirmed CPAM type 2.Case 2: A one-month-old female presented with recurrent respiratory distress and infections. Imaging showed a large cystic lesion in the right middle lobe. Right middle lobectomy confirmed CPAM type 3.Case 3: A two-month-old male presented with dyspnea. Imaging showed a large bullae in the right chest with mediastinal shift. Right upper lobectomy confirmed CPAM type 1. Clinical discussionCPAM is a rare congenital lung malformation characterized by abnormal bronchial development and localized glandular overgrowth. Management involves surgical resection, with the timing of surgery dependent on symptomatology. Early identification and intervention are crucial for preventing complications such as pulmonary hypoplasia and recurrent infections. Histopathological examination post-resection is essential for accurate classification and management of CPAM. ConclusionThis case series provides valuable insights into the clinical, radiological, and histopathological features of CPAM, including the types encountered in each case. It underscores the importance of early diagnosis and timely surgical intervention, advocating for routine prenatal and postnatal screenings to effectively identify and manage CPAM.

CPLANE protein INTU regulates growth and patterning of the mouse lungs through cilia-dependent Hh signaling

Congenital lung malformations are fatal at birth in their severe forms. Prevention and early intervention of these birth defects require a comprehensive understanding of the molecular mechanisms of lung development. We find that the loss of inturned (Intu), a cilia and planar polarity effector gene, severely disrupts growth and branching morphogenesis of the mouse embryonic lungs. Consistent with our previous results indicating an important role for Intu in ciliogenesis and hedgehog (Hh) signaling, we find greatly reduced number of primary cilia in both the epithelial and mesenchymal tissues of the lungs. We also find significantly reduced expression of Gli1 and Ptch1, direct targets of Hh signaling, suggesting disruption of cilia-dependent Hh signaling in Intu mutant lungs. An agonist of the Hh pathway activator, smoothened, increases Hh target gene expression and tubulogenesis in explanted wild type, but not Intu mutant, lungs, suggesting impaired Hh signaling response underlying lung morphogenetic defects in Intu mutants. Furthermore, removing both Gli2 and Intu completely abolishes branching morphogenesis of the lung, strongly supporting a mechanism by which Intu regulates lung growth and patterning through cilia-dependent Hh signaling. Moreover, a transcriptomics analysis identifies around 200 differentially expressed genes (DEGs) in Intu mutant lungs, including known Hh target genes Gli1, Ptch1/2 and Hhip. Genes involved in muscle differentiation and function are highly enriched among the DEGs, consistent with an important role of Hh signaling in airway smooth muscle differentiation. In addition, we find that the difference in gene expression between the left and right lungs diminishes in Intu mutants, suggesting an important role of Intu in asymmetrical growth and patterning of the mouse lungs.

Retrospective evaluation of neonates with fatal congenital lung malformation: A single center 15-year forensic autopsy experience.

Congenital lung malformation (CLM) is a leading cause of infant mortality. Clinical methods for diagnosing CLM mainly rely on computed tomography, magnetic resonance imaging, ultrasonography, and Doppler. However, forensic identification of the cause of death in neonates is challenging. Unequivocal classification criteria for CLM are missing as its forensic identification is ambiguous. Therefore, we aimed to analyze neonatal death cases at our center to assist in identifying those with congenital lung malformation. This retrospective study identified and classified the causes of deaths of neonates autopsied between January 2008 and April 2023. All cases born alive and died within 28 days with a clear time of death were selected, and forensic experts reviewed their records. The manner, cause of death, and other characteristics were noted and discussed. This retrospective study reveals a steady increase in autopsy cases from 2008 to 2015, attributed to improved parental consent, heightened awareness of autopsy importance, and enhanced medical resources. However, a subsequent decline post-2015 is observed, potentially influenced by advancements in medical technology and prenatal examination protocols. The top causes of neonatal mortality include respiratory diseases, asphyxia, congenital dysplasia, and fetal distress. Congenital lung malformations, particularly bronchopulmonary malformations, constitute a significant portion of congenital anomalies. This study underscores the importance of standardized autopsies and histopathological examinations in diagnosing and understanding CLM. Future research should focus on expanding case collections and elucidating the genetic basis of CLM to improve forensic management and outcomes.

High Dependency Unit (HDU) requirement following thoracoscopic resection for congenital lung malformation

High Dependency Unit (HDU) requirement following thoracoscopic resection for congenital lung malformation

Role of Multidetector Computed Tomography with Contrast in Evaluation of Congenital Lung Malformations in Pediatric Age Group at a Tertiary Care Hospital: An Observational Study

Background: Computed tomography (CT) has a larger role in the noninvasive examination of congenital lung abnormalities, thanks to the introduction of multidetector CT (MDCT). If a surgical lesion is suspected, it is often necessary to use cross-sectional imaging, such as CT, for diagnosis confirmation, further lesion definition, and preoperative evaluation. Aims and Objectives: The aim of this study was to enhance comprehension of the occurrence and varieties of congenital lung abnormalities in children, as well as to assess and compare the diagnostic precision of MDCT with that of surgical intervention. Methodology: MDCT with contrast CT was performed in children with respiratory complaints and prenatal history of suspicion in targeted imaging for fetal anomalies (TIFFA) or congenital lung malformations (CLMs) using TOSHIBA 8 slice CT; the study population consisted of 40 pediatric patients with a history of chest anomalies during pregnancy in TIFFA or children with abnormal chest X-ray suspicion of lung anomalies for 18 months. Results: In a study of 40 pediatric patients who underwent MDCT with contrast to evaluate CLMs, 6 were excluded from further evaluation with contrast due to the absence of pulmonary anomalies on plain CT, leaving a possible diagnosis of s CLM in the remaining 34 cases. Of these, cystic pulmonary airway malformations (CPAM) (41.1%) were the most common, followed by congenital lobar emphysema (17.6%), bronchogenic cyst (14.7%), lung agenesis (11.6%), and phylogenetic lung. Conclusion: To notice the characteristics and related anomalies of CLMs and aid in the presurgical evaluation of CLMs, the study concludes that MDCT with contrast is the imaging of choice.

Congenital Lung Malformations: A Pictorial Review of Imaging Findings and a Practical Guide for Diagnosis.

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.

A Clinical Comparative Study of Thoracoscopic Anatomical Lesion Resection and Lobectomy in the Treatment of Congenital Lung Malformations

BackgroundTo explore the safety and feasibility of HuaXi thoracoscopic anatomical lesion resection (HX-TALR) in the treatment of congenital lung malformations (CLMs) in children. MethodsA retrospective review of clinical data was conducted for patients who underwent HX-TALR and thoracoscopic lobectomy (TL) in our hospital from October 2017 to March 2023. Intraoperative and postoperative outcomes were compared between the HX-TALR and TL groups. ResultsThere were 485 patients in this study, 267 of whom underwent HX-TALR and 218 of whom underwent TL. All patients underwent thoracoscopic surgery without conversion to open surgery. No patients had major complications, including bronchopleural fistula, hemorrhage, atelectasis, recurrence or reoperation. The operative time, intraoperative bleeding volume, cases with thoracic drainage tubes, postoperative hospital stay, and cases with postoperative fever in HX-TALR were greater than those in TL (P < 0.05). ConclusionsHX-TALR is safe, feasible, and retains all normal lung tissue while removing the lesion, which is expected to become the preferable operation for the treatment of CLMs. HX-TALR is a new and technically challenging procedure that needs to be carried out after training. Level of EvidenceLevel IV.

Operative Volumes and Postoperative Outcomes After Elective Resection of Congenital Lung Malformations in Infants.

To determine whether hospital- and surgeon-level operative volumes are associated with differences in postoperative outcomes among infants undergoing elective lung surgery for a congenital lung malformation (CLM). Infant lung surgery is a relatively uncommon procedure performed by pediatric surgeons nationwide. The relationship between surgical volume and postoperative outcomes remains unknown. A retrospective cohort study of asymptomatic infants who underwent elective lung resection of a CLM was conducted using the Pediatric Health Information System database (2016-2020). Multivariable linear and poisson regressions were performed based on annual lung resection tertiles. There were 1420 infants managed by 48 hospitals and 309 primary surgeons. Institutions that performed seven or fewer CLM resections per year (56%) were associated with significantly higher postoperative complication rates compared to medium- and high-volume hospitals (low: 134 [34%], medium: 110 [21%], high: 144 [29%]; P<0.001). Surgeons who performed one or fewer CLM resections per year (82%) were associated with significantly higher complication rates compared to medium- and high-volume surgeons (low: 171 [31%], medium: 75 [26%], high: 119 [24%]; P=0.02). Multivariable analyses confirmed that low-volume hospitals were associated with higher complications (OR 1.81, CI 1.38-2.37; P<0.001), and low-volume surgeons had an increased risk of complications (overall: OR 1.37, CI 1.01-1.84; P=0.04). In this cohort study of infants undergoing elective lung resection for a CLM, lower volume providers were associated with higher postoperative patient morbidity. These findings represent an opportunity to inform quality improvement initiatives on pediatric lung resection and the debate on surgical subspecialization for this unique patient population.

The impact of undergoing elective pediatric lung resection during respiratory syncytial virus peak season on patient outcomes: A nationwide retrospective analysis.

Observational data to support delaying elective pediatric thoracic surgery during peak respiratory viral illness season is lacking. This study evaluated whether lung surgery during peak viral season is associated with differences in postoperative outcomes and resource utilization. A retrospective observational cohort study was performed using the Pediatric Health Information System (PHIS). Patients with a congenital lung malformation (CLM) who underwent elective lung resection between 1 January 2016 and 29 February 2020 were included. Respiratory syncytial virus (RSV) incidence was used as a proxy for respiratory viral illness circulation. Monthly hospital-specific RSV incidence was calculated from PHIS data, and peak RSV season was defined by Centers for Disease Control data. Multivariable regression models were built to identify predictors of postoperative mechanical ventilation, which was the main outcome measure, as well as secondary outcomes including 30-day readmission after lung resection, postoperative length of stay (LOS) and hospital billing charges. Of 1542 CLM patients identified, 344 (22.3%) underwent lung resection during peak RSV season. 38% fewer operations were performed per month during peak RSV season than during off-peak months (p < .001). Children who underwent surgery during peak RSV season did not differ from the off-peak group in terms of age at operation, race, or comorbid conditions (i.e., congenital heart disease, newborn respiratory distress, and preoperative pneumonia). There was no association between hospital-specific RSV incidence at the time of surgery and postoperative mechanical ventilation, postoperative LOS, 30-day readmission rate or hospital billing charges. Performing elective lung surgery in children with CLMs during peak viral season is not associated with adverse surgical outcomes or increased utilization of healthcare resources.

Congenital lung malformations: a nationwide survey on management aspects by the Italian Society of Pediatric Surgery.

Over the years, congenital lung malformations (CLM) management remains a controversial topic in pediatric thoracic surgery. The Italian Society of Pediatric Surgery performed a national survey to study the current management variability among centers, trying to define national guidelines and a standardized approach of children with congenital lung malformations. Following a National Society approval, an electronic survey including 35 items on post-natal management was designed, focusing on surgical, anesthesiology, radiology and pneumology aspects. The survey was conducted contacting all pediatric surgical units performing thoracic surgery. 39 pediatric surgery units (97.5%) participated in the study. 13 centers (33.3%) were classified as high-volume (Group A), while 26 centers (66.7%) were low volume (Group B). Variances in diagnostic imaging protocols were observed, with Group A performing fewer CT scans compared to Group B (p = 0.012). Surgical indications favored operative approaches for asymptomatic CLM and pulmonary sequestrations in both groups, while a wait-and-see approach was common for congenital lobar emphysema. Surgical timing for asymptomatic CLM differed significantly, with most high-volume centers operating on patients younger than 12months (p = 0.02). Thoracoscopy was the preferred approach for asymptomatic CLM in most of centers, while postoperative long-term follow-up was not performed in most of the centers. Thoracoscopic approach seems uniform in asymptomatic CLM patients and variable in symptomatic children. Lack of uniformity in surgical timing and preoperative imaging assessment has been identified as key areas to establish a common national pattern of care for CLM.

Clinicopathological and genetic characteristics of congenital cystic adenomatoid malformation of lung and its associated lung cancer in adults

Objective: To investigate the clinicopathological features and genetic characteristics of congenital cystic adenomatoid malformation (CCAM) of lung and CCAM associated lung cancer in adults. Methods: A total of 13 cases of CCAM of lung in adults, diagnosed from June 2015 to May 2023, were collected from the Department of Pathology, Shanghai Pulmonary Hospital, Tongji University School of Medicine, China. Their histopathological features were correlated with probable development into lung cancer. Next-generation sequencing was performed on the benign and malignant areas of all cases. Results: The pathological classification of all cases were of CCAM of lung type 1. There were 4 male and 9 female cases, age ranged from 18 to 65 years, with a mean age of 41 years. Six cases were accompanied by lung cancer, all of them were mucinous adenocarcinoma. Next-generation sequencing showed no gene mutation in 2 of the 13 cases; KRAS mutations in exon 2 were detected in 7 cases, in which there were 6 cases complicated with lung mucinous adenocarcinoma and no matter in the malignant or benign regions, the same case exhibited the same mutation sites in KRAS gene. Conclusions: CCAM of the lung is a congenital disease, and in adults, type 1 is most commonly found in the pathological classification, and it is often accompanied by cancer. Gene mutations are frequently detected in CCAM of the lung, KRAS being the most recurrent mutation which may play an important role in the carcinogenesis.

The clinical features and operation experience of multilobar involved congenital lung malformation: A retrospective cohort study.

Congenital lung malformation (CLM) is usually characterized by single-lobe involvement and multilobe involvement is uncommon. There is a lack of experience in the clinical features and surgical treatment of unilateral multilobar CLM. Therefore, this study aims to summarize the clinical characteristics and evaluate the safety and feasibility of thoracoscopic lung sparing resection in the treatment of unilateral multilobar CLM. A retrospective study was conducted on 34 patients with unilateral multilobar CLM, and 34 unilobar patients were randomly selected as the control group in West China Hospital of Sichuan University from 2014.1 to 2021.1. Clinical, operation and follow-up outcomes were compared between the two groups. Twelve (35.2%) patients developed preoperative symptoms in the multilobar group more than 4(11.7%) the unilobar group (p = 0.02), the main preoperative symptom was infection; The multilobar group and unilobar group showed significant differences in mean intraoperative blood loss (13.3 mL vs. 7.5 mL; p = 0.02) and mean surgical time (95.6 min vs. 47.5 min; p = 0.037). The median length of postoperative hospital stay and the median chest tube placement time in the multilobar group were significantly longer (5 d vs.3 d, p = 0.045; 2 d vs.1 d, p = 0.031). There were one (2.9%) patient in unilobar group and 4(11.7%) patients in multilobar group developed complications postoperatively, which is no significant differences between the two groups(p = 0.16). No complications such as thoracic deformity, mediastinal deviation, scoliosis or recurrence were observed in either group at follow-up. Unilateral multilobar CLM cases are more susceptible to develop symptoms than unilobar cases. Thoracoscopic lung sparing resection in the treatment of unilateral multilobar CLM has high safety and can effectively avoid serious complications of unilateral multiple lobectomy or pneumonectomy.

Clinical and morphological characteristics of lung sequestration

To study the clinical and morphological manifestations of lung sequestration. Surgical material (lung tissue) of 18 patients (2017-2021) and 8 archival observations (1972-1994) of confirmed lung sequestration were examined. Histological staining (hematoxylin and eosin, Schiff reagent, Ziehl-Neelsen, picrofuchsin according to Van Gieson) and immunohistochemical reactions with antibodies to SMA, TTF1, CK5/6 (DAKO) were performed. The ratio of men to women was 5:4; the average age of the patients was 35 years. Lung sequestration is localized mainly in the lower lobe segments, the left lung is involved in the pathological process more often. The cystic variant of lung sequestration with a chronic inflammatory process prevailed. Histological examination revealed the similarity of lung sequestration with congenital adenomatous lung malformation type 2 and rhabdomyomatous dysplasia. The respiratory and bronchial epithelium lost differentiation in the sequestered area. The morphological picture of lung sequestration is nonspecific and can manifest itself in various types of inflammatory reactions and remodeling of lung tissue.

Congenital Pulmonary Airway Malformation in a Retroviral Exposed Infant: A Case Report and Review of Literature

Abstract Congenital pulmonary airway malformation (CPAM) is a rare congenital lung malformation accounting for 25% of congenital malformations and 95% of congenital lung lesions. Diagnosis prenatally is by ultrasonography while postnatal diagnosis follows presentation with respiratory distress symptoms and radiographic imaging. Although they are often misdiagnosed radiologically, most cases of CPAM are manageable with proper assessment, diagnosis and interventions.

Pediatric thoracoscopic lung resections: a comprehensive analysis of congenital lesion cases

Background Congenital lung lesions in pediatric patients may be managed conservatively or by video assisted thoracoscopic surgery (VATS). This study aimed to determine the complications after VATS for congenital lung lesions in children. Methods All children undergoing a lung resection between January 2009 and June 2022 were retrospectively identified. Children undergoing a primary open lobectomy or a resection other than a congenital lung lesion were excluded. Both early (<30 days) and late postoperative pulmonary complications were determined. The primary endpoint was postoperative complications within 30 days. Results In total, 56 patients were included, with a median age of 13 months (IQR 9-37). A VATS lobectomy were performed in 46 patients (82%), an extralobar sequestration in 8 patients (14%), an wedge resection in 1 patient and a segment resection in 1 patient. During the COVID pandemic, fewer resections were performed with an increase in symptomatic patients. A conversion to open occurred in 6 patients (11%), of which a preoperative lung infection was associated with an increased risk thereof(p = 0.004). The median follow-up was 22 months (IQR 7-57) and all patients were alive. A postoperative complication (Clavien Dindo ≥3) occurred in 9 patients and complications without the need of intervention in 6 patients. During follow-up a pneumonia occurred in 11 patients. Conclusion There seems to be a shift towards delayed surgery with an increase in symptomatic congenital lung malformations, which might lead to an increase in postoperative complications