Malformation Of Lung Research Articles

Congenital Lung Malformations: Outcomes after Minimally Invasive and Open Surgery in Infancy.

The management of congenital lung malformations (CLM) remains controversial. The aim of this study was to analyze the timing, approach, and outcomes of surgical treatment in children with CLM.A cohort study was conducted comparing children with CLM who underwent thoracoscopic resection with open resection via thoracotomy. All children were treated according to an institutionalized multidisciplinary coordinated treatment algorithm.Between 2002 and 2019, 68 children with CLM were treated. Thoracoscopic resection of CLM (n=44) resulted in a statistically significant longer operative time (mean 169 vs. 97 minutes, p=0.04) but shorter hospital stay (mean 7.2 vs. 16.7/12 days, p=0.01) compared to thoracotomy (n=15) or after conversion to thoracotomy (n=9). There were no major complications in either group. At a mean follow-up of 32.2 months (range 0.5-163), patients after thoracoscopic resection had statistically significantly fewer rip fusions than patients after open resection (n=0 vs. 2/2, p≤0.004) and less chest wall asymmetry than after conversion (n=0 vs. 2, p=0.004).Thoracotomy proved to be a fast and safe surgical approach in respiratory unstable CLM patients. In respiratory stable CLM patients, thoracoscopic resection was feasible in early infancy with good surgical and musculoskeletal outcomes.Thoracoscopic resection of CLM offers advantages over resection by thoracotomy in terms of surgical recovery and musculoskeletal function. Therefore, it should be offered to respiratory-stable patients in specialized centers.

Standardized Note Template Expedites Completion of Consults for Surgical Fetal Anomalies.

We developed standardized electronic medical record templates (EMR-temp) for use in ambulatory prenatal surgical consultations for surgical fetal anomalies (SFAs). Our aim was to evaluate EMR-temp impact in provider documentation in prenatal care of SFA. Prenatal consultations for SFAs at a single institution were retrospectively reviewed (2019-2022). Disease-specific EMR-templates were developed. Note content and time to encounter closure (TEC) were collected. Descriptive statistics, chi-square, Fisher's Exact Test, and Wilcoxon rank sum tests were used. Seven hundred twenty-four prenatal consultations were analyzed, with 131 consultations (18%) using EMR-temp for 55 (42%) congenital diaphragmatic hernia, 50 (38%) congenital lung malformations, 9 (7%) neural tube defects, 8 (6%) gastroschisis, and 9 (7%) heterotaxy. Overall median TEC was 23.9hs [interquartile range (IQR) 1.3-128.6]. EMR-temp use decreases TEC from 28.4hs [IQR 1.6-159.4] to 2.07hs [IQR 0.85-76.2] (P<0.001). The impact of EMR-temp varies depending on pathology. In congenital diaphragmatic hernia, EMR-temp decreased TEC 61%, from 52.0 [IQR 2.6-171.1] to 20.3hs [IQR 1.5-55.5] (P=0.01). In neural tube defect, EMR-temp decreased TEC 98% from 48.6 [IQR 2.6-157.1] to 1.02hs [IQR 0.5-1.5] (P<0.001). There were no differences in TEC with EMR-temp use in congenital lung malformation, gastroschisis and heterotaxy (P>0.05). Across all diseases, EMR-temp had more comprehensive documentation of the following content: pathophysiology (92%), pregnancy planning (92%), delivery planning (84%), postnatal planning (95%), and long-term follow-up (90%) compared to nontemplates (P<0.01). EMR templates in SFA consultations improves encounter closure with comprehensive documentation of disease-specific pathophysiology and plans. However, impact varies across pathology, suggesting other factors are also important.

Elective thoracoscopic surgery for congenital lung malformations: what age to operate?

This study aimed to evaluate the characteristics of congenital lung malformations (CLMs) in patients from infancy to adulthood, and to determine the optimal timing for thoracoscopic surgery. All patients with CLMs who underwent thoracoscopic surgery between 2017 and 2023 were retrospectively reviewed. The patients were divided into five age groups: 1-6 months, 6-12 months, 1-4 years, 4-16 years, and >16 years. Clinical characteristics and surgical outcomes were compared and analyzed for variance among the age groups. A total of 173 patients with CLM were included. Thirteen (7.5%) patients were categorized in the 1-6 months age group, 44 (25.4%) in the 6-12 months age group, 58 (33.5%) in the 1-4 years age group, 30 (17.3%) in the 4-16 years age group, and 28 (16.2%) in the >16 years age group. The median age at surgery was 2.3 years (range: 1 month to 74 years). The operative time was shortest in the 1-4 years age group at 86.5 min (interquartile range: 57.3-131.5 min, p < 0.01). The results of the multivariate regression analysis indicated that age >4 years (odds ratio [OR]: 8.70, p < 0.01) and previous infection (OR: 3.75, p < 0.01) increased the risk of operative adhesions, which increased the risk of major complications and conversion to open thoracotomy. Thoracoscopic surgery for CLMs is safe and feasible at all ages.Age < 4 yearsmay predict uneventful surgical outcomes due to fewer operative adhesions.

ESR Essentials: imaging of common paediatric pulmonary diseases-practice recommendations by the European Society of Paediatric Radiology.

Chest imaging in children presents unique challenges due to varying requirements across age groups. For chest radiographs, achieving optimal images often involves careful positioning and immobilisation techniques. Antero-posterior projections are easier to obtain in younger children, while lateral decubitus radiographs are sometimes used when expiratory images are difficult to obtain and for free air exclusion. Chest CT protocols should be age-dependent to minimise radiation exposure and motion artefacts. MRI is primarily used in specialised centres to reduce radiation exposure, requiring specific expertise and sedation in younger children. Respiratory distress syndrome is a leading cause of morbidity in preterm neonates, diagnosed through characteristic radiographic findings and a history of prematurity. Bronchopulmonary dysplasia is the most common complication of extreme preterm birth and chronic oxygen therapy; imaging is used for predicting outcomes for the assessment of severe cases. Transient tachypnoea of the newborn and meconium aspiration syndrome are common in term infants, with specific imaging characteristics aiding in their differentiation. Congenital lung malformations present diagnostic and management challenges, with imaging playing a crucial role in diagnosis and surgical planning. Finally, imaging is essential in detecting complications from pneumonia in children, such as empyema and necrotic pneumonia, or in identifying foreign object aspiration. CLINICAL RELEVANCE STATEMENT: This review summarises current radiology practice of paediatric chest pathologies, aiding in the accurate diagnosis and management of neonatal and congenital pulmonary conditions and pneumonia complications, ultimately improving patient outcomes through precise imaging interpretation and targeted clinical intervention. KEY POINTS: Chest radiographs should be systematically assessed for pathology. Ensure accurate differential diagnosis of neonatal lung diseases by collecting information on gestational age, method of delivery, presenting symptoms, ventilation type, and fetal ultrasound findings. Radiographs and ultrasound are initial diagnostic tools for paediatric pulmonary disease; CT should be reserved for complex cases. Referral to paediatric hospital should be considered when the use of chest MRI is indicated.

A Series of 40 Congenital Lung Malformation Cases and the Informative Value of CPAM Lesion Ratios.

In this single-center retrospective analysis, we present case data and insights gathered over the past eight years. Additionally, we computed postnatal, pre-therapy lesion-to-lung ratios of Congenital Pulmonary Airway Malformations (CPAMs) to retrospectively assess potential outcome prediction using lesion-to-lung ratios. Data were collected between 2015 and 2022. Information such as chosen therapy, surgical duration, postoperative hospital stay, and follow-up was obtained from electronic case records. Pre-therapy pulmonary lesion volumes [mm3], lesion-to-ipsilateral-lung ratio, and lesion-to-both-lungs ratio of CPAMs were retrospectively calculated from computed tomography images using specialized software. Of the 40 identified cases, 27 had CPAM, 7 had pulmonary sequestration, 4 had bronchogenic cysts, and 2 had congenital lobar emphysema. Histological examinations of resected specimens revealed no malignancy. For CPAMs, the median surgery age was 7 months (interquartile range (IQR): 0.45-11), averaging 9.54 ± 15.01 months. The CPAM surgery lasted on average 126 ± 53 min (median 124 min (IQR 108-172)). The mean length of hospital stay was 6 ± 1.41 days for thoracoscopic surgery and 17 ± 18.23 days for open surgery. No clear link was found between the lesion ratio and management choice or surgical length. Notably, larger lesions tended to result in longer postoperative stays. CPAMs with a lesion-to-ipsilateral-lung ratio of ≤0.11 were asymptomatic. If patients present no symptoms, mild symptoms, or smaller CPAM lesions, "wait and watch" and a CT scan of the thorax up to approximately six months of age remain a reasonable approach. The true risk of malignancy remains ambiguous, especially since there was no evidence of malignancy in our biopsies. On the other hand, prophylactic surgery before symptoms arose led to earlier discharge and overall low intraoperative complications compared to symptomatic counterparts. Ultimately, the adopted therapy pathway remains a parental choice. For CPAMs, an increased lesion-to-lung ratio correlated with extended hospital stay and symptomatic presentation. However, there was no cut-off value for conservative or surgical treatment.

Fluorescence imaging-assisted thoracoscopic anatomical lesion resection in treating congenital lung malformation

Identification of lesion demarcation during thoracoscopic anatomical lesion resection is fundamental for treating children with congenital lung malformation. Existing lesion demarcations do not always meet the needs of clinical practice. This study aimed to explore the safety and efficacy of near-infrared fluorescence imaging with nebulized inhalation of indocyanine green for thoracoscopic anatomical lesion resection in children with congenital lung malformation. Under the fluorescence scope, the area of uniform green fluorescence was produced when indocyanine green was distributed into normal lung tissue, and lesions showed little or no green fluorescence, thus delineating a clear fluorescent lesion demarcation. Under the guidance of fluorescent demarcation, all the patients except one case of extralobar sequestration underwent thoracoscopic anatomical lesion resection successfully.

Management of chest tube after thoracoscopic lung resection in children with congenital lung malformation: a multicenter retrospective study

This study aimed to investigate the safety and effect of omitting chest tubes after thoracoscopic lobectomy in children with congenital lung malformation. A multicenter retrospective study was performed with 632 thoracoscopic lobectomy CLM patients in four hospitals between 2014.1 and 2023.1, which were divided into non-chest tube (NCT)group and chest tube (CT)group. Baseline data, operation and follow-up outcomes were compared. In total, 312 patients were included in the NCT group, and 320 in the CT group. There were no statistically significant differences in baseline data between the two groups. The FLACC scale score in the NCT group was less than the CT group (2.7 ± 0.43 vs. 5.8 ± 0.26 p = 0.027). The median length of postoperative hospital stay in the CT group was significantly longer than the NCT group (5 d vs.3 d, p = 0.045). Eight (2.5%) patients developed chest tube related infections in the CT group(p = 0.004). Six patients developed atelectasis in the NCT group, which was significantly less than the 18 patients in the CT group(p = 0.014). No chest tube placement in selected CLM pediatric patients may be safe and avoid chest tube-related complications, which may also contribute to a rapid recovery.

Factors affecting inflammatory changes in congenital lung malformations.

Patients with congenital lung malformation (CLM) may present pulmonary inflammatory changes. However, little is known about the factors influencing local inflammation. The aim of this study was to evaluate the factors that may affect inflammatory changes in CLM. Patients with CLM operated upon between 2005 and 2021 were included. The grade of inflammation was defined with a purpose-made inflammatory score (IS) ranging from 0 to 5. The association of type of CLM and age at surgery with IS was analyzed. Results are means (standard deviation). Data from 105 patients with CLM were collected, 56 had congenital pulmonary airways malformation (CPAM), 24 bronchopulmonary sequestration (BPS), and 25 congenital lobar emphysema (CLE). 91 patients (87%) had inflammatory changes. IS was 2.1 (1.5), 1.2 (1.0), and 1.3 (1.5) in CPAM, BPS, and CLE respectively (One-way ANOVA p = 0.0101). CPAM showed a significantly higher IS as compared with BPS (p = 0.0242) and CLE (p = 0.0495). Age at operation significantly correlated to IS (r2 = 0.14; p < 0.0001). Patients aged below 6months at operation had lower IS [1.4 (1.2)] as compared to those over 6months [2.0 (1.6)] (p = 0.018). Age at operation significantly correlated with the IS in CPAM (r2 = 0.17; p = 0.0016) and CLE (r2 = 0.47; p < 0.0001) patients. Patients with CLMs often present inflammatory changes in their lungs. Grade of inflammation significantly correlates with age at surgery and type of anomaly, with CPAMs having the highest grade. These findings support early resection in patients with CLM, especially in case of CPAM.

Respiratory Morbidity Among Children Undergoing Surgical Resection for a Congenital Lung Malformation.

Congenital lung malformations (CLMs) are the most common condition requiring lung surgery in children. Although surgical resection is generally well tolerated in the immediate postoperative period, long-term outcomes are not well studied. In this paper, we sought to define the risk of childhood respiratory morbidity, specifically asthma and pneumonia, in patients who underwent CLM resection. After IRB approval, a retrospective study was conducted on all pediatric CLM resections performed at a single tertiary care children's hospital between January 2013 and December 2022. The primary outcome measures were the diagnosis of asthma and/or pneumonia more than 30 days after resection. Clinical and demographic characteristics were evaluated in univariate analysis and multivariable logistic regression as appropriate (p < 0.05). Of the 54 patients who underwent CLM resection, 36 (67%) met inclusion criteria. The median age at resection was 6.2 months (IQR: 4.3-9.0), and the median follow-up period was 3.7 years (IQR: 1.7-5.7). Twelve were subsequently diagnosed with asthma at a median age of 3.3 years (IQR: 2.1-4.5). These observed asthma rates were significantly higher than expected when compared to nonlung surgery controls (33% vs. 6%, p < 0.01). Seven (21%) children were diagnosed with pneumonia, but this was not significantly different. These data suggest that children undergoing lung resection for a CLM may be at an increased risk for the development of early childhood asthma. Given the potential implications for preoperative counseling and postoperative follow-up, multicenter studies to validate these findings are warranted.

Thoracoscopic anatomical lesion resection by fluorescence imaging on congenital lung malformation

Thoracoscopic anatomical lesion resection (TALR) is characteristic of removal of the lesion and preservation of all normal lung in treating congenital lung malformation(CLM). However, to conduct TALR is technically demanding for a beginner. To make the procedure easier to understand, the details for TALR are demonstrated in this report. TALR technique for CLM consisted of eight steps: step 0, ICG inhalation; step 1, mark the external lesion border; step 2, dissociate of the trunk of pulmonary vain; step 3, dissociate the pulmonary vain plane; step 4, split the parenchyma; step 5, dissect the tubular structures running into the lesion; step 6, remove the lesion; step 7, seal the wound, flush the thoracic cavity with normal saline, reinflate the lung to make sure there is no large air leak, then place 16 F chest tube. The external lesion boundary is delineated via flurorescence imaging with inhaled indocyanine green (the normal lung displays flurorescent green while the lesion hardly shows green). TALR procedure are able to split the lung along the external border combined with the internal lesion border (pulmonary vain plane), during the process, all the tubular structure including vessels and bronchi running into the lesion can be separately sealed and divided. Thereafter, complete resection of the lesion and preservation of all normal lung can be achieved. TALR procedure is characteristic of dissection of the lesion along its borders, sealing and dividing the aberrant tubular structures, then the lesion can be exclusively removed and all the normal lung preserved, which may be considered as a preferable method in treating congenital lung malformation.

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

The 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. Retrospective 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. 90 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). Thoracoscopic 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. III.

7819 Hypoglycemia with Hereditary Hemorrhagic Telangiextsia in a Boy After Liver Transplantation

Abstract Disclosure: H. Miyagi: None. M. Igarashi: None. E. Tanimoto: None. R. Horikawa: None. Introduction: Hereditary hemorrhagic telangiectasia (HHT) causes arteriovenous malformation (AVM) in liver, lung, and brain. It is not clear whether it also causes hypoglycemia. We show a case of HHT combined with hypoglycemia after liver transplantation, improved by nutrition management and growth hormone treatment. Case: 11 months old boy born from a mother with HHT was diagnosed as having with AVM prenatally. He was once operated for the liver AVM but developed a new collateral circulation which caused severe heart failure and lead to liver transplantation. He showed severe growth retardation and malnutrition. His body weight was 4.6 kg and height were 60 cm (-5.8 SD) when he was 1 year old, and blood test showed low albumin for 3 g/dL (3.4 - 4.7 g/dL), and total protein for 4 g/dL (5.7 - 7.5 g/dL). Hypoglycemia was found while switching his nutrition from parenteral to enteral nutrition after operation. His insulin was under 0.3 µU/mL when his blood glucose was 49 mg/dL IGF1 was 17 ng/mL (ref.11 - 149 ng/mL), suggested growth hormone (GH) deficiency. GH stimulation test showed blunted response. GH treatment was started. Other pituitary hormones were within normal range. Nutritional support was added using high-calory milk. After GH treatment, IGF1 level increased to 87 ng/mL. GH and nutritional management improved not only his weight and height gain but also his glycemic control. Discussion: Hypoglycemia may be caused by both GHD and malnutrition. GHD and low IGF1 might be caused by malnutrition. Retesting GH secretion after improvement of nutrition is planned to clarify his pituitary function. Presentation: 6/2/2024

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

Congenital 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. We 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. Of 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). Our 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

Congenital 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 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. CPAM 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. This 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.

Surgical Management Of Congenital Thoracic Disorders: A 15-Year Center Experience.

Congenital thoracic disorders represent a spectrum of fetal lung bud development abnormalities, which may affect breathing capacity and quality of life. We aim to evaluate the impact of surgery in the treatment of 4 major congenital conditions. We performed a retrospective cohort analysis of patients who underwent surgical treatment in our tertiary center, from 2007 to 2022. Over the 15-year period, we treated 33 patients, with a male predominance of 55%. 22 patients (67%) were asymptomatic. When symptomatic, the recurrence of respiratory infections was the most common clinical presentation (18%). In 13 patients (39%), diagnosis was achieved through fetal ultrasonography. This study encompassed 13 patients with pulmonary sequestration (39%), 11 patients with bronchogenic cysts (33%), 7 patients with congenital pulmonary airway malformation (21%) and 2 patients with congenital lobar emphysema (6%). Considering solely lung malformation conditions, we accounted 22 patients with a median age of 3 [1-67] years-old. Surgery comprised bilobectomy (9%), lobectomy (77%), lobectomy with wedge resection (5%), segmentectomy (5%) and wedge resection (5%). Concerning bronchogenic cysts, we treated 11 patients with a median age of 19 [14-66] years-old. We identified 1 hilar, 1 intrapulmonary and 9 mediastinal lesions, of which 4 were paraesophageal, 4 were subcarinal and 1 was miscellaneous. Overall, surgery was conducted by thoracotomy in 61% of patients, VATS in 33% and RATS in 6%. The median drainage time was 3 [1-40] days and median hospital stay was 4 [1-41] days. There were no cases of mortality. Ensuing, 94% of patients experienced clinical improvement after surgery. Early diagnosis of congenital thoracic malformations increased considerably with the improvement in imaging technology and prenatal screening. Treatment may include expectant conservative treatment. However, in selected cases, surgery may play an important role in symptomatic control and prevention of disease progression.

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.