Cystic Adenomatoid Malformation Research Articles

Clinical Symptoms Affect Treatment and Prognosis in Pediatric Patients with Congenital Pulmonary Airway Malformation: A Propensity Score Matching Retrospective Cohort Study

ObjectivesSurgery for asymptomatic congenital pulmonary airway malformation patients is still debatable at this time. This study aims to investigate the safety and efficacy of surgery for asymptomatic patients, as well as the factors influencing the symptoms of this group of patients. MethodsAn institutional database was sampled for congenital pulmonary airway malformation patients. Patients were divided into the symptomatic group and the asymptomatic group. Propensity score matching (PSM) analysis selected patients in each group to compare perioperative outcomes. A multivariable logistic regression analysis was performed to investigate the potential influences on symptomatic lesions. ResultsThe asymptomatic group had better perioperative results than the symptomatic group, including shorter operating times (119.39 ± 49.42 min vs 100.73 ± 23.09 min, P = 0.031), shorter postoperative mechanical ventilation (2 h [0.5–46] vs 1 h [0.5–5], P = 0.002), shorter chest tube durations (4d [2-29] vs 3d [2-10], P = 0.007), and shorter postoperative hospital stays (10d [6–36] vs 8d [6-16], P < 0.001). With the conversion to thoracotomy and postoperative complications, there was no statistically significant difference between the two PSM-matched groups (P > 0.05). Age (p = 0.037), postnatal diagnosis (p = 0.018), and maximum cyst diameter (p = 0.032) were found to be independent variables associated with symptomatic lesions by multivariable logistic regression. ConclusionsPatients with congenital pulmonary airway malformation appear to have better perioperative outcomes before the beginning of symptoms. Symptomatic pulmonary lesions were associated with age, postnatal diagnosis, and maximum cyst diameter. Level of evidenceLevel III.

A Multi-Disciplinary Management of a Rare Case of a Complete Lung Congenital Pulmonary Airway Malformation in a Newborn from a Developing Country

A Multi-Disciplinary Management of a Rare Case of a Complete Lung Congenital Pulmonary Airway Malformation in a Newborn from a Developing Country

Application of second-generation sequencing in congenital pulmonary airway malformations

To investigate the differential expression of genes in whole transcripts of congenital pulmonary airway malformation (CPAM) using second-generation sequencing (also known as next-generation sequencing, NGS) technology. Children with CPAM were strictly screened after setting the criteria, and grouped by taking CPAM parietal tissue and CPAM lesion tissue respectively, and RNA-Seq libraries were established separately using second-generation sequencing technology, followed by differential expression analysis and GO (gene ontology) functional enrichment analysis, KEGG (Kyoto encyclopedia of genes and genomes, a database) pathway analysis and GSEA (Gene Set Enrichment Analysis) analysis. Five cases were screened from 36 children with CPAM, and high-throughput sequencing was performed to obtain 10 whole transcripts of samples with acceptable sequence quality and balanced gene coverage. One aberrantly expressed sample (3b) was found by analysis of principal components, which was excluded and then subjected to differential expression analysis, and 860 up-regulated genes and 203 down-regulated genes. GO functional enrichment analysis of differentially expressed genes demonstrates the functional class and cellular localization of target genes. The whole transcript of CPAM shows obvious gene up and down-regulation, differentially expressed genes are located in specific cells and belong to different functional categories, and NGS can provide an effective means to study the transcriptional regulation of CPAM from the overall transcriptional level.

Pulmonary congenital cystic adenomatoid malformation: a rare congenital abnormality in adults and review of literature

Congenital cystic adenomatoid malformation of the lung (CCAM) is characterized by an adenomatoid proliferation of bronchiole-like structures and cysts formation. The condition is most commonly found in newborns and children and may be associated with other malformations; rarely, the presentation is delayed until adulthood. We herein report two cases of CCAM in adult patients. 22 years old healthy female with pre-employment health screening chest X-ray showed a lesion in the upper lobe of the right lung. In another case, a computed tomographic scan of the thorax (CT) confirmed a mass in the upper right lung. A 28-year-old male presented with recurrent respiratory tract infection resistant to antimicrobial therapy. CT scan of the thorax showed a mass in the left lung upper zone. Surgical resection was performed in both cases, and histopathology of the resected specimen showed both cases were consistent with the CCAM.

Congenital Cystic Adenomatoid Malformation, Type II: A Rare Cause of Haemoptysis

Congenital cystic adenomatoid malformation (CCAM) occurs secondary to the cystic adenomatous over-growth of terminal bronchioles, which results in the secondary inhibition of alveolar growth. In most of the cases, respiratory distress is the presenting feature during the neonatal period. In about 90% of patients, recurrent respiratory infections necessitating chest imaging reveal CCAM before the age of two years. We describe here the occurrence of congenital cystic adenomatoid malformation of right lung in a 12-year-old girl presenting with haemoptysis and hypovolaemic shock. She underwent right middle lobectomy; and histopathological examination confirmed the diagnosis. She has been doing well on follow-up.

Congenital lung malformations: Dysregulated lung developmental processes and altered signaling pathways

Congenital lung malformations comprise a diverse group of anomalies including congenital pulmonary airway malformation (CPAM, previously known as congenital cystic adenomatoid malformation or CCAM), bronchopulmonary sequestration (BPS), congenital lobar emphysema (CLE), bronchogenic cysts, and hybrid lesions. Little is known about the signaling pathways that underlie the pathophysiology of these lesions and the processes that may promote their malignant transformation. In the last decade, the use of transgenic/knockout animal models and the implementation of next generation sequencing on surgical lung specimens have increased our knowledge on the pathophysiology of these lesions. Herein, we provide an overview of normal lung development in humans and rodents, and we discuss the current state of knowledge on the pathophysiology and molecular pathways that are altered in each congenital lung malformation.

Thoracoscopic approach of a left accessory lobe associated with a congenital pulmonary airway malformation

Thoracoscopic approach of a left accessory lobe associated with a congenital pulmonary airway malformation

Perinatal outcomes of prenatal diagnosis of congenital pulmonary airway malformation: an experience.

This study aimed to assess the perinatal outcomes of pregnancies with a prenatal diagnosis of congenital cystic adenomatoid malformation. We conducted a retrospective cohort study based on information contained in the medical records of pregnant women whose fetuses had been prenatally diagnosed with congenital cystic adenomatoid malformation by ultrasonography. Sample analysis was based on 21 singleton pregnancies with confirmed isolated fetal congenital cystic adenomatoid malformations. The mean maternal±standard deviation age was 28±7.7 years. Types I, II, and III congenital cystic adenomatoid malformation were detected in 19% (4/21), 52.4% (11/21), and 28.6% (6/21), respectively. All fetuses presented with unilateral congenital cystic adenomatoid malformation (21/21) without associated anomalies, and 52.3% (11/21) were in the right lung. In total, 33.3% (7/21) of fetuses presented a "congenital cystic adenomatoid malformation volume ratio" >1.6 and were managed with maternal betamethasone administration. The mean gestational age at the time of steroid administration was 28.5±0.9 weeks, with a reduction in the lesion dimensions of 9.5% (2/21) (Types I and III of congenital cystic adenomatoid malformation). The mean gestational age at delivery was 38.7±2.4 weeks, and a cesarean section was performed in 76.2% (16/21) cases. Postsurgical resection was necessary for 23.8% (5/21) of the patients, and 4.7% (1/21) of them died because of respiratory complications after surgery. Pulmonary hypoplasia occurred in 9.5% (2/21) of the patients, and 4.7% (1/21) of them died because of respiratory insufficiency. The survival rate was 90.5% (19/21), and 57.2% (12/21) remained asymptomatic. Despite the isolated prenatal diagnosis of congenital cystic adenomatoid malformation, which showed good survival, congenital cystic adenomatoid malformation is associated with significant perinatal morbidity. Maternal betamethasone administration did not significantly reduce fetal lung lesion dimensions.

SOX2 and SOX21 in Lung Epithelial Differentiation and Repair.

The lung originates from the ventral foregut and develops into an intricate branched structure of airways, alveoli, vessels and support tissue. As the lung develops, cells become specified and differentiate into the various cell lineages. This process is controlled by specific transcription factors, such as the SRY-related HMG-box genes SOX2 and SOX21, that are activated or repressed through intrinsic and extrinsic signals. Disturbances in any of these processes during the development of the lung may lead to various pediatric lung disorders, such as Congenital Diaphragmatic Hernia (CDH), Congenital Pulmonary Airway Malformation (CPAM) and Broncho-Pulmonary Dysplasia (BPD). Changes in the composition of the airways and the alveoli may result in reduced respiratory function and eventually lead to chronic lung disorders. In this concise review, we describe different intrinsic and extrinsic cellular processes required for proper differentiation of the epithelium during development and regeneration, and the influence of the microenvironment on this process with special focus on SOX2 and SOX21.

Thoracoscopic Clockwise Lobectomy May Be a Stylized Procedure for Treating Children with Congenital Lung Malformations.

Background: Thoracoscopic lobectomy is a challenging procedure in children with congenital lung malformations (CLMs). This study aims to evaluate the safety and efficacy of thoracoscopic clockwise lobectomy (TCL) in CLMs in children and its potential to be a stylized procedure. Methods: All patients with CLMs who received TCL from 2015 to 2019 in our hospital were retrospectively reviewed. Clinical information was extracted from medical records, including patient demographics, operative details, and outcomes. Results: A total of 184 patients with a median age of 6.8 months (range, 3-156) and a median weight of 9 kg (range, 6-45) received TCL. Lesions were all located in the lower lobe and included congenital pulmonary airway malformation (n = 133), intralobar sequestration (n = 44), bronchiectasis (n = 4), and congenital lobar emphysema (n = 3). The mean (±standard deviation [SD]) operating time was 46 ± 7.5 minutes (range, 35-113). The mean (±SD) blood loss was 3.5 ± 0.8 mL (range, 1-60). Three patients converted to thoracotomy, and 162 patients did not have a chest tube placed. The postoperative course was uneventful in all patients except 2 patients who developed air leaks and 23 patients who developed a mild fever. The median length of postoperative hospital stay was 2 days. A total of 163 patients were followed up for more than 1 year without any complications. Conclusion: TCL is suitable for lower lobectomy and is safe and effective in standard and complicated thoracoscopic lobectomy. It could be recommended as a stylized procedure in treating children with CLMs.

Hospital Volume and Postoperative Outcomes after Elective Lung Resection in Infants with Congenital Lung Malformations

INTRODUCTION: The relationship between hospital volume and patient outcomes after pediatric lung resection remains unknown. This study evaluated the association between hospital volume and postoperative outcomes in infants undergoing elective lung resection for congenital lung malformations (CLM). METHODS: After IRB approval, infants >30 days of age who underwent elective CLM resection were identified in the Pediatric Health Information System database (2016 to 2020). Those with previous symptomatic disease were excluded. Annual lung resection tertiles were used to classify hospitals into low (1 to 7 cases), medium (8 to 11 cases), or high (>11 cases) volume centers. Primary outcomes were hospital length of stay (LOS) and postoperative complications. Linear or Poisson multivariable regressions were performed. RESULTS: There were 1,420 infants from 48 hospitals. CLMs included congenital pulmonary airway malformations (n = 623, 43.9%) and bronchopulmonary sequestrations (n = 364, 25.6%). The mean annual cases at low-, medium-, and high-volume hospitals were 4.6 ± 1.6, 8.9 ± 1.2, and 36.4 ± 23.6, respectively. The median LOS was 2 days (interquartile range 2 to 3), and there were no differences based on center volume (p = 0.139). Overall postoperative complications were significantly increased at low-volume centers (31.1%) compared with medium-volume (16%) and high-volume (25%) centers (p < 0.001; Table 1). Respiratory complications were significantly increased in low-volume centers (28.8%) compared with medium-volume (14%) and high-volume (23%) centers (p < 0.001). Adjusted odd ratios showed a 51% reduction in overall complications (95% CI 0.37 to 0.65, p < 0.001) at medium/high-volume centers. Table 1. - Patient Outcomes by Center Volume Variable Low (n =89 patients) Medium (n = 534 patients) High (n = 497 patients) p Value Operative approach Thoracoscopic, n (%) 295 (75.8) 403 (75.5) 248 (49.9) <0.001 Open, n (%) 121 (31.1) 83 (15.5) 124 (24.9) <0.001 Respiratory complications, n (%) 112 (28.8) 73 (13.7) 112 (22.5) <0.001 Postoperative ventilation, n (%) 25 (6.4) 32 (6.0) 15 (3.0) 0.034 Length of stay (d), median (IQR) 2.00 (2–4) 2.00 (2–3) 2.00 (2–3) 0.139 Mortality, n (%) 1 (0.3) 1 (0.2) 0 (0) 0.7366 30-day readmission, n (%) 22 (5.7) 16 (3.0) 14 (2.8) 0.062 CONCLUSION: For elective infant lung resections, these data suggest that low-volume hospitals are associated with increased postoperative complications. Further study of CLM outcomes, including the role of surgeon-specific volume, is warranted.

Perioperative Anesthetic Care of Children with Congenital Cystic Adenomatous Malformation: A Report of 3 Cases

Congenital cystic adenomatoid malformation (CCAM) is a rare developmental entity. Symptomatic patients mustundergo thoracic surgery for lobectomy. Perioperative anesthetic management is challenging because it involvesthoracotomy in a young patient that may lead to hemodynamic compromise and inadequate ventilation. We present 3 cases of pediatric patients (6 months - 2 years old) with CCAM who underwent lobectomy.

CYSTIC ADENOMATOID MALFORMATION OF THE LUNG IN AN ADULT: A CASE REPORT

CYSTIC ADENOMATOID MALFORMATION OF THE LUNG IN AN ADULT: A CASE REPORT

Enfermedades bronquiales y pulmonares hereditarias y congénitas

Congenital diseases of the respiratory system comprise a large number of embryonic development diseases that can compromise the development of the larynx, trachea, bronchi, pulmonary parenchyma, vessels, diaphragm, or thoracic wall. Prenatal ultrasound has made it possible for a large number of lung malformations to be detected before birth. Imaging techniques (radiography, ultrasound, computed tomography) are essential for diagnosis. The treatment is surgical in most cases. We will study embryology, lung development abnormalities, cystic adenomatoid malformation, sequestration, bronchogenic cyst, congenital lobar emphysema, and vascular abnormalities.Hereditary diseases originate from genetic abnormalities. They can affect the respiratory tract, parenchyma, and vascular structures of the lung. They include very diverse diseases such as alpha-1 antitrypsin deficiency, primary ciliary dyskinesia, or cystic fibrosis which, given its frequency and clinical relevance, is the disease we will study in this update.

Utility of Postnatal Chest X-Ray in Newborns for the Evaluation of Prenatally Suspected Congenital Pulmonary Airway Malformation: A Single-Center Experience.

The aim of the study is to assess the necessity of chest X-ray (CXR) during the newborn hospitalization for all patients with prenatally suspected congenital pulmonary airway malformation (CPAM). This is a retrospective chart review of all infants delivered with prenatally suspected CPAM at our high-risk delivery hospital from January 2013 through April 2020 (n = 44). Nonparametric tests assessed the association between postnatal CXR findings, prescribed follow-up timeline, and neonatal outcomes. Mean follow-up period recommended was 6.4 weeks regardless of CXR findings in the neonatal period (p = 0.81). Additionally, patients who required respiratory support at or after birth were not more likely to have a lesion identified on chest X-ray (odds ratio [OR] = 0.72, 95% confidence interval [CI], 0.18-2.64, p = 0.71). Neonatal hospital course and future follow-up plan of patients with prenatally suspected CPAM were not altered by information from the CXR obtained in the immediate neonatal period, suggesting that this CXR may not be necessary in the asymptomatic patient. · Immediate postnatal X-ray of prenatally diagnosed CPAM does not alter planned follow-up interval.. · Immediate postnatal X-ray does not alter surgical plan for CPAM.. · Postnatal X-ray is not absolutely required for asymptomatic newborns with CPAM..

Prenatal ultrasound-and MRI-based imaging predictors of respiratory symptoms at birth for congenital lung malformations

BackgroundCongenital lung malformations (CLM) are rare developmental anomalies of the fetal lung with a minority of patients exhibiting symptoms around the time of birth. Although ultrasound remains the gold standard, fetal MRI has recently been incorporated as an adjunct imaging modality in the workup and prenatal counseling of patients with CLM as it is thought to more accurately delineate lesion boundaries and diagnose lesion type. We evaluate what prenatal variables correlate with postnatal respiratory symptoms. MethodsWe performed a retrospective review of patients with prenatal diagnosis of CLM treated at our institution between 2006-2020. Fetal ultrasound and magnetic resonance imaging (MRI) parameters including maximal congenital pulmonary airway malformation volume ratio (CVR), absolute cyst volume, and observed to expected normal fetal lung volume (O/E NFLV) were correlated with outcomes including postnatal respiratory symptoms, need for supplementary oxygen or mechanical ventilation, delay in tolerating full feeds, resection in the neonatal period. ResultsOur study included 111 patients, all of whom underwent fetal ultrasound with 64 patients additionally undergoing fetal MRI. Postnatal respiratory symptoms were noted in 22.5% of patients, 19.8% required supplemental oxygen, 2.7% mechanical ventilation and two patients requiring urgent resection. Ultrasound parameters including absolute cyst volume and maximal CVR correlated with need for mechanical ventilation (p=0.034 and p=0.024, respectively) and for urgent resection (p=0.018 and p=0.023, respectively) and had a marginal association with postnatal respiratory symptoms (p=0.050 and p=0.052). Absolute cyst volume became associated with postnatal respiratory symptoms (p=0.017) after multivariable analysis controlling for maternal steroid administration and gestational age. O/E NFLV did not correlate with perinatal outcomes. ConclusionWe have found that ultrasound-based measurements correlate with postnatal respiratory symptoms, while MRI derived O/E NFLV does not. Further studies are needed to elucidate the role of MRI in the prenatal workup of congenital lung malformations. Type of StudyStudy of Diagnostic Test. Level of EvidenceLevel I.

“ROLE OF FETAL MRI IN EVALUATION OF CONGENITAL ANOMALIES”

When US evaluation of the second and third-trimester foetus is limited, MR imaging might be used as a supplemental test. Due to a lack of an acceptable acoustic window, US is often limited in cases of oligohydramnios or breech presentation. In both cases, however, MR imaging can clearly show the foetal anatomy in detail. Ossification of the calvaria[1], which inhibits view of the posterior fossa at US, can also impede third trimester foetal assessment. US frequently suggests intracranial pathologic abnormalities such as posterior fossa anomalies (Chiari II syndrome, cerebellar aplasia or hypoplasia). MR imaging can corroborate the US findings, and it can also provide a diagnosis in some circumstances, such as neuronal migrational anomalies. MR imaging certainly has the advantage of being able to analyse the foetus in many planes within a greater field of view. T1-weighted MR imaging can show acute and chronic germinal matrix bleeding, as well as ischemia alterations. Although ultrasound is helpful in detecting corpus callosum abnormalities, it can miss many of the central nervous system problems associated with corpus callosum agenesis. Because the prognosis of agenesis of the corpus callosum is so unpredictable, detecting concomitant central system defects on MR imaging can help with prenatal and postnatal medical care. Although the majority of prenatal MR imaging applications are related to the cranial nervous system, new developments in foetal surgical intervention have expanded the utility of foetal MR imaging in thoracoabdominal illness detection. Fetal MR imaging can help diagnose abnormalities such congenital cystic adenomatoid malformation, congenital diaphragmatic hernia, cystic hygroma, and bronchopulmonary sequestration. Accurate assessment of foetal lung and liver sizes is critical in foetal surgical planning and postnatal prognosis in the case of congenital diaphragmatic hernia. Some high-risk pregnancies may also benefit from obstetric MR imaging, according to preliminary research. With a combination of foetal weight estimates and liver volume measurements, birth weight may be correctly predicted and the diagnosis of intrauterine growth retardation or macrosomia can be made with confidence.

Congenital cystic adenomatoid malformations of the lung: a retrospective study of diagnosis, treatment strategy and postoperative morbidity in surgically treated patients.

The purpose of this study was to evaluate the diagnosis of, clinical signs of and strategy for congenital cystic adenomatoid malformations (CCAM). In this retrospective study, patients who had thoracic surgery for CCAM lesions at the University Hospitals of Leuven from July 1993 to July 2016 were identified. Data on diagnosis, prenatal ultrasound findings, clinical signs, lesion site, CCAM type, associated anomalies, imaging, surgical approach and postoperative morbidity were reviewed. The Fisher exact and Mann-Whitney tests were used as appropriate. A total of 55 patients were identified with CCAM. In 65% (n = 36/55), CCAM was detected on prenatal ultrasound scans. Prenatal symptoms due to hydrops or mass effect were present in 22% (n = 8/36), 6 of whom eventually needed prenatal intervention (thoracoamniotic shunting or intrauterine puncture). Elective surgery was performed in 40% of patients (n = 22/55); others developed clinical signs that indicated the need for semi-urgent surgery, with clinical signs of pulmonary infection and respiratory distress being the most common. Most patients had a single lobectomy via a minithoracotomy. Postoperative complications and length of stay were significantly higher in patients with CCAM with preoperative clinical signs. Surgery in asymptomatic patients with CCAM can be performed safely with few postoperative complications and can be planned at a young age in patients with a high risk of developing clinical signs later in life.

Prospective analysis of the prognostic value of prenatal MRI measurement of cystic volume ratio in fetal congenital cystic adenomatoid malformation.

The cystic volume ratio (CVR) has been used to predict the prognosis of fetal lung disease, but most of them are reported on ultrasound. The purpose of this study was to investigate the prognostic value of prenatal magnetic resonance imaging (MRI) measurement of CVR in fetal congenital cystic adenomatoid malformation (CCAM). To collect follow-up data on postnatally confirmed CCAM fetuses. According to the size of CVR, they were divided into a CVR ≥1.26 group and a CVR <1.26 group. The lesions were divided into macrocystic (diameter ≥5 mm) and microcystic (diameter <5 mm) types according to the size of cysts in the lesions. The collected contents also included the location of the lesions, prenatal symptoms, postpartum clinical prognosis, and pregnancy outcomes. In all, 51 cases were collected. Of these, 11 were placed into the CVR ≥1.26 group, and 40 were placed into the CVR <1.26 group; meanwhile 34 were classified as macrocystic lesions and 17 as microcystic lesions; 7 cases involved a terminated pregnancy. In the CVR ≥1.26 group, 81.82% (9/11) and 36.36% (4/11) had displacement of large vessels/heart and increased amniotic fluid, respectively; meanwhile, in the CVR <1.26 group, these phenomena occurred in 35.00% (14/40) and 2.50% (1/40) of cases (P=0.006, 0.010). Additionally, 83.33% (5/6) of live infants in the CVR ≥1.26 group had dyspnea, a significantly higher proportion than the 21.05% (8/38) in the CVR <1.26 group (P=0.011). Moreover, 45.45% (5/11) of the cases in the CVR ≥1.26 group were terminated pregnancies, a significantly higher proportion than the 5.26% (2/38) in the CVR <1.26 group (P=0.001). Finally, the CVR of macrocystic lesions was 0.55 (0.34-1.31), which was significantly greater than the 0.34 (0.17-0.57) of microcystic lesions (P=0.022). Logistical regression analysis identified that CVR is an independent factor associated with the postpartum prognosis of CCAM. Prenatal symptoms and postpartum prognosis were worse than CVR <1.26 when fetal CVR ≥1.26 measured by prenatal MRI; and the measurement of CVR of CCAM through prenatal MRI has considerable practical value in prenatal consultation, evaluation, and postpartum treatment.

Clinical Presentation and Outcome of Congenital Thoracic Malformations in Children Beyond Neonatal Period

Objective: To describe the clinical spectrum of presentation and outcome of children with congenital thoracic malformations beyond neonatal age. Methodology: Cross sectional study conducted at inpatient department of National institute of child health from Jan-Dec 2021. All patients hospitalized in study duration with diagnosis of congenital thoracic formations from 1 month till 12 years of age were enrolled. History, examination, laboratory tests, treatment and outcome were recorded. Results: Total 44 children were enrolled with mean age of 7.7+10.8 months. Common congenital thoracic malformations were congenital diaphragmatic hernia 24(54.5%), congenital cystic adenomatoid malformation 6(13.6%), congenital lobar emphysema5(11.4%) and eventration of diaphragm 4(9.1%). Three (6.9%) patients had associated cardiac anomalies. Surgical intervention was done in 33 (75%) patients with a postoperative survival rate of 95.4%. Conclusion: Congenital diaphragmatic hernia and cystic adenomatoid malformation are common congenital thoracic malformations that present beyond neonatal age and are associated with good postsurgical outcome. Keywords: congenital diaphragmatic hernia, congenital cystic adenomatoid malformation, beyond neonate