Search

Background Risk for infection and potential malignant degeneration are the most common arguments for resecting asymptomatic Congenital Pulmonary Airway Malformations (CPAM). We aimed to investigate if CT- imaging characteristics can be used to predict histopathological features, by using an objective quantitative CT scoring method. Methods Archival CPAM tissue samples were histologically re-assessed and patients who had a pre-operative volumetric CT-scan were included. Lung disease was quantified using the newly-developed congenital lung abnormality quantification(CLAQ) scoring method and obtained percentages were used to predict histopathological signs of inflammation and presence of mucinous proliferation (MP). Because MP is presumed a precursor for mucinous adenocarcinoma in situ (AIS) this method was also used to compare CT-scans of patients with AIS to those with only CPAM. Results Thirty-three CPAM patients were included of which 13(39%) had histological signs of inflammation and 8(24%) had a MP. Patients with inflammation had a significantly smaller lesion (14% vs 38%) while those with MP had more extensive disease (54%vs17%). Patients with AIS had a significantly smaller lesion compared to CPAM patients (5%vs29%). Significant predictors for inflammation were smaller lesion size and percentage hypodensity within lesions while a larger lesion size and percentage parenchymal hyperdensity (solid lung tissue components) were predictors for MP as well as AIS. Conclusions Smaller CPAM lesions may be more susceptible to inflammation while larger lesions may be associated with the presence of MP. Parenchymal hyperdensity is found as a predictor for MP as well as AIS and should therefore elicit more extensive gross sampling. Level of evidence Level III.

Pleuropulmonary blastomas are rare, potentially aggressive embryonal cancers of the lung parenchyma and pleural surfaces that account for 0.25%-0.5% of primary pulmonary malignancies in children. Pleuropulmonary blastomas are classified as cystic (type I), mixed cystic and solid (type II), and solid (type III). Pleuropulmonary blastoma occurs in the same age group (0-6 years) as other more common solid tumors such as neuroblastoma and Wilms tumor. Differential diagnosis includes metastasis from Wilms tumor and macrocystic congenital pulmonary airway malformation (CPAM). A key pathologic and genetic discriminator is the DICER1 germline mutation found in patients with pleuropulmonary blastoma. Imaging, histopathologic, and clinical data are important to use in conjunction in order to determine the diagnosis and risk stratification of pleuropulmonary blastomas. Survival varies from poor to good, depending on type. However, the spectrum of pleuropulmonary blastoma is insufficiently understood due to the variable presentation of this rare disease. We present a current review of the literature regarding pleuropulmonary blastomas in this article.

Indications for pulmonary lobectomy in infants and children include cystic pulmonary adenomatoid malformation, congenital lobar emphysema, chronic infection, and malignancy. These procedures can now all be done thoracoscopically avoiding the short- and long-term morbidity of an open thoracotomy. In this article we describe the technique of thoracoscopic lobectomy as well as the preoperative and postoperative care.

Background: It has been postulated that video-assisted thoracoscopic surgery (VATS) is superior than conventional thoracotomy for resection of congenital pulmonary airway malformation (CPAM) in terms of shorter hospital length of stay, reduced post-operative complication and better long-term pulmonary outcome. However, there is limited available data concerning the effect of VATS and conventional thoracotomy on musculoskeletal aspects in patients with CPAM. This study aims to compare the long-term effects of both surgical techniques on musculoskeletal outcomes in this group of patients. Methods: Thirty-five patients with mean age of 11.38 years old who underwent VATS or conventional thoracotomy for CPAM resection were recruited in this study. The mean follow-up time was 10.42 years (5.48 to 17.71 years). Musculoskeletal examination included screening of musculoskeletal deformities (elevation of shoulder, asymmetry of nipple level, breast/ pectoral muscle maldevelopment, winging of scapula, scoliosis), measurement of bilateral chest wall in relation to anatomical parameter, and testing of shoulder range of motion and power. Results: The prevalence of winged scapula was significantly lower in patients who underwent VATS than thoracotomy (13% vs 58%, p = 0.008). There is also a smaller reduction in operated-side chest wall vertical distance in VATS patients (0.982 vs 0.956, p = 0.058). There were no significant difference in prevalence of other musculoskeletal deformities, other chest wall measurements, shoulder range of motion and power between two groups. Conclusion: Thoracoscopy decreases the incidence of scapular winging but has no effect on effect on other musculoskeletal deformities.

To identify, in fetuses with a congenital lung malformation (CLM), prenatal predictors of the need for postnatal respiratory support and the need for surgery by calculating the CLM volume ratio (CVR), and to evaluate the concordance between the prenatal appearance and the postnatal type of CLM.This was an analysis of prenatal, perinatal and postnatal data from fetuses diagnosed with a CLM at the Erasmus University Medical Center - Sophia Children's Hospital in Rotterdam, The Netherlands, between January 2007 and December 2016. For all included fetuses, CVR was measured retrospectively on stored ultrasound images obtained at 18 + 1 to 24 + 6 weeks (US1), 25 + 0 to 29 + 6 weeks (US2) and/or 30 + 0 to 35 + 6 weeks' gestation (US3). Postnatal diagnosis of CLM was based on computed tomography or histology. Primary outcomes were the need for respiratory support within 24 h and surgery within 2 years after birth.Of the 80 fetuses with a CLM included in this study, 14 (18%) required respiratory support on the first postnatal day, and 17 (21%) required surgery within 2 years. Only the CVR at US2 was predictive of the need for respiratory support, with a cut-off value of 0.39. Four of 16 (25%) fetuses which showed full regression of the CLM prenatally required respiratory support within 24 h after birth. The CVR at US1, US2 and US3 was predictive of surgery within 2 years. Overall, the prenatal appearance of the CLM showed low concordance with the postnatal type. Prenatally suspected microcystic congenital pulmonary airway malformation (CPAM) was shown on computed tomography after birth to be congenital lobar overinflation in 15/35 (43%) cases. Respiratory support within 24 h after birth and surgical resection within 28 days after birth were needed in all cases of macrocystic CPAM.CVR can predict the need for respiratory support within 24 h after birth and for surgery within 2 years. Regression of a CLM prenatally does not rule out respiratory problems after birth. © 2020 The Authors. Ultrasound in ObstetricsGynecology published by John WileySons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology. - Legal Statement: This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.

OBJECTIVE To investigate the relationship between mild congenital pulmonary airway malformation (CPAM) and its long-term prognosis in childhood and to explore whether surgery is necessary. METHODS We conducted a retrospective cohort of fetuses with mild CPAM diagnosed prenatally with available long-term outcomes in childhood from 2004 to 2016. The patients were divided into two groups according to the fetal CPAM-to-volume ratio (CVR) of less than 1.0 and 1.0-1.6. The primary outcome was a postnatal composite outcome including CPAM-associated respiratory symptoms and surgical resection of the lesion. The secondary outcomes included neonatal asphyxia, perinatal morbidity and mortality. RESULTS Forty-two fetuses were identified as having CVR

Large solitary cystic lesions are a rare finding, and their differential diagnosis includes cystic airspaces associated with lung cancer, congenital pulmonary airway malformations and pneumatoceles. Here, we report 3 consecutive patients who presented with a large solitary pulmonary cyst on chest computed tomography. All underwent surgical resection, and the histopathological findings were different in all 3 cases. In one patient, a very rare finding of squamous cell carcinoma arising from the cystic lesion in the left lower lobe was confirmed. Therefore, in carefully selected cases, pulmonary cysts should be resected based on the potential risk for recurrent infection and the development of malignancy. ispartof: Interact Cardiovasc Thorac Surg vol:34 issue:6 pages:1157-1159 ispartof: location:England status: published

BACKGROUND: Congenital pulmonary airway malformation (CPAM) is the most common prenatally-diagnosed lung malformation. This lesion, classified as macrocystic or microcystic, can lead to significant fetal compromise. Management options include observation, maternal antenatal steroid administration, and fetal surgical intervention. Current evidence suggests that microcystic (but not macrocystic) lesions and those with a cyst volume ratio (CVR) >1.6 are responsive to steroid therapy. The objective of this study was to identify patterns of prenatal steroid administration for the management of CPAMs and to identify characteristics of CPAMs prompting steroid administration. METHODS: An 18-question survey was distributed to obstetricians from the Pregnancy-Related Care Research Network (PRCRN) and the North American Fetal Therapy Network (NAFTNet), from January to April 2019, to capture antenatal steroid prescribing patterns. RESULTS: Response rates were 28.3% (138/487) for PRCRN and 63.3% (19/30) for NAFTNet. Among PRCRN members, 16.8% administered prenatal steroids, with most (77.2%) doing so for both microcystic and macrocystic CPAMs; corresponding percentages for NAFTNet members were 90.9% and 52.6%. Two thirds (65.6%) of obstetricians who administer steroids do so for a CVR > 1.6, without evidence of mediastinal shift or hydrops fetalis. CONCLUSIONS: There is a lack of consensus among obstetricians as to the CPAM characteristics that should prompt administration of prenatal steroids. Many surveyed obstetricians do not use cyst type or CVR to guide decision-making regarding steroid therapy.

Objectives To develop a clinical guideline for structured assessment and uniform reporting of congenital lung abnormalities (CLA) on Computed Tomography (CT)-scans. Materials and Methods A systematic literature search was conducted for articles describing CT-scan abnormalities of congenital pulmonary airway malformation (CPAM), bronchopulmonary sequestration (BPS), congenital lobar emphysema (CLE) and bronchogenic cyst (BC). A structured report using objective features of CLA was developed after consensus between a pediatric pulmonologist, radiologist and surgeon. Results Of 1581 articles identified, 158 remained after title-abstract screening by two independent reviewers. After assessing full-texts, we included 28 retrospective cohort-studies. Air-containing cysts and soft tissue masses are described in both CPAM and BPS while anomalous arterial blood supply is only found in BPS. Perilesional low-attenuation areas, atelectasis and mediastinal shift may be found in all aforementioned abnormalities and can also be seen in CLE as a cause of a hyperinflated lobe. We have developed a structured report, subdivided into five sections: Location & Extent, Airway, Lesion, Vascularization and Surrounding tissue. Conclusions CT-imaging findings in CLA are broad and nomenclature is variable. Overlap is seen between and within abnormalities, possibly due to definitions often being based on pathological findings, which is an unsuitable approach for CT imaging. We propose a structured assessment of CLA using objective radiological features and uniform nomenclature to improve reporting.

Background: This study aimed to report a European multi-institutional experience about thoracoscopic management of children with congenital lung malformations. Methods: The records of 102 patients (49 girls and 53 boys) with median age at surgery of 1 year (range 6 months-1.5 years), who underwent thoracoscopic lobectomy in five European Pediatric Surgery units, were retrospectively collected. Indications for surgery included congenital pulmonary airway malformation (CPAM) (n = 47), intra- and extralobar pulmonary sequestration (n = 34), hybrid lesion (CPAM/intralobar sequestration) (n = 2), severe bronchiectasis (n = 9), congenital lobar emphysema (n = 8), and others (n = 2). The condition was asymptomatic in 77/102 (75.5%), whereas symptoms such as recurrent pneumonia and/or respiratory distress were present in 25/102 (24.5%). Results: Surgical procedures included 18 upper, 20 middle, and 64 lower lobe resections. No conversions to open were reported. A 3 mm sealing device and 5 mm stapler were adopted in the last 48/102 patients (47%). The median operative time was 92.2 minutes (range 74-141). The median operative time significantly decreased in patients in whom the vessel division and bronchial sealing were performed using sealing devices (75.5 minutes) compared with suture ligations (118.9 minutes) (P = .001). The median hospital stay was 3.7 days (range 2-6.2). Three/102 patients (2.9%) developed postoperative complications, including air leakage requiring pleural drainage (n = 1) (Clavien IIIb) and respiratory infection (n = 2) (Clavien II). A reoperation was required in one patient with residual pleuropulmonary blastoma (0.9%). All symptomatic patients reported resolution of symptoms postoperatively. Conclusions: Thoracoscopic lobectomy is a safe and effective procedure with excellent cosmetic outcome, in expert hands. Based upon our experience, we strongly recommend surgery in patients with congenital lung malformations by the first year of life, to reduce the risk of infection and make the procedure technically easier, despite the small patients' size. Surgeon's experience and use of miniaturized instruments and sealing devices remain key factors for successful outcome.

Introduction Infection is undoubtedly the most important factor in influencing the timing and surgical strategy of congenital pulmonary airway malformation (CPAM) surgery. However, there have been no studies on the optimal timing of surgery for patients based on the probability of infection. The aim of this study was performed to explore the optimal timing of surgery of CPAM in children from the risk of infection. Materials and Methods The correlation of age distribution and pulmonary infection of 237 children diagnosed by pathology from January 2012 to January 2020 in Guangzhou Women and Children's Medical Center were analyzed retrospectively. We defined the cases with preoperative computed tomographic findings of infection, pathological findings of large number of neutrophils, tissue cells, and abscess formation as the infection group. Results The rate of infection in patients less than 2 years old was significantly lower than in patients over 2 years old (11.4% vs. 45.7%, p Conclusion In consideration of the high risk of infection, lower minimally invasive surgery rate, an increased rate of pulmonary lobectomy, and more blood loss in patients over 2 years old, our study also supports early surgical treatment. Therefore, we suggest that for asymptomatic patients with CPAM I and CPAM II, surgical treatment should be performed when they are less than 2 years old, providing more options for surgical strategies and monitoring of CPAM patients.

Aims: Mucinous adenocarcinoma arising in congenital pulmonary airway malformation (CPAM) is a rare complication, with little being known about its natural course. The aims of this article are to describe a series of mucinous adenocarcinomas arising from CPAMs, and present their clinicopathological features, genetics, and clinical outcome. Methods and results: Thirty-seven cases were collected within a 34-year period, and the subtype of adenocarcinoma and CPAM, tumour location, stage, growth patterns, molecular data and follow-up were recorded. The cohort comprised CPAM type 1 (n = 33) and CPAM type 2 (n = 4). Morphologically, 34 cases were mucinous adenocarcinomas (21 in situ; 13 invasive), and three were mixed mucinous and non-mucinous adenocarcinoma. Seventeen cases showed purely extracystic (intra-alveolar) adenocarcinoma, 15 were mixed intracystic and extracystic, and five showed purely intracystic proliferation. Genetically, nine of 10 cases tested positive for KRAS mutations, four with exon 2 G12V mutation and five with exon 2 G12D mutation. Residual disease on completion lobectomy was observed in two cases, and three cases recurred 7, 15 and 32 years after the original diagnosis. Two patients died of metastatic invasive mucinous adenocarcinoma. Conclusions: Most adenocarcinoma that arise in type 1 CPAMs, are purely mucinous, and are early-stage disease. Intracystic proliferation is associated with lepidic growth, an absence of invasion, and indolent behaviour, whereas extracystic proliferation may be associated with more aggressive behaviour and advanced stage. Most cases are cured by lobectomy, and recurrence/residual disease seems to be associated with limited surgery. Long-term follow-up is needed, as recurrence can occur decades later.

Congenital-pulmonary-airway-malformation (CPAM) and bronchopulmonary-sequestration (BPS) are rare conditions. The objective of this study was to describe the sonographic characteristics and prenatal course of fetal lung mass.A retrospective study on 20 pregnancies with CPAM and BPS diagnosed during 2018-2018 was performed. Data evaluated included gestational age (GA) at diagnosis, size of lesion, CPAM-volume-ratio (CVR), associated sonographic findings and pregnancy outcome.Fourteen cases of CPAM and six cases of BPS were evaluated. The average GA at diagnosis was 22 weeks'-gestation (range 17-26). 66% were macrocystic-multicystic. Five cases of BPS (71.4%) had other structural anomalies. None of the fetuses with CPAM had additional anomalies. Four pregnancies (20%) underwent termination due to severe hydrops fetalis. Two cases of CPAM (15%) had spontaneous resolution by 30 week's-gestation; three cases (20%) continued to grow during pregnancy and all the rest reached their largest size at 25-28 week's-gestation. Most cases delivered at term (average 37.6 range 32.2-41.4). Two children had lobectomy at 11 and 12 months of life.Careful follow-up during pregnancy may result in term delivery of adequate-for-gestational-age newborns, with no need for admission to the NICU, with only 10% need for lobectomy during the first year of life.

Abstract Background: Congenital pulmonary airway malformation is a rare congenital lung lesion. Infants with large Congenital pulmonary airway malformation can present with a series of serious symptoms. Surgery is still the main treatment. Thoracoscopic lobectomy for neonates is rarely reported. Case presentation The authors report a case of a congenital pulmonary airway malformation located in the left lower lung of a 4-day-old female infant. Prenatally, the cystic adenomatoid malformation volume ratio was 2.99 according to ultrasound scan. After birth, thoracoscopic lobectomy was performed to alleviate respiratory failure and mediastinal hernia. The patient’s clinical symptoms and the X-ray re-examination showed good postoperative recovery. Conclusions The purpose of this study is to indicate that a safe and effective minimally invasive surgery for the giant congenital pulmonary airway malformation is feasible, even for infants only 4 days old.

OBJECTIVES To compare the prognostic factors of fetuses with microcystic and macrocystic congenital pulmonary airway malformations (CPAMs). METHODS We retrospectively evaluated fetuses with CPAMs at Asan Medical Center. The CPAM size, mass effect, and maximum cyst size in macrocystic CPAMs were evaluated prenatally. The adverse postnatal outcomes, including respiratory symptoms, mechanical ventilation, and surgery, were evaluated. RESULTS In 118 cases, 2 fetal deaths and 1 neonatal death occurred. All cases of fetal hydrops and complete regression after birth were in the macrocystic and microcystic CPAM groups, respectively. Twenty-four neonates (20.7%) had respiratory symptoms, and 18 (15.5%) required mechanical ventilation. Sixty-three neonates (54.3%) underwent surgery, of whom 21 (33.3%) required surgery in the neonatal period. The maximum congenital pulmonary airway malformation volume ratio was significantly associated with all postnatal outcomes (P < .05), and the optimal cutoff values were lower for respiratory symptoms, mechanical ventilation, and neonatal surgery in the macrocystic CPAMs. The maximum cyst size was also associated with all postnatal outcomes in macrocystic CPAMs (P < .05). CONCLUSIONS Different cutoff values for the maximum congenital pulmonary airway malformation volume ratio should be applied according to the CPAM type for the prediction of postnatal outcomes. The maximum cyst size can also be a useful prognostic factor in macrocystic CPAMs.

The potential for malignant degeneration is the most common reason for some practitioners to resect asymptomatic congenital pulmonary airway malformations (CPAMs). We aimed to investigate the potential of various immunohistochemical (IHC) and genomic biomarkers to predict the presence of mucinous proliferations (MPs) in CPAM. Archival CPAM tissue samples were re-assessed and underwent IHC analysis using a panel of differentiating markers (TTF1/CDX2/CC10/MUC2/MUC5AC/p16/p53/DICER1). In each sample, intensity of IHC staining was assessed separately in normal lung tissue, CPAM, and MP tissue, using a semiquantitative approach. Likewise, next-generation targeted sequencing of known adult lung driver mutations, including KRAS/BRAF/EGFR/ERBB2, was performed in all samples with MP and in control samples of CPAM tissue without MP. We analyzed samples of 25 CPAM type 1 and 25 CPAM type 2 and found MPs in 11 samples. They were all characterized by strong MUC5AC expression, and all carried a KRAS mutation in the MP and adjacent nonmucinous CPAM tissue, whereas the surrounding normal lung tissue was negative. By contrast, in less than half (5 out of 12) control samples lacking MP, the CPAM tissue also carried a KRAS mutation. KRAS mutations in nonmucinous CPAM tissue may identify lesions with a potential for malignant degeneration and may guide histopathological assessment and patient follow-up.

Background The increased and earlier use of prenatal ultrasound has facilitated the detection of congenital thoracic malformations (CTMs). Our Pediatric Pulmonology Institute follows an increasing number of patients with CTMs. Thus, we aimed to evaluate and describe prenatal sonographic findings of CTM, and to estimate changes in detection rates of CTMs over a period of 16 years. Methods A retrospective, cross-section analysis of prenatal ultrasound (US) screening tests carried out in a large community-based clinic, comparing two periods: 2001-2007 and 2007-2017. Results A total of 34 716 prenatal US were performed at a median gestational age of 15.4 weeks (range, 11.6-23.9) and 15.7 weeks (range, 12-33.6) in 2001-2007 and 2007-2017, respectively. In 2001-2007, 12 016 prenatal US tests detected 19 CTMs, compared to 30 CTMs in 22 700 tests in 2007-2017. Detection rates did not change (1.58/1,000 in 2001-2007 versus 1.32/1,000 in 2007-2017, P = 0.64). The most common abnormality was congenital pleural effusion (CPE) (17 cases, 34.7%), followed by congenital pulmonary airway malformation) and congenital diaphragmatic hernia; 13 cases each, 26.5%. Twenty CTMs, mainly congenital diaphragmatic hernia and CPE, were associated with other fetal lesions. Conclusions Congenital diaphragmatic hernia and CPE tend to appear with multiple lesions and warrant further attention. The incidence rates stayed stable when comparing the last decade to previous years. Thus, the increased referral of CTM can be attributed to an increase in the number of prenatal screening studies performed, rather than a true higher incidence.

Introduction: A congenital lung abnormality (CLA) is often found in conjunction with other abnormalities but screening guidelines for newborns with CLA have not yet been reported. We aimed to assess the incidence of associated anomalies in CLA patients born or followed up at our centre and the need for additional screening of newborns with a CLA. Methods: From a retrospective chart review of all patients born with a CLA between January 1999 and January 2019, we identified patients diagnosed with a congenital pulmonary airway malformation, bronchopulmonary sequestration, congenital lobar overinflation, bronchogenic cyst, or lung agenesis. Associated anomalies were noted and categorized according to the affected organ system. Results: Twenty-eight (14%) of 196 CLA patients had a major associated anomaly. This was most frequent in conjunction with a lung agenesis (100%) or bronchogenic cyst (29%). Congenital heart defects (32%) and gastrointestinal defects (18%) were the most frequently associated anomalies. Examination of newborns with a CLA should focus on the cardiovascular and gastrointestinal tract, and a chest and abdominal radiograph may be useful to assess signs of major associated anomalies, regardless of the clinical course.

Background: Congenital pulmonary airway malformations (CPAM), bronchopulmonary sequestrations (BPS), and CPAM-BPS hybrid lesions are most commonly solitary; however, >1 lung congenital lung lesion may occur. Objectives: To assess the frequency of multiple congenital thoracic anomalies at a high-volume referral center; determine prenatal ultrasound (US) and magnetic resonance imaging (MRI) features of these multifocal congenital lung lesions that may allow prenatal detection; and determine the most common distribution or site of origin. Methods: Database searches were performed from August 2008 to May 2019 for prenatally evaluated cases that had a final postnatal surgical diagnosis of >1 congenital lung lesion or a lung lesion associated with foregut duplication cyst (FDC). Lesion location, size, echotexture, and signal characteristics were assessed on prenatal imaging and correlated with postnatal computed tomographic angiography and surgical pathology. ­Results: Of 539 neonates that underwent surgery for a thoracic lesion, 35 (6.5%) had >1 thoracic abnormality. Multiple discrete lung lesions were present in 19 cases, and a lung lesion associated with an FDC was present in 16. Multifocal lung lesions were bilateral in 3 cases; unilateral, multilobar in 12; and, unilobar multisegmental in 4. Median total CPAM volume/head circumference ratio for multifocal lung lesions on US was 0.66 (range, 0.16–1.80). Prenatal recognition of multifocal lung lesions occurred in 7/19 cases (36.8%). Lesion combinations were CPAM-CPAM in 10 cases, CPAM-BPS in 5, CPAM-hybrid in 2, hybrid-hybrid in 1, and hybrid-BPS in 1. Of 5 unilateral, multifocal lung lesions, multifocality was prenatally established through identification of a band of normal intervening lung or intrinsic differences in lesion imaging features. Conclusions: Although less common, multiple thoracic abnormalities can be detected prenatally. Of multifocal lung lesions, the most common combination was CPAM-CPAM, with a unilateral, multilobar distribution. Prenatal recognition is important for pregnancy counseling and postnatal surgical management.

Congenital lung masses (CLM) the rare group of causes of acute respiratory insufficiency (RI) in newborns include congenital airway pulmonary malformation (CAPM), congenital overinflation, bronchopulmonary sequestration, and bronchial atresia. The presenting group consists of 13 newborns who were admitted to the Neonatal Department of Intensive Medicine (NDIM) during January 1st 2015-December 31st 2019 (8 males, 5 females, 2 premature/11 term newborns, spontaneous delivery: 2, caesarean section: 11) with positive prenatal diagnosis of CAPM in all cases. In 2 cases prenatal intervention was performed (drainage of the amniotic fluid, attempt of thoracentesis). Signs of acute RI immediately after delivery were seen in 5 newborns. Postnatal echocardiographic investigation confirmed the presence of increased pulmonary pressure in 8 patients, no patient had congenital heart abnormality. A thorax x-ray was positive also in asymptomatic patients. Computed tomography in patients brought detailed information about the position, size and character of CAPM. Six patients underwent surgery. In 15.4 % right lungs were affected by cystic malformation and in 23 % left lungs were affected. A final diagnosis of CAPM was confirmed in 5 patients using histopathologic examination. Multidisciplinary cooperation during prenatal as well as postnatal period is necessary.

International audience; AimOesophageal atresia is frequently associated with other malformations, and our aim was to use computed tomography (CT) to explore intrathoracic malformations in patients with this condition.MethodThis was retrospective study of children aged 0-16 with oesophageal atresia who were born in 1996-2013 and followed up at the French reference centre for rare oesophageal diseases at the University of Lille. Computed tomography scans were available for 48 of the 234 patients during follow-up visits, and these were reviewed by a thoracic radiologist.ResultsMore than two-thirds of the scans were performed to explore persistent respiratory symptoms. We found that six patients had a pulmonary malformations: four lobar agenesis, one right pulmonary aplasia and one congenital cystic adenomatoid malformation. Computed tomography enabled us to diagnose unexpected thoracic malformations in 16 patients: four lobar agenesis, six arteria lusoria, five persistent left superior vena cava and one partial anomalous pulmonary venous return. It also confirmed the diagnoses of suspected malformations in five patients: one congenital cystic adenomatoid malformation, one pulmonary hypoplasia, two right-sided aortic arches and one communicating bronchopulmonary foregut malformation.ConclusionIntrathoracic anomalies were frequently associated with oesophageal atresia, and contrast-enhanced chest CT scans should be performed on patients with persistent respiratory symptoms.

Authors discuss novel approach to the management of fetal congenital pulmonary cystic malformation (CPAM) and possible benefit of routine administration of betamethasone, which is currently recommended only for severe cases. The article presents authors' own experience with antenatally diagnosed CPAM and describes 4 cases of prenatally diagnosed CPAM without hydrops treated by two doses of betamethasone at 21-31 weeks of gestation with the aim of improving the perinatal prognosis by effect on not only mortality but also postnatal morbidity. Article also summarizes current knowledge on all aspects of the prenatal CPAM focusing on its treatment options. Data were obtained from the literature search based on the PubMed and Scopus database with additional search of particular articles from reference list of the selected publications.All 4 patients from the case reports showed regression of the pulmonary mass after maternal administration of betamethasone with 100 % survival rate. Even though it is not possible to say if the CPAM regression was due to the betamethasone treatment, we did not observe any complication in relation to the treatment and all cases survived until discharge. During the literature search, we did not find any data on betamethasone administration in non-hydropic fetuses with CPAM in relation to the overall perinatal and postnatal morbidity, neither data comparing the outcome between the treated versus observed only fetuses.Routine betamethasone treatment should be discussed in antenatally diagnosed CPAM cases without fetal hydrops in order to reduce the perinatal morbidity associated with CPAM (Tab. 1, Ref. 47). Keywords: betamethasone, CPAM (congenital pulmonary adenomatoid malformation), fetal therapy.

Purpose To investigate the characteristic thoracic multidetector computed tomography (MDCT) findings of pathologically proven combined congenital lung lesion consisting of bronchial atresia (BA) and congenital pulmonary airway malformation (CPAM) in children. Materials and methods All pediatric patients (age ≤ 18 years) with a known pathological diagnosis of a combined BA-CPAM congenital lung lesion, who underwent thoracic MDCT studies from January 2011 to January 2021 were included. Two pediatric radiologists independently evaluated thoracic MDCT studies for the presence of abnormalities in the lung, including nodule, mass, cyst, ground-glass opacity, and consolidation. When a lung abnormality was present, the number, size, composition (solid, cystic, or combination of both), borders (well-circumscribed vs. ill-defined), contrast enhancement pattern (nonenhancement vs. enhancement), and location (laterality, and lobar distribution) were also evaluated. Interobserver agreement between two independent reviewers was evaluated with κ statistics. Results Eighteen contrast-enhanced thoracic MDCT studies from 18 individual pediatric patients (8 males (44%) and 10 females (56%); mean age: 4.9 months; SD: 2.6; range: 1-10 months) with a pathological diagnosis of combined BA-CPAM congenital lung lesion comprised the final study population. The most frequent MDCT finding of combined BA-CPAM congenital lung lesion in children was a solitary (18/18; 100%), well-circumscribed (18/18; 100%), both solid and cystic (17/18; 94%) lesion with nonenhancing (17/17; 100%) nodule, reflecting the underlying BA component, adjacent to a well-circumscribed multicystic mass (18/18; 100%), representing the underlying CPAM component. This combined congenital lung lesion occurred in all lobes with similar frequency. There was almost perfect interobserver κ agreement between the two independent reviewers for detecting abnormalities on thoracic MDCT studies (k = 0.98). Conclusion The characteristic thoracic MDCT findings of a combined BA-CPAM congenital lung lesion are a solitary, well-circumscribed solid and multicystic mass, with a nonenhancing nodule, reflecting the BA component, adjacent to a cystic mass, representing the CPAM component. Accurate recognition of these characteristic MDCT findings of combined BA-CPAM congenital lung lesion has great potential to help differentiate this combined congenital lung lesion from other thoracic pathology in children.

Fetal lung masses complicate approximately 1 in 2000 live births. Our aim was to determine whether obstetric and neonatal outcomes differ by final fetal lung mass histology.A review of all pregnancies complicated by a prenatally diagnosed fetal lung mass between 2009 and 2017 at a single academic center was conducted. All cases included in the final analysis underwent surgical resection and histology diagnosis was determined by a trained pathologist. Clinical data were obtained from review of stored electronic medical records which contained linked maternal and neonatal records. Imaging records included both prenatal ultrasound and magnetic resonance imaging. Fisher's exact test was used for categorical variables and the Kruskal-Wallis test was used for continuous variables. The level of significance wasOf 61 pregnancies complicated by fetal lung mass during the study period, 45 cases underwent both prenatal care and postnatal resection. Final histology revealed 10 cases of congenital pulmonary airway malformation (CPAM) type 1, nine cases of CPAM type 2, and 16 cases of bronchopulmonary sequestration. There was no difference in initial, maximal, or final CPAM volume ratio between groups, with median final CPAM volume ratio of 0.6 for CPAM type 1, 0.7 for CPAM type 2, and 0.3 for bronchopulmonary sequestration (In our cohort, there was no significant association between histologic lung mass subtypes and any obstetric or neonatal morbidity including respiratory distress.

BackgroundCongenital Pulmonary Airway Malformation (CPAM) has an estimated prevalence between 0.87 and 1.02/10,000 live births and little is know about their pathogenesis. To improve our knowledge on these rare malformations, we analyzed the cellular origin of the two most frequent CPAM, CPAM types 1 and 2, and compared these malformations with adjacent healthy lung and human fetal lungs.MethodsWe prospectively enrolled 21 infants undergoing surgical resection for CPAM. Human fetal lung samples were collected after termination of pregnancy. Immunohistochemistry and proteomic analysis were performed on laser microdissected samples.ResultsCPAM 1 and 2 express mostly bronchial markers, such as cytokeratin 17 (Krt17) or α-smooth muscle actin (ACTA 2). CPAM 1 also expresses alveolar type II epithelial cell markers (SPC). Proteomic analysis on microlaser dissected epithelium confirmed these results and showed distinct protein profiles, CPAM 1 being more heterogeneous and displaying some similarities with fetal bronchi.ConclusionThis study provides new insights in CPAM etiology, showing clear distinction between CPAM types 1 and 2, by immunohistochemistry and proteomics. This suggests that CPAM 1 and CPAM 2 might occur at different stages of lung branching. Finally, the comparison between fetal lung structures and CPAMs shows clearly different protein profiles, thereby arguing against a developmental arrest in a localized part of the lung.

International audience; Objectives To assess prenatal changes in the volume of congenital pulmonary malformations (CPM) and examine whether these changes differ in lesions that appear cystic on ultrasound compared with hyperechoic lesions, and to study the relationship between CPM volume and risk of fetal compression. Methods We conducted a nationally representative, multicenter, prospective cohort study, which included 579 ultrasound examinations in 176 pregnant women with a diagnosis of fetal CPM, between March 2015 and November 2016. Several ultrasound examinations were performed between diagnosis and delivery, including measurement of CPM volume. We modeled changes in CPM volume ratio (CVR) as a function of gestational age, overall and for cystic/mixed vs hyperechoic malformations, and examined the association between CVR and signs of compression during pregnancy. Results When modeling CVR changes over time, there was a statistically significant decrease in CVR with increasing gestational age (P 0.4 vs CVR ≤ 0.4 cm2. Conclusions Predicted changes in CVR during pregnancy differ between cystic and hyperechoic malformations. This may be the result of different pathophysiological mechanisms or differences in the timing of occurrence of these different types of CPM. CVR measured at the initial diagnostic ultrasound examination was strongly associated with the odds of subsequent compression. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.

Background The aim of this study was to demonstrate the feasibility and safety of the Nuss procedure for patients with pectus excavatum (PE) with a history of intrathoracic surgery. Patients From April 2010 to December 2013, we performed 6 cases of PE repair in patients with a history of intrathoracic surgery. The causes of previous operations were congenital cystic adenomatoid malformation in 4 patients and congenital diaphragmatic hernia in 2. The patients’ median age was 5 years (range, 4–9 years) and median preoperative pectus severity index was 4.63 (range, 3.42–10.03). Their intraoperative and postoperative courses were reviewed retrospectively. Results The mean overall operation time was 127.5 ± 17.0 minutes, and the mean operation time for endoscopic pneumolysis was 28.8 ± 12.3 minutes. Intraoperative exploration for pleural adhesion revealed that the endoscopic approach in the previous operation was associated with low pleural adhesion, and the open thoracotomy or laparotomy approach was associated with low to high pleural adhesion. One patient developed a pneumothorax on the first postoperative day. All the other patients had uneventful postoperative courses. All the patients received bar removal 2–3 years after bar insertion. One patient developed atelectasis after bar removal. All the other patients had an uneventful postoperative course. The mean postoperative follow-up time after bar removal was 20.1 ± 14.7 months. Conclusions History of intrathoracic surgery seems not a contraindication for the Nuss procedure. However, perioperative complications should be carefully monitored in both the bar insertion and removal operations.

Congenital pulmonary airway malformation (CPAM), is a rare disease known as a developmental abnormality of the lower respiratory tract. It may occur in the neonatal period due to respiratory distress and patients usually die in the first few months of life. Rarely, it may remain asymptomatic until adulthood. In this study we describe an adult case and present a review of the literature. A 19-year-old male with no relevant medical history was admitted to our clinic with cough and wheeze. The patient had a history of frequent lower respiratory tract infection during childhood. Chest radiograph revealed a diffuse opacity and volume loss in the right hemithorax. High resolution tomography showed hypoplasia of the right hemithorax, multiple cysts in all of the lobes and segments on the right side, ground glass opacity and interlobular septal thickening of the whole right lung parenchyma. Right pneumonectomy was performed with the pre-diagnosis of congenital pulmonary airway malformation and the pathological examination was compatible with CPAM. CPAM is a rare disease in adulthood. We should consider CPAM in the differential diagnosis of patients with frequent recurrent pulmonary infection and cystic lung lesions. In order to prevent infections and to eliminate the risk of malignancy, surgical treatment should be applied for definite diagnosis and treatment. Konjenital pulmoner hava yolu malformasyonu (KPHM), alt solunum yollarında gelişimsel bir anormallik olarak bilinen nadir bir hastalıktır. Yenidoğan döneminde solunum sıkıntısı nedeniyle ortaya çıkabilir ve hastalar genellikle yaşamın ilk birkaç ayında ölür. Nadiren yetişkinliğe kadar asemptomatik kalabilir. Bu çalışmada tanımlanan erişkin bir olgu eşliğinde literatürün gözden geçirilmesini sunuyoruz. Bilinen kronik hastalığı olmayan 19 yaşında erkek öksürük ve hışıltı ile kliniğimize başvurdu. Hastanın çocukluk döneminde sık alt solunum yolu infeksiyonu öyküsü vardı. Akciğer grafisinde sağ hemitoraksta difüz opasite ve hacim kaybı izlendi. Yüksek çözünürlüklü akciğer tomografisinde sağ hemitoraksta hipoplazi, sağda tüm loblarda ve segmentlerde çok sayıda kist, buzlu cam opasitesi ve sağ akciğer parankiminin tamamında interlobüler septal kalınlaşmalar görüldü. KPHM ön tanısı ile sağ pnömonektomi yapıldı ve patoloji sonucu KPHM ile uyumlu bulundu. KPHM erişkin yaşta nadir görülen bir hastalıktır. Sık sık tekrarlayan akciğer infeksiyonu ve kistik akciğer lezyonu olan hastaların ayırıcı tanısında KPHM’yi düşünmeliyiz. İnfeksiyonları önlemek ve malignite riskini ortadan kaldırmak, kesin tanı ve tedavi için cerrahi tedavi uygulanmalıdır.

Purpose To examine postsurgical outcomes of a consecutive series of children treated with elective operations for congenital lung malformations (CLM). Methods A retrospective review was performed on a prospectively collected dataset of all fetuses evaluated for a CLM between July 2001 and June 2016. Prenatal findings, operative treatment and postnatal outcomes were collected. Children having elective operations were divided in two groups based on age at time of surgery. Results Of 220 fetuses, 143 had operations and follow-up at our center. Six had open fetal lobectomy, 17 had EXIT-to-resection, 16 infants had urgent resection for symptoms and 110 with asymptomatic lesions had elective resection. Of these 110, the median fetal maximum CVR was 0.8 [range 0.1–2.2], and median age at operation was 4 (1.5–60) months (58% had resection at ≤ 4 months). Overall complication rate, including air-leak and pleural effusion, was 15%. When comparing those having resection at ≤ 4 months to those > 4 months, there were no significant differences in complication rates or length of stay. Operative time was shorter for patients with early resection (154 ± 59 vs 181 ± 89, p = 0.05). No infant having resection at ≤ 4 months was readmitted. Overall, children survived with good recovery. Conclusion Early elective resection of congenital lung malformations prior to 4 months of age is feasible and not associated with increased operative risk. Type of study Restropective study. Level of evidence Level III.

Asymptomatic cystic lung lesions-congenital pulmonary airway malformations (CPAMs), sequestrations, and bronchogenic cysts-are commonly diagnosed prenatally. Indications to resect are to eliminate risk of malignancy or infection. CPAMs consist of a spectrum of malformations, with type 1 historically considered the most common. Mucinous cell clusters, seen almost exclusively in type 1, are premalignant lesions at risk for progression to mucinous adenocarcinoma. We reviewed and classified 2.5 years of consecutive, prenatally diagnosed lesions as extralobar sequestration, intralobar sequestration, type 1 CPAM, type 2 CPAM/bronchial atresia, or "other" to determine the distribution of lesion types and risk of malignancy. One hundred eighty-four lesions in 174 patients showed type 1 CPAM to be least common subtype. Type 1 CPAMs had more severe presentation, infrequently had features of obstruction, and usually had cysts ≥2 cm. Fifteen of eighteen type 1 CPAMs had mucinous cell clusters (total risk, 8%), with mucous cells outside main cyst in 12/15. No pleuropulmonary blastomas were identified. Additional historic cases were reviewed to further evaluate risk of malignancy. Over 14 years, 28 infants with fetal/type 1 lesions were identified, with clusters of mucinous cells in 75% of cases. A total of 9 pleuropulmonary blastomas were diagnosed in 6 patients over 16 years. Contrary to historical studies, type 1 CPAMs are much less common than type 2, likely related to detection of asymptomatic lesions prenatally. A majority of type 1 CPAMs contain mucinous cell clusters. This data is useful in management of patients in centers that do not resect asymptomatic lesions.

BACKGROUND The objective of this study was to determine acyl-CoA synthetase 5 (ACSL5) and Wnt2B expression patterns in human congenital pulmonary airway malformations (CPAMs) and to identify the possible roles of ACSL5 and Wnt2B in the pathogenesis of CPAM. METHODS Expression of ACSL5 and Wnt2B was evaluated by immunohistochemical staining, Western blotting, and quantitative real-time polymerase chain reaction, which were performed on surgical specimens of CPAM and adjacent normal lung tissues as controls. RESULTS Immunohistochemistry revealed that ACSL5 and Wnt2B immunopositive cells were predominantly detected in the mesenchymal cell nucleus, and there were lower expressions of ACSL5 and Wnt2B immunopositive cells in CPAM tissues than those in adjacent normal lung tissues. Western blotting and quantitative real-time polymerase chain reaction showed that ACSL5 and Wnt2B protein and mRNA expressions were significantly decreased in CPAM tissues as compared to the adjacent normal lung tissues (P

Background Fetal MRI-based differential diagnosis of congenital lung malformations is difficult because of the paucity of well-described imaging markers. Purpose To characterize the hyperintense bronchus sign (HBS) in in vivo fetal MRI of congenital lung malformation cases. Materials and Methods In this retrospective two-center study, fetal MRI scans obtained in fetuses with congenital lung malformations at US (January 2002 to September 2018) were reviewed for the HBS, a tubular or branching hyperintense structure within a lung lesion on T2-weighted images. The frequency of the HBS and respective gestational ages in weeks and days were analyzed. Areas under the curve (AUCs), 95% CIs, and P values of the HBS regarding airway obstruction, as found in histopathologic and postnatal CT findings as the reference standards, were calculated for different gestational ages. Results A total of 177 fetuses with congenital lung malformations (95 male fetuses) and 248 fetal MRI scans obtained at a median gestational age of 25.6 weeks (interquartile range, 8.9 weeks) were included. The HBS was found in 79% (53 of 67) of fetuses with bronchial atresia, 71% (39 of 55) with bronchopulmonary sequestration (BPS), 43% (three of seven) with hybrid lesion, 15% (six of 40) with congenital cystic adenomatoid malformation, and 13% (one of eight) with bronchogenic cyst at a median gestational age of 24.9 weeks (interquartile range, 9.7 weeks). HBS on MRI scans at any gestational age had an AUC of 0.76 (95% CI: 0.70, 0.83; P = .04) for the presence of isolated or BPS-associated airway obstruction at histopathologic analysis and postnatal CT. The AUC of HBS on fetal MRI scans obtained until gestational age of 26 weeks (AUC, 0.83; 95% CI: 0.75, 0.91; P < .001) was significantly higher (P = .045) than that for fetal MRI scans obtained after gestational age 26 weeks (AUC, 0.69; 95% CI: 0.57, 0.80; P = .004). Conclusion The hyperintense bronchus sign is a frequently detectable feature at fetal MRI and is associated with airway obstruction particularly before gestational age 26 weeks. © RSNA, 2021 Online supplemental material is available for this article. See also the editorial by Dubinsky in this issue.

Objective To study the effect of an early childhood education machine on sedation and analgesia in children after cardiothoracic surgery. Methods A prospective randomized controlled study was conducted in a provincial hospital in China. Fifty-two patients (aged from 1 to 5 years) underwent cardiothoracic surgery (including: ventricular septal defect, patent ductus arteriosus, atrial septal defect, pulmonary stenosis, pulmonary sequestration and congenital cystic adenomatoid lung malformation) were divided into the study group (n = 26) and the control group (n = 26). The patients in the study group underwent intervention with an early childhood education machine (uniform type) in addition to routine standard treatment and nursing, while the patients in the control group only received routine standard treatment and nursing. Richmond agitation sedation score (RASS) and face, legs, activity, cry, consolability (FLACC) score of all of the patients were evaluated, and the negative emotions (self-rating anxiety scale (SAS) score and self-rating depression scale (SDS) score) of the parents of the two groups were compared. Results There was no significant difference in the general clinical data between the two groups. The RASS and FLACC scores in the study group were significantly lower than those in the control group, and the SAS and SDS scores of the parents in the study group were significantly lower than those in the control group. Conclusion The application of an early childhood education machine for children after cardiothoracic surgery can effectively reduce postoperative agitation, improve sedation and analgesia of the patients, and ease the pessimistic mood of the patients’ parents.

We present the case of a baby boy, with antenatal finding of bilateral enlarged echogenic lungs, who developed severe postnatal respiratory failure. Two important differential diagnoses, which have an impact on antenatal and postnatal management, are discussed. Fetal ultrasound, MRI and postmortem MRI images are presented and findings correlated with the autopsy findings.

Background Congenital pulmonary airway malformation (CPAM) with partial anomalous pulmonary venous connection presenting as episodic retrosternal chest pain on exertion in an adult has not been described. Case presentation A 21-year-old female, non-smoker, presented with a 4-year history of sharp, episodic, retrosternal chest pains brought on with exercise. A contrast-enhanced computed tomography (CT) scan showed a grossly overinflated left lower lobe with partial anomalous pulmonary venous drainage into the left hemi-azygos vein. Lobectomy, the recommended treatment of choice, carried out thoracoscopically, was curative with satisfactory mid-term results. Histology confirmed type-II congenital pulmonary airway malformation. Conclusions CPAM can present in young adults with unusual symptoms of chest pain on exertion. When suspected a contrast-enhanced CT scan is the gold standard for establishing the diagnosis. An anatomical lung resection is curative with satisfactory medium term results.

Congenital pulmonary airway malformation (CPAM), previously known as congenital cystic airway malformation, is a developmental disorder of the lower respiratory tract. It is subdivided into 5 types based on clinical and pathologic features. Type 3, an adenomatoid type of CPAM, is the second rarest form of CPAM, occurring in approximately 5% of all CPAM cases. This article reports an autopsy of a nearly 11-week-old male infant, found unresponsive in bed with his mother. She had fallen asleep after breastfeeding a few hours prior. Although the autopsy and additional technical examinations did not uncover the exact cause of death, CPAM type 3 was eventually identified on histological examination. Taking into account the context of this case, in which accidental asphyxia/neglect could not be ruled out, it is thought that the presence of CPAM might have contributed to the demise of the infant. As CPAM is a rare congenital disorder, the diagnosis could easily be missed. Therefore, this article aims to raise awareness of this diagnosis and points out the clinical and pathologic features of this disorder.

Purpose The purpose of this study is to evaluate the potential advantages of thoracoscopic versus open resection for symptomatic congenital pulmonary airway malformation (CPAM) in neonates. Methods A retrospective review of the medical records of neonates (age ≤ 28 days) who underwent surgery for symptomatic CPAM from 2010 to 2020. Results Of the 24 patients, 14 patients underwent thoracoscopic resection and 10 patients underwent open resection. 4 patients with CPAM located in the upper or middle lobes underwent lobectomy, and 20 underwent lung-preserving wedge resection in the lower lobe. Between the two groups, there were no statistically significant differences in related preoperative variables, including gestational age at birth, body weight, head circumference, lesion size, cystic adenomatoid malformation volume ratio (CVR), and age at operation (P > .05). The differences in intraoperative variables were statistically significant. The length of the surgical incision was significantly shorter in thoracoscopic resection group than in open resection group (1.4 cm [1.3–1.8] vs. 6.0 cm [5.0–8.0], P = .000), along with significantly less operative blood loss (3 ml [1–6] vs. 5 ml [2–10], P = .030) but significantly longer operation time (159 min [100–220] vs. 110 min [70–170], P = .003). Regarding postoperative variables, ventilator days, duration of chest tube use and length of hospital stay were not statistically significant (P > .05). Conclusion Both thoracoscopic and open resection for symptomatic CPAM achieve good clinical outcomes, even in neonates. Thoracoscopic resection has minimal aesthetic effects and does not increase the risk of surgical or postoperative complications. Lung-preserving resection may be feasible for neonatal CPAM surgery.

There are limited data on congenital lung malformations (CLM) and their clinical course from developing countries. A 10-year retrospective chart review of records of children with CLM attending pediatric chest clinic at an Indian tertiary care center was conducted. Among the 48 children (24 boys) included in the review, the malformations included congenital lung hypoplasia/agenesis in 24 (50%), cystic pulmonary airway malformation in 9 (19%), bronchogenic/foregut cyst in 8 (18%), and congenital lobar emphysema in 4 (9%). Median (IQR) age at symptom onset and diagnosis were 1.5 (0.4,9.5) and 24 (3,62) months, respectively. Median (IQR) weight for age for age z-score at presentation was −2.4 (−1.4,− 3.4). More than a third (37.5%) children underwent surgical removal of resectable lesions at median (IQR) age of 14 (6,42) months. 14 (27%) children had associated congenital heart disease. Median duration of follow-up was 13 months. In children with lung hypoplasia, median (IQR) number of hospitalizations in follow-up were significantly less than that prior to diagnosis 0 (0,0) vs 1(0,2) (P=0.001). Median (IQR) numbers of hospitalizations in follow up were significantly less than that of prior to surgical resection 0 (0,0) vs 1(1,1) (P=0.016) in children with CPAM. Lung hypoplasia was the most common congenital lung malformation in our setup. Detection of malformation during antenatal period was poor. Age of diagnosis and surgical intervention is often delayed. Regular follow up and definitive and/or supportive management decreased the morbidity.

Objectives Mucinous adenocarcinoma arising in unresected congenital pulmonary airway malformation (CPAM) is rare. Underlying driver mutations in addition to KRAS gain-of-function mutations in this setting and the long-term outcomes of these patients are unknown. Methods We report a case of metastatic mucinous adenocarcinoma harboring both KRAS and GNAS mutations arising in a type 1 CPAM of a 14-year-old male. A literature review was performed. Results Next-generation sequencing revealed identical KRAS (G12V) mutations in both the CPAM and metastatic adenocarcinoma and a missense mutation in the GNAS (R201C) gene in the metastatic adenocarcinoma only. Median survival was 23 and 4 years for patients with localized (no or limited spread within the same lobe of CPAM) and distant involvement (spread to any different lobe of CPAM) of mucinous cells, respectively (95% confidence interval, 23-23 and 1.5-22 years, respectively; P = .017). Conclusions Mucinous cell proliferation associated with type 1 CPAM has exceptionally good long-term outcomes if confined within the same lobe of CPAM. A second oncogenic mutation in the GNAS gene may be necessary for progression to malignancy and distant spread.

Trisomy mosaicism of chromosome 5 is uncommon with few cases described. Case report: A 41-year-old woman underwent ultrasound (US) at 16 weeks, which showed oligohydramnios and intrauterine growth ...

Background Severe primary fetal hydrothorax (PFH) and fetal lung lesions (FLL) such as congenital pulmonary airway malformation (CPAM) and Bronchopulmonary sequestration (BPS) are often treated by thoraco-amniotic shunt (TAS). Objectives To compare short and long-term outcome of fetuses treated by TAS due to FLL to those treated due to PFH. Method A retrospective analysis was performed for all fetuses treated by TAS, between the years 2004–2015, evaluating the short and long term neurodevelopmental outcome. Long term neurodevelopment was additionally analyzed prospectively by Vineland adaptive behavioral scale (VABS) standardized questionnaires. Results 38 fetuses were treated by 52 TAS insertions; of which 13 (35%) due to FLL and 25 due to PFH. Perinatal survival was high (87.9%) with 3 neonatal death and one termination of pregnancy (TOP). High survival rate persisted even in cases requiring recurrent shunt insertion (80% survival). There was no significant difference in short or long term outcome including perinatal survival (84% Vs 90%, P = 0.64) and hydrops resolution (91% Vs 63%, p = 0.19). Long term outcome, including rate of neurodevelopmental abnormalities (23.5% Vs 20%) and VABS score (91.3 ± 13.3 Vs 96.4 ± 14.7), were similar for both groups. Conclusion TAS insertion is effective and resulting in high perinatal survival even in cases when sequential insertion is needed. Short and long- term outcome of neonates with FLL treated by TAS are comparable to neonates treated due to PFH.

Video-assisted thoracoscopic (VATS) resection of CPAM in children is an established, albeit controversial strategy for its management. We report a 10-year single center experience.All children underwent VATS (2008-2017) and their current status was reviewed. Patients were grouped: 'symptomatic-P' (if parents reported recurrent lower respiratory tract infections etc.) or 'symptomatic-S' (neonates presenting with respiratory distress/difficulty) or 'asymptomatic'.73 children, aged 10 m (4d-14yrs) underwent VATS; a neonate as an emergency ('symptomatic-S') and all others electively. The lesion was unilateral in all but one case. Histologically none were malignant. Of the elective 72 cases, 7 (10%) required conversion to open thoracotomy. Twenty (27.7%) were 'symptomatic-P' and the duration of surgery when compared to 'asymptomatic' children was longer 269 (range 129-689) versus 178 (range 69-575) minutes (P = 0.01). Post operatively, 8 children (11%) had a grade III/IV (Clavien-Dindo) complication; persistent air leak/pneumothorax (n = 5), chylothorax (n = 1), pleural effusion (n = 1) and seizure/middle cerebral artery thrombosis (n = 1). There was no mortality. Twenty-four children (33.3%) were reported 'symptomatic-P' post-surgery after a median follow up of 2.18 years. The surgical intervention had no impact on 'symptomatic-P' status (P = 0.46).The risks of surgery may outweigh benefit in asymptomatic children. CLINICALTRIALS.NCT04449614.

Background Congenital lung malformation is an umbrella term and consist of various kind of parenchymal and mediastinal pathologies. Surgical resection is often required for diagnosis and curative treatment. We aimed to review our experience in surgical treatment for congenital lung disease and present the role of minimally invasive surgery. Methods Surgical resections performed for benign lesions of the lung and mediastinum between January 2009 and May 2019 were retrospectively analyzed. Patients who were found to have congenital lung malformation as a result of pathological examination were included in our study. Distribution characteristics of the patients according to congenital lung malformation subtypes, differences in surgical approach and postoperative results were investigated. Results A total of 94 patients who underwent surgical resection and were diagnosed with the bronchogenic cyst, sequestration, bronchial atresia, congenital cystic adenomatoid malformation (CCAM), or enteric cyst as a result of pathological examination were included the study. There were no significant differences between pathological subtypes in the postoperative length of hospital stay and drainage duration however, perioperative complication rate was higher in the sequestration group. In addition, in the first three days postoperatively, the mean pain score was found to be lower in the VATS group compared to thoracotomy. Conclusions Congenital lung malformations consist of a heterogeneous group of diseases and the surgical treatment in these patients can range from a simple cyst excision to pneumonectomy. Video-assisted thoracoscopic surgery should be considered as the first choice in the surgical treatment of these patients in experienced centers.

Fetal therapy has been defined as any therapeutic intervention either invasive or noninvasive for correcting or treating any fetal malformation or condition. Invasive fetal therapy have its own set of maternal and fetal complications and invasive approach is not feasible in many of fetal conditions that are candidate for fetal therapy. Many such fetal conditions have been treated successfully by medical or noninvasive management. In medical fetal therapy, mothers are treated with medications which are transferred to fetus through placenta and exert positive effect on the fetus, thus avoiding complications that are seen secondary to invasive fetal therapy. The fetal conditions that have been managed with medical therapy includes fetal and neonatal alloimmune thrombocytopenia, neural tube defect, congenital adrenal hyperplasia, perinatal infections, respiratory distress syndrome, inborn error of metabolism, and congenital cystic adenomatoid malformation. This review will cover the medical or noninvasive aspect of fetal therapy and will highlight the progress made in the management of these fetal conditions.

Elective resection of congenital lung malformations (CLMs) is still debatable. The two main risks are malignant transformation and recurrent pulmonary infections. Our study aimed to assess the effect of previous pulmonary infection on the intraoperative and postoperative courses of thoracoscopic surgery for CLMs.This is a retrospective study including all thoracoscopic lung resections for CLMs between 2010 and 2019. Ninety patients were included. There was a history of previous pulmonary infection in 28 patients (group A) and no such history in 62 patients (group B).The median age at operation for group A was 20.4 months (IQR:14.9-41.4) versus 15.1 months (IQR:9.7-20.8) in group B (p = 0.006). There were 10 conversions (35.7%) in group A and 8 (12.9%) in group B (p = 0.02). The operative time was significantly shorter in group B (p0.002). In group A, 32.1% of patients experienced postoperative fever versus 11.3% of group B (p = 0.03), with higher antibiotics requirement (28.6% versus 6.5% respectively, p = 0.007). However, no significant differences were found in terms of postoperative complications (p = 0.99).Earlier intervention for CLMs before the development of pulmonary infection carries higher chances for the success of the thoracoscopic approach with shorter operative time and more uneventful postoperative courses.

INTRODUCTION Our aim is to compare thoracoscopy to thoracotomy in the treatment of congenital lung malformations (CLM) in children. MATERIALS AND METHODS We report a retrospective monocentric cohort study. Patients treated at our Center for CLM (1991-2020) were divided in two groups: patients treated with video-assisted thoracoscopic surgery (VATS) and open thoracotomy (OT). Characteristics of the two groups were compared through statistical analysis (GraphPad Prism7). A p value less than .05 was considered statistically significant. RESULTS One hundred six patients were included: 58 in VATS group, 48 in OT group. Prenatal diagnosis was possible in 73.6%. The most frequent surgical procedures were lobectomy (43.4%) and sequestrectomy (22.6%). All VATS patients underwent lung exclusion, mostly by endobronchial blocker (69%). Mean operative time was 146.1 min (±52.04 SD) in VATS and 159.2 (±46.53 SD) in OT (p = .1973). Conversion to OT was necessary in 20.6% of VATS patients, but decreased in the last 5 years (6.2%). There were not any intraoperative complication. Respectively in VATS and OT group, length of stay (LOS) was 4.5 days ± 3.6 SD versus 7.7 ± 3.4 SD (p

Objective: Fetal thoraco-amniotic shunts (TASs) can dislodge in utero, migrating internally into the fetal thorax or externally into the amniotic cavity. Our objective was to evaluate the perinatal and long-term outcome of fetuses with TAS dislodgement and conduct a review of the literature. Methods: This is a retrospective review of all TAS inserted for primary pleural effusions and macrocystic congenital pulmonary airway malformations (CPAMs) in a tertiary fetal medicine center (1991–2020). Antenatal history, procedural factors, and perinatal and long-term outcomes were reviewed in all fetuses with dislodged shunts and compared to fetuses with shunts that did not dislodge. Results: Of 211 TAS inserted at a mean gestational age of 27.8 weeks ± 5.47 (17.4–38.1 weeks), 187 (89%) were inserted for pleural effusions and 24 (11%) for macrocystic CPAMs. Shunts dislodged in 18 fetuses (8.5%), 17 (94%) of which were for pleural effusions. Shunts migrated into the chest wall/amniotic cavity or into the thorax among 7/18 (39%) and 11/18 (61%) fetuses, respectively. Eleven (61%) fetuses were initially hydropic, which resolved in 8 (72%) cases. Effusions were bilateral in 9 (50%), amnioreduction was required in 6 (33%), and fetal rotation in 8 cases (44%). Four (22%) fetuses underwent repeat shunting, 12 (67%) neonates required ventilatory support, and 2 (11%) neonates required chest tubes. There was no significant difference in technical factors or outcomes between infants with shunts that dislodged and those that did not. Among 11 intrathoracic shunts, 2 (18%) were removed postnatally and the remainder are in situ without any shunt-related or respiratory complications over a follow-up period of 9 months to 22 years. Conclusion: TAS dislodged antenatally in 8.5% of fetuses, with 2/3 of shunts migrating into the thorax, and nearly 25% requiring re-shunting. Retained intrathoracic shunts were well tolerated and may not necessarily require surgical removal after birth.