Congenital Lung Malformations Research Articles

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

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

Outcome of fetal congenital pulmonary malformations: a systematic review and meta-analysis.

To report the outcome of fetuses with a prenatal diagnosis of congenital lung malformation (CLM) diagnosed on ultrasound by performing a comprehensive assessment of these outcomes through a systematic review and meta-analysis. CLMs are a heterogeneous group of anomalies that involve the lung parenchyma and its bronchovascular structures. Their presentation and evolution are variable, from entirely asymptomatic lesions with sonographic regression in utero to hydropic fetuses requiring fetal therapy, intrauterine death or neonatal morbidity. A systematic review was conducted in Medline, Embase and Cochrane databases including studies on fetuses with CLM diagnosed prenatally in order to report the in-utero natural history of these lesions. Thirty-nine studies (2,638 fetuses) were included in the final review. Regression/reduction in size of the lung lesion during pregnancy was reported in 31 % of cases, while its increase in 8.5 % of cases. Intra-uterine death complicated 1.5 % of pregnancies with fetal CLM, while neonatal and perinatal death were 2.2 and 3 %, respectively. Neonatal morbidity occurred in 20.6 % of newborns with CLM; 46 % had surgery, mainly elective. In fetuses with CLM and hydrops, fetal/perinatal loss occurred in 42 %. Assessment of the role of fetal therapy in improving the outcomes of pregnancies complicated by CLM was hampered by the small number of included cases and heterogeneity of type of interventions. Fetuses with CLM prenatally diagnosed have a generally favorable outcome. Conversely, there is a low quality of evidence on the actual role of fetal therapy in improving the outcome of fetuses presenting with these anomalies.

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

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

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

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

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

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

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

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

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

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

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

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

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

IntroductionCongenital lung malformations (CLMs) have a variable natural history. Larger lesions with CLM volume ratio (CVR) ≥ 1.6 are associated with hydrops and fetal mortality. The purpose of this study is to describe the management and outcomes of high-risk (CVR ≥ 1.6) CLM patients. MethodsA retrospective cohort study was performed for all fetuses evaluated between May 2015 and May 2022. Demographics, prenatal imaging factors, prenatal and postnatal treatment, and outcomes were collected. Descriptive statistics were used to compare the cohorts. ResultsOf 149 fetal CLM patients referred to our fetal center, 21/149 (14%) had CVR ≥ 1.6. One CLM patient had intrauterine fetal demise, and 2 patients were lost to follow-up. Of the remaining 18 patients, 11/18 (67%) received maternal steroids. Seven out of 18 patients (39%) underwent resection at the time of delivery with 1/7 (14%) undergoing exutero intrapartum treatment (EXIT)-to-resection, 5/7 (71%) undergoing EXIT-to-exteriorization-to-resection, and 1/7 (14%) undergoing a coordinated delivery to resection; among those undergoing resection, there were 2 fatalities (28.5%). Seven out of 18 (39%) patients required urgent neonatal open lobectomies, and the remaining 4/18 (22%) patients underwent elective thoracoscopic lobectomies with no mortality. ConclusionsThe natural history and outcomes of severe CLM patients remain highly variable. The EXIT-to-exteriorization-to-resection procedure may be a safe and effective approach for a subset of CLM patients with persistent symptoms of mass effect and severe mediastinal shift due to the observed decreased operative time requiring placental support observed in our study.

Experience of video-assisted thoracic surgery treatment of congenital pulmonary airway malformation in infants less than 3 months of age.

Congenital pulmonary airway malformation (CPAM) is the most common type of congenital lung malformation, some children were born with serious respiratory and circulatory disorders, these children tend to have larger cystic lesions and emergency surgery should be taken after birth. This article aimed to explore the feasibility of video-assisted thoracic surgery (VATS) for CPAM in infants less than 3 months of age and analyze the early and mid-term postoperative outcomes. A retrospective analysis was performed on 12 children with CPAM who were admitted to Shanghai Children's Hospital from January 2019 to December 2022. Among them, there were ten boys and two girls. The age ranged from 13 to 89 days, with an average of 60.09±30.13 days. The body weight ranged from 2.8 to 7.5 kg, averaging 5.12±1.56 kg. All patients had severe respiratory symptoms such as persistently tachypneic, recurrent pneumonia or respiratory failure. All of the included children underwent VATS with no cases converted to thoracotomy. The operation time ranged from 40 to 190 minutes, with an average of 124.17 minutes. Among the children in this study, ten cases underwent lobectomy; one underwent segmental resection; one underwent irregular resection. Postoperative mechanical ventilation time ranged from 3 to 49 hours, with an average of 12.75 hours. The indwelling time of the thoracic drainage tube ranged from 2 to 9 days, with an average of 3.92 days. The postoperative hospital length of stay ranged from 5 to 12 days, averaging 8.42 days. None of the children had postoperative complications such as bleeding, bronchopleural fistula or atelectasis. The follow-up time ranged from 3 months to 4 years, and all the children were discharged within 12 days of postoperative hospitalization without need for readmission. Furthermore, no residual lesions were found in the reexamined chest computerized tomography (CT), the lung inflammation dissipated, and no residual cavity was in the affected chest. Infants with symptomatic CPAM less than 3 months of age should be operated on as soon as possible. With the improvement of minimally invasive technology and anesthesia management, VATS is relatively safe and effective in treating children with such conditions.

Percutaneous transthoracic catheter drainage prior to surgery in treating neonates with congenital macrocystic lung malformation presenting with respiratory distress

Percutaneous transthoracic catheter drainage prior to surgery in treating neonates with congenital macrocystic lung malformation presenting with respiratory distress

The complexities in diagnosing intradiaphragmatic extrapulmonary sequestration: a case report

BackgroundExtralobar pulmonary sequestration–a congenital lung malformation characterized by nonfunctional lung tissue with its own visceral pleura and without a connection to the normal tracheobronchial tree–is often surgically resected given the potential for infectious complications. We report a case of a child with extralobar pulmonary sequestration in a rare and challenging intradiaphragmatic location, which made preoperative identification and planning difficult.Case presentationA 2-year-old boy presented for follow-up of a large left extralobar pulmonary sequestration initially diagnosed on an antenatal ultrasound. Follow-up imaging, including prenatal MRI and postnatal CT angiography, was inconclusive on the precise location of the extralobar pulmonary sequestration in relation to the diaphragm but did reveal a subdiaphragmatic arterial supply from a branch off the celiac trunk. Planned resection with diagnostic thoracoscopy revealed the mass to seemingly be below the diaphragm; however, subsequent abdominal laparoscopy identified it to be within the diaphragm. Once the supplying vessel off the celiac trunk was controlled and divided, the mass was circumferentially excised from the edges of the diaphragmatic muscle. The remaining diaphragmatic defect was then closed, and the patient did well postoperatively.ConclusionsThis case demonstrates the difficulty of making a definitive diagnosis of intradiaphragmatic extralobar pulmonary sequestration without operative intervention. CT angiography and identification of the sequestration’s arterial supply may not be conclusive in determining the precise location if in close proximity to the diaphragm.

Diagnostic accuracy of imaging compared to histology in congenital lung malformations

IntroductionThe aim of this study was to evaluate the accuracy of imaging tests (prenatal ultrasound [US] and postnatal computed tomography [CT]) in comparison to histology for diagnosis of congenital lung malformations (CLMs). Material and methodsRetrospective study of patients with a prenatal diagnosis of CLM whose postnatal follow-up included thoracic CT scan and histological examination of the lesion. We collected data on demographic variables, gestational age at diagnosis, US findings and the history of multiple gestation. We used the kappa coefficient to determine the level of agreement between the findings of prenatal US and postnatal tests (CT and histology).We analysed paired data on the size of the lesion, its location and the presence or absence of systemic arterial vascularization. ResultsThe sample included 56 patients with 57 lesions. The mean gestational age at diagnosis was 22.42 weeks (SD, 3.94) and 57% were male. Malformations most frequently involved the left lung and the lower lobes. The agreement between CT and histology in the detection of cystic lesions was moderate (κ = 0.55) but stronger compared to the agreement between US and histology (κ = 0.10). The agreement between CT and histology was substantial (κ = 0.66) in the detection of systemic vascularization of the lesion and stronger compared to the agreement between US and histology. Both imaging methods were highly accurate in the identification of the location of the pulmonary lesions. Conclusionspostnatal CT offers a substantial concordance with histological findings, especially in the detection of systemic vascularization, and an accurate prediction of the anatomy of the lesion.

Novel Clinical Algorithm for Prenatal Monitoring of Congenital Lung Malformations

IntroductionCongenital lung malformations (CLMs) are readily identified early in pregnancy with a variable natural history. Monitoring for lesion size and mediastinal shift (MS) is recommended following diagnosis. The purpose of this study is to propose a risk-stratified clinical algorithm for prenatal monitoring of CLM. MethodsAfter ethical approval, all fetuses with CLMs evaluated at our fetal center from January 2015 to June 2022 were retrospectively reviewed. Patient demographics, imaging characteristics, and fetal interventions were collected. Lesions were stratified by congenital lung malformation volume ratio (CVR) and the presence of MS. Descriptive statistics and receiver operating characteristic curves were employed in the analysis. ResultsWe analyzed 111 patients with a mean of 23.4 wk gestational age, a median CVR of 0.5 (interquartile range, 0.3-1.2), and MS in 76 of 111(68%) patients on initial evaluation. Among low-risk patients (CVR ≤1.1), 96% remained low-risk on final evaluation. No patients transitioned from low to high risk during the growth period. Patients with CVR >1.1 often had persistent MS (P < 0.001). Hydrops (5/111, 5%) and fetal intervention (4/111, 4%) only occurred in patients with CVR >1.1 (P < 0.001, P = 0.002) and MS (P = 0.144, P = 0.214). On receiver operating characteristic curve analysis, initial CVR >1.1 had 100% sensitivity and negative predictive value for hydrops and fetal intervention. ConclusionsCLMs with initial CVR ≤1.1 are low risk for hydrops and fetal intervention. We propose a risk-stratified algorithm for the monitoring of CLM during the growth period based on CVR. While our experience suggests that patients with CLM and MS are at higher risk, the current subjective assessment of MS is not adequately predictive. Incorporating an MS grading system may further refine risk stratification in the management of CLM.

Prenatal Measurements of Congenital Lung Malformations: Can They Predict Postnatal Size?

IntroductionPrenatally diagnosed congenital lung malformations (CLMs) are monitored via ultrasound and measured by congenital pulmonary airway malformation volume ratios (CVRs) which can predict postnatal respiratory symptoms. This study compared CVR to postnatal lesion size to help guide prenatal counseling. MethodsA retrospective chart review evaluated the prenatal imaging and postnatal outcomes for patients who were prenatally diagnosed with CLMs and had a postnatal computed tomography (CT) scan at one institution. ResultsFifty-seven patients were included. Four had symptoms requiring urgent resection. The remaining were discharged and had clinic follow-up with CT scan to determine next steps: five had no identified lesions, eight had lesions whose diagnosis did not warrant an operation, and 40 had lesions whose diagnosis rendered size a factor in operative decision-making. Of these 40, 26/40 patients (65%) underwent elective resection (median maximum CVR 0.97; median lesion size 4 cm) and 14/40 patients (35%) were observed without resection (median maximum CVR 0.5; median lesion size 3 cm). There was a positive correlation between prenatal CVR and postnatal lesion size, with R-squared = 0.46. Maximum CVRs were better than last CVRs when predicting whether postnatal CT size would fall above or below our institution's level of recommended resection, with an area under the curve of 0.85 and a CVR cut-point of 0.61. ConclusionsFor newborns with asymptomatic CLMs, higher maximum CVRs correlated with larger size on postnatal CT. A maximum CVR ≤0.6 was correlated with a smaller postnatal CT size that may be eligible for nonoperative management. While these results are not intended to recommend surgery based on higher CVRs alone, this information could potentially be used to reassure expectant parents whose babies’ prenatal imaging demonstrate lower maximum CVRs.

Pulmonary function after lobectomy in children: a systematic review and meta-analysis

BackgroundThe influence of lobectomy on pulmonary function in children was still controversial. A systematic review and meta-analysis were essential to explore whether pulmonary function was impaired after lobectomy in children.MethodsPubMed,...

Pulmonary sequestrations

Congenital lung malformations account for 2.2 – 6.6% of all congenital abnormalities of the lungs. This is a pretty rare problem as compared to acquired lung diseases. Pulmonary sequestrations account for 0.15 – 1.8% in the incidence of all lung malformations, occupying the second place after the complex abnormalities called “lung agenesis-hypoplasia”. Pulmonary sequestrations are common in children and adolescents so one could encounter them in pediatric practice. But pulmonary sequestrations may be associated with insignificant symptoms or even be asymptomatic in selected pediatric cases. Thereby, adult general practitioners, pulmonologists, and thoracic surgeons may encounter such patients. The aim. Review had the purpose to inform the general practitioners, pulmonologists, and thoracic surgeons about pulmonary sequestrations and their diagnostic, clinics course, and treatment. Conclusion. There is a wide range of congenital abnormalities of human body. Pulmonary sequestrations are only a part of this huge problem. But modern medicine has a vast selection of methods for revealing and treating these conditions (including minimally invasive surgery).

Prognosis of the risk progression of post-inflammatory pneumofibrosis in children with chronic nonspecific lung diseases

Introduction. Pneumofibrosis (PF) is a morphological outcome of acute and chronic lung diseases, the progression of which leads to organ deficiency.Aim. Development of a prognostic algorithm for assessing the risk of progression of post-inflammatory PF in children with chronic nonspecific lung diseases (CNSLD).Materials and methods. The examination of 52 children with CNSLD with focal post-inflammatory PF was conducted, of which 26 children with progressive PF and 26 children with non-progressive PF. The children were selected according to the “case-control” scheme. The patients had a dynamic clinical and laboratory examination with multispiral computed tomography of the lungs. Determination of the “zero” genotypes of the detoxification genes GSTM1, GSTT1 was carried out by means of polymerase chain reaction. The odds ratio indicator was used to assess the relative risk.Results. Based on the analysis of medico-social, clinical characteristics and genetic polymorphism, an individual prognostic algorithm for the risk of progression of post-inflammatory PF in children with CNSLD was developed. The algorithm includes the calculation of the total score (TS) of 7 medical and social indicators (living in an urban area; the presence of passive smoking; the duration of a pulmonological history of 4-9 years; the presence of congenital lung malformation; the number of acute respiratory infections (ARI) 4 or more times/year; the duration of ARI 11 days or more; taking antibiotics for more than 3 once/year) and the presence of deletions in the detoxification genes GSTM1 and GSTT1. With a value of TS 6.97 or more, a high risk of progression of PF is predicted, with TS 3.47-6.96 points, a moderate risk of progression of PF is diagnosed, with TS 3.46 or less points, a minimal risk of progression of PF.Conclusion. The proposed algorithm allows predicting the risk of progression of post-inflammatory PF in children with CNSLD at the stage of early diagnosis and will prevent the spread of the process in the lungs with the help of preventive measures and preventive therapy, which will increase the duration and improve the quality of life of the patient.

Pulmonary Sequestration and Congenital Pulmonary Airway Malformation (CPAM) 1 Presented as A Left Suprarenal Mass:ACase Report

Introduction Pulmonary sequestration is a rare condition accounting for 0.1% to 6% of all congenital lung malformations. The correlation with Congenital Pulmonary Airway Malformation (CPAM) type 1 has not been adequately elucidated. Case presentation From routine fetal sonographic screening, a 20-day-old female baby's ultrasound revealed hydronephrosis with enlargement above the left kidney. Magnetic resonance imaging during a follow-up appointment uncovered a left suprarenal lesion of approximately 30 mm, which was not suspected to be adrenal neuroblastoma. After resection, histological analysis was consistent with pulmonary sequestration and confirmed the connection with CPAM type 1. Conclusion Surgery remains the only effective therapeutic choice for intra-abdominal pulmonary sequestration. The definitive diagnosis is confirmed through histological examination.

Concordancia entre el diagnóstico por imagen y la histología en las malformaciones pulmonares congénitas

IntroductionThe aim of this study was to evaluate the accuracy of imaging tests (prenatal ultrasound [US] and postnatal computed tomography [CT]) in comparison to histology for diagnosis of congenital lung malformations (CLMs). Material and methodsRetrospective study of patients with a prenatal diagnosis of CLM whose postnatal followup included thoracic CT scan and histological examination of the lesion. We collected data on demographic variables, gestational age at diagnosis, US findings and the history of multiple gestation. We used the kappa coefficient to determine the level of agreement between the findings of prenatal US and postnatal tests (CT and histology). We analysed paired data on the size of the lesion, its location and the presence or absence of systemic arterial vascularization. ResultsThe sample included 56 patients with 57 lesions. The mean gestational age at diagnosis was 22.42 weeks (SD, 3.94) and 57% were male. Malformations most frequently involved the left lung and the lower lobes. The agreement between CT and histology in the detection of cystic lesions was moderate (κ=.55) but stronger compared to the agreement between US and histology (κ=.10). The agreement between CT and histology was substantial (κ=.66) in the detection of systemic vascularization of the lesion and stronger compared to the agreement between US and histology. Both imaging methods were highly accurate in the identification of the location of the pulmonary lesions. ConclusionsPostnatal CT offers a substantial concordance with histological findings, especially in the detection of systemic vascularization, and an accurate prediction of the anatomy of the lesion.