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

INTRODUCTION: 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. METHODS: 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. RESULTS: 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 12 months (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. CONCLUSION: 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.

Anatomical Variations of the External Genitalia in Posterior Cloaca: Clinical Consequences of Misdiagnosis-A Systematic Review of the Literature and the ARM-Net Consortium Experience.

PURPOSE: All types of cloacal malformations may be associated with anatomic variations of the external genitalia, including hypoplasia of the labia minora and enlarged clitoris; these variations could be even higher in posterior cloacas (PCs). If a careful physical examination is not performed, patients may be misdiagnosed with ambiguous genitalia (AG), leading to subsequent unnecessary testing, surgeries, or even wrong gender assignment. The aim was to analyze data of patients with PC within the ARM-Net registry, focusing on the description of the genitalia, gender assignment, and its consequences. Additionally, we investigated the presence of AG diagnosis in utero or at birth in patients with PC in the literature. METHODS: The ARM-Net registry was scanned for PC cases and data on diagnosis were collected. A systematic literature search was conducted using the PubMed, EMbase, and Web-of-Science databases. Descriptive statistics was used to report data. RESULTS: Nine patients with PC were identified in the ARM-Net registry. Five patients (55%) were diagnosed with AG, two (22%) were assigned as males and only two patients were correctly assigned as females and diagnosed with PC with respective variations of external genitalia. All patients diagnosed with AG had extensive blood testing including karyotype and hormonal studies. One of the patients who was diagnosed as a male, had surgery for pelvic cystic mass removal, which ultimately led to unaware salpingo-oophorectomy, hysterectomy, and vaginectomy. In the literature we identified 60 patients, 14 (23%) with AG, 1 with clitorolabial transposition and 1 with undeveloped vulva and vagina; 4 patients had normal anatomy. In 40 (67%) patients the anatomy of genitalia was not mentioned. CONCLUSION: Patients with PC are at high risk of being diagnosed with AG or even assigned the wrong gender at birth. In our series two patients were assigned as males, and consequently one of them underwent a highly mutilating surgery. A thorough physical examination together with a high index of suspicion and laboratory workup are mandatory to identify these variations, avoiding further investigations, unnecessary surgeries, and parental stress.

Congenital Lung Malformations: Pulmonary Function Assessment Before and After Surgery.

BACKGROUND: The management of asymptomatic congenital lung malformations (CLM) is debated, and pulmonary function of patients with CLM is seldom discussed. Short-term respiratory outcome in patients operated for CLM was assessed in order to better define surgical impact. METHODS: A retrospective study on patients operated for CLM between 2012 and 2021 was performed. Respiratory function was assessed with tidal breath analysis (TBA) in spontaneous sleep within 2 years of life. Patients with comorbidities affecting pulmonary function were excluded. Three variables were studied: tPTEF/tE (time to peak tidal expiratory flow/total expiratory time), tV (tidal volume), RR (respiratory rate). Results were assessed as z-score (normal ±1.64) and expressed as median (range). Pre- and post-operative results were compared, as well as post-operative results of disease and surgery type subgroups. RESULTS: Thirty-nine patients were included. Median pre- and post-operative tPTEF/tE were normal (pre: -0.32 (-2.12; +1.56); post: -0.18 (-1.62; +2.43)). Patients with extralobar sequestration had pre-operative high median RR, which improved after sequestrectomy (pre: +1.99 (-2.49; +7.43); post -0.22 (-3.01; +1.18)). All groups had reduced pre-operative median tV, which restored after surgery (pre: -2.15 (-9.75; +0.91); post: -0.35 (-6.65; +3.14)). Patients undergoing lobectomy and patients with intralobar sequestration showed greater improvement. Median post-operative TBA results were normal for all sub-groups except for reduced tV in patients operated with thoracotomy (-2.27 (-6.12; +5.26)). CONCLUSIONS: Patients with clinically asymptomatic CLM can have pathologic pulmonary function tests, which improve after surgery. These results add to the elements in favor of an interventional approach. LEVEL OF EVIDENCE: III, Treatment study.

Health status of petrochemical workers: a narrative review.

SUMMARY: Professional exposure to benzene has been extensively investigated by occupational medicine, leading to strict regulation of exposure threshold values. However, the petrochemical industry utilizes many chemical substances, whose exposure, without effective control and mitigation actions, could influence the health status over time. The aim of this narrative review is to describe health status of petrochemical workers related to occupational exposures, inquiring literature from 1980 to present. We used the PubMed and Web of Science search engines. As regards non-neoplastic diseases, despite heterogeneous prevalence estimates, we could say that standardized mortality rate (SMR) for hypertension, hypercholesterolemia and diabetes does not increase overall, compared to reference populations; a possible explanation may be the "healthy worker effect". Attention should be paid to color disperception and respiratory symptoms, due to toxic or irritating substances exposure. Studies concerning neoplastic pathology have mainly investigated mortality outcomes, finding no increase in cancer, except for melanoma or other skin cancers and leukemia. As regards the former, however, it is not excluded that other risk factors may contribute (e.g. UV rays in offshore workers), while for leukemia, only the most recent studies have analyzed various subtypes of hematopoietic tumors, highlighting a possible risk for the development of myelodysplastic syndrome. The risk of pleural mesothelioma was also increased, likely due to asbestos exposures, while the risk of death from prostate cancer remains controversial.

Bowel function and associated risk factors at preschool and early childhood age in children with anorectal malformation type rectovestibular fistula: An ARM-Net consortium study.

BACKGROUND: Outcome of patients operated for anorectal malformation (ARM) type rectovestibular fistula (RVF) is generally considered to be good. However, large multi-center studies are scarce, mostly describing pooled outcome of different ARM-types, in adult patients. Therefore, counseling parents concerning the bowel function at early age is challenging. Aim of this study was to evaluate bowel function of RVF-patients at preschool/early childhood age and determine risk factors for poor functional outcome. METHODS: A multi-center cohort study was performed. Patient characteristics, associated anomalies, sacral ratio, surgical procedures, post-reconstructive complications, one-year constipation, and Bowel Function Score (BFS) at 4-7 years of follow-up were registered. Groups with below normal (BFS < 17; subgroups 'poor' ≤ 11, and 'fair' 11 < BFS < 17) and good outcome (BFS ≥ 17) were formed. Univariable analyses were performed to detect risk factors for outcome. RESULTS: The study included 111 RVF-patients. Median BFS was 16 (range 6-20). The 'below normal' group consisted of 61 patients (55.0%). Overall, we reported soiling, fecal accidents, and constipation in 64.9%, 35.1% and 70.3%, respectively. Bowel management was performed in 23.4% of patients. Risk factors for poor outcome were tethered cord and low sacral ratio, while sacral anomalies, low sacral ratio, prior enterostomy, post-reconstructive complications, and one-year constipation were for being on bowel management. CONCLUSIONS: Although median BFS at 4-7 year follow-up is nearly normal, the majority of patients suffers from some degree of soiling and constipation, and almost 25% needs bowel management. Several factors were associated with poor bowel function outcome and bowel management. LEVEL OF EVIDENCE: Level III.

Intrauterine Ultrasound-Guided Laser Coagulation as a First Step for Treatment of Prenatally Complicated Bronchopulmonary Sequestration: Our Experience and Literature Review.

INTRODUCTION: Prenatal ultrasound-guided laser coagulation (USLC) for complicated bronchopulmonary sequestrations has been described but a consensus on the procedure and on the following management is still lacking. We present our experience and provide a literature review. METHODS: Retrospective review of patients treated in our center. Literature review and combined analysis of perinatal data were performed. RESULTS: Five cases were treated at our center, all presenting with severe hydrothorax. Four met the criteria for fetal hydrops. Four cases underwent postnatal computed tomography (CT) scan: in one case, there was no evidence of persistent bronchopulmonary sequestration. The other three underwent thoracoscopic resection, in two, a viable sequestration was found. Including our series, 57 cases have been reported, with no mortality and a success rate of 94.7%. Mean gestational age (GA) at the procedure was 28 ± 3.4 weeks and mean GA at birth and birth weight (BW) were 38.6 ± 2.3 weeks and 3,276 ± 519.8 g, respectively. In 80.6% of the cases investigated postnatally, a residual mass was found, 50% of cases who showed prenatal arterial flow cessation had a persistent sequestration postnatally, and 26.3% of cases underwent postnatal sequestrectomy. Both patients in our series had pathology examination confirming a viable bronchopulmonary sequestration. CONCLUSION: Prenatal USLC seems to be a valid option for bronchopulmonary sequestration complicated by severe hydrothorax and/or fetal hydrops. Authors believe that this procedure should aim to reverse fetal distress and allow pregnancy continuation, and it should not be considered a definitive treatment. The currently available data do not support changes of the common postnatal management.

Urological Impact of Epididymo-orchitis in Patients with Anorectal Malformation: An ARM-Net Consortium Study.

INTRODUCTION: To investigate the current experience of the ARM-Net Consortium in the management of epididymo-orchitis (EO) in patients with anorectal malformations (ARMs), and to identify specific risk factors and the need for urological care involvement. MATERIALS AND METHODS: We retrospectively collected data of EO in patients with ARM between 2015 and 2019. Data on urological aspects, ARM type, surgical approach, associated anomalies, diagnosis, and treatment of EO were collected and analyzed. RESULTS: Twenty-nine patients were reported by 12 centers. Twenty-six patients with EO (90%) had ARM with a rectourinary fistula. Median age at first EO was 2 years (range: 15 days-27 years). Twenty patients (69%) experienced multiple EO, and 60% of recurrences were ipsilateral. Associated urological anomalies included vesicoureteral reflux (48%), urethral anomalies (41%), neurogenic bladder (41%), and ectopic vas (10%). A positive urine culture during EO was present in 69%. EO was treated with antibiotics (90%), limiting surgical exploration to 14%. Prevention of recurrences included surgery (bulking agents 15%, vasectomy 15%, and orchiectomy 5%) and antibiotic prophylaxis (20%). CONCLUSION: Urologists may encounter patients with EO in ARM patients, frequently with positive urine culture. An appropriate urologic work-up for most ARM patients is necessary to identify and treat underlying risk factors. A practical scheme for the work-up is suggested for a close collaboration between pediatric surgeons and urologists.

Cerebral Oxygenation by Near-Infrared Spectroscopy in Infants Undergoing Thoracoscopic Lung Resection.

Background: Thoracoscopic resection is the standard of care for congenital lung malformations (CLMs) in infants. However, there is rising concern that capnothorax may affect cerebral perfusion and oxygenation, carrying potential long-term effects on neurodevelopmental behavior. The aim of our study was to investigate, using near-infrared spectroscopy (NIRS), the regional cerebral oxygenation (CrSO2) in infants undergoing thoracoscopic lung resection; the secondary aim was to assess the relationship between rSO2 and standard monitoring. Methods: In this retrospective study, we reviewed all infants (<1 year old, ASA II) who underwent thoracoscopic CLM resection in double-lung ventilation under fixed capnothorax parameters (5 mmHg of pressure, 1 L/minute flow), standardized anesthetic protocol, standard monitoring, and multisite NIRS in our center. We focused our attention on 8 anesthetic and surgical maneuvers, potentially affecting tissue oxygen saturation. Results: Ten infants met the inclusion criteria. At surgery, median age was 5.5 (4-7) months, median weight 7.2 (6.6-8) kg, median operative time 110 (55-180) minutes, and median capnothorax duration 79 (34-168) minutes. No conversion to open surgery occurred. CrSO2 values remained within clinically accepted values during thoracoscopy, beside a CrSO2 drop >20% of basal value in 1 patient, during capnothorax induction. Renal NIRS added very little to standard monitoring, which appeared generally inadequate to consistently appraise end-organ perfusion. ETCO2 best correlated with CrSO2 variations, suggesting to be able to realistically predict them. Conclusions: The thoracoscopic treatment of CLMs under the given conditions appears well tolerated in infants, pending the continuous adjustment of ventilator settings by an experienced anesthetist, confident with NIRS technology.

Mentoring in Pediatric Thoracoscopy: From Theory to Practice.

Introduction: Thoracoscopy represents the most challenging area of pediatric minimally invasive surgery due to its technical difficulty. A standardized training program would be advisable. The aim of this study is to evaluate the results of our surgical training. Materials and Methods: A retrospective, single-center, cohort study was performed. The following four-step program was tested: (1) theoretical part; (2) experimental training; (3) training in centers of reference; (4) personal operative experience. Particular attention was focused on the choice of mentor. Times and modality of adherence to the program were evaluated. The effectiveness and safety of the training were evaluated according to the surgical results of esophageal atresia (EA/TEF) repair and resection of congenital lung malformations (CLM). The study was conducted from January 2014 to May 2020. Attending surgeons with previous experience in neonatal and pediatric laparoscopy were selected for the training program after being evaluated by the head of Department. Results: The training program was fully completed in 2 years. Twenty-four lobectomies, 9 sequestrectomies, 2 bronchogenic cyst resections and 20 EA/TEF repair were performed. Thoracoscopy was always feasible and effective, with no conversion. The operative times progressively decreased. Only three minor complications were recorded, all treated conservatively. Conclusions: A standardized training program is highly desirable to learn how to safely perform advanced pediatric thoracoscopy. The 4-steps design seems a valid educational option. The choice of the mentor is crucial. An experience-based profile for pediatric surgeons who may teach thoracoscopy is advisable.

The coagulation profile as a marker for acute appendicitis in the paediatric population: Retrospective study.

INTRODUCTION: Appendicitis is the commonest and most frequently misdiagnosed acute abdominal surgical illness in the paediatric population worldwide. The aim of this study is to evaluate the role of coagulation profile in acute appendicitis (AA) in children. MATERIALS AND METHODS: we retrospectively collected data of patients submitted to appendectomy from 2011 to 2017. According to histopathology, patients were divided into three groups: not histologically confirmed AA (NAA), simple AA (SAA) and complicated AA (CAA). White blood cell (WBC) count, relative neutrophilia (Neutr%), C-reactive protein (CRP), prothrombin time ratio (PTratio), activated partial thromboplastin time ratio (APTTratio) and fibrinogen (Fib) were compared among groups. RESULTS: Three hundred and seven patients were included: 57 NAA, 184 SAA and 66 CAA. WBC was significantly different among groups: CAA (mean 16.67 × 103/ml), SAA (14.73 × 103/ml, P= 0.01) and NAA (10.85 × 103/ml, P< 0.0001). Significant differences were found for Neutr% (mean CAA 81.14 vs. SAA 77.03 P= 0.006, vs. NAA 63.86 P< 0.0001) and CRP (mean NAA 2.56, SAA 3.26, CAA 11.58, P< 0.0001). PTratio and Fib increased with the severity of AA receiver operator characteristic curves were similar for CRP (0.739), Fib (0.726), WBC (0.746) and Neutr% (0.754), while for PTratio and aPTTratio were 0.634 and 0.441, respectively. CONCLUSIONS: extrinsic coagulation pathway is altered in AA, especially in CAA. Coagulation can be useful in the diagnostic and perioperative anaesthetic management of AA in children. Fib seems to have the highest accuracy.

Inter- and Intraobserver Variation in the Assessment of Preoperative Colostograms in Male Anorectal Malformations: An ARM-Net Consortium Survey.

Aim: Male patients with anorectal malformations (ARM) are classified according to presence and level of the recto-urinary fistula. This is traditionally established by a preoperative high-pressure distal colostogram that may be variably interpreted by different surgeons. The aim of this study was to evaluate the inter- and intraobserver variation in the assessment by pediatric surgeons of preoperative colostograms with respect to the level of the recto-urinary fistula. Materials and Methods: Sixteen pediatric surgeons from 14 European centers belonging to the ARM-Net Consortium twice scored 130 images of distal colostograms taken in sagittal projection at a median age of 66 days of life (range: 4-1,106 days). Surgeons were asked to classify the fistula in bulbar, prostatic, bladder-neck, no fistula, and "unclear anatomy" example. Their assessments were compared with the intraoperative findings (kappa) for two scoring rounds with an interval of 6 months (intraobserver variation). Agreement among the surgeons' scores (interobserver variation) was also calculated using Krippendorff's alpha. A kappa over 0.75 is considered excellent, between 0.40 and 0.75 fair to good, and below 0.40 poor. Surgeons were asked to score the images in "poor" and "good" quality and to provide their years of experience in ARM treatment. Results: Agreement between the image-based rating of surgeons and the intraoperative findings ranges from 0.06 to 0.45 (mean 0.31). Interobserver variation is higher (Krippendorff's alpha between 0.40 and 0.45). Years of experience in ARM treatment does not seem to influence the scoring. The mean intraobserver variation between the two rounds is 0.64. Overall, the quality of the images is considered poor. Images categorized as having a good quality result in a statistically significant higher kappa (mean: 0.36 and 0.37 in the first and second round, respectively) than in the group of bad-quality images (mean: 0.25 and 0.23, respectively). Conclusions: There is poor agreement among experienced pediatric colorectal surgeons on preoperative colostograms. Techniques and analyses of images need to be improved in order to generate a homogeneous series of patients and make comparison of outcomes reliable.

Is Fetal Endoscopic Tracheal Occlusion (FETO) a Predisposing Factor for Acid Gastro-Esophageal Reflux in Infants With Congenital Diaphragmatic Hernia?

Introduction: Various anatomical defects predispose patients with congenital diaphragmatic hernia (CDH) to develop gastroesophageal reflux disease (GERD). The fetal endoscopic tracheal occlusion (FETO) has increased the survival of patients with severe CDHs. The aim of this study was to study GERD in patients who underwent FETO. Materials and Methods: We included patients with CDH treated with or without FETO ("FETO" and "no-FETO" group, respectively) from 2013 to 2016. Data on gestational age (GA), birth weight (BW), initial observed/expected lung to head ratio (O/E LHR), final O/E LHR, duration of ventilation and hospitalization, maximal tracheal diameter, and pulmonary volume were collected. All patients underwent pH-metry after 1 year of life, and the results were compared between groups and correlated to risk factors. Results: Thirty-two patients were included in the study: 10 FETO and 22 no-FETO. No significant differences were observed in the pH-metric results of the two groups. No correlation was found between GA, BW, initial O/E LHR, maximal tracheal diameter, pulmonary volume, and pH-metric results. pH-metric results were correlated with the total duration of ventilation (R = 0.5, p = 0.003) and of hospitalization (R = 0.54, p = 0.001). Gastric herniation is associated with the worse pH-metric result. Conclusions: The FETO procedure does not seem to represent an independent risk factor for GERD. However, patients with the most severe CDH have the worst GERD.

Combined Pre- and Postnatal Minimally Invasive Approach to Complicated Pulmonary Sequestrations.

Pulmonary sequestration (PS) is mostly asymptomatic but there is a proportion of fetuses that develop hydrops, leading to fetal or neonatal death. Fetal treatments are available, but postnatal management of the residual lesions is not uniformly defined. We present two cases of combined pre- and postnatal minimally invasive approach to complicated extra-lobar PS. Patient 1 presented with complicated PS at 31 weeks of gestation. Ultrasound-guided laser coagulation of the anomalous artery was successful. The patient was born asymptomatic at 38 weeks. Neonatal magnetic resonance imaging (MRI) showed a residual mass, confirmed by computed tomography (CT) at 6 months. No systemic artery was described, but perfusion was present. We decided for thoracoscopic resection. A residual artery was identified and sealed. Patient 2 presented with complicated PS at 25 weeks of gestation, underwent laser coagulation of the anomalous artery and was born asymptomatic at 38 weeks. Neonatal MRI showed persistence of the lesion, confirmed by CT scan at 4 months. We proceeded with thoracoscopic resection. A residual vessel was ligated. The patients 1 and 2 are now 24 and 21 months old, respectively, and healthy. Prenatal treatment of complicated PS is a life-saving procedure. Postnatal thoracoscopic resection of the residual lesion is feasible and safe; we believe it is the best course of treatment to grant the complete excision of the malformation.

Perineal Groove: An Anorectal Malformation Network, Consortium Study.

OBJECTIVE: To review the Anorectal Malformation Network experience with perineal groove (PG) focusing on its clinical characteristics and management. STUDY DESIGN: Data on patients with PG managed at 10 participating Anorectal Malformation Network centers in 1999-2019 were collected retrospectively by questionnaire. RESULTS: The cohort included 66 patients (65 females) of median age 1.4 months at diagnosis. The leading referral diagnosis was anal fissure (n = 20 [30.3%]): 23 patients (34.8%) had anorectal malformations. Expectant management was practiced in 47 patients (71.2%). Eight (17%) were eventually operated for local complications. The median time to surgery was 14 months (range, 3.0-48.6 months), and the median age at surgery was 18.3 months (range, 4.8-58.0 months). In the 35 patients available for follow-up of the remaining 39 managed expectantly, 23 (65.7%) showed complete or near-complete self-epithelization by a mean age 15.3 months (range, 1-72 months) and 4 (11.4%) showed partial self-epithelization by a mean age 21 months (range, 3-48 months). Eight patients showed no resolution (5 were followed for ≤3 months). Nineteen patients (28.7%) were primarily treated with surgery. In total, 27 patients were operated. Dehiscence occurred in 3 of 27 operated patients (11.1%). CONCLUSIONS: PG seems to be an underestimated anomaly, frequently associated with anorectal malformations. Most cases heal spontaneously; therefore, expectant management is recommended. When associated with anorectal malformations requiring reconstruction, PG should be excised in conjunction with the anorectoplasty.

Thoracoscopic Surgery for Congenital Lung Malformation Using Miniaturized 3-mm Vessel Sealing and 5-mm Stapling Devices: Single-Center Experience.

Aim: To evaluate the outcomes of thoracoscopic resection of congenital lung malformations (CLM) by using JustRight® (Bolder Surgical) 3-mm vessel sealing system and 5-mm stapler. Methods: Patients who underwent thoracoscopic resection of CLM in our Center from January 2016 to July 2019 were selected and divided into two groups: G1 (2018-2019), treated with the new JustRight instruments, and G2 (2016-2017), treated with Ligasure® and Hem-o-lok. Surgical outcomes in term of length of surgery and complications were compared between groups, stratifying the groups for type of resection (lobectomy, extralobar sequestration [ES] resection, and bronchogenic cyst [BC] resection). Results: Thirteen patients were included in G1, and 16 patients were included in G2. Seven lobectomies, 5 ES resections, and 1 BC resection were performed in G1; whereas 12 lobectomies, 3 ES resections, and 1 BC resection were performed in G2. Mean age at the time of surgery was 7.8 ± 6 months (G1) and 6.8 ± 3.3 months (G2) (P = .57). The average length of surgery was shorter for G1 (lobectomies: 120.7 ± 28.2 versus 171.7 ± 37.5, P = .006; ES resection: 63 ± 21.4 versus 91.7 ± 29.3, P = .15; BC 40 minutes versus 100 minutes). No significant difference was found for length of stay (4 ± 1 days versus 5 ± 2 days, P = .18). Neither the need for conversion nor major complications were observed in either group. Conclusion: In our experience, thoracoscopic resections of CLM with the new JustRight instruments were revealed to be safe and effective. On account of having adequate dimensions for small cavities, these instruments can facilitate the procedure and help to reduce the length of surgery.

Thoracoscopic Resection of Congenital Lung Malformation: Looking for the Right Preoperative Assessment.

INTRODUCTION: Consensus on the best postnatal radiological evaluation of congenital lung malformations (CLMs) is still lacking. In recent years, the interest on magnetic resonance imaging (MRI) has grown, but its role is still unknown. AIM: The aim of the study was to identify the best preoperative diagnostic assessment for CLM. MATERIALS AND METHODS: All patients with a prenatal suspicion of CLM between January 2014 and February 2018 were studied. Asymptomatic newborns underwent MRI, during spontaneous sleep without contrast. Patients with a positive MRI were scheduled for computed tomography (CT) within the fourth month of life. Thoracoscopic resection was performed in cases with a pathological CT. MRI, CT, and surgical findings were compared based on dimension, localization, and features of the CLM using the Cohen's kappa test (K). RESULTS: A total of 20 patients were included (10 males). No difference was found in the diameter and site of the lesions always localized in the same side (K = 1) and in the same pulmonary lobe (K = 1). Infants who underwent thoracoscopic resection included: three congenital pulmonary airway malformations (CPAMs), five extralobar and eight intralobar sequestrations (bronchopulmonary sequestrations [BPSs]), three bronchogenic cysts, and one congenital emphysema. The concordance between MRI and CT and between radiological investigations and pathology was satisfactory for the greatest part of the studied variables. MRI showed sensitivity of 100%, specificity of 82%, positive predictive value of 50% and negative predictive value of 100% for CPAM and 77, 100, 100, and 80% for BPS, respectively. CONCLUSION: MRI proved to be a reliable diagnostic investigation for CLM with high sensitivity and specificity. Early MRI in spontaneous sleep without contrast and preoperative contrast CT scan is a valuable preoperatory assessment.