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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.

Fresh frozen plasma (FFP) is largely misused in the neonatal setting. The aim of the study is to evaluate the impact of a Thromboelastography (TEG)-based Quality Improvement (QI) project on perioperative FFP use and neonatal outcomes. Retrospective pre-post implementation study in a level-III NICU including all neonates undergoing major non-cardiac surgery before (01–12/2017) and after (01–12/2019) the intervention. In 2018, the intervention included the following: (1) Training on TEG, (2) Implementation of TEG, and (3) Algorithm for TEG-directed FFP administration in surgical neonates. We compared pre- vs post-intervention patient characteristics, hemostasis, and clinical management. Linear and logistic regression models were used to evaluate the impact of the project on main outcomes. We analyzed 139 neonates (pre-intervention: 72/post-intervention: 67) with a mean (± SD) gestational age (GA) 34.9 (± 5) weeks and birthweight 2265 (± 980) grams which were exposed to 184 surgical procedures (pre-intervention: 91/post-intervention: 93). Baseline characteristics were similar between periods. In 2019, prothrombin time (PT) was longer (14.3 vs 13.2 s; p p p CI: 0.070, 0.371). Conclusion: The TEG-based QI project for the management of FFP during neonatal surgery reduced intraoperative FFP exposure. What is Known:• PT and aPTT are poor predictors of bleeding risk in acquired neonatal coagulopathy, leading to likely unnecessary fresh frozen plasma (FFP) transfusion in the Neonatal Intensive Care Setting. • As neonatal hemostasis is a delicate balance between the concomitant reduction of pro- and anti-coagulants drivers, thromboelastography (TEG) is a promising alternative for coagulation monitoring. What is New:• The implementation of TEG, training, and shared protocols contributed to reduced intraoperative FFP use, which was not associated with increased mortality or bleeding events.• These findings inform future research showing that there is clinical equipoise to allow for larger studies to confirm the use of TEG in NICUs and to identify TEG cut-offs for transfusion practice.

Background: Trauma is the leading cause of morbidity and mortality in the pediatric population. During the Covid-19 pandemic (C-19), different trends for Pediatric Trauma (PT) were described. This study aims to explore the trend over time of PT in our center, also considering the effects of C-19, focusing on trauma mechanisms, violence-related trauma (VRT) and intentionality, especially suicide attempts (SA).Methods: All PT patients accepted at Niguarda Trauma Center (NTC) in Milan from January 2015 to December 2020 were retrospectively analyzed. We considered demographics and clinical variables and performed descriptive and year comparison analyzes. Results: 684 PT were accepted at NTC: 84 in 2015, 98 in 2016, 125 in 2017, 119 in 2018, 114 in 2019 and 144 in 2020 (pConclusions: NTC is the adult level I referral trauma center for the Milan urban area, with pediatric commitment. During C-19, every traumatic emergency was centralized to NCT. In 2020 we observed an increasing trend of SA and VRT among PTs. The psychological impact of the C-19 restriction could explain this evidence. The long-term effects of C-19 on the mental health of the pediatric population should not be underestimated. Focused interventions on psychological support and prevention of SA and VRT should be implemented, especially during socio-demographic storms like the last pandemic.

In newborns with congenital diaphragmatic hernia (CDH), the mediastinal shift caused by the herniated organs negatively affects lung development. Assessment of the fetal magnetic resonance imaging (MRI) mediastinal shift angle (MSA) was shown to have an inverse correlation with the total fetal lung volume (TFLV), being associated with neonatal survival. However, a possible association with postnatal morbidity has never been investigated. We hypothesize that the degree of the mediastinal shift could be associated with higher respiratory and cardiocirculatory impairment, requiring intensive treatments and extended hospitalization in survivors. We retrospectively consider a cohort of isolated, left-sided CDH, for whom we calculated the MSA and the observed/expected (O/E) TFLV at fetal MRI. We performed a data collection regarding inotropic or vasoactive support, treatment with pulmonary vasodilators, mechanical ventilation, and length of stay. General linear models were performed. The MSA and O/E TFLV were inversely correlated (Pearson’s coefficient − 0.65, p < 0.001), and deceased patients showed higher MSA values then survivors (p = 0.011). Among survivors, an increase in MSA was associated with longer pharmacological treatments (dobutamine: p = 0.016; dopamine: p = 0.049; hydrocortisone: p = 0.003; nitric oxide: p = 0.002; sildenafil: p = 0.039; milrinone: p = 0.039; oxygen: p = 0.066), and mechanical ventilation (p = 0.005), with an increasing trend in the length of hospitalization (p = 0.089). Conclusion: The MSA indirectly reflects lung hypoplasia and is associated with a higher neonatal intensity of cares. However, further studies are needed to consolidate the results. Trial registration: The study is an exploratory post hoc analysis of the registered NeoAPACHE protocol at ClinicalTrials.gov with the identifier NCT04396028.

Neonatal extracorporeal membrane oxygenation (ECMO) is a life-saving procedure for critically ill neonates suffering from a potentially reversible disease, causing severe cardiac and/or respiratory failure and refractory to maximal conventional management. Since the 1970s, technology, management, and clinical applications of neonatal ECMO have changed. Pulmonary diseases still represent the principal neonatal diagnosis, with an overall 74% survival rate, and up to one-third of cases are due to congenital diaphragmatic hernia. The overall survival rate in cardiac ECMO is lower, with congenital heart defect representing the main indication. This review provides an overview of the available evidence in the field of neonatal ECMO. We will address the changing epidemiology, basic principles, technologic advances in circuitry, and monitoring, and deliver a current multidisciplinary management framework, focusing on ECMO applications, complications, and long-term morbidities. Lastly, areas for further research will be highlighted.Conclusions: ECMO is a life support with a potential impact on long-term patients' outcomes. In the next years, advances in knowledge, technology, and expertise may push neonatal ECMO boundaries towards more premature and increasingly complex infants, with the final aim to reduce the burden of ECMO-related complications and improve overall patients' outcomes. What is Known: • ECMO is a life-saving option in newborns with refractory respiratory and/or cardiac failure. • The multidisciplinary ECMO management is challenging and may expose neonates to complications with an impact on long-term outcomes. What is New: • Advances in technology and biomaterials will improve neonatal ECMO management and, eventually, the long-term outcome of these complex patients. • Experimental models of artificial placenta and womb technology are under investigation and may provide clinical translation and future research opportunities.

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.

BACKGROUND: Although complete video-assisted thoracic surgery (C-VATS) has been demonstrated to have several advantages compared with conventional thoracotomy, there are few reports on the clinical feasibility of C-VATS for CPAM in infants. METHODS: We retrospectively evaluated 13 consecutive patients (neonates 4; infants 9) surgically treated for congenital pulmonary airway malformation (CPAM) from 1 January 2008 to 31 March 2017. RESULTS: In the group of neonates, all 4 cases were prenatally diagnosed and they underwent semi-emergent surgery after birth due to respiratory failure. In the group of 9 infants, 5 cases were prenatally diagnosed and 4 cases were diagnosed at age >2.5 years due to symptoms associated with pulmonary cystic infection. Pulmonary resection consisted of the following: 8 lobectomies, 1 segmentectomy, 2 wedge resection, 1 fractionated lung resection and 1 lobectomy with segmentectomy. Overall, there were 9 thoracotomy and 4 thoracoscopic surgeries. Mean operation time was 162 min (range, 67–290 min) and blood loss was 21 mL (range, 0–74 mL) on average. There were no complications such as thoracic deformity or respiratory failure, however in 2 of those who underwent segmentectomy the cystic remnant remained. No statistically significant differences were observed between the thoracotomy Group and C-VATS group in terms of age and height at intervention, operation time, blood loss, postoperative day of drain removal, and length of hospital stay after surgery. However, only the average body weight was heavier in C-VATS group (P=0.03). CONCLUSIONS: Since early surgical resection of asymptomatic CPAM is often recommended for the prevention of infections and the development of lung malignancy, we recommend performing surgery after the age of 1 year if the patient’s condition is stable. Furthermore, C-VATS lobectomy may be feasible if they are older than 18 months or weigh more than 10 kg.

Background: Trauma is the leading cause of morbidity and mortality in the pediatric population. During the COVID-19 pandemic (COVID-19), different trends for pediatric trauma (PT) were described. This study aims to explore the trend over time of PT in our center, also considering the effects of COVID-19, focusing on trauma mechanisms, violence-related trauma (VRT) and intentionality, especially suicide attempts (SAs). Methods: All PT patients accepted at Niguarda Trauma Center (NTC) in Milan from January 2015 to December 2020 were retrospectively analyzed. We considered demographics and clinical variables and performed descriptive and year comparison analyses. Results: There were 684 cases of PT accepted at NTC: 84 in 2015, 98 in 2016, 125 in 2017, 119 in 2018, 114 in 2019 and 144 in 2020 (p < 0.001), 66.2% male, mean age 9.88 (±5.17). We observed a higher number of traffic-related, fall-related injuries and an increasing trend for VRT and SAs, peaking in 2020. We report an increasing trend over time for head trauma (p = 0.002). The Injury Severity Score did not significantly change. During COVID-19 we recorded a higher number of self-presenting patients with low priority codes. Conclusions: NTC is the adult level I referral trauma center for the Milan urban area with pediatric commitment. During COVID-19, every traumatic emergency was centralized to NCT. In 2020, we observed an increasing trend in SAs and VRT among PT patients. The psychological impact of the COVID-19 restriction could explain this evidence. The long-term effects of COVID-19 on the mental health of the pediatric population should not be underestimated. Focused interventions on psychological support and prevention of SAs and VRT should be implemented, especially during socio-demographic storms such as the last pandemic.

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 ( 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.

Esophageal lung is a rare bronchopulmonary foregut malformation characterized by an anomalous origin of one of the main bronchi which arises from the esophagus. Less than 30 cases are reported in the literature. Therefore, there are no standardized guidelines for the treatment of this condition. We report a case of right esophageal lung diagnosed in a neonate. The patient was treated with thoracoscopic closure of the ectopic main bronchus in the neonatal period, followed by delayed pneumonectomy at 5 months of age. No prosthetic substitute was implanted in the ipsilateral hemithorax after pneumonectomy. The patient is now 4 years old and doing well, postpneumonectomy syndrome was never observed. Our strategy and the possible alternatives are discussed here.

Introduction. In newborns with congenital diaphragmatic hernia (CDH), the mediastinal shift caused by the herniated organs negatively affects lung development. Assessment of the fetal magnetic resonance imaging (MRI) mediastinal shift angle (MSA) was shown to have an inverse correlation with the total fetal lung volume (TFLV), being associated with neonatal survival. However, a possible association with postnatal morbidity has never been investigated. We hypothesize that the degree of the mediastinal shift could be associated with higher respiratory and cardiocirculatory impairment, requiring intensive treatments and extended hospitalization in survivors.Method. We retrospectively consider a cohort of isolated, left-sided CDH, for whom we calculated the MSA and the observed/expected (O/E) TFLV at fetal MRI. We performed a data collection regarding inotropic or vasoactive support, treatment with pulmonary vasodilators, mechanical ventilation, and length of stay. General linear models were performed. Results. The MSA and O/E TFLV were inversely correlated (Pearson’s coefficient -0.65, p < 0.001) and deceased patients showed higher MSA values then survivors (p = 0.011). Among survivors, an increase in MSA was associated with longer pharmacological treatments (dobutamine: p = 0.016; dopamine: p = 0.049; hydrocortisone: p = 0.003; nitric oxide: p = 0.002; sildenafil: p = 0.039; milrinone: p = 0.039; oxygen: p = 0.066), and mechanical ventilation (p = 0.005), with an increasing trend in the length of hospitalization (p = 0.089). Conclusions. The MSA indirectly reflects lung hypoplasia and is associated with the higher neonatal intensity of cares. Further studies are needed to consolidate the results. Clinical Trial Registration: The study is an exploratory post-hoc analysis of the registered NeoAPACHE protocol at ClinicalTrials.gov with the identifier NCT04396028

In newborns with congenital diaphragmatic hernia (CDH), the mediastinal shift caused by the herniated organs negatively affects lung development. Assessment of the fetal magnetic resonance imaging (MRI) mediastinal shift angle (MSA) was shown to have an inverse correlation with the total fetal lung volume (TFLV), being associated with neonatal survival. However, a possible association with postnatal morbidity has never been investigated. We hypothesize that the degree of the mediastinal shift could be associated with higher respiratory and cardiocirculatory impairment, requiring intensive treatments and extended hospitalization in survivors. We retrospectively consider a cohort of isolated, left-sided CDH, for whom we calculated the MSA and the observed/expected (O/E) TFLV at fetal MRI. We performed a data collection regarding inotropic or vasoactive support, treatment with pulmonary vasodilators, mechanical ventilation, and length of stay. General linear models were performed. The MSA and O/E TFLV were inversely correlated (Pearson's coefficient - 0.65, p 0.001), and deceased patients showed higher MSA values then survivors (p = 0.011). Among survivors, an increase in MSA was associated with longer pharmacological treatments (dobutamine: p = 0.016; dopamine: p = 0.049; hydrocortisone: p = 0.003; nitric oxide: p = 0.002; sildenafil: p = 0.039; milrinone: p = 0.039; oxygen: p = 0.066), and mechanical ventilation (p = 0.005), with an increasing trend in the length of hospitalization (p = 0.089).Conclusion: The MSA indirectly reflects lung hypoplasia and is associated with a higher neonatal intensity of cares. However, further studies are needed to consolidate the results.Trial registration: The study is an exploratory post hoc analysis of the registered NeoAPACHE protocol at ClinicalTrials.gov with the identifier NCT04396028. What is Known: • In congenital diaphragmatic hernia, the lung size, liver position, and defect side are the most common prenatal prognostic parameters used in clinical practice for morbidity and mortality prediction. • Lung hypoplasia, strictly associated with lung size, is estimated by observed/expected lung to head ratio and observed/expected total fetal lung volume with prenatal ultrasound and fetal magnetic resonance imaging, respectively. What is New: • A new, faster, more straightforward, and less operator-dependent tool to assess CDH severity could be the mediastinal shift angle calculation with fetal magnetic resonance imaging. • Postnatal clinical severity, considered as a postnatal cardiovascular and respiratory impairment that indirectly reflects lung hypoplasia, is associated with an increased mediastinal shift angle calculation.

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.

Introduction Outcome predictions of patients with congenital diaphragmatic hernia (CDH) still have some limitations in the prenatal estimate of postnatal pulmonary hypertension (PH). We propose applying Machine Learning (ML), and Deep Learning (DL) approaches to fetuses and newborns with CDH to develop forecasting models in prenatal epoch, based on the integrated analysis of clinical data, to provide neonatal PH as the first outcome and, possibly: favorable response to fetal endoscopic tracheal occlusion (FETO), need for Extracorporeal Membrane Oxygenation (ECMO), survival to ECMO, and death. Moreover, we plan to produce a (semi)automatic fetus lung segmentation system in Magnetic Resonance Imaging (MRI), which will be useful during project implementation but will also be an important tool itself to standardize lung volume measures for CDH fetuses. Methods and analytics Patients with isolated CDH from singleton pregnancies will be enrolled, whose prenatal checks were performed at the Fetal Surgery Unit of the Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico (Milan, Italy) from the 30th week of gestation. A retrospective data collection of clinical and radiological variables from newborns’ and mothers’ clinical records will be performed for eligible patients born between 01/01/2012 and 31/12/2020. The native sequences from fetal magnetic resonance imaging (MRI) will be collected. Data from different sources will be integrated and analyzed using ML and DL, and forecasting algorithms will be developed for each outcome. Methods of data augmentation and dimensionality reduction (feature selection and extraction) will be employed to increase sample size and avoid overfitting. A software system for automatic fetal lung volume segmentation in MRI based on the DL 3D U-NET approach will also be developed. Ethics and dissemination This retrospective study received approval from the local ethics committee (Milan Area 2, Italy). The development of predictive models in CDH outcomes will provide a key contribution in disease prediction, early targeted interventions, and personalized management, with an overall improvement in care quality, resource allocation, healthcare, and family savings. Our findings will be validated in a future prospective multicenter cohort study. Registration The study was registered at ClinicalTrials.gov with the identifier NCT04609163.

Introduction: Thoracoscopy is generally considered the approach of choice for the treatment of uncomplicated congenital pulmonary airway malformation (CPAM) in infants and children.1–3 The...

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.

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 ...

Foreign body and caustic ingestions in children are usually the most common clinical challenges for emergency physicians, general pediatricians and pediatric gastroenterologists. Management of these conditions often requires different levels of expertise and competence. Endoscopy is often necessary but there is a high risk of misusing this tool with incorrect timing and indications. The imprecise clinical history frequently leaves clinicians uncertain about timing and nature of the ingestion. Few clinical guidelines regarding management of these ingestions in children have been published, none of which from the Italian Society of Pediatric Gastroenterology, Hepatology and Nutrition (SIGENP). An expert panel of Italian endoscopists was convened by the SIGENP Endoscopy Working Group to produce the present article that outlines practical clinical approaches to the pediatric patient with a variety of foreign body and caustic ingestions. The Italian Association of Hospital Gastroenterologists and Endoscopists (AIGO) has also endorsed the project since many adult endoscopists usually manage children with these conditions. Differently from the other published guidelines, the proposed one focuses on the role of the endoscopists (regardless of whether they are adult or pediatric gastroenterologists) in the diagnostic process of children with foreign body and caustic ingestions.

Aim: To evaluate tracheal diameters and their clinical impact in patients with congenital diaphragmatic hernia (CDH) after fetal endoscopic tracheal occlusion (FETO). Methods: Patients born with CDH between January 2012 and August 2016 were divided into two groups: noFETO and FETO. Tracheal diameters at three levels (T1, carina, and maximum tracheal dilation) on chest X-ray at 1, 3, 6, 12, 24, and 36 months of follow-up, requirements of invasive and noninvasive respiratory support, the incidence of respiratory infections, and results of pulmonary function tests (PFT) were compared. Results: A total of 71 patients with CDH were born in the study period, and there were 34/41 survivors in the no­FETO group (82.9%) and 13/30 in the FETO group (43.3%). The maximum tracheal diameter was significantly greater in the FETO group at all ages. No differences were observed in the diameters at T1 and the carina, in the requirements of invasive and noninvasive respiratory support, and in the incidence respiratory infections. At the PFT (6–12 months), the FETO group presented higher respiratory rates (46.1 ± 6.2 vs. 36.5 ± 10.6, p = 0.02). No differences in PFT results were found between the groups after the 1st year of life. Conclusions: The FETO procedure leads to persistent tracheomegaly. However, the tracheomegaly does not seem to have a significant clinical impact.

Introduction We present a single-center experience with very low birth weight (VLBW) infants with focal intestinal perforation (FIP), comparing the results of primary anastomosis (PA) and stoma opening (SO). Materials and Methods Clinical records of VLBW infants with FIP who underwent surgery between 2006 and 2015 were reviewed. Patients were divided into two groups according to the procedure performed: limited bowel resection and PA or SO. Patients with gastric perforation or patients who underwent clip and drop were excluded. Information regarding birth weight (BW), gestational age (GA), weight at surgery (WS), number of abdominal reoperations, duration of parenteral nutrition (PN), and demise was recorded. Results In this study, 40 patients were included: 22 in PA group and 18 in SO group. BW was 865 g in PA and 778 in SO (p-value: 0.2). GA was 26.1 weeks in PA and 25.6 in SO (p-value: 0.3). WS was 1,014 g in PA and 842 in SO (p-value: 0.09). Duration of surgery was 115 minutes in PA and 122 in SO (p-value: 0.67). Five patients (23%) belonging to PA group developed complications and required SO. Five patients (23%) demised in PA group and six (33%) in SO (p-value: 0.2). Seventeen abdominal reoperations were performed in PA group and 22 in SO group (p-value: 0.08). Conclusion Both procedures appear to be safe. When possible, PA should be performed as it reduces the number of abdominal reinterventions.

Introduction The aim of this study was to stratify anastomotic strictures (AS) following esophageal atresia (EA) repair and to establish predictors for the need of dilations. Materials and Methods A retrospective study on children operated on for EA between 2004 and 2014 was conducted. The stricture index (SI) was measured both radiologically (SIXR) and endoscopically (SIEND). A correlation analysis between the SI and the number of dilations was performed using Spearman's test and linear regression analysis. Results In this study, 40 patients were included: 35 (87.5%) presented with Gross's type C EA, 3 (7.5%) type A, 1 (2.5%) type B, and 1 (2.5%) type D. The mean follow-up time was 101 ± 71.1 months (range: 7.8–232.5, median: 97.6). The mean SIXR was 0.56 ± 0.16 (range: 0.15–0.86). The mean SIEND was 0.45 ± 0.22 (range: 0.15–0.85). Twenty-four patients (60%) underwent a mean of 2 endoscopic dilations (range: 1–9). The number of dilations was poorly correlated with SIXR, while significantly correlated with SIEND. Patients who did not need dilations had a SIEND Conclusion SIEND is a useful tool to classify AS and can represent a predictor of the need for endoscopic dilation. The role of the SIEND becomes even more important as clinical characteristics have a low predictive value for the development of an AS and the need for subsequent endoscopic esophageal dilatations.

To investigate the prevalence and the characteristics of gastroesophageal reflux disease (GERD) in infants with apparent life threatening events (ALTE). Materials and methods: Infants with at least one episode of ALTE in absence of predisposing factors were included. All infants underwent a cardiorespiratory recording with simultaneous 24-hour pH-monitoring. Patients were divided into 3 groups according to the severity of GERD: A. Reflux Index (RI) 7%. Monthly evaluations were performed and the anti-reflux therapy was maintained till normalization of monitoring and clinic. Results: 41 infants were enrolled. GERD was found in 80% of patients (moderate in 54%, severe in 27%). A normalization of the cardiorespiratory tracks was recorded on average after 1 month for group A, 7 months for the group B and 9.5 months for group C. A significant difference was registered between group A and both group B and C (P < 0.0001), as well as between the group B and C (P < 0.05). Conclusion: GERD influences significantly the time of normalization of the cardiorespiratory monitoring in infants with ALTE. GERD diagnosis and treatment are mandatory in these patients.

An extralobar pulmonary sequestration (EPS) associated with a contralateral aorto-to-pulmonary vein fistula is rare. We report the case of a female newborn with left EPS fed by an artery originating from the distal thoracic aorta and, symmetrically on the controlateral side, an artery shunting in the inferior right pulmonary vein. Echocardiography showed dilatation of the left atrium. On the 34th day since birth (weight 4500 g), the patient was operated on thoracoscopically. The EPS was closed with a 3-mm sealing system, divided and removed. A window in the mediastinal pleura was created, and the origin of the fistula was identified and sealed. The postoperative course was uneventful. The patient was discharged on Day 4 with no echocardiographic signs of persistence of the fistula and of the congestive heart failure. This is the first case report of a thoracic large systemic circulation-to-pulmonary vein fistula causing heart failure associated with EPS. The thoracoscopic monolateral approach and the availability of 3-mm instruments guaranteed a maximum level of minimal invasiveness.

Cornelia de Lange spectrum (CdLSp) is a rare genetic condition characterized by intellectual disability, facial dysmorphisms, major malformations, growth impairment, and development delay. Approximately 80% of CdLSp patients have gastroesophageal reflux disease (GERD) with a varied clinical presentation. The aim of this study is to define potential clinical/genetic risk factors based on the clinical phenotype description of CdLSp patients with severe GERD who underwent surgical treatment. We retrospectively collected data from 23 CdLSp patients, 13 females and 10 males. Mean age of the patients undergoing surgical treatment was of 4 years. 21/23 (91%) had a molecular characterization, of which 21/21 (100%) had a NIPBL gene mutation, while 2/23 (9%) did not have a genetical characterization, only a clinical diagnosis. Most of our patients presented a moderate-severe severity score (21/23, 91%) with limb malformations evidenced in 10/23 (44%) of our patients and a moderate-severe intellectual disability in 20/23 (87%). Therefore, CdLSp patients harboring NIPBL variants, upper limb malformations and severe psychomotor delay are more likely to suffer from severe GERD, not responsive to proton pump inhibitors treatment. These features should be considered as clinical markers for potentially severe GERD that might require surgical treatment.

OBJECTIVERecent trials have shown the safety and benefits of fetoscopic treatment of myelomeningocele (MMC). The authors’ aim was to report their preliminary results of prenatal fetoscopic treatment of MMC using a biocellulose patch, focusing on neurological outcomes, fetal and maternal complications, neonatal CSF leakage, postnatal hydrocephalus, and radiological outcomes.METHODSPreoperative assessment included clinical examination, ultrasound imaging, and MRI of the fetus. Patients underwent purely fetoscopic in utero MMC repair, followed by postoperative in utero and postnatal MRI. All participants received multidisciplinary follow-up.RESULTSFive pregnant women carrying fetuses affected by MMC signed informed consent for the fetoscopic treatment of the defect. The mean MMC size was 30.4 mm (range 19–49 mm). Defect locations were L1 (2 cases), L5 (2 cases), and L4 (1 case). Hindbrain herniation and ventriculomegaly were documented in all cases. The mean gestational age at surgery was 28.2 weeks (range 27.8–28.8 weeks). Fetoscopic repair was performed in all cases. The mean gestational age at delivery was 33.9 weeks (range 29.3–37.4 weeks). After surgery, reversal of hindbrain herniation was documented in all cases. Three newborns developed signs of hydrocephalus requiring CSF diversion. Neurological outcomes in terms of motor level were favorable in all cases, but a premature newborn died due to CSF infection and sepsis.CONCLUSIONSThe authors’ preliminary results suggest that fetoscopic treatment of MMC is feasible, reproducible, and safe for mothers and their babies. Neurological outcomes were favorable and similar to those in the available literature. As known, prematurity was the greatest complication.

Introduction Gastroesophageal reflux disease (GERD) is a frequent comorbidity after congenital diaphragmatic hernia (CDH) repair. Our aim was to evaluate the clinical and endoscopic prevalence of esophagitis in a long-term follow-up of CDH patients. Materials and Methods Patients operated on for posterolateral CDH and undergoing general anesthesia for concomitant pathologies between January and October 2013 were included in the study. GERD was investigated both clinically (Manterola questionnaire) and endoscopically. The severity of esophagitis was evaluated according to the Hetzel–Dent classification and multiple biopsies were performed. The correlation between clinical score and severity of esophagitis was evaluated. Results Twelve patients were included in the study (mean age: 14.5 years; range, 9–18 years). Only three children (25%) had a pathological questionnaire. At endoscopy, three children (25%) were affected by grade 1 esophagitis, six (50%) by grade 2, two (17%) by grade 3, and one (8%) by grade 4. One of the children presented Barrett esophagus. A moderate negative correlation was found between clinical data and endoscopic findings (r: −0.54 and p : 0.067). Conclusion Even in the absence of symptoms, esophagitis revealed to have a high prevalence in CDH patients. A long-term clinical and instrumental follow-up is mandatory to early diagnose and treat GERD.