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Background: Aim of study is to report our preliminary experience with magnetic anastomosis (magnamosis) treating long-gap esophageal atresia (LGEA), the most challenging condition of esophageal atresia continuum. Magnamosis has been reported in 20 patients worldwide as an innovative and marginally invasive option., Methods: Prospective evaluation of all LGEA patients treated with magnamosis was performed (study registration number: 2535/2021). Main outcomes considered were demographic and surgical features, postoperative complications and feeding within 6-month of follow-up., Results: Between June 2020 and January 2021, 5 LGEA patients (Type A, Gross classification) were treated. Median preoperative gap was 5 vertebral bodies. Magnetic bullets were placed at an average age of 81 days of life, leading to successful magnamosis in all cases: 4 infants had primary magnetic repair (one after thoracoscopic mobilization of the pouches), 1 patient had a delayed magnamosis after Foker's procedure. Esophageal anastomosis was achieved after an average of 8 days. No anastomotic leak was found. All patients developed anastomotic stenosis at 6-month follow-up, requiring a mean of 6 dilations each. Full oral feeding was achieved in 3 patients, while 2 were still on oral-gastrostomy feeding. One patient experienced small esophageal perforation after dilation (3 months after magnamosis), distal to the anastomotic stricture and subsequently developed oral aversion., Conclusions: Our preliminary results suggest magnamosis a safe and effective minimally invasive option in patients with LGEA. Absence of postoperative esophageal leaks may represent a major advantage of magnamosis over conventional surgery, although possible high rate of esophageal stenosis should be further evaluated., Levels of Evidence: IV (Case series with no comparison group)., Competing Interests: Declaration of Competing Interest The Authors have no conflicts of interest to disclose None of the Authors have any conflict of interest., (Copyright © 2022. Published by Elsevier Inc.)

Background: Infants affected by Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) may require non-invasive ventilation (NIV) in the post-operative period after elective extubation, especially if born preterm. The aim of the paper is to evaluate the role of different ventilation strategies on anastomotic complications, specifically on anastomotic leak (AL)., Materials and Methods: Retrospective single Institution study, including all consecutive neonates affected by EA with or without TEF in a 5-year period study (from 2014 to 2018). Only infants with a primary anastomosis were included in the study. All infants were mechanically ventilated after surgery and electively extubated after 6-7 days. The duration of invasive ventilation was decided on a case-by-case basis after surgery, based on the pre-operative esophageal gap and intraoperative findings. The need for non-invasive ventilation (NCPAP, NIPPV, and HHHFNC) after extubation and extubation failure with the need for mechanical ventilation in the post-operative period were assessed. The primary outcome evaluated was the rate of anastomotic leak., Results: 102 EA/TEF infants were managed in the study period. Sixty-seven underwent primary anastomosis. Of these, 29 (43.3%) were born preterm. Patients who required ventilation (n = 32) had a significantly lower gestational age as well as birthweight (respectively p = 0.007 and p = 0.041). 4/67 patients had an AL after surgical repair, with no statistical differences among post-operative ventilation strategies., Conclusion: We found no significant differences in the rate of anastomotic leak (AL) according to post-operative ventilation strategies in neonates operated on for EA/TEF., Competing Interests: Declaration of Interests All authors state that they have no conflicts of interest or competing interests., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background In congenital diaphragmatic hernia (CDH) survivors, failure to thrive is a well-known complication, ascribed to several factors. The impact of lung volume on growth of CDH survivors is poorly explored. Our aim was to evaluate if, in CDH survivors, lung volume (LV) after extubation correlates with growth at 12 and 24 months of life. Methods LV (measured as functional residual capacity-FRC) was evaluated by multibreath washout traces with an ultrasonic flowmeter and helium gas dilution technique, shortly after extubation. All CDH survivors are enrolled in a dedicated follow-up program. For the purpose of this study, we analyzed the correlation between FRC obtained shortly after extubation and anthropometric measurements at 12 and 24 months of age. We also compared growth between infants with normal lungs and those with hypoplasic lungs according to FRC values. A p < 0.05 was considered as statistically significant. Results We included in the study 22 CDH survivors who had FRC analyzed after extubation and auxological follow-up at 12 and 24 months of age. We found a significant correlation between FRC and weight Z-score at 12 months, weight Z-score at 24 months and height Z-score at 24 months. We also demonstrated that CDH infants with hypoplasic lungs had a significantly lower weight at 12 months and at 24 months and a significantly lower height at 24 months, when compared to infants with normal lungs. Conclusion We analyzed the predictive value of bedside measured lung volumes in a homogeneous cohort of CDH infants and demonstrated a significant correlation between FRC and growth at 12 and 24 months of age. An earlier identification of patients that will require an aggressive nutritional support (such as those with pulmonary hypoplasia) may help reducing the burden of failure to thrive., (Copyright © 2021. Published by Elsevier Inc.)

Background: Near-infrared spectroscopy (NIRS) is a noninvasive technique for monitoring tissue oxygenation and perfusion. The aim of this study was to evaluate cerebral and splanchnic NIRS changes in CDH operated infants enrolled into the VICI trial and therefore randomized for ventilatory modalities., Materials and Methods: CDH newborns enrolled into the VICI trial (Netherlands Trial Register, NTR 1310) were randomized at birth for high-frequency oscillatory ventilation (HFOV) or conventional mechanical ventilation (CMV) according to the trial. Cerebral oxygenation (rSO2C) and splanchnic oxygenation (rSO2S) were obtained by NIRS (INVOS 5100; Somanetics, Troy, MI) before and after surgery. Variations in rSO2C and rSO2S were evaluated. Mann-Whitney test and one-way ANOVA were used as appropriate. p<0.05 was considered significant., Results: Thirteen VICI trial patients underwent surgical repair between March 2011 and December 2012, and were enrolled in the study. Seven patients were assigned to HFOV and six to CMV group respectively. During surgery, a significant reduction in rSO2C (p=0.0001) and rSO2S (p=0.005) were observed. HFOV patients experienced prolonged reduction in rSO2C value (p=0.003) while rSO2S did not vary between HFOV and CMV (p=0.94)., Conclusions: Surgical CDH repair was associated with decrease of cerebral and splanchnic oxygenation, regardless of ventilation. Patients ventilated by HFOV need a longer time interval to recovery normal rSO2C values, than those ventilated by CMV. This may be owing to a different impact of HFOV on patients' hemodynamic status with a higher impairment on total venous return and its negative consequences on cardiac output., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Neurodevelopmental impairment is one of the most significant morbidities among CDH survivors., Purpose: Assess correlation between ventilatory time (VT) and short-term neurodevelopmental outcome in congenital diaphragmatic hernia (CDH) survivors., Methods: A prospective longitudinal study was conducted between 2008 and 2012. Assessment of mental and motor development was performed at 6 and 12months by Bayley Scales of Infant and Toddler Development - 3rd Edition (BSID-III). ROC curve analysis was used., Results: Forty-two subjects were included in the study. There was a significant inverse correlation between neurodevelopment at 6 and 12months and VT during first admission (p<0.0001). VT predicting the risk of moderate (BSID-III <85) and severe (BSID-III <70) delay was 13 and 28days, respectively (area under the curve - delay <85: 6months mental 0.943 and motor 0.992; 12months mental 0.877 and motor 0.925; delay <70: 6months mental 0.934 and motor 0.943; 12months mental 0.906 and motor 0.975; p<0.0001)., Conclusions: VT should be considered an important marker to identify subjects at risk for short-term neurodevelopmental delay in CDH survivors. Early follow-up intervention therapy should be activated in every baby with a history exceeding 13days of VT., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Introduction: Functional residual capacity (FRC) and lung clearance index (LCI) are sensitive parameters for early detection of airway disease in infancy. The closed helium dilution method has been applied to assess lung volume and ventilation inhomogeneity (VI) in spontaneously breathing infants., Aims: The aims of this study were as follows: (1) to assess applicability of the helium gas dilution technique in mechanically ventilated infants with high-risk congenital diaphragmatic hernia (CDH) and to evaluate changes in breathing patterns, lung volume, and VI during the first days of life before and after surgery, and (2) to analyze the possible correlation between changes in lung volume, cerebral hemodynamics, and oxygenation before and after surgical correction of CDH through near-infrared spectroscopy (NIRS) monitoring., Methods: Lung function tests were performed by multibreath washout traces with an ultrasonic flowmeter and helium gas dilution technique. For all babies, three acceptable FRC and LCI measurements were collected for each test (mean and SD of three measurements were calculated) before surgery (T0), 24 h after surgery (T1) during mechanical ventilation, and within 24 h after extubation in spontaneous breathing (T2). Cerebral and splanchnic hemodynamics were continuously monitored by NIRS during mechanical ventilation to evaluate relationships between changes in lung volume and capillary-venous oxyhemoglobin saturation in tissues. Fraction of inspired oxygen delivered was adjusted to keep oxygen saturation between 90% and 95%., Results: Thirteen CDH infants were studied; median GA = 38 weeks (range 35-41) and median BW = 3000 g (range 1850-3670). FRC and LCI significantly improved after extubation when compared with pre-surgical values. No differences were found in tidal volume (Vt) and NIRS monitoring before and after surgery and after extubation. Neither LCI nor FRC was correlated with NIRS values., Conclusions: Helium gas dilution technique is an applicable and reliable technique to measure lung volumes and ventilation inhomogeneity also in ventilated infants. NIRS is a non-invasive technique to monitor tissue oxygenation during surgery and mechanical ventilation. In CDH newborns these preliminary data show an improvement in both FRC and LCI after extubation., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Purpose: The aim of our study was to elucidate if the defect size reflects the magnitude of the developmental defect in patients with CDH., Methods: All patients recorded in the CDH Study Group registry between January 1, 2007, and December 31, 2010, and with defect classification were included in the study. They were divided according to defect size (A-D) and compared for: gestational age (GA), birth weight (BW), number of abnormal organ systems, prevalence of associated anomalies, cardiovascular malformations (CVM), chromosomal anomalies, liver in the chest, and hernia sac., Results: A total of 1350 of 1778 patients had defect classification: 173 A, 557 B, 438 C, and 182 D. Mortality rate was 0.6%, 5.3%, 22.6%, and 45.6% in group A, B, C, and D, respectively, (p<0.0001; p for trend <0.0001). GA, BW, prevalence of associated anomalies, particularly CVM, number of abnormal organ systems, and prevalence of sac were significantly different between the groups, with a significant reduction of GA, BW, and prevalence of sac. There was an increase in prevalence of associated anomalies, liver in the chest, and number of abnormal systems as the defect size increased., Conclusion: Defect size is directly correlated with mortality rate, prevalence of other anomalies (particularly CVM), and number of abnormal systems, and inversely with GA, BW, and prevalence of hernia sac. The defect size may be a marker for the magnitude of developmental abnormality, thereby explaining its relationship with the outcome., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Aim of the Study: Infants with congenital diaphragmatic hernia (CDH) are at high risk of sensorineural hearing loss (SNHL). Extracorporeal membrane oxygenation is known to increase this risk, but little is known about other potential causes. We evaluated the impact of several risk factors on SNHL development in CDH survivors not treated with extracorporeal membrane oxygenation., Methods: All high-risk CDH survivors consecutively treated between 1999 and 2005 were included. SNHL was diagnosed based on formal assessment with standard audiologic tests. Patients with and without SNHL were compared for patient-related and treatment-related risk factors. Subsequently, a logistic regression analysis was performed to identify independent risk factors associated with SNHL development., Main Results: Out of 87 CDH survivors, 82 had a formal audiologic evaluation and 40 (49%) had SNHL. Patients with SNHL had significantly lower gestational age (P = .045); higher prevalence of sepsis (P < .001); older age at audiologic examination (P < .001); more episodes of hypocapnia (P = .045); higher prevalence of inhaled nitric oxide use (P = .005); longer mechanical ventilation (P = .009); and longer aminoglycosides (P = .006), furosemide (P = .004), and pancuronium bromide (P = .001) treatments. On logistic regression analysis, the only variable independently associated with the development of SNHL was patient's age at audiologic follow-up (P = .012)., Conclusions: Several risk factors were associated with SNHL development at univariate analysis. After logistic regression, only age at evaluation remained independently associated with SNHL. Routine audiologic follow-up is advocated in all CDH patients. Further studies are needed to define if other (genetic) factors may be involved in the pathogenesis of SNHL in patients with CDH.

Background: Short bowel syndrome (SBS) is a severe malabsorption caused by bowel loss. Congenital intestinal anomalies (CIA) detectable by prenatal ultrasound as jejunoileal atresia, meconium peritonitis, complicated meconium ileus, and fetal volvulus can be responsible for SBS., Aims: This study aims to investigate either frequency of SBS or the morbidity in CIA population during the first admission., Material and Methods: Records of CIA treated from 1997 to 2003 were reviewed. The prenatal ultrasound findings were correlated with SBS. Student's t and chi(2) tests were performed to analyze epidemiological data, growth at discharge, sepsis, liver disease, catheter-related complications, motor developmental delay, and hospital stay in CIA with and without SBS., Results: Forty-four CIA: SBS developed in 43%, ranging from 83% in volvulus to 0% in complicated meconium ileus. Thirty-six prenatal diagnoses: a strong correlation with SBS was observed in isolated dilated bowel (58%). In SBS neonates, birth weight, gestational age, and growth at discharge were statistically lower, whereas sepsis, motor delay, and hospital stay were statistically higher., Conclusions: Many neonates with CIA detectable by prenatal ultrasound develop SBS. Short bowel syndrome presents a significant higher morbidity. The counseling should stress the frequent association between CIA and SBS as well as the significant morbidity in SBS.

Background: Despite improvements in clinical management, mortality of congenital diaphragmatic hernia (CDH) remains high. Early prediction of mortality risk helps in comparing strategies and/or performances of different centers. Birth weight (BW), Apgar Score at 5 minutes, and modified McGoon Index (MGI) calculated by the ratio between the diameters of pulmonary arteries and the descending aorta have been used to determine mortality of CDH., Aim: The purpose of this study is to evaluate the relationship between early detectable variables and survival in newborns with CDH intubated at birth, managed with "gentle" ventilation and delayed surgery., Methods: All medical records of patients affected by high-risk CDH and treated with a standardized protocol at Bambino Gesù Children's Hospital, Rome, Italy, between January 2002 and September 2004 were reviewed. Prenatal diagnosis, gestational age, BW, sex, side of hernia, and MGI were recorded on admission. The relationship with mortality of each variable was evaluated by univariate analysis. Subsequently, a predictive model of mortality was developed using a logistic regression: the explanatory variables, BW, and MGI were dichotomized in high (HBW and HMGI) and low (LBW and LMGI) according to the best cutoff found with receiver-operating characteristic curves., Results: Thirty-four newborns with CDH, treated with a standardized protocol, were studied. The main characteristics of the 34 patients were BW, 2886 g (1500-3620 g); gestational age, 37.7 weeks (32-42 weeks); male/female, 22/12; right/left, 8/26; prenatal diagnosis, 29; MGI, 1.31 (0.9-1.85). Only BW and MGI were significantly (P < .05) associated with mortality at the univariate analysis. The best cutoff values were 2755 g for BW (sensitivity, 70%; specificity, 74%) and 1.25 for MGI (sensitivity, 73%; specificity, 78%). Using these limits, BW and MGI resulted independently associated with mortality in the multivariate analysis. Using the 4 possible combinations, the LBW associated with the LMGI presented the highest prediction of mortality (80%)., Conclusions: Birth weight and MGI, variously combined, were predictive of mortality. Because they are not influenced by subsequent modalities of care, they can be considered as valid early severity scores in CDH and used for comparing strategies and/or performances of different centers.

Background: Cardiorespiratory stabilization is recommended before surgical repair of congenital diaphragmatic hernia (CDH) because surgery may induce a transitory deterioration of chest compliance and gas exchange. It is not known if surgical intervention can affect cerebral circulation and oxygenation., Aim: The aim of the study was to assess noninvasively, by near-infrared spectroscopy, the possible changes in cerebral hemodynamics and oxygenation associated with surgical repair of CDH., Subjects: Twenty-five newborns with severe CDH (birth weight, 3057 +/- 354 g; gestational age, 37.8 +/- 1.8 weeks; male/female newborns, 15/10; left/right CDH, 19/6) were sedated, paralyzed, and mechanically ventilated by conventional gentle ventilation and surgically corrected at a median age of 2.7 days (min-max, 2-14 days) after cardiorespiratory stabilization., Methods: Heart rate (HR [beats per minute]), preductal transcutaneous oxygen saturation (tcSaO2 [%]), carbon dioxide tension (tcPCO2 [Torr]), and mean arterial blood pressure (mm Hg) were continuously monitored. Inspired fractional oxygen concentration (FIO2) was adjusted to maintain and preductal tcSaO2 of greater than 80%, whereas the ventilator's settings were kept unchanged throughout the surgical procedure. Cerebral hemodynamics was assessed by near-infrared spectroscopy (NIRO 300, Hamamatsu Photonics, Japan), recording continuously and noninvasively the relative changes in concentration of oxygenated (DeltaO2Hb [micromol/L]), deoxygenated (DeltaHHb [micromol/L]), and total (DeltatHb [micromol/L]) hemoglobin; the tissue oxygenation index (TOI [%]) was also calculated (TOI = O2Hb/O2Hb + HHb). Total hemoglobin concentration is considered to be representative of cerebral blood volume. Arterial blood gases were also measured at the beginning (T1) and at the end of surgery (T2). For all measurements, results at T1 and at T2, as well as the differences between T1 and T2, have been expressed as means or medians and SDs or 95% confidence intervals or ranges. The differences between T1 and T2 were considered statistically significant for a P value of less than .05 by the Student t test for paired values., Results: At T1, mean tcSaO2% was 94.1 % (SD, 4.6) with a FIO2 of 0.25 (SD, 0.1); at T2, to obtain similar values of tcSaO2 (93.4%; SD, 4.4), it was necessary to increase the FIO2 to 0.37 (SD, 0.14; P < .001). Mean HR at T1 was 149.5 beats per minute (SD, 9.1) and increased significantly (P < .05) at T2 (165.2 beats per minute; SD, 14.2). Mean arterial blood pressure was 54.7 mm Hg (SD, 7.7) at T1 and did not change appreciably at T2 (55.6 mm Hg; SD, 8.1). Moreover, tcPCO2 did not change significantly during the procedure (mean tcPCO2 = 49.9 Torr [SD, 12.8] at T1 and 57.3 mm Hg [SD, 17.9] at T2). O2Hb and tHb decreased (P < .001 and <.005) and HHb increased (P < .05) significantly during the surgical procedure (mean Delta [SD]: DeltaO2Hb= -10.9 micromol/L [9.7], DeltatHb = -7.5 micromol/L [11.7], and DeltaHHb = -3.5 micromol/L [6.8]). Mean TOI was 70% at T1 (normal values >60%) and decreased significantly at T2 (mean DeltaTOI = -6.1% [SD, 10.6]). In all infants, the greatest changes occurred when the viscera were positioned into the abdomen., Conclusions: Notwithstanding the initial cardiorespiratory stabilization, surgical repair of CDH was associated with a rise in HR and oxygen requirement and a drop in cerebral tHb and O2Hb, suggesting a reduction in cerebral blood volume and oxygenation. These events were probably due to the combined effects of an increase in right to left shunting (as indicated by the increased oxygen requirement) and a decrease in venous return (possibly due to compression of the inferior vena cava by the viscera positioned into the abdomen). These preliminary results reinforce the importance of achieving a good cardiorespiratory stability before undertaking surgical correction of CDH to minimize the possible interference of the procedure with cerebral circulation and oxygenation.