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Objectives: To quantify mediastinal shift in isolated congenital diaphragmatic hernia (CDH), by the introduction of a new ultrasonographic (US) marker, defined as mediastinal shift angle (MSA) and to evaluate its ability in predicting postnatal survival at discharge.Methods: Twenty-four consecutive fetuses from singleton pregnancies with isolated left-sided CDH were included in the study group and then subdivided into group A (16 survivors) and group B (8 nonsurvivors). The study group was matched with a control group of 95 fetuses from singleton pregnancies free from structural and/or chromosomal anomalies. On the same US stored images commonly used for lung-to-head ratio (LHR) measurement, a landmark line was drawn from a point on the posterior face of the vertebral body, splitting it into two equal parts, to the mid-posterior surface of the sternum. Another landmark line was then traced from the same point of the vertebral body to touch tangentially the lateral wall of the right atrium. The angle between these two lines was used to quantify mediastinal shift and called "mediastinal shift angle" (MSA).Results: Median MSA was significantly different between group A (34.3° range 29.3-45.9°) and group B (42.7° range 34.1-58.9°) (p < .001) and between study group as a whole and the control group (19° range 13.8-25.9°) (p < .001). Statistical analysis confirmed an inverse correlation between MSA values and survival (p = .004). The best cutoff value for MSA was 43.7°, which demonstrated the highest discriminatory power (sensitivity 63%; specificity 93.75%).Conclusions: In fetuses with isolated CDH, the mediastinal shift may be quantified using mediastinal shift angle (MSA) and this US marker, similarly to the widely accepted and used US prenatal prognostic indicators (LHR and O/E LHR), seems to reliably predict survival. [ABSTRACT FROM AUTHOR]

Purpose: Daily postoperative anal dilations after endorectal pull-through for Hirschsprung disease (HD) are still considered a common practice. We analyzed the potential risks of this procedure and its effectiveness compared to a new internal protocol., Methods: All infants (< 6 months of age) who underwent transanal endorectal pull-through between January 2021 and January 2023 were prospectively enrolled in a new postoperative protocol group without daily anal dilations (Group A) and compared (1:2 fashion) to those previously treated by postoperative anal dilations (Group B). Patients were matched for age and affected colonic tract. Patients with associated syndromes, extended total intestinal aganglionosis, and presence of enterostomy were excluded. Outcomes considered were: anastomotic complications (stenosis, disruption/leakage), incidence of enterocolitis, and constipation., Results: Eleven patients were included in group A and compared to 22 matched patients (group B). There were no significant differences in the occurrence of anastomotic complications between the two groups. We found a lower incidence of enterocolitis and constipation among group A (p = 0.03 and p = 0.02, respectively)., Conclusion: A non-dilation strategy after endorectal pull-through could be a feasible alternative and does not significantly increase the risk of postoperative anastomotic complications. Moreover, some preliminary advantages such as lower enterocolitis rate and constipation should be further investigated., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

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.)

In patients affected by CDH, survival beyond the neonatal period continues to increase thanks to technological and pharmacological improvements. Conversely, patients, families and caregivers are more and more frequently facing "new" complex late comorbidities, including chronic pulmonary and cardiac dysfunctions, neurodevelopmental challenges, and specific nutritional requirements, that often require ongoing long-term medical or surgical care. Therefore, late morbidity is now a key focus in clinical care of CDH. The aims of this paper are to stress some of the most important "unsolved problems" for CDH patients at long-term follow-up., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Valfré, Conforti, Morini, Patel, Bevilacqua, Cianci, Bagolan and Braguglia.)

Background: A high prevalence (9.5-30%) of eosinophilic esophagitis (EoE) in patients with esophageal atresia (EA) has been reported. The application of the EoE criteria to EA patients might be problematic. To date, only studies using a "routine" biopsy approach, even in asymptomatic patients, have been performed. The aim of the study was to establish the prevalence of EoE among symptomatic EA patients (EA/EoE group) without anastomotic stricture (AS) and to compare their characteristics with those of EoE patients from general population (EoE group)., Methods: From 2005 to 2018, we reviewed charts of children with EA and EoE. "Selective" biopsy approach only in EA children without AS and/or endoscopic feature of EoE was performed. Characteristics of EA/EoE and EoE groups were compared., Results: Among 370 EA and 118 EoE, 15 EA/EoE patients were detected (4.0% of EA patients). Male predominance and a high prevalence of allergy without differences between EA/EoE and EoE groups was observed. EA/EoE children were significantly younger (p < 0.0001). PPI-responder patients were significantly more prevalent in EA/EoE group (p = 0.045)., Conclusion: Our data confirm that EA patients are at high risk for developing EoE. High incidence, early onset, and high prevalence of PPI-responders might suggest that esophageal motility disorders interact to increase propensity to EoE in EA patients. However, our study also suggests that overdiagnosis of EoE may occur in EA and that adapted criteria for EoE diagnosis should be developed for EA patients., Trial Registration: Not applicable for this retrospective study., (© 2020 The Authors.)

Background: Children with congenital esophageal atresia (EA) and tracheoesophageal fistula (TEF) have chronic respiratory symptoms including recurrent pneumonia, wheezing and persistent cough. The aim of this study is to describe the clinical findings of a large group of children with EA and TEF surgically corrected and the instrumental investigation to which they have undergone in order to better understand the patient's needs and harmonize the care., Methods: A retrospective data collection was performed on 105 children with EA and TEF followed at Department of Pediatric Medicine of Bambino Gesù Children's Hospital (Rome, Italy) between 2010 and 2015., Results: 69/105 (66%) children reported lower respiratory symptoms with a mean age onset of 2.2 ± 2.5 years and only 63/69 (91%) performed specialist assessment at Respiratory Unit. Recurrent pneumonia (33%) and wheezing (31%) were the most reported symptoms. The first respiratory evaluation was performed after surgically correction of gastroesophageal reflux (GER) at mean age of 3.9 ± 4.2 years. Twenty nine patients have undergone to chest CT with contrast enhancement detecting localized atelectasis (41%), residual tracheal diverticulum (34%), bronchiectasis (31%), tracheal vascular compression (21%), tracheomalacia (17%) and esophageal diverticulum (14%). Fifty three patients have undergone to airways endoscopy detecting tracheomalacia (66%), residual tracheal diverticulum (26%), recurrent tracheoesophageal fistula (19%) and vocal cord paralysis (11%)., Conclusions: Our study confirms that respiratory symptoms often complicate EA and TEF; their persistence despite medical and surgical treatment of GER means that other etiological hypothesis must be examined and that a complete respiratory diagnostic work up must be considered.

Feeding and swallowing disorders are commonly seen in clinical practice in infants and children treated for esophageal atresia with or without tracheoesophageal fistula. Nevertheless, only few authors have addressed these issues. This review aims to describe the feeding and swallowing disorders encountered, focusing on pathophysiology, normal development of swallowing and feeding abilities, and possible rehabilitation therapies to prevent or correct these disorders., (Georg Thieme Verlag KG Stuttgart · New York.)

Objective: Near infrared spectroscopy (NIRS) gradually became the gold standard to guide anesthetic conduction during cardiac surgery, and nowadays, it is commonly utilized to monitor cerebral oxygenation during invasive procedures. Preterm babies also benefit from this non-invasive monitoring to prevent neurological sequelae. However, few data are available on NIRS perioperative changes in newborn operated on for major non-cardiac malformations. Aim of the present study is to evaluate the usefulness of NIRS assessment during and after esophageal atresia (EA) correction and its correlation with clinical behavior., Patients and Methods: All patients treated for EA from May 2011 were prospectively enrolled in the study. All infants underwent "open" correction of EA and cerebral and splanchnic NIRS was applied up to 48h after surgery. Body temperature, blood pressure, pH, paSO2, paCO2, and urine output, were recorded during NIRS registration. Mann-Whitney test and 1-way ANOVA (Kruskal-Wallis and Dunn's multiple comparison tests) were used as appropriate., Results: Seventeen patients were enrolled into the study and 13 were available for the analysis. Four patients were excluded because of poor NIRS registration. Cerebral and renal NIRS values significantly decreased at 24h post-operatively (p<0.05). Interestingly, all parameters studied as possible confounders in NIRS remained stable during the study period. Urine output significantly decreased., Conclusion: Our data confirmed that perioperative monitoring of tissue oxygenation during neonatal esophageal surgery is feasible. Cerebral and renal NIRS evaluation, as for cardiac patients, may guide anesthetic conduction and postoperative care. Out data suggest a newly observed hemodynamic reorganization during esophageal surgery involving renal and, probably, splanchnic blood flow redistribution, demonstrated by the observed subsequent significant post-operative transitory decrease in urinary output. Reducing the decrement in cerebral and renal NIRS values may improve, and ideally eliminate, the well-known late sequelae linked to hemodynamic changes during surgery. More studies are needed to better understand the causes of the NIRS described hemodynamic changes and, therefore, correct them., (Copyright © 2014 Elsevier Inc. All rights reserved.)