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• Magnamosis represents a minimally invasive treatment for LGEA with limited knowledge available. • A prospective evaluation of 5 LGEA patients consecutively treated with magnetic anastomosis is here reported. • Also highlighting 6-months postoperative outcomes. 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. 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. 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. 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. IV (Case series with no comparison group) [ABSTRACT FROM AUTHOR]

• What is currently known about this topic? Concerns have been addressed about a higher risk of anastomotic leak and mediastinitis in neonates operated on for EA/TEF who required non-invasive ventilation after extubation in previous studies, with contrasting results. • What new information is contained in this article? Post-operative non-invasive respiratory support could be safely used in EA/TEF infants without a significant increase in risk of anastomotic leak. This should be considered especially in preterm infants in which a prolonged invasive ventilation contribute to the development of bronchopulmonary dysplasia and therefore it should be carefully shifted to non-invasive ventilation as soon as possible, using low pressures and, if necessary, a mild sedation. 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). 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. 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. 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. [ABSTRACT FROM AUTHOR]

Aim Esophageal atresia (EA) and tracheoesophageal fistula (TEF) consist of a spectrum of rare congenital abnormalities. Although EA surgical treatment is well established, the outcome of EA with proximal fistula (type B and D EA) or isolated H-type fistula (type E EA) is poorly explored. These forms of EA shared a common surgical step: the need of a cervical approach to close the fistula. Therefore, the aim of present study is to evaluate postoperative outcomes of patients treated for Gross type B–D and E EA, on regards of their cervical surgery. Materials and methods A retrospective case series analysis of all patients affected by type B–D and E EA, and admitted to our tertiary care center between January 2003 and December 2014 was performed. All patients underwent preoperative flexible laryngo-tracheobronchoscopy (LTBS) as part of our standardized preoperative diagnostic assessment to define the diagnosis, evaluate preoperative vocal cord motility and to cannulate the fistula when required. Fistula closure was always performed through a right cervical access. Analysis of all cases and comparison between type B–D and E EA were performed. Mann–Whitney test, Chi-squared test and unpaired t test were used as appropriate; p < 0.05 was considered significant. Results During the study period, 180 EA newborns were treated. Proximal or isolated TEF was found in 18 patients (10%): 7 type B, 11 type E EA. Patients affected by type B and E EA/TEF frequently present associated major malformations (27%), and major cardiac abnormalities (44%). Major postoperative complications were: vocal cord paralysis (5 patients), bilateral in 2 infants requiring tracheostomy, cerebral ischemia (1 patient), and cardiac failure (1 patient). Conclusion Patients affected by type B and E EA have a high rate of associated abnormalities, and risk of possible sequelae. Postoperative complications are common, with possible transient vocal cord dismotility, but in some cases persistent paralysis may require tracheostomy. Therefore, both preoperative and postoperative LTBS is highly recommended to evaluate the presence of a proximal fistula, and vocal cord motility, even in asymptomatic patients, to rule out any possible intraoperative “surprise” and any vocal cord abnormality and to possibly define its pathogenesis (congenital vs. iatrogenic). [ABSTRACT FROM AUTHOR]

Background Newborn babies with esophageal atresia/tracheoesophageal fistula (EA/TEF) are prone to respiratory tract disorders. Functional residual capacity (FRC) and lung clearance index (LCI) are commonly considered useful and sensitive tools to investigate lung function and early detecting airways diseases. The aim of the present study is to report the first series of EA/TEF infants prospectively evaluated for FRC and LCI. Methods Prospective observational cohort study of all patients treated for EA/TEF. Lung volume and ventilation inhomogeneity were measured by helium gas dilution technique using an ultrasonic flow meter. Babies were studied both in assisted controlled ventilation (sedated) and in spontaneous breathing (quiet sleep). Three consecutive FRC and LCI measurements were collected for each test at three different time points: before surgery (T0), 24 hours after surgery (T1) and after extubation (T2). Results 16 EA newborns were eligible for the study between December 2011 and July 2013. Three were excluded because of technical problems. At T0 FRC values were in the normal range regardless the presence of TEF but worsened afterwards at T1, with a subsequent recovering after extubation; a significant improvement after surgery was observed concerning LCI while no differences were found in tidal volume. Conclusion Helium gas dilution technique is a suitable method to measure the effect of surgery on lung physiology, even in ventilated infants with EA. The changes observed could be related to the ventilatory management and lung compression during surgical procedure. [ABSTRACT FROM AUTHOR]

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 (rSO 2 C) and splanchnic oxygenation (rSO 2 S) were obtained by NIRS (INVOS 5100; Somanetics, Troy, MI) before and after surgery. Variations in rSO 2 C and rSO 2 S 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 rSO 2 C (p = 0.0001) and rSO 2 S (p = 0.005) were observed. HFOV patients experienced prolonged reduction in rSO 2 C value (p = 0.003) while rSO 2 S 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 rSO 2 C 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. [ABSTRACT FROM AUTHOR]

Introduction Refractory chylothorax is a severe clinical issue, particularly in neonates. Conventional primary approach is based on diet with medium-chain fatty acids and/or total parenteral nutrition. In nonresponders, proposed second line treatments include chemical or surgical pleurodesis, thoracic duct ligation, pleuroperitoneal shunting and pleurectomy but none of these have been shown to be superior to other in terms of resolution rate and safety. Our aim is to report our experience on povidone–iodine use for chemical pleurodesis in newborn infants with chylothorax unresponsive to conservative treatment. Our aim is to report our experience on povidone–iodine use for chemical pleurodesis in newborn infants with chylothorax unresponsive to conservative treatment. Methods Since 2013, povidone–iodine pleurodesis was attempted in all patients with persistent chylothorax who failed conservative treatment (no response to at least 10 days of total parenteral nutrition and maximum dosage of intravenous octreotide). Pleurodesis consisted in the injection of 2 ml/kg of a 4% povidone–iodine solution inside the pleural space, leaving the pleural tube clamped for the subsequent 4 hours. Results Five patients were treated with chemical pleurodesis of persistent chylothorax. Four of 5 patients had their pleural effusion treated by one single povidone–iodine infusion. Median time for resolution was 4 days. A patient with massive superior vena cava thrombosis did not benefit from pleurodesis. None of the patients experienced long term side effects of the treatment. Conclusion Our data suggest that povidone–iodine pleurodesis may be considered a safe and effective option to treat refractory chylothorax in newborns. [ABSTRACT FROM AUTHOR]

Introduction Postpneumonectomy syndrome (PPS) is an ominous complication, caused by mediastinal shift following massive lung resection. Presentation of the case A neonate with oesophageal atresia and tracheo-oesophageal fistula developed acute respiratory distress shortly after surgery, despite mechanical ventilation. The patient was found to have an associated oesophageal right lung that collapsed after oesophageal atresia repair and a left pulmonary artery sling causing left main bronchus stenosis mimicking a postpneumonectomy syndrome. Discussion We will describe the diagnostic work-up and the therapeutic measures used both in the acute phase and as definitive treatment in this challenging case. Conclusions Neonatologists and paediatric surgeons should be aware of this rare association that may cause acute life threatening and worsening of patient’s clinical status. Prompt realignment of the mediastinum in the normal position is critical to obtain rapid improvement of the patient’s clinical conditions. [ABSTRACT FROM AUTHOR]

Although most patients with esophageal atresia (EA) and tracheo-esophageal fistula (TEF) may benefit from “standard” management, which is deferred emergency surgery, some may present unexpected elements that change this paradigm. Birth weight, associated anomalies, and long gap can influence the therapeutic schedule of the patients with EA/TEF and can make their treatment tricky. As a consequence, detailed information on these aspects gives the power to develop a decision-making process as correct as possible. In this article, we will review the most important factors influencing the treatment of patients with EA/TEF and will share our experience on the diagnostic and therapeutic tips that may provide pivotal help in the management of such patients. [ABSTRACT FROM AUTHOR]

Abstract: 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 &y& Elsevier]

Abstract: Yerba Mate (Ilex paraguariensis) tea consumption is higher in Argentina and other South American countries than those of coffee or tea (Camellia sinensis). The effects of Yerba Mate on bone health have not previously been explored. From a program for osteoporosis prevention and treatment, postmenopausal women who drank at least 1L of Yerba Mate tea daily during 4 or more years (n=146) were identified, and matched by age and time since menopause with an equal number of women who did not drink Yerba Mate tea. Their bone mineral density (BMD) was measured by dual-energy X-ray absorptiometry (DXA) at the lumbar spine and femoral neck. Yerba Mate drinkers had a 9.7% higher lumbar spine BMD (0.952g/cm2 versus 0.858g/cm2: p <0.0001) and a 6.2% higher femoral neck BMD (0.817g/cm2 versus 0.776g/cm2; p =0.0002). In multiple regression analysis, Yerba Mate drinking was the only factor, other than body mass index, which showed a positive correlation with BMD at both the lumbar spine (p <0.0001) and the femoral neck (p =0.0028). Results suggest a protective effect of chronic Yerba Mate consumption on bone. [Copyright &y& Elsevier]

Objectives: To describe the prevalence and pathogenesis of symptomatic vocal cord paresis/paralysis (VCP) in patients treated for esophageal atresia (EA), tracheo-esophageal fistula (TEF) or both. Study design: Retrospective study of all patients treated for EA/TEF in our center (1995 to 2009). Patients with and without symptomatic VCP were compared for gestational age, birth weight, associated anomalies, referrals, long-gap EA (>3cm or 3 vertebral bodies), cervical esophagostomy, anastomotic leakage, length of ventilation, and major cardiac surgery. Prevalence or median (IQR) is reported. Results: Of 174 patients, 7 (4%) had symptomatic VCP. Prevalence of referrals (5/7 versus 21/167; P = .0009), long gap (5/7 versus 41/167; P = .0146), previous cervical esophagostomy (5/7 versus 7/167; P < .0001), and anastomotic leakage (3/7 versus 10/167; P = .0097) was higher, and ventilation longer (8.5 days [7.0 to 15.5] versus 6.0 days (5.0 to 7.0); P = .0072) in patients with VCP. Conclusions: In infants treated for EA/TEF, VCP should be ruled out in case of persistent respiratory morbidity or, when present, cautiously monitored. Surgical risk factors should be actively controlled. Further studies are needed to define the prevalence of acquired and congenital VCP in patients with EA/TEF. [ABSTRACT FROM AUTHOR]

Abstract: Congenital chylous ascites is a rare entity, and surgical treatment is confined to selected intractable cases. We report 2 cases of refractory congenital chylous ascites successfully treated with preoperative administration of lipophilic dye (Sudan III) followed by abdominal systematic surgical exploration, cauterization, and fibrin glue application. [Copyright &y& Elsevier]

Abstract: Background/Purpose: The increased survival rate reached in infants with congenital diaphragmatic hernia (CDH) has shown a concomitant increase in late morbidity. A recent report from CDH Study Group showed that dimension of diaphragmatic defect is the only independent risk factor of mortality. However, the influence of defect size on late morbidity is still controversial. The aim of the study was to evaluate the influence of patch repair (proxy of diaphragmatic defects size) on midterm morbidity. Methods: All high-risk (prenatal diagnosis and/or respiratory symptoms within 6 hours of life) CDH survivors treated at our institution from 2004 to 2008 were followed up in a multidisciplinary outpatient clinic as part of a longitudinal prospective study. Auxological, gastroesophageal, pulmonary, and orthopedic evaluations were performed at 6, 12, and 24 months of age. Patient outcomes were compared with respect to +/− patch repair. Results: Of 70 survivors, 61 (87%) were enrolled and prospectively evaluated in follow-up. Poorer auxological outcome, increased rate of gastroesophageal reflux, and altered pulmonary function test were observed during follow-up. Conclusions: Patch repair correlates with higher pulmonary, auxological, and gastroesophageal morbidity without increasing chest wall deformities at long-term follow-up. [ABSTRACT FROM AUTHOR]

Abstract: Congenital diaphragmatic hernia (CDH) retains high mortality due to lung hypoplasia and pulmonary hypertension. Efforts to improve survival and outcome have included fetal intervention, delivery at specialist centres, elective operation after stabilisation of labile physiology and minimising barotrauma. Permissive hypercapnea (‘gentle ventilation’) represents a significant advance in therapy gaining wider acceptance in centres worldwide. Human genetic studies are underway to identify candidate genes for the birth defect. Progress in the basic sciences may uncover critical aspects of developmental biology fundamental to CDH. Clinical trends in perinatal management of CDH are highlighted, which underpin the challenges of this lethal human anomaly. [Copyright &y& Elsevier]

Objective: To analyze the findings of both multichannel intraluminal impedance with pH (MII-pH) and endoscopy/histopathology in children with esophageal atresia at age 1 year, according to current recommendations for the evaluation of gastroesophageal reflux disease (GERD) in esophageal atresia.Study Design: We retrospectively reviewed both MII-pH and endoscopy/histopathology performed in 1-year-old children with esophageal atresia who were followed up in accordance with international recommendations. Demographic data and clinical characteristics were also reviewed to investigate factors associated with abnormal GERD investigations.Results: In our study cohort of 48 children with esophageal atresia, microscopic esophagitis was found in 33 (69%) and pathological esophageal acid exposure on MII-pH was detected in 12 (25%). Among baseline variables, only the presence of long-gap esophageal atresia was associated with abnormal MII-pH. Distal baseline impedance was significantly lower in patients with microscopic esophagitis, and it showed a very good diagnostic performance in predicting histological changes.Conclusions: Histological esophagitis is highly prevalent at 1 year after esophageal atresia repair, but our results do not support a definitive causative role of acid-induced GERD. Instead, they support the hypothesis that chronic stasis in the dysmotile esophagus might lead to histological changes. MII-pH may be a helpful tool in selecting patients who need closer endoscopic surveillance and/or benefit from acid suppression. [ABSTRACT FROM AUTHOR]

Abstract: This is the unusual case of a 17-year-old girl affected by Rett Syndrome (RS) who suffered acute abdominal distension and constipation for a week. Laparotomy showed massive gastric dilatation, with total necrosis and perforation. Total gastrectomy and Y-Roux esophagojejunostomy were performed. We believe the clinical status was caused by the mechanism of air swallowing, present in our patient and typical in RS. In fact, as reported, massive air bloat may result in a decrease of the intramural blood flow with consequential ischemia of the gastric wall. We stress the importance of early detection of the gastroenterological symptoms in these patients, with timely positioning of nasogastric tube and gastrostomy, to prevent serious complications potentially life-threatening as massive gastric necrosis. [Copyright &y& Elsevier]