Minimally invasive surgical approach in children treated for oesophageal atresia is associated with attention problems at school age: a prospective cohort study.

The long-term neurodevelopment of children born with oesophageal atresia (OA) is unclear. Therefore, we assessed the neurocognitive domains and their predictors. Patients born with OA between February 2006 and December 2014, who were routinely seen at eight years as part of a structured prospective longitudinal follow-up program, were included. Main outcome measures were need for school support, performance in various neurocognitive domains and potential predictors of neurocognitive problems. We analysed data of 65 children with a mean (SD) age of 8.1 (0.2) years, of whom 89% with OA type C. Thirty-five (54%) surgical corrections were minimally invasive; the median (interquartile range) duration of exposure to anaesthetics in the first 24 months was 398 (296 - 710) minutes. Forty-four (68%) attended regular education without extra support and intelligence was within normal range (99-108). More than 50% had z-scores ≤ -2 on one or more neurocognitive domains, of which attention was the most frequently affected domain. The speed on the sustained attention task was significantly below normal (z-score -1.48 (2.12), p < .001), as was fluctuation of sustained attention (z-score -3.19 (3.80), p < .001). The minimally invasive approach and a lower socio-economic status (both p = 0.006) proved significant predictors for sustained attention problems in multivariable analyses.  Conclusion: Children who undergo minimally invasive surgery for OA correction are at risk for sustained attention problems at school age. Future studies unravelling the effects of perioperative events on neurodevelopment should lead to optimal surgical, anaesthesiological, and intensive care management in the neonatal period. What is Known: • School-aged children born with oesophageal atresia have normal intelligence but problems with sustained attention at eight years. What is New: • Oesophageal atresia patients, who undergo minimally invasive surgery or who have a background of lower socioeconomic status are at serious risk for sustained attention problems at school age. • Moreover, those who have been intubated for a longer period are at risk for stronger fluctuations in sustained attention.

Surgical management of Encapsulating Peritoneal Sclerosis (EPS) in children: international case series and literature review.

BACKGROUND: Encapsulating Peritoneal Sclerosis (EPS) is a rare phenomenon in paediatric patients with kidney failure treated with peritoneal dialysis (PD). This study highlights clinical challenges in the management of EPS, with particular emphasis on peri-operative considerations and surgical technique. METHODS: Retrospective analysis of all paediatric patients with EPS treated at the Manchester Centre for Transplantation. RESULTS: Four patients were included with a median duration of 78 months on PD. All patients had recurrent peritonitis (> 3 episodes), and all had symptoms within three months of a change of dialysis modality from PD to haemodialysis or transplant. In Manchester, care was delivered by a multi-disciplinary team, including surgeons delivering the adult EPS surgical service with a particular focus on nutritional optimisation, sepsis control, and wound management. The surgery involved laparotomy, lavage, and enterolysis of the small bowel + / - stoma formation, depending on intra-abdominal contamination. Two patients had a formal stoma, which were reversed at three and six months, respectively. Two patients underwent primary closure of the abdomen, whereas two patients had re-look procedures at 48 h with secondary closure. One patient had a post-operative wound infection, which was managed medically. One patient's stoma became detached, leading to an intra-abdominal collection requiring re-laparotomy. The median length of stay was 25 days, and patients were discharged once enteral feeding was established. All patients remained free of recurrence with normal gut function and currently two out of four have functioning transplants. CONCLUSIONS: This series demonstrates 100% survival and parenteral feed independence following EPS surgery. Post-operative morbidity was common; however, with individualised experience-based decision-making and relevant additional interventions, patients made full recoveries. Health and development post-surgery continued, allowing the potential for transplantation. A higher resolution version of the Graphical abstract is available as Supplementary information.

A novel method for monitoring abdominal compliance to optimize insufflation pressure during laparoscopy.

BACKGROUND: Abdominal compliance describes the ease of expansion of the abdominal cavity. Several studies highlighted the importance of monitoring abdominal compliance (Cab) during the creation of laparoscopic workspace to individualize the insufflation pressure. The lack of validated clinical monitoring tools for abdominal compliance prevents accurate tailoring of insufflation pressure. Oscillometry, also known as the forced oscillation technique (FOT), is currently used to measure respiratory mechanics and has the potential to be adapted for monitoring abdominal compliance. This study aimed to define, develop and evaluate a novel approach which can monitor abdominal compliance during laparoscopy using endoscopic oscillometry. MATERIALS AND METHODS: Endoscopic oscillometry was evaluated in a porcine model for laparoscopy. A custom-built insufflator was developed for applying an oscillatory pressure signal superimposed onto a mean intra-abdominal pressure. This insufflator was used to measure the abdominal compliance at insufflation pressures ranging from 5 to 20 hPa (3.75 to 15 mmHg). The measurements were compared to the static abdominal compliance, which was measured simultaneously with computed tomography imaging. RESULTS: Endoscopic oscillometry recordings and CT images were obtained in 10 subjects, resulting in 76 measurement pairs for analysis. The measured dynamic Cab ranged between 0.0216 and 0.261 L/hPa while the static Cab based on the CT imaging ranged between 0.0318 and 0.364 L/hPa. The correlation showed a polynomial relation and the adjusted R-squared was 97.1%. CONCLUSIONS: Endoscopic oscillometry can be used to monitor changes in abdominal compliance during laparoscopic surgery, which was demonstrated in this study with a comparison with CT imaging in a porcine laparoscopy model. Use of this technology to personalize the insufflation pressure could reduce the risk of applying excessive pressure and limit the drawbacks of insufflation.

Recommendations for endoscopic surveillance after esophageal atresia repair in adults.

BACKGROUND: Endoscopic surveillance of adults with esophageal atresia is advocated, but the optimal surveillance strategy remains uncertain. This study aimed to provide recommendations on appropriate starting age and intervals of endoscopic surveillance in adults with esophageal atresia. METHODS: Participants underwent standardized upper endoscopies with biopsies. Surveillance intervals of 3-5 years were applied, depending on age and histopathological results. Patient's age and time to development of (pre)malignant lesions were calculated. RESULTS: A total of 271 patients with esophageal atresia (55% male; median age at baseline endoscopy 26.7 (range 15.6-68.5) years; colon interposition n = 17) were included. Barrett's esophagus was found in 19 (7%) patients (median age 32.3 (17.8-56.0) years at diagnosis). Youngest patient with a clinically relevant Barrett's esophagus was 20.9 years. Follow-up endoscopies were performed in 108 patients (40%; median follow-up time 4.6 years). During surveillance, four patients developed Barrett's esophagus but no dysplasia or cancer was found. One 45-year-old woman with a colon interposition developed an adenoma with high-grade dysplasia which was radically removed. Two new cases of esophageal carcinoma were diagnosed in patients (55 and 66 years old) who were not under surveillance. One of them had been curatively treated for esophageal carcinoma 13 years ago. CONCLUSIONS: This study shows that endoscopic screening of patients with esophageal atresia, including those with a colon interposition, can be started at 20 years of age. Up to the age of 40 years a surveillance interval of 10 years appeared to be safe. Endoscopic surveillance may also be warranted for patients after curative esophageal cancer treatment.

An international survey on anastomotic stricture management after esophageal atresia repair: considerations and advisory statements.

BACKGROUND: Endoscopic dilatation is the first-line treatment of stricture formation after esophageal atresia (EA) repair. However, there is no consensus on how to perform these dilatation procedures which may lead to a large variation between centers, countries and doctor's experience. This is the first cross-sectional study to provide an overview on differences in endoscopic dilatation treatment of pediatric anastomotic strictures worldwide. METHODS: An online questionnaire was sent to members of five pediatric medical networks, experienced in treating anastomotic strictures in children with EA. The main outcome was the difference in endoscopic dilatation procedures in various centers worldwide, including technical details, dilatation approach (routine or only in symptomatic patients), and adjuvant treatment options. Descriptive statistics were performed with SPSS. RESULTS: Responses from 115 centers from 32 countries worldwide were analyzed. The preferred approach was balloon dilatation (68%) with a guidewire (66%), performed by a pediatric gastroenterologist (n = 103) or pediatric surgeon (n = 48) in symptomatic patients (68%). In most centers, hydrostatic pressure was used for balloon dilatation. The insufflation duration was standardized in 59 centers with a median duration of 60 (range 5-300) seconds. The preferred first-line adjunctive treatments in case of recurrent strictures were intralesional steroids and topical mitomycin C, in respectively 47% and 31% of the centers. CONCLUSIONS: We found a large variation in stricture management in children with EA, which confirms the current lack of consensus. International networks for rare diseases are required for harmonizing and comparing the procedures, for which we give several suggestions.

Towards integrative neuromonitoring of the surgical newborn: A systematic review.

BACKGROUND: The altered neurodevelopment of children operated on during the neonatal period might be due to peri-operative changes in the homeostasis of brain perfusion. Monitoring of vital signs is a standard of care, but it does not usually include monitoring of the brain. OBJECTIVES: To evaluate methods of monitoring the brain that might be of value. We also wanted to clarify if there are specific risk factors that result in peri-operative changes and how this might be evaluated. DESIGN: Systematic review. DATA SOURCES: A structured literature search was performed in MEDLINE in Ovid, Embase, Cochrane CENTRAL, Web of Science and Google Scholar. ELIGIBILITY CRITERIA: Studies in neonates who received peri-operative neuromonitoring were eligible for inclusion; studies on neurosurgical procedures or cardiac surgery with cardiopulmonary bypass and/or deep hypothermia cardiac arrest were excluded. RESULTS: Nineteen of the 24 included studies, totalling 374 infants, reported the use of near-infrared spectroscopy. Baseline values of cerebral oxygenation greatly varied (mean 53 to 91%) and consequently, no coherent results were found. Two studies found a correlation between cerebral oxygenation and mean arterial blood pressure. Five studies, with in total 388 infants, used (amplitude-integrated) electro-encephalography to study peri-operative brain activity. Overall, the brain activity decreased during anaesthesia and epileptic activity was more frequent in the peri-operative phase. The association between intra-operative cerebral saturation or activity and neuro-imaging abnormalities and/or neurodevelopmental outcome was investigated in six studies, but no association was found. CONCLUSION: Neuromonitoring with the techniques currently used will neither help our understanding of the altered neonatal pathophysiology, nor enable early detection of deviation from the norm. The modalities lack specificity and are not related to clinical (long-term) outcome or prognosis. Accordingly, we were unable to draw up a monitoring guideline.

Perioperative management of esophageal atresia/tracheo-esophageal fistula: An analysis of data of 101 consecutive patients.

BACKGROUND: The perioperative management of esophageal atresia/tracheo-esophageal fistula by open or thoracoscopic approach can be complicated by metabolic derangements. Little is known, however, about the severity of derangements of vital and metabolic parameters in the perioperative period. AIM: The aim of this study is to describe the perioperative courses of vital and metabolic parameters in 101 consecutive neonates undergoing surgical repair of esophageal atresia type C. METHOD: In a retrospective cohort study, we extracted all data from the electronic anesthetic and medical charts of patients who underwent esophageal atresia type C repair within 30 days of life (2007-2017). We distinguished three types of surgery: primary open, primary thoracoscopic, and primary thoracoscopic surgery converted to open surgery. Descriptive analysis was applied. RESULTS: The charts of 117 patients were reviewed: data of 101 were included. The perioperative anesthetic management was not standardized; various methods and medications were used for anesthesia induction and maintenance. Intraoperative blood gas analysis data of 72 patients were available and showed derangements regardless of type of surgery. The median pH-value decreased to 7.21 [IQR 7.14-7.30] and a pH-value below 7.20 was found in 29 patients; in four cases below 7.0, with the lowest value 6.83. The median PaCO2 reached an upper level of 7.5kPa [IQR 5.8-9.2]; in 13 cases above 10.0kPa, with a peak value of 25.8kPa. These high PaCO2 levels fluctuated with lowest measured PaCO2 of median 5.6 [IQR 4.5-6.6], with the lowest value 2.8kPa. The median PaO2 level reached an upper level of 16.9kPa [IQR 11.8-25.7], in 22 cases above 20.0kPa, with a peak value of 50.0kPa. These high levels fluctuated with lowest measured PaO2 levels of median 8.3kPa [IQR 6.73-10.5]; the lowest PaO2 value was 4.7 kPa. CONCLUSION: Open and thoracoscopic correction of esophageal atresia were associated with periods of severe metabolic derangements. These events need to be taken into account for the evaluation of esophageal atresia (surgical) care and in evaluations of short- and long-term outcomes.

Thoracoscopic versus open repair of CDH in cardiovascular stable neonates.

BACKGROUND: Thoracoscopic surgery is an increasingly popular surgical technique to repair congenital diaphragmatic hernia (CDH). However, acidosis during surgery and the higher recurrence rate are considerable risk factors. The aim of this retrospective study is to compare the outcome of open versus thoracoscopic repair of the diaphragm in neonates with CDH with the same degree of cardiovascular and pulmonary illness who meet the criteria for thoracoscopic repair. METHODS: Retrospective analysis of all patients of two large national reference centers for CDH born in the years 2008 through 2012, and meeting the criteria for surgical repair on cardiopulmonary and physiological criteria according to the CDH EURO consortium consensus and meeting the criteria for thoracoscopic repair according to the review by Vijfhuize et al. The surgical technical aspects were comparable in both centers. RESULTS: 108 patients were included, of whom 75 underwent thoracoscopic repair and 34 underwent open repair. The gestational age and lung-to-head ratio were significantly lower and stay on the ICU significantly longer in the open-repair group. The operation time was longer (178 vs. 150 min, p = .012) and the recurrence rate higher (18.9 vs. 5.9 %, p = .036) in the thoracoscopic-repair group. The arterial pH, pO2, pCO2 and base excess before and after thoracoscopic repair were all significantly different. CONCLUSION: After critical selection for thoracoscopic repair of left-sided CDH based on the patient's preoperative condition, the outcomes of open repair were almost identical to those of thoracoscopic repair. A notable exception is the recurrence rate, which was significantly higher in the thoracoscopic-repair group. For the time being, thoracoscopic primary closure seems a safe and effective procedure, but efficacy of thoracoscopic patch repair has not been established.

Optimizing working space in laparoscopy: CT-measurement of the effect of neuromuscular blockade and its reversal in a porcine model.

OBJECTIVE: The objective of this paper was to determine the effect of neuromuscular blockade (NMB) on working space in a porcine laparoscopy model. BACKGROUND: Conflicting results on the effect of NMB on laparoscopic working space are found in literature. Almost all studies are limited by absence of objective assessment of working space or use surrogate outcomes. METHODS: In a standardized porcine laparoscopy model, laparoscopic working-space dimensions with and without NMB were investigated in 16 animals using computed tomography at intra-abdominal pressures of 0, 5, 10, and 15 mmHg during multiple runs of abdominal insufflation. RESULTS: No statistically significant effect of NMB on abdominal dimensions and laparoscopic working-space volume was found during CO2 pneumoperitoneum. In contrast, the effect of pre-stretching of the abdominal wall by a previous abdominal insufflation was found to be significant. CONCLUSIONS: This experimental study confirms the results from several clinical studies that NMB does not influence laparoscopic working space. Studies dealing with working space during laparoscopy should take note of pre-stretching bias.

Optimizing working space in laparoscopy: CT measurement of the effect of pre-stretching of the abdominal wall in a porcine model.

BACKGROUND: Determinants of working space in minimal access surgery have not been well studied. Using computed tomography (CT) to measure volumes and linear dimensions, we are studying the effect of a number of determinants of CO2 working space in a porcine laparoscopy model. Here we report the effects of pre-stretching of the abdominal wall. METHODS: Earlier we had noted an increase in CO2 pneumoperitoneum volume at repeat insufflation with an intra-abdominal pressure (IAP) of 5 mmHg after previous stepwise insufflation up to an IAP of 15 mmHg. We reviewed the data of this serendipity group; data of 16 pigs were available. In a new group of eight pigs, we also explored this effect at repeat IAPs of 10 and 15 mmHg. Volumes and linear dimensions of the CO2 pneumoperitoneum were measured on reconstructed CT images and compared between the initial and repeat insufflation runs. RESULTS: Previous stepwise insufflation of the abdomen with CO2 up to 15 mmHg significantly (p < 0.01) increased subsequent working-space volume at a repeat IAP of 5 mmHg by 21 %, 7 % at a repeat IAP of 10 mmHg and 3 % at a repeat IAP of 15 mmHg. The external anteroposterior diameter significantly (p < 0.01) increased by 0.5 cm (14 %) at repeat 5 mmHg. Other linear dimensions showed a much smaller change. There was no statistically significant correlation between the duration of the insufflation run and the volume increase after pre-stretching at all IAP levels. CONCLUSIONS: Pre-stretching of the abdominal wall allows for the same surgical-field exposure at lower IAPs, reducing the negative effects of prolonged high-pressure CO2 pneumoperitoneum on the cardiorespiratory system and microcirculation. Pre-stretching has important scientific consequences in studies addressing ways of increasing working space in that its effect may confound the possible effects of other interventions aimed at increasing working space.

Optimizing working space in porcine laparoscopy: CT measurement of the effects of intra-abdominal pressure.

BACKGROUND: Several factors may affect volume and dimensions of the working space in laparoscopic surgery. The precise impact of these factors has not been well studied. In a porcine model, we used computed tomographic (CT) scanning for measuring working space volume and distances. In a first series of experiments, we studied the relationship between intra-abdominal pressure (IAP) and working space. METHODS: Eleven 20 kg pigs were studied under standardized anesthesia and volume-controlled ventilation. Cardiorespiratory parameters were monitored continuously, and blood gas samples were taken at different IAP levels. Respiratory rate was increased when ETCO2 exceeded 7 kPa. Breath-hold CT scans were made at IAP levels of 0, 5, 10, and 15 mmHg. Insufflator volumes were compared to CT-measured volumes. Maximum dimensions of pneumoperitoneum were measured on reconstructed CT images. RESULTS: Respiratory rate had to be increased in three animals. Mild hypercapnia and acidosis occurred at 15 mmHg IAP. Peak inspiratory pressure rose significantly at 10 and 15 mmHg. CT-measured volume increased relatively by 93 % from 5 to 10 mmHg IAP and by 19 % from 10 to 15 mmHg IAP. Comparing CT volumes to insufflator volumes gave a bias of 76 mL. The limits of agreement were -0.31 to +0.47, a range of 790 mL. The internal anteroposterior diameter increased by 18 % by increasing IAP from 5 to 10 mmHg and by 5 % by increasing IAP from 10 to 15 mmHg. At 15 mmHg, the total relative increase of the pubis-diaphragm distance was only 6 %. Abdominal width did not increase. CONCLUSIONS: CT allows for precise calculation of the actual CO2 pneumoperitoneum volume, whereas the volume of CO2 released by the insufflator does not. Increasing IAP up to 10 mmHg achieved most gain in volume and in internal anteroposterior diameter. At an IAP of 10 mmHg, higher peak inspiratory pressure was significantly elevated.

Optimizing working-space in laparoscopy: measuring the effect of mechanical bowel preparation in a porcine model.

BACKGROUND: Adequate working space is a prerequisite for safe and efficient minimal access surgery. No objective data exist in literature about the effect of mechanical bowel preparation (MBP) on working space in laparoscopic surgery. We objectively measured this effect with computed tomography in a porcine laparoscopy model. METHODS: Using standardized anesthesia, twelve 20-kg pigs without MBP and eight 20-kg pigs with MBP were studied with computed tomography at intra-abdominal pressure (IAP) levels of 0, 5, 10, and 15 mmHg. Volumes and dimensions of the pneumoperitoneum were measured on reconstructed CT images and compared between the pigs with and those without MBP. RESULTS: A reproducible and statistically significant increase of approximately 500 ml in pneumoperitoneum volume was found in the MBP group at all levels of IAP. This represents a 43 % relative increase at a pneumoperitoneum pressure of 5 mmHg, 21 % at IAP 10 mmHg, and 18 % at IAP 15 mmHg. Peak inspiratory pressure was lower at IAP 0 and 5 mmHg in the MBP group. Anteroposterior diameter in the group with MBP was lower at 0 mmHg, but abdominal dimensions were similar in both groups at all other IAPs. This shows that the gain in working space is due to a diminished volume of the intra-abdominal content and not to compression or displacement of the bowel. CONCLUSIONS: MBP increases working space by reducing bowel content. Especially at low intra-abdominal working pressures, the increase in working space associated with MBP could represent an important benefit in challenging laparoscopic surgery.

Automated control for investigation of the insufflation-ventilation interaction in experimental laparoscopy.

In laparoscopic surgery the abdominal cavity is insufflated with pressurized carbon dioxide gas to create workspace. This pressure is exerted through the diaphragm onto the lungs, competing with ventilation and hampering it. In clinical practice the difficulty of optimizing this balance can lead to the application of harmfully high pressures. This study set out to create a research platform for the investigation of the complex interaction between insufflation and ventilation in an animal model. The research platform was constructed to incorporate insufflation, ventilation and relevant hemodynamic monitoring devices, controlling insufflation and ventilation from a central computer. The core of the applied methodology is the fixation of physiological parameters by applying closed-loop control of specific ventilation parameters. For accurate volumetric measurements the research platform can be used in a CT scanner. An algorithm was designed to keep blood carbon dioxide and oxygen values stable, minimizing the effect of fluctuations on vascular tone and hemodynamics. This design allowed stepwise adjustment of insufflation pressure to measure the effects on ventilation and circulation. A pilot experiment in a porcine model demonstrated adequate platform performance. The developed research platform and protocol automation have the potential to increase translatability and repeatability of animal experiments on the biomechanical interactions between insufflation and ventilation.

First genome-wide association study of esophageal atresia identifies three genetic risk loci at CTNNA3, FOXF1/FOXC2/FOXL1, and HNF1B.

Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) is the most common congenital malformation of the upper digestive tract. This study represents the first genome-wide association study (GWAS) to identify risk loci for EA/TEF. We used a European case-control sample comprising 764 EA/TEF patients and 5,778 controls and observed genome-wide significant associations at three loci. On chromosome 10q21 within the gene CTNNA3 (p = 2.11 × 10-8; odds ratio [OR] = 3.94; 95% confidence interval [CI], 3.10-5.00), on chromosome 16q24 next to the FOX gene cluster (p = 2.25 × 10-10; OR = 1.47; 95% CI, 1.38-1.55) and on chromosome 17q12 next to the gene HNF1B (p = 3.35 × 10-16; OR = 1.75; 95% CI, 1.64-1.87). We next carried out an esophageal/tracheal transcriptome profiling in rat embryos at four selected embryonic time points. Based on these data and on already published data, the implicated genes at all three GWAS loci are promising candidates for EA/TEF development. We also analyzed the genetic EA/TEF architecture beyond the single marker level, which revealed an estimated single-nucleotide polymorphism (SNP)-based heritability of around 37% ± 14% standard deviation. In addition, we examined the polygenicity of EA/TEF and found that EA/TEF is less polygenic than other complex genetic diseases. In conclusion, the results of our study contribute to a better understanding on the underlying genetic architecture of ET/TEF with the identification of three risk loci and candidate genes.

The Safety of Intralesional Steroid Injections in Young Children and Their Effectiveness in Anastomotic Esophageal Strictures-A Meta-Analysis and Systematic Review.

OBJECTIVE: Intralesional steroid injections (ISI) are a widely used technique for various pediatric indications and represent a possible adjuvant treatment for anastomotic esophageal strictures. Yet, no consensus has been reached neither on their safety in the pediatric population or their effectiveness in esophageal atresia patients. This systematic review aimed to assess the safety of ISI in young children through a meta-analysis and to summarize the current knowledge on the effectiveness of ISI in anastomotic esophageal strictures. METHODS: A systematic literature search was performed in Embase, Medline, Web of Science Core Collection, Cochrane Central Register of Controlled Trials and Google Scholar up to August 16 2021. Studies focusing on ISI and involving children up to 2 years were included in the meta-analysis for the safety assessment. All studies evaluating the use of ISI as adjuvant treatment in anastomotic esophageal strictures in children were included in the systematic review to assess the effectiveness of the intervention. RESULTS: The literature search yielded 8,253 articles. A total of 57 studies were included, of which 55 for the safety and five for the effectiveness assessment. The overall complication rate was 7%, with a greater incidence of local complications compared to systemic complications. Six studies (with a total of 367 patients) evaluated adrenocorticotropic hormone and cortisol levels, of which four reported hypothalamic-pituitary axis suppression. Two children (0.6%) received replacement therapy and all patients recovered uneventfully. A mean number of 1.67 ISI were performed per esophageal atresia (EA) patient. A reduction of needed dilatations was seen after ISI, compared to the number of dilatations performed before the intervention (5.2 vs. 1.3). CONCLUSION: The insufficient data emphasized the need for further prospective and comparative studies. Results from this meta-analysis and systematic review address ISI as a safe and effective technique. Close clinical follow-up and growth curve evaluation are advisable in patients receiving ISI. SYSTEMATIC REVIEW REGISTRATION: PROSPERO, identifier: CRD42021281584.

Primary repair of esophageal atresia is followed by multiple diagnostic and surgical procedures.

BACKGROUND: Children born with esophageal atresia (EA) face comorbidities and complications often requiring surgery and anesthesia. We aimed to assess all procedures performed under general anesthesia during their first 12 years of life. METHODS: We performed a retrospective cohort study about subsequent surgeries and procedures requiring general anesthesia in children born with type C EA between January 2007 and December 2017, with follow-up to March 2019. RESULTS: Of 102 eligible patients, 63 were diagnosed with comorbidities, of whom 18 had VACTERL association. Follow-up time for all patients varied between 14 months and 12 years (median 7 years). The patients underwent total 637 procedures, median 4 [IQR2-7] per patient. In the first year of life, 464 procedures were performed, in the second year 69 and in the third year 29. Thirteen patients underwent no other procedures than primary EA repair. In 57 patients, 228 dilatations were performed. Other frequently performed procedures were esophagoscopy (n=52), urologic procedures (n=44) and abdominal procedures (n=33). CONCLUSIONS: Patients with EA frequently require multiple anesthetics for a variety of procedures related to the EA, complications and comorbidities. This study can help care providers when counselling parents of a patient with an EA by giving them more insight into possible procedures they can be confronted with during childhood.

The effect of intralesional steroid injections on esophageal strictures and the child as whole: A case series.

BACKGROUND: The most frequent complication after esophageal atresia repair remains anastomotic stricture formation. The initial treatment is endoscopic dilatation. Intralesional steroid injection (ISI) might be an effective adjuvant treatment in case of recurrent strictures. In this series we present our initial experience with this intervention. METHODS: Data on primary surgery, stricture treatment, postoperative complications, outcome and growth were retrospectively collected from electronic patient records. Findings were analyzed by descriptive statistics and mixed model analysis. RESULTS: Between 2014 and 2017, ISI was performed for severe recurrent anastomotic strictures in six patients (median age at injection 12.4 (2.1-34.7) months) after a median of 6 (2-20) dilatations. In five patients ISI was successful and the stenosis was cleared. No postoperative complications were reported, especially none related to acute adrenal suppression. Comparing the year before with the year after ISI, a significant positive change for weight (r = 0.70, p = 0.003) was calculated versus a negative change for height (r = -0.87, p = 0.003). CONCLUSIONS: We found ISI to be an effective adjuvant treatment to recurrent anastomotic stricture dilatation after esophageal atresia repair, without postoperative complications or symptoms of adrenal suppression. It remains important, however, to monitor growth effects. Further evaluation is required in a large prospective study. TYPE OF STUDY: Treatment study, Level IV (case series).

Interventions with Music in PECTus excavatum treatment (IMPECT trial): a study protocol for a randomised controlled trial investigating the clinical effects of perioperative music interventions.

INTRODUCTION: Pectus excavatum repair is associated with substantial postoperative pain, despite the use of epidural analgesia and other analgesic regimens. Perioperative recorded music interventions have been shown to alleviate pain and anxiety in adults, but evidence for children and adolescents is still lacking. This study protocol describes a randomised controlled trial that evaluates the effects of recorded music interventions on postoperative pain relief in children and adolescents after pectus excavatum repair. METHODS: A multicentre randomised controlled trial was set up comparing the effects of perioperative recorded music interventions in addition to standard care with those of standard care only in patients undergoing a Nuss procedure for pectus excavatum repair. One hundred and seventy subjects (12-18 years of age) will be included in three centres in the Netherlands. Patient inclusion has started in November 2018, and is ongoing. The primary outcome is self-reported perceived pain measured on the visual analogue scale. Secondary outcomes are anxiety level, analgesics consumption, vital parameters such as heart rate, blood pressure and respiratory rate, length of hospital stay, postoperative complications, quality of life and cost-effectiveness. ETHICS AND DISSEMINATION: This study is being conducted in accordance with the Declaration of Helsinki. The Medical Ethics Review Board of Erasmus University Medical Centre Rotterdam, The Netherlands, has approved this protocol. Results will be disseminated via peer-reviewed scientific journals and conference presentations. TRIAL REGISTRATION NUMBER: NL6863.

Effects of Neonatal Thoracoscopic Surgery on Tissue Oxygenation: A Pilot Study on (Neuro-) Monitoring and Outcomes.

INTRODUCTION: Multiple reports have questioned the feasibility of neonatal thoracoscopic repair of congenital diaphragmatic hernia (CDH) and esophageal atresia (EA). The aim of this study is to examine the effects of CO2 pneumothorax on cerebral and renal rSO2 and to assess the potential predictive value of these data on neurodevelopmental outcome after neonatal thoracoscopic surgery for CDH or EA. MATERIALS AND METHODS: A prospective observational pilot study. Cerebral and renal regional tissue oxyhemoglobine saturation (rSO2) rSO2 were assessed using near-infrared spectroscopy (NIRS) during thoracoscopic surgery in neonates with CDH and with EA, in addition to routine anesthesia monitoring. Cerebral and renal rSO2, linked to repeated arterial blood gas analyses, heart rate, blood pressure, and to structured longitudinal neurodevelopmental follow-up. RESULTS: Baseline estimated marginal means of cerebral rSO2 values (CDH: 82%, EA: 91%) did not change significantly during pneumothorax (CDH: 81%, EA 79% [n.s. versus baseline]) despite severe acidosis (lowest pH, CDH: 6.99, EA: 7.1). Neurodevelopmental outcomes at 24 months were normal in all 7 patients who were available for evaluation. CONCLUSION: Neonatal thoracoscopic repair of CDH and EA using CO2-pneumothorax leads to severe acidosis. Cerebral rSO2 remained within clinical acceptable limits during intraoperative periods of acidosis. Neurodevelopmental outcome was favorable within the first 24 months. The potential of NIRS to further improve perioperative care and long-term outcome in this specific patient group deserves further investigation.