The aim of the study was to investigate the prevalence of esophageal atresia (EA), frequency of associated anomalies, and mortality from 2004 to 2017 in the Finnish population. We hypothesized the Spitz classification and the presence of other congenital malformations would predict mortality in patients with EA as well as assumed the survival to be high among patients with EA.

 This retrospective, population-based study was based on the registries maintained by THL Finnish Institute for Health and Welfare and Statistics Finland. The cases were identified and classified according to the World Health Organization's International Classification of Diseases and Health Related Problems (ICD) revisions 9 and 10 (ICD-9 and ICD-10) codes and accompanying written diagnoses. Associated anomalies were classified based on the EUROCAT criteria, and minor anomalies were excluded. All statistical tests were performed as a two-sided significance level set at p < 0.05. The chi-square or Fisher's exact test was utilized for categorical variables. The change in prevalence rates during the study period was evaluated with linear regression.

 In total, 337 cases with EA were identified including 295 (87.5%) live births, 17 (5.0%) stillbirths, and 25 (7.4%) terminations of pregnancy. The total prevalence for EA in Finland was 4.17/10,000 births with no significant change during the study period, p = 0.35. Neonatal mortality was 5% (n = 15) and 1-year survival was 91.5%. Mortality was associated with syndromic cases (p = 0.002). The Spitz classification predicted neonatal mortality better than cardiac anomalies alone (p < 0.001 and p = 0.6, respectively). Type C was the most common atresia type (65.9%) followed by type A (14.8%) and B (6.8%). The most common group of associated malformations were heart defects (35.0%) followed by other gastrointestinal tract malformations (15.3%) and limb anomalies (12.2%). Syndromic cases (12.2%) were associated with type A and B atresias (p = 0.001). VACTERL association was observed in 16.6% of the cases.

 The overall prevalence of EA remains stable and relatively high in Finland. Despite the high prevalence of co-occurring malformations, the overall survival rate is high. Spitz classification predicted neonatal survival well.

Post-operative muscle paralysis aims to reduce anastomotic complications following primary esophageal atresia (EA) repair. This study evaluates the impact of paralysis on outcomes in children enrolled in the EUPSA Esophageal Atresia Registry (EAR).

Patients with type B, C, and D EA enrolled in the EAR between 2014 and 2017 who underwent primary EA repair were included. They were divided into two groups based on the use of paralysis (Group P) or not (Group NP). Comparisons included demographics, associated malformations, surgical approach, complications, and hospital stay. Multivariate logistic regressions analyses were performed.

Of 316 patients, 126 were in Group P and 190 in Group NP. Group P had significantly lower gestational age and birth weight. Ventilation duration (7.9 ± 10.4 vs. 4.0 ± 4.4 days; p < 0.001) and hospital stay (43.7 ± 93.7 vs. 27.5 ± 31.8 days; p < 0.001) were significantly longer in Group P. The overall complication rate was higher in Group P (39.7 % vs. 28.4 %; p = 0.036), but mortality rates did not differ significantly. Post-operative paralysis was not associated with a significant reduction in anastomotic leaks or strictures.

Post-operative paralysis may provide stability in high-risk cases, such as neonates with low birth weight or associated malformations, but it does not significantly reduce surgical complications and may prolong recovery. Its use should be carefully considered and limited to individualized scenarios where the benefits outweigh the risks.

Recurrent Tracheoesophageal Fistula (rTEF) is a complication of Esophageal Atresia (EA) that can lead to severe respiratory symptoms. RTEF can be corrected via endotracheal treatment (ET) or surgical treatment (ST). The efficacy of these techniques varies in literature. This study presents the outcome of rTEF treatment after EA correction at the Wilhelmina Children's Hospital in Utrecht.

From 2000 until 2023, patients with EA (n = 251) underwent thoracoscopic EA correction by using the native esophagus at the Wilhelmina Children's Hospital in Utrecht. All EA patients who had developed rTEF (n = 19) were evaluated. Success rates, number of treatment attempts, and complication rates of ET and ST were collected in our EA database. Data was expressed as medians with interquartile ranges (IQR).

In total, 19/251 (8 %) patients had developed an rTEF after EA correction. For 10/19 (53 %) patients, primary ET was performed, with a median number of 2 procedures per patient [range 1-3]. In 8/10 patients, ET failed, therefore, these eight patients underwent secondary ST. For 9/19 (47 %) patients, primary ST was performed, with a median number of 1 procedure per patient [range 1-3]. In all patients, primary ST was performed via thoracoscopy with a success rate (absence of recurrence after 1 procedure) of 78 % (7/9) and no conversions. The complication rate was 10 % for primary ET and 44 % for primary ST, and 75 % for secondary ST. All complications were classified as minor.

Thoracoscopic correction of rTEF has a high success rate. Similar to previous studies, the Utrecht success rate of ET for rTEF treatment is poor.

Retrospective cohort study.

IV.

This review examines the common risk factors associated with anastomotic stricture (AS) following esophageal atresia (EA) repair and evaluates the current understanding and clinical practices concerning esophageal dilation, which remains the predominant therapeutic approach. We present emerging evidence on adjunctive therapies for recurrent and refractory strictures, including esophageal stenting, endoscopic incision therapy, mitomycin C application, local steroid injections, cell sheet transplantation, and surgical interventions. A comprehensive literature review was conducted using PubMed, with a cutoff date of December 31, 2024, encompassing all relevant studies and reviews pertinent to this topic, with a particular emphasis on refractory and recurrent strictures. Given the scarcity of evidence-based data on AS in children with EA, we also critically analyze findings from adult literature and studies addressing esophageal strictures caused by various etiologies to provide a more comprehensive understanding. The pathogenesis of AS is multifactorial, with a key contributing factor being the significant gap between the proximal and distal esophageal segments. This anatomical disparity increases anastomotic tension, thereby elevating the likelihood of stricture formation. A thorough identification and detailed understanding of the risk factors associated with AS are crucial for enhancing patient outcomes and developing effective preventive strategies. Currently, the evidence regarding esophageal dilation is insufficient to establish the superiority of any single technique, particularly between balloon and bougie dilators. Recurrent and refractory strictures remain a challenge, as no pharmacological and mechanical adjunctive interventions have demonstrated sufficient feasibility, efficacy, and safety to fundamentally change clinical practice. While some results show promise, transformative outcomes have not yet been achieved, and further research is needed to establish evidence-based best treatment strategies.

Background and objectives: Outcomes of neonates diagnosed with esophageal atresia (EA), a rare congenital malformation, vary widely. Due to limited and fragmented data globally, major regional centers offer a crucial opportunity to better understand EA's epidemiology and the management. This study aimed to address these gaps by determining total birth prevalence and early treatment outcomes of EA in southern Croatia. Methods: All EA cases (1991-2020) were retrospectively ascertained from medical documentation at the only tertiary referral center for EA in southern Croatia, with birth data collected from the entire background population. We collected data on neonates' status and diagnosis, operative findings, early postoperative complications, and treatment outcome from this single center. Results: A total of 53 cases were identified, with an average total birth prevalence of 2.44 per 10,000 total births/year. No significant sex differences were found (p = 0.339), and most cases were complex Vogt 3B. The mortality rate dropped from 87 to 8% over a 30-year period (p < 0.001). The 1-year survival rate was 54% (95% CI 40-68%) for liveborns and 64% (50-79%) for liveborns who underwent surgery and intensive care. However, 16% of patients died before surgery due to hemodynamic instability, and among those operated on, high sepsis, pneumonia, and atelectasis rates were observed. Conclusions: EA prevalence in southern Croatia aligns with European data. Survival improved significantly after 2002, coinciding with a dedicated pediatric ICU and enhanced pediatric care training. Further advancements in early diagnosis and a multidisciplinary approach are needed to further reduce mortality. Strengthened postoperative infection control and optimized postoperative respiratory support are also crucial to minimizing complications.

Esophageal atresia is one of the most common life-threatening congenital malformations and is defined as an interruption in the continuity of the esophagus with or without fistula to the trachea or bronchi. Definitive treatment is surgical ligation of the fistula if present and esophageal end-to-end anastomosis of the two pouches, thereby reconstructing the continuity of the esophagus. During this procedure, the surgeon may choose to either ligate or preserve the azygos vein, a major draining vein for the esophagus and surrounding structures, but no definitive consensus on the matter exists.

To assess the benefits and harms of preservation of the azygos vein versus ligation of the azygos vein during primary surgical repair of congenital esophageal atresia.

We identified trials from the Cochrane Gut Specialized Register, Cochrane Central Register of Controlled Trials (CENTRAL) via Ovid Evidence-Based Medicine Reviews Database (EBMR), MEDLINE, Embase, and CINAHL (Cumulative Index to Nursing and Allied Health Literature). We also conducted a search of ClinicalTrials.gov, LILACS, Science Citation Index Expanded and Conference Proceedings Citation Index - Science (Web of Science), and the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP). We searched all databases from their inception to 22 May 2024, with no restriction on language of publication.

We included randomized clinical trials (RCTs) assessing preservation of the azygos vein versus ligation of the azygos vein during primary surgical repair of congenital esophageal atresia in infants less than three months of age.

Pairs of two review authors independently screened titles and abstracts, screened relevant full-text reports, and identified RCTs for inclusion. We recorded the selection process in a PRISMA flow diagram. We assessed the risk of bias of the included studies (using RoB 2) and the certainty of the evidence (using the GRADE approach) according to the Cochrane Handbook for Systematic Reviews of Interventions. We contacted the study authors if data were missing or unclear.

Six trials met the inclusion criteria, including 390 participants in total. We were able to assess all our primary outcomes: all-cause mortality, serious adverse events, and anastomosis leakage, as well as two of our three secondary outcomes: sepsis or mediastinitis and esophageal stricture. However, none of the six trials assessed recurrent tracheoesophageal fistula. All trials had either some concerns or high risk of bias, and the certainty of the evidence for all outcomes was either low or very low. Meta-analyses showed that preservation of the azygos vein may result in a large reduction in mortality (risk ratio (RR) 0.44, 95% confidence interval (CI) 0.26 to 0.73; low-certainty evidence), serious adverse events (RR 0.33, 95% CI 0.21 to 0.50; very low-certainty evidence), and anastomosis leakage (RR 0.44, 95% CI 0.26 to 0.76; low-certainty evidence) when compared to ligation of the azygos vein in neonates undergoing primary surgical repair of congenital esophageal atresia. Meta-analysis of our secondary outcomes showed that preservation of the azygos vein may result in a large reduction in mediastinitis or sepsis (RR 0.34, 95% CI 0.21 to 0.53; very low-certainty evidence). Esophageal stricture was only reported in two studies with 114 participants. The effects on esophageal stricture were unclear (RR 0.75, 96% CI 0.35 to 1.63; very low-certainty evidence), but the evidence is very uncertain.

Current evidence suggests that preserving the azygos vein during primary surgical repair for esophageal atresia may result in large reductions in overall mortality, serious adverse events, anastomosis leakage, and sepsis or mediastinitis. No included data suggested that routine ligation of the azygos vein was beneficial. However, all the evidence was of low to very low certainty. Further research is still warranted as the results of this review may not be applicable to all newborns with congenital esophageal atresia.

Esophageal atresia (EA) is a complex malformation. Multidisciplinary management is necessary, with the operative repair being the most challenging step in the treatment algorithm. The complete care structure for children with EA in Germany has not been analyzed yet.

In the observed period 2016-2022, inpatient EA cases were analyzed 1) during the hospital stay of birth, and 2) during the hospital stay of corrective surgery in patients aged up to 365 days, both based on national hospital discharge data. Patients' comorbidities, hospital caseload, treatment characteristics (e.g. surgical approach) and outcome parameters were analyzed.

1) 1190 newborn EA cases were treated in 260 hospitals during the perinatal period. 54% had at least one additional malformation, and 16% had a birthweight below 1500 g. In-hospital mortality was 8.4%. 2) 1475 corrective operations for EA were performed in 111 hospitals with a consistent median annual caseload of 2 (P25-P95 1-8) per hospital. At least one indicator for a complicated perioperative course was documented in 63.7% of cases. The use of bronchoscopy was coded in 50% of cases. Median ventilation time during the entire hospital stay was 176 h (P25-P95: 95-1759 h).

Newborns with EA are complex and early postoperative complications are common. The care structure is decentralized, and there was no trend towards centralization in the observed period. The low documented use of bronchoscopy is noteworthy. Centralization of the highly complex and schedulable corrective surgery for EA is necessary to evaluate outcomes and might improve the quality of care and resource utilization. CATEGORY OF THE MANUSCRIPT AND TYPE OF STUDY: original article, observational cross-sectional study, secondary data analysis.

 Anastomotic stricture (AS) is the second most common complication after esophageal atresia (EA) repair. We aimed to evaluate the data in the Turkish Esophageal Atresia Registry to determine the risk factors for AS development after EA repair in a large national cohort of patients.

 The data between 2015 and 2021 were evaluated. Patients were enrolled into two groups according to the occurrence of AS. Patients with AS (AS group) and without AS (non-AS group) were compared according to demographic and operative features, postoperative intubation status, and postoperative complications, such as anastomotic leaks, fistula recanalization, and the presence of gastroesophageal reflux (GER). A multivariable logistic regression analysis was performed to define the risk factors for the development of AS after EA repair.

 Among the 713 cases, 144 patients (20.19%) were enrolled into the AS group and 569 (79.81%) in the non-AS group. The multivariable logistic regression showed that, being a term baby (odds ratio [OR]: 1.706; p = 0.006), having a birth weight over 2,500 g (OR: 1.72; p = 0.006), presence of GER (OR: 5.267; p < 0.001), or having a recurrent tracheoesophageal fistula (TEF, OR: 4.363; p = 0.006) were the risk factors for the development of AS.

 The results of our national registry demonstrate that 20% of EA patients developed AS within their first year of life. In patients with early primary anastomosis, birth weight greater than 2,500 g and presence of GER were risk factors for developing AS. When patients with delayed anastomosis were included, in addition to the previous risk factors, being a term baby, and having recurrent TEF also became risk factors.

 III.

Children undergoing surgical anastomosis for long-gap esophageal atresia (LGEA) often suffer from complications related to delayed oral feeding, which may impair their early development. Clinical swallow evaluation (CSE) is an effective technique to improve feeding outcomes. However, there are limited evidences on the application of CSE in these children.

Since 2020, serial CSEs have been consistently implemented for children undergoing anastomosis for LGEA in our hospital. We conducted a retrospective study comparing 19 children who received CSE with 31 historical controls who did not. Inverse probability of treatment weighting (IPTW) was applied to balance preoperative characteristics. We compared the time from surgery to full oral feeding and the rate of postoperative complications between the two groups. Growth curves for length-for-age Z score (LAZ) and weight-for-age Z score (WAZ) up to age 3 were fitted using generalized additive mixed models.

The median time to full oral feeding was 1.1 months [interquartile range (IQR), 0.8-2.4] in the CSE group and 1.5 months (IQR, 0.6-5.7) for controls. After IPTW, CSE was associated with a shorter time to full oral feeding, with a weighted hazard ratio of 2.26 [95% confidence interval (CI), 1.21 to 4.24]. LAZ growth curves significantly differed between groups (P = 0.001).

CSE was associated with the expedited achievement of full oral feeding and a more favorable growth pattern before 3 years of age.

Challenges regarding feeding difficulties and nutrition in children with esophageal atresia (EA) have been sparsely studied. The aim of this study was to explore parent-reported feeding difficulties in children with EA by applying Montreal Children's Hospital-Feeding Scale (MCH-FS), and to further explore associations between feeding difficulties and clinical factors, growth and nutritional intake.

Parents of EA children born between 2012 and 2017 were invited. Clinical data were collected from medical records. In a prospective cohort-study parent-reported feeding difficulties (by MCH-FS) were reported at two assessments, and at the second assessment, dietary data were collected by using the 24-h food-recall method.

Out of 55 eligible participants, we evaluated 53 children at median age of 1.6 years (Q1:Q3 1.0:2.9) (first assessment) and 38 at median age of 4.2 years (Q1:Q3 1.0:2.9) (second assessment). Feeding difficulties were reported by 34% and 31% of the parents, respectively, but no particular profile of concerns could be identified. Children's energy intake and weight-for-age were correlated with feeding difficulties (MCH-FS total score) (p < 0.02).

Parent-reported feeding difficulties were identified in one-third of children with EA and related to low energy intake and low weight-for-age, but not to clinical factors. This implies that feeding difficulties must be screened for during follow-up in all EA children and may facilitate early detection of challenges and intervention if needed.

This study investigates the long-term outcomes of palliative and definitive surgeries for esophageal atresia (EA) in patients with trisomy 18 syndrome. A retrospective study included 25 cases undergoing EA surgery at our center between 2008 and 2022. The Palliative group (n = 16) comprised 13 cases with esophageal banding and 3 with tracheoesophageal fistula (TEF) division. The Definitive group (n = 9) included 5 cases with primary repair and 4 with staged repair following TEF division. The patient characteristics exhibited no significant differences between the groups. In the Definitive group, 56% (5/9) were successfully weaned off mechanical ventilation, compared with none in the Palliative group (p = 0.002). Survival-to-discharge rates were 31% (5/16) in the Palliative group and 67% (6/9) in the Definitive group. Home ventilator management was required for all 5 cases that required ventilation in the Palliative group, whereas only 17% (1/6) in the Definitive group needed it. The Palliative group also required continuous oral suction for persistent saliva removal, with two cases undergoing laryngotracheal separation. Overall, definitive surgery for EA in patients with trisomy 18 syndrome may provide enhanced respiratory stability, thereby improving the survival-to-discharge rate and overall quality of life for patients and their families.

Long-gap esophageal atresia (LGEA) is an abnormality in the development of the esophagus resulting in the disruption of the continuity of the esophageal lumen with no feasible primary repair due to the "long gap" between two esophageal stumps. There is controversy regarding both the precise definition and the treatment protocol of this congenital condition. Methods such as delayed primary repair, open external traction, thoracoscopic external traction, and thoracoscopic internal traction were used in the treatment of patients with varied outcomes. Thoracoscopic internal traction was employed in the treatment of the described patient, who had a good functional outcome. Complications were managed conservatively with no need for additional surgeries. The four-week waiting period between both stages of the surgery made it possible to extubate the patient, forgo the postoperative need for paralysis, and reduce the period of sedation to the minimum. We believe that minimally invasive surgery can be used in the treatment of LGEA with good outcomes, provided the surgeon has sufficient experience.

The objectives of this study were to bring out the results of application of pleural wrap in primary repair of tracheo - esophageal fistula (TEF) with long-gap oesophageal atresia (LGEA) and also define the role of neonatologists and paediatric intensivists in post-operative management in these patients by laying down standard neonatal management protocol.

This was a retrospective descriptive observational study conducted between March 2011 and April 2019 on 23 cases of LGEA with TEF. The study was conducted at two tertiary care paediatric surgery centres in Northern part of India wherein these newborn babies were operated by two paediatric surgeons with variable experience of 8-12 years. It also describes the neonatal management protocol used in post-operative period.

Out of 23 patients, 15 were boys and 8 were girls, with a mean age of 32.25 weeks and a mean birth weight of 2.02 kg. The mean hospital stay was 23.5 ± 8 days. Eleven cases had gap between 3 and 3.5 cm, 8 cases between 3.5 and 4 cm and 4 cases had gap more than 4 cm. The incidence of associated anomalies was 52%. Anastomotic leak rate was 8.69%, and 3 (13.04%) patients died in the post-operative period. All the operated patients were managed postoperatively as per strict neonatal management protocol exclusively by the team of neonatologists and neonatal intensivists.

Application of pleural wrap over anastomosis following primary repair of LGEA with TEF significantly reduced the incidence of anastomotic leak in our study. Apart from the pleural wrap, the key to successful outcome also is contributed by the little prolonged, controlled ventilation and patience and perseverance in post-operative feeds. This post-operative management protocol that has been followed by us in our study is easily reproducible and can be adopted by paediatric surgeons working alongside neonatologists as a team.

Vertebral defects, anorectal anomalies, cardiac anomalies, tracheoesophageal fistula (TEF)/esophageal atresia, renal anomalies, and limb abnormalities (VACTERL) association is a rare congenital disorder presenting with a constellation of birth defects. The diagnosis is primarily clinical, and patients exhibit at least three of these anomalies. These patients' management involves a multidisciplinary approach tailored to the individual's condition. Anesthetic management is particularly challenging due to the diverse and complex anomalies. This article discusses the anesthetic management of a term newborn male (39 weeks, six days gestation, 3340 g) diagnosed with VACTERL association. The newborn was admitted to the neonatal intensive care unit (NICU) and scheduled for the surgical repair of TEF and derivative colostomy on the second day of life. To mitigate the risk of air leak and abdominal distension from positive pressure ventilation, a derivative colostomy was performed first under regional anesthesia preserving spontaneous ventilation. To achieve that, the patient was sedated with ketamine and dexmedetomidine, and an ultrasound-guided single-shot caudal block with ropivacaine was performed. Post-abdominal decompression, general anesthesia was induced, and intubation was managed via videolaryngoscopy. Thoracoscopic TEF repair required several pauses for ventilation and hemodynamic optimization. Dopamine was administered intraoperatively for blood pressure support. The newborn was extubated and started on enteral feeding by the seventh postoperative day, progressing well by the time of discharge. In this case, a derivative colostomy before TEF repair avoided positive pressure ventilation complications. Ultrasound-guided caudal block provided effective regional anesthesia with high success rates. Ketamine and dexmedetomidine offered balanced sedation with minimal respiratory compromise. Dopamine was used effectively to maintain adequate perfusion, monitored with invasive blood pressure and cerebral oximetry. Anesthetic management of newborns with VACTERL association undergoing simultaneous repair of TEF and anal atresia demands meticulous and tailored planning to address the specific needs and minimize associated risks. This case highlights the importance of comprehensive anesthetic management and its impact on the patient's outcome.

Esophageal atresia/tracheoesophageal fistula (EA/TEF) is a congenital malformation that occurs in about 1 in 2500-4000 live births. After surgical repair, despite the lack of evidence supporting the routine use of postoperative esophagram, most surgeons report obtaining an esophagram prior to enteral feeding. We hypothesized that abnormal indicators in vital signs, drain characteristics, and chest radiograph (CXR) could be used to screen for anastomotic leak, thus reducing the need for a routine esophagram.

A single institution, retrospective chart review of all patients born with EA with or without TEF between 2009 and 2022 was performed. Vital signs, postoperative CXR, chest drain characteristics, and esophagram results were analyzed for patients who underwent repair.

Forty-five patients who underwent EA/TEF repair were included in the study, and 40 patients had routine esophagram. Out of the twenty-two patients who had at least one abnormal indicator, 14 (64%) had an anastomotic leak. Seventeen patients (43%) had the absence of abnormalities of all three indicators, and none of these patients had an anastomotic leak (100% negative predictive value). Moreover, changes in drain characteristics and vital signs together presented high sensitivity (87.5%), specificity (90%), and negative predictive value (94%).

In the absence of abnormalities in vital signs, CXR, and drain characteristics in patients undergoing EA/TEF repair, routine esophagram can be safely avoided prior to enteral feeding. Abnormalities in drain characteristics and vital signs together were highly sensitive and specific for anastomotic leak, thus potentially eliminating the need for routine CXR and thereby minimizing radiation exposure and cost.

This study aimed to analyze the results, feasibility and safety of the thoracoscopic approach for patients with esophageal atresia with tracheoesophageal fistula (EA/TEF) depending on the patient's birth weight.

The study involved only type C and D EA/TEF. Among the analyzed parameters were the patients' characteristics, surgical treatment and post-operative complications: early mortality, anastomosis leakage, anastomosis strictures, chylothorax, TEF recurrence, and the need for fundoplication or gastrostomy.

145 consecutive newborns underwent thoracoscopic EA with TEF repair. They were divided into three groups-A (N = 12 with a birth weight < 1500 g), B (N = 23 with a birth weight ≥ 1500 g but < 2000 g), and C-control group (N = 110 with a birth weight ≥ 2000 g). Primary one-stage anastomosis was performed in 11/12 (91.7%) patients-group A, 19/23 (82.6%)-group B and 110 (100%)-group C. Early mortality was 3/12 (25%)-group A, 2/23 (8.7%)-group B, and 2/110 (1.8%)-group C and was not directly related to the surgical repair. There were no significant differences in operative time and the following complications: anastomotic leakage, recurrent TEF, esophageal strictures, and chylothorax. There were no conversions to an open surgery. Fundoplication was required in 0%-group A, 4/21 (19.0%)-group B, and 2/108 (1.9%)-group C survivors. Gastrostomy was performed in 1/9 (11.1%)-group A, 3/21 (14.3%)-group B and 0%-group C.

In an experienced surgeon's hands, even in the smallest newborns, the thoracoscopic approach may be safe, feasible, and worthy of consideration. Birth weight seems to be not a direct contraindication to the thoracoscopic approach.

Esophageal atresia (EA) is a congenital defect that causes esophageal discontinuity, often with an associated tracheo-esophageal fistula (TEF) in 70%-90% of cases. When the distance between esophageal ends precludes primary anastomosis, it results in long gap esophageal atresia (LGEA), complicating the surgical management. This study retrospectively reviewed LGEA cases from the past decade, treated with the goal of preserving the native esophagus, comparing surgical techniques and outcomes with current literature.

The data of patients treated for LGEA between 2013 and 2024 were collected from medical charts, focusing on patients treated with the preservation of their native esophagus.

Ten patients were enrolled for this study. All of them had a gap between the esophageal ends equal to or greater than three vertebral bodies. Four patients (40%) underwent a delayed primary anastomosis (DPA) procedure, while the remaining six (60%) underwent a traction staged repair. All patients were treated with open surgery. The follow-up period extended from 3 months to 10 years.

Preserving the native esophagus in patients with LGEA is a challenging but feasible goal, with delayed primary anastomosis and traction techniques playing key roles. We advocate for the preservation of the native esophagus as the preferred approach for ensuring a high quality of life for patients, as it helps to avoid severe long-term complications associated with esophageal substitution.

Esophageal squamous cell carcinoma (ESCC) remains the most common malignancy of the esophagus worldwide. Environmental and lifestyle exposures such as alcohol and tobacco have been well defined in the pathogenesis of ESCC, acting in concert with cell intrinsic epigenomic, genomic and transcriptomic changes. However, a variety of nonenvironmental etiologies including Fanconi anemia, lichen planus, chronic mucocutaneous candidiasis, esophageal epidermoid metaplasia, epidermolysis bullosa, tylosis, esophageal atresia, and achalasia receive minimal attention despite a high risk of ESCC in these diseases. The goal of this review was to promote clinical recognition and suggest a diagnostic framework for earlier detection of ESCC in patients with these rare diseases. In all the discussed conditions, a change in symptoms should trigger a prompt endoscopic evaluation, and endoscopic surveillance programs with advanced imaging techniques and chromoendoscopy should be considered. Moreover, we leverage the convergence of these diseases on ESCC to identify common mechanisms underlying malignant transformation including aberrant proliferation, mucosal barrier dysfunction, increased inflammation, and genome instability. In this study, we summarize the clinical presentation, pathologic findings, potential screening strategies, and common mechanisms of malignant transformation associated with these rare diseases that drive ESCC.

Babies with oesophageal atresia (OA) who cannot achieve a primary anastomosis (complex OA) may be treated by attempted oesophageal lengthening. We contrast reported outcomes of lengthening with our experience of managing complex OA.

A consecutive series treated in an English regional centre was completed. Outcomes of interest were the rate of retention of the native oesophagus, complications requiring thoracotomy, rates of anastomotic leak, stricture, fundoplication, and mortality. Possible explanatory variables were the surgical techniques applied.

29/215 (13%) OA were complex, and 25/207 survived to repair. 14/25 (56%) had no distal fistula, pure OA, while 11/25 (44%) had a long gap with distal fistula. 18/25 (72%) had delayed primary anastomosis, while 7/25 (28%) required oesophageal replacement. However, 2 of the replacements were salvage procedures following failed traction. Only 4/207 (2%) of OA were potentially treatable by traction. Salvage surgery was required in 2/23 (9%) complex OA not subjected to lengthening. The native oesophagus was retained without utilising lengthening in 200/207 (97%). Amongst complex OA where traction techniques had not been attempted, the native oesophagus was retained in 18/23 (78%) of cases, with median time to oesophageal continuity of 77 days. There was no in hospital mortality following treatment of complex OA, and overall survival was identical to non-complex OA among cases surviving to anastomosis.

Management of complex OA without lengthening procedures leads a similar rate of retention of the native oesophagus as reports describing lengthening, but with significantly less morbidity. We see little need for oesophageal lengthening in the management of complex OA.

IV.

We report a low-birth-weight child (1.8 kg) with neonatal type III congenital esophageal atresia (CEA) combined with symptomatic patent ductus arteriosus (PDA). After comprehensive evaluation, esophageal anastomosis was performed on postnatal day 11 after excluding surgical contraindications, and arterial catheter ligation was performed at the same time. Concurrent surgery for CEA combined with PDA has not been clearly reported in the literature.

We report a 6-day-old female child with type III CEA and PDA. The patient presented with foam at the mouth after birth, cough and shortness of breath after feeding. At another hospital, she was considered to have neonatal pneumonia, neonatal jaundice and congenital heart disease and transferred to our hospital. After iodine oil radiography of the esophagus and echocardiography we confirmed diagnosis of CEA and PDA. The diameter of the PDA was 8 mm, with obvious left to right shunting. We performed right rear extrapleural orificium fistula ligation and esophageal anastomosis, and ligation of PDA via left axilla straight incision after 5 d of hospitalization. The operations were successful, and the incision healed after 12 d, and the patient was discharged. We re-examined the patient 1 mo after surgery. She did not vomit when she ate rice flour. Esophageal angiography showed no stricture of the anastomotic stoma. The patient weighed 3.2 kg.

For CEA patients with multiple risk factors, comprehensive, timely and accurate diagnosis and evaluation, and early treatment may improve prognosis.

This case report details the emergency management and successful surgical repair of a tracheoesophageal fistula (TEF) in a newborn delivered by lower segment cesarean section. Despite immediate crying after birth, the neonate's distress was evident, with an Apgar score of 4, prompting an urgent referral to the Neonatal ICU (NICU). Diagnostic investigations, including ultrasonography and two-dimensional echocardiography (2D Echo), revealed associated anomalies, such as a patent ductus arteriosus, arterial septal defect, and a TEF. An anaesthetist was urgently involved due to postnatal desaturation, leading to challenging intubation and surgical repair performed under general anaesthesia, which involved separating the trachea from the oesophagus. Postoperative imaging confirmed the successful closure of the fistulous connection. This case highlights the significance of prompt diagnosis, collaborative management, and surgical intervention in optimising outcomes for neonates with complex congenital anomalies like TEF.

Oesophageal atresia-tracheoesophageal fistula (EA-TEF) is a common congenital digestive disease. Patients with EA-TEF face gastrointestinal, surgical, respiratory, otolaryngological, nutritional, psychological and quality of life issues in childhood, adolescence and adulthood. Although consensus guidelines exist for the management of gastrointestinal, nutritional, surgical and respiratory problems in childhood, a systematic approach to the care of these patients in adolescence, during transition to adulthood and in adulthood is currently lacking. The Transition Working Group of the International Network on Oesophageal Atresia (INoEA) was charged with the task of developing uniform evidence-based guidelines for the management of complications through the transition from adolescence into adulthood. Forty-two questions addressing the diagnosis, treatment and prognosis of gastrointestinal, surgical, respiratory, otolaryngological, nutritional, psychological and quality of life complications that patients with EA-TEF face during adolescence and after the transition to adulthood were formulated. A systematic literature search was performed based on which recommendations were made. All recommendations were discussed and finalized during consensus meetings, and the group members voted on each recommendation. Expert opinion was used when no randomized controlled trials were available to support the recommendation. The list of the 42 statements, all based on expert opinion, was voted on and agreed upon.

To determine the incidence, management and outcomes of esophageal atresia/tracheo-esophageal fistula (EA/TEF) over a 15-y period in South Africa.

A retrospective chart review of neonates with EA/TEF presenting at the main tertiary referral hospital in the KwaZulu-Natal province between 2002 and 2017 was conducted. Data collection comprised patient and maternal demographics, clinical presentations, laboratory and radiologic investigations, surgical procedures, and outcomes. A multivariate logistic regression determined the risk factors associated with mortality.

Among 180 neonates, mean (SD) age of diagnosis was four (three) days postnatal with Gross Type C (n = 165, 92%) being the most common and the incidence was one per 10,000 live births. Majority were born term (n = 95, 53%) at peripheral hospitals (n = 167, 93%) with a mean birth weight of 2369 (736) grams. Overall HIV exposure rate was 27% (n = 48). Most (n = 138, 77%) patients presented with established pneumonia, 44% (n = 61) of whom required prolonged (>7 d) ventilator support. The median (IQR) hospital stay was 11 (8-20) d. Overall survival rate was 70% (n = 126). Birth weight <1500 g, life threatening anomalies, ventilation >30 d and postoperative sepsis contributed to mortality.

Incidence, disease types and presentations were similar to developed countries. Despite advances in technology and neonatal care in Africa, EA/TEF surgical outcomes remain suboptimal likely due to caregivers' inability to care for these infants in disadvantaged socioeconomic circumstances with poor sanitation, etc. Research is needed to identify strategies tailored for disadvantaged communities which may contribute to improved outcomes in the perioperative and postoperative period.

Esophageal atresia (EA) is the most common malformation of the upper gastrointestinal tract. The estimated incidence of EA is 1 in 3500 births. EA is more frequently observed in boys and in twins. The exact cause of isolated EA remains unknown; a multifactorial etiology, including epigenetic gene expression modifications, is considered. The study included six pairs of twins (three pairs of monozygotic twins and three pairs of dizygotic twins) in which one child was born with EA as an isolated defect, while the other twin was healthy. DNA samples were obtained from the blood and esophageal tissue of the child with EA as well as from the blood of the healthy twin. The reduced representation bisulfite sequencing (RRBS) technique was employed for a whole-genome methylation analysis. The analyses focused on comparing the CpG island methylation profiles between patients with EA and their healthy siblings. Hypermethylation in the promoters of 219 genes and hypomethylation in the promoters of 78 genes were observed. A pathway enrichment analysis revealed the statistically significant differences in methylation profile of 10 hypermethylated genes in the Rho GTPase pathway, previously undescribed in the field of EA (ARHGAP36, ARHGAP4, ARHGAP6, ARHGEF6, ARHGEF9, FGD1, GDI1, MCF2, OCRL, and STARD8).

An Oesophageal atresia (OA) affects almost 1 in 3500 newborn and its aetiology for development between 4th to 6th pregnancy weeks had not yet been clarified.

This retrospective, observational control group-supported (n = 30) examination was performed with OA mothers (n = 20) who were interrogated via specialized questionnaires and interviews to evaluate data on medical pregnancy history including hormonal treatment, changing of life habits (nutrition, diet, medication), drug consumption (alcohol, smoking, illicit drugs) as well as exposure to nuclear waste or poisonous substance exposition. Aim was to assess risk factors during pregnancy and in particular in the first trimester.

The body mass index (BMI) prior pregnancy of OA mothers was significant lower (p = 0.022) and the number of mothers with obesity (BMI>30) did not differ to control group mothers (p = 0.081). They had additionally more pre-existing illnesses (p = 0.009) and allergies (p = 0.001). Control group mothers changed dietary habits with higher meat (p = 0.032) and caffeine intake (p = 0.012) compared to OA mothers, which had higher rates of abnormalities during pregnancy (p < 0.001) and 38.7% of them suffered of ≥1 abortion in the past. The ethnical background of OA mothers was more often German (p < 0.033), while OA fathers had a lower socioeconomic status (p = 0.039).

Maternal factors like previous abortions, obesity and immunological predispositions like existence of allergies combined with increased daily dairy consumption influenced obviously the occurrence of OA.

Organoids have been developed in the last decade as a new research tool to simulate organ cell biology and disease. Compared to traditional 2D cell lines and animal models, experimental data based on esophageal organoids are more reliable. In recent years, esophageal organoids derived from multiple cell sources have been established, and relatively mature culture protocols have been developed. Esophageal inflammation and cancer are two directions of esophageal organoid modeling, and organoid models of esophageal adenocarcinoma, esophageal squamous cell carcinoma, and eosinophilic esophagitis have been established. The properties of esophageal organoids, which mimic the real esophagus, contribute to research in drug screening and regenerative medicine. The combination of organoids with other technologies, such as organ chips and xenografts, can complement the deficiencies of organoids and create entirely new research models that are more advantageous for cancer research. In this review, we will summarize the development of tumor and non-tumor esophageal organoids, the current application of esophageal organoids in disease modeling, regenerative medicine, and drug screening. We will also discuss the future prospects of esophageal organoids.

Radiologic evaluation of neonatal bowel obstruction is challenging owing to the overlapping clinical features and imaging appearances of the most common differential diagnoses. The key to providing an appropriate differential diagnosis comes from a combination of the patient's gestational age, clinical features, and imaging findings. While assessment of radiographs can confirm bowel obstruction and indicate whether it is likely proximal or distal, additional findings at upper or lower gastrointestinal contrast study together with use of US are important in providing an appropriate differential diagnosis. The authors provide an in-depth assessment of the appearances of the most common differential diagnoses of proximal and distal neonatal bowel obstruction at abdominal radiography and upper and lower gastrointestinal contrast studies. These are divided into imaging patterns and their associated differential diagnoses on the basis of abdominal radiographic findings. These findings include esophageal atresia variants including the "single bubble," "double bubble," and "triple bubble" and distal bowel obstruction involving the small and large bowel. Entities discussed include esophageal atresia, hypertrophic pyloric stenosis, pyloric atresia, duodenal atresia, duodenal web, malrotation with midgut volvulus, jejunal atresia, ileal atresia, meconium ileus, segmental volvulus, internal hernia, colonic atresia, Hirschsprung disease, and functional immaturity of the large bowel. The authors include the advantages of abdominal US in this algorithm, particularly for hypertrophic pyloric stenosis, duodenal web, malrotation with midgut volvulus, and segmental volvulus. ©RSNA, 2023 Online supplemental material is available for this article. Quiz questions for this article are available through the Online Learning Center.

Background: VACTERL association consists of Vertebral, Anorectal, Cardiac, Tracheo-Esophageal, Renal, and Limb defects. The diagnosis depends on the presence of at least three of these structural abnormalities. Methods: The clinical presentation and diagnostic prenatal imaging of VACTERL association are comprehensively reviewed. Results: The most common feature is a vertebral anomaly, found in 60-80% of cases. Tracheo-esophageal fistula is seen in 50-80% of cases and renal malformations in 30% of patients. Limb defects including thumb aplasia/hypoplasia, polydactyly, and radial agenesis/hypoplasia are present in 40-50% of cases. Anorectal defects, like imperforate anus/anal atresia, are challenging to detect prenatally. Conclusion: The diagnosis of VACTERL association mostly relies on imaging techniques such as ultrasound, computed tomography, and magnetic resonance. Differential diagnosis should exclude similar diseases such as CHARGE and Townes-Brocks syndromes and Fanconi anemia. New insights into genetic etiology have led to recommendations of chromosomal breakage investigation for optimal diagnosis and counseling.

The combination of duodenal atresia (DA) and esophageal atresia (EA) is very rare. With improvements in prenatal sonography and the use of fetal magnetic resonance imaging (MRI), these malformations can be diagnosed in a more accurate and timely manner; polyhydramnios remains the most common sign despite having a low specificity. The high rate of associated anomalies (in 85% of cases) can also impact neonatal management and increase the morbidity rate; thus, it is of paramount importance to look for every possible associated malformation, such as VACTERL and chromosomic anomalies. The surgical management of this combination of atresias is not well defined and changes according to the patient's clinical status, the type of EA, and the other associated malformations. Management ranges from a primary approach for one of the atresias with delayed correction of the other (56.8%) to a simultaneous repair of both atresias (33.8%) with or without gastrostomy, or total abstention (9.4%). We suggest that a simultaneous approach can be safely performed on patients in good physical condition, with a birth weight over 1500 g, and with no major respiratory distress; this method begins by closing the tracheoesophageal fistula to protect the lung and then repairing the DA. The mortality rate has decreased over the years, dropping from 71% before 1980 to 24% after 2001. In this review, we present the available evidence on these conditions, focusing mostly on the epidemiology, prenatal diagnosis, neonatal management strategies, and outcome, with the aim of determining how the different clinical features and surgical approaches may impact on morbidity and mortality.

To explore the experience of healthcare transition from paediatric to adult health care for adults born with oesophageal atresia and tracheo-oesophageal fistula (OA/TOF) and parents.

OA/TOF is a rare and chronic health condition that can require lifelong medical follow-up and management. There is evidence to suggest that transitioning from paediatric to adult health care can be problematic for people with rare and chronic conditions, including OA/TOF. The previous literature suggests that the experience of transitioning with a rare condition is more complex than transitioning with a common chronic condition.

The current study was a qualitative, cross-sectional, survey-based study.

Data were collected through an online survey. Parents of children born with OA/TOF (n = 23) and adults born with OA/TOF (n = 16) were recruited through a UK-based OA/TOF patient charity. Data from six open-ended questions were analysed using a hybrid approach combining elements of inductive and deductive thematic analyses. Throughout the research process, the SRQR were followed.

Five themes were constructed during the analysis, reflecting the experience of parents and adults transitioning from paediatric to adult health care: thrown into the unknown; a cultural shift; stepping back and stepping up; 'no transition as such'; and living with uncertainty.

The findings suggested that a formalised, managed healthcare transition is not commonly experienced by people born with OA/TOF and parents.

We recommend a formalised healthcare transition process in OA/TOF, including preparation for transition and having a named key worker to manage the multidisciplinary transition process. The results also highlighted the need for adults born with OA/TOF to have access to a specialist health service with knowledge and understanding of issues related to OA/TOF.

Oesophageal atresia (EA) is the most common congenital anomaly of the oesophagus. Despite improvement of survival observed over the previous two decades in developed countries, the mortality remains very high and the management greatly challenging in resource-poor settings such as Cameroon. We report our experience of management of EA in this environment, with a successful outcome.

We prospectively assessed patients diagnosed with EA and operated in January 2019, at the University Hospital Centre of Yaounde. Records were reviewed for demographics, history and physical examinations, radiological findings, surgical procedures and outcomes. The study has received approval from the Institutional Ethics Committees.

In total, six patients (three males and three females, sex ratio, 0.5; mean age at diagnosis, 3.6 days; range, 1-7 days) were assessed. A past history of polyhydramnios was found in one patient (16.7%). All patients were classified Waterston Group A at diagnosis, with Ladd-Swenson type III atresia. Early primary repair was performed in four patients (66.7%) and delayed primary repair in two patients (33.3%). Operative repair mainly involved resection of the fistula, suture of trachea and oesophagus end-to-end anastomosis, followed by interposition of vascularised pleural flap. Patients were followed up 24 months. With one late death, the survival rate was 83.3%.

Improvement has been achieved in the outcomes of neonatal surgery in Africa in the past two decades, but EA-related mortality remains relatively too high. Using simple techniques and available, reproducible equipment can improve survival in resource-poor settings.

Unexpected cardiac arrest in the perioperative period is a devastating complication. Owing to immaturity of organ systems, and presence of congenital malformations, morbidity and mortality are higher in neonates. There is abundant literature about early recognition and management of perioperative adverse events in children, but similar data and guidelines for surgical neonates is lacking. The current neonatal resuscitation guidelines cater to a newborn requiring resuscitation at the time of birth in the delivery room. The concerns in a newborn undergoing transition from intrauterine to extra uterine life is significantly different from a neonate undergoing surgery. This review highlights the causes and factors responsible for peri-arrest situations in neonates in the perioperative period, suggests preoperative surveillance for prevention of these conditions, and finally presents the resuscitation protocol of the surgical neonate. All these are comprehensively proposed as Neonatal Peri-operative Resuscitation (NePOR) protocol.

Esophageal atresia (EA) is a life-threatening congenital malformation of the esophagus. Despite considerable recent advances in perinatal resuscitation and neonatal care, EA remains an important cause of mortality and morbidity, especially in low-income countries. The aim of this study was to assess risk factors for adverse outcomes following surgical repair of EA at a single center in Tunisia. We performed a retrospective analysis using medical records of neonates with surgical management of EA at our institution from 1 January 2007 to 31 December 2021. In total, 88 neonates were included with a mortality rate of 25%. There were 29 girls and 59 boys. The diagnosis of EA was suspected prenatally in 19 patients. The most common associated anomalies were congenital heart diseases. Prematurity, low birth weight, outborn birth, age at admission >12 hours, congenital heart disease, postoperative sepsis, and anastomotic leak were risk factors for mortality following surgical repair of EA. Anastomotic tension was the only factor associated with short-term complications and the occurrence of short-term complications was predictive of mid-term complications. This study provides physicians and families with contemporary information regarding risk factors for adverse outcomes following surgical repair of EA. Thus, any effort to reduce these risk factors would be critical to improving patient outcomes and reducing cost. Future multi-institutional studies are needed to identify, investigate, and establish best practices and clinical care guidelines for neonates with EA.

Postoperative ventilatory strategies in patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) may have an impact on early postoperative complications. Our national Esophageal Atresia Registry was evaluated to define a possible relationship between the type and duration of respiratory support on postoperative complications and outcome.

Among the data registered by 31 centers between 2015 and 2021, patients with esophago-esophageal anastomosis (EEA)/tracheoesophageal fistula (TEF) were divided into two groups; invasive ventilatory support (IV) and noninvasive ventilatory support and/or oxygen support (NIV-OS). The demographic findings, gestational age, type of atresia, associated anomalies, and genetic malformations were evaluated. We compared the type of repair, gap length, chest tube insertion, follow-up times, tensioned anastomosis, postoperative complications, esophageal dilatations, respiratory problems requiring treatment after the operation, and mortality rates.

Among 650 registered patients, 502 patients with EEA/TEF repair included the study. Four hundred and seventy of patients require IV and 32 of them had NIV-OS treatment. The IV group had lower mean birth weights and higher incidence of respiratory problems when compared to NIV-OS group. Also, NIV-OS group had significantly higher incidence of associated anomalies than IV groups. The rates of postoperative complications and mortality were not different between the IV and NIV-OS groups.

We demonstrated that patients who required invasive ventilation had a higher incidence of low birth weight and respiratory morbidity. We found no relation between mode of postoperative ventilation and surgical complications. Randomized controlled trials and clinical guidelines are needed to define the best type of ventilation strategy in children with EA/TEF.

The objective was to determine the prevalence of oesophageal atresia (OA) and describe the characteristics of OA cases diagnosed before the first year of life, born between 2007 and 2019, and residents in the Valencian Region (VR), Spain. Live births (LB), stillbirths (SB), and termination of pregnancy for fetal anomaly (TOPFA) diagnosed with OA were selected from the Congenital Anomalies population-based Registry of VR (RPAC-CV). The prevalence of OA per 10,000 births with 95% confidence interval was calculated, and socio-demographic and clinical variables were analyzed. A total of 146 OA cases were identified. The overall prevalence was 2.4/10,000 births, and prevalence by type of pregnancy ending was 2.3 in LB and 0.03 in both SB and TOPFA. A mortality rate of 0.03/1000 LB was observed. A relationship was found between case mortality and birth weight (p-value < 0.05). OA was primarily diagnosed at birth (58.2%) and 71.2% of the cases were associated with another congenital anomaly, mainly congenital heart defects. Significant variations in the prevalence of OA in the VR were detected throughout the study period. In conclusion, a lower prevalence in SB and TOPFA was identified compared to EUROCAT data. As several studies have identified, an association between OA cases and birth weight was found.

Introduction: During embryonic development, the trachea emerges from an area of the foregut, which is often referred to as "anterior" or "common" foregut tube or simply foregut. To explain this process of differentiation, four competing models exist to date. The outgrowth and watershed models propose a foregut that remains constant in length. In the outgrowth model, the trachea buds off and elongates from the foregut, while in the watershed model, a mesenchymal wedge splits the growing foregut into the trachea and esophagus. In contrast, the septation model proposes a cranial splitting and thus a shortening of the "common" foregut tube into the trachea and esophagus by an emerging septum. Finally, the splitting and extension model describes an interaction of cranial splitting of the foregut and simultaneous caudal tracheal and esophageal growth. Methods: Here we examine the development of the undifferentiated foregut by micro computed tomography, which allows precise measurements. Results: Our results show that this area of the foregut transforms into the larynx, a process, which is independent from tracheal and esophageal development. Discussion: These observations are only consistent with the outgrowth model.

Esophageal biomechanical studies are being performed to understand structural changes resulting from stretches during repair of esophageal atresias as well as to obtain biomechanical values for tissue-engineered esophagus. The present study offers insights into ultrastructural changes after stretching of the ovine esophagus using uniaxial stretch tests. In vitro uniaxial stretching was performed on esophagi (n = 16) obtained from the abattoir within 4-6 h of 1-month-old lambs. Esophagi were divided into 4 groups (4 esophagi/group): control, Group1 (G1), Group2 (G2), Group3 (G3) stretched to 20%, 30% and 40% of their original length respectively. Force and lengthening were measured with 5 cycles performed on every specimen. Transmission electron microscopic (TEM) studies were performed on the 4 groups. During observational TEM study of the control group there were no significant differences in muscle cell structure or extracellular matrix. In all stretched groups varying degrees of alterations were identified. The degree of damage correlated linearly with the increasing level of stretch. Distance between the cells showed significant difference between the groups (control (μ = 0.41 μm, SD = 0.26), G1 (μ = 1.36 μm, SD = 1.21), G2 (μ = 2.8 μm, SD = 1.83), and G3 (μ = 3.01 μm, SD = 2.06). The diameter of the cells (control μ = 19.87 μm, SD = 3.81; G1 μ = 20.38 μm, SD = 4.45; G2 μ = 21.7 μm, SD = 6.58; G3 μ = 24.48 μm, SD = 6.69) and the distance between myofibrils (control μ = 0.23 μm, SD = 0.08; G1 μ = 0.27 μm, SD = 0.08; G2 μ = 0.4 μm, SD = 0.15; G3 μ = 0.61 μm, SD = 0.2) were significantly different as well ( p < 0.05 was considered to be significant). Esophageal stretching > 30% alters the regular intracellular and extracellular structure of the esophageal muscle and leads to disruption of intra- and extracellular bonds. These findings could provide valuable insights into alterations in the microscopic structure of the esophagus in esophageal atresias repaired under tension as well as the basis for mechanical characterization for tissue engineering of the esophagus.

An electronic literature search was performed using the keywords "tracheoesophageal fistula," "endoscopic," and "children" in the four major medical databases (Ovid, Embase, PubMed, and Web of Science) right from inception to September 2022. All English language articles describing the endoscopic interventional therapies of TEF in children were reviewed. Two independent researchers screened eligible articles at the title and abstract level. Full texts of potentially relevant articles were then screened again, and reference lists were screened manually to identify additional studies. Relevant data were extracted and analyzed. A synthesis of the relevant data was presented in descriptive form because of the heterogeneity of the included articles. The Chi-Squared test was used with a significance level of 5% (P < 0.05).

Among the 1,167 retrieved papers, a total of 46 studies describing 170 TEF patients with an age range of 0.3-175 months were included, including 11 cases of acquired tracheoesophageal fistula, 144 cases of recurrent tracheoesophageal fistula, and 15 cases of congenital tracheoesophageal fistula (H-type TEF). A total of 119 out of 170 fistulas were successfully blocked via endoscopic techniques with an overall success rate of 70.0%, while 48 fistulas failed to close by endoscopic interventions, following which the procedure was converted to open surgery. No obviously severe intraoperative/postoperative complications occurred during the follow-up period, but only a mild esophageal stricture was noticed in six patients and grade II tracheal stenosis in one patient. Two patients died from causes unrelated to endoscopic procedures, with a mortality rate of approximately 1.2%. A comparative assessment of different endoscopic interventional techniques for TEF that detected endotracheal stenting was performed in six patients and one fistula was successfully blocked (16.7%). De-epithelialization alone was performed in 65 patients and the fistula healed in 47 of them (72.3%), with the mean number of successful treatments required being 2.3 times. Chemical sealant injection was administered in 33 patients and success was achieved in 21 (63.6%). The average requirement for endoscopic procedures was 1.5 times. De-epithelialization, in combination with chemical sealant injection, was performed in 62 patients, achieving the highest success rate of 77.4% (48 patients). Other treatment methods were performed in four patients and successfully treatment outcomes were reported in two of them (50.0%). The mean number of successful treatments required was four times, and a treatment was converted to surgery in one patient (25.0%). An assessment of different TEF types showed that 9 out of 15 congenital TEFs, 7 out of 11 acquired TEFs, and 103 out of 144 recurrent TEFs were successfully occluded. A comparison of the success rate across multiple groups showed a significant difference with a score of P < 0.05, while there was no significant difference in the success rate of different TEF-type groups (P > 0.05).

Endoscopic intervention is currently a preferred treatment modality for children with TEF because of its less-invasive nature, less complications, and high success rate. Among all interventional techniques, de-epithelialization, in combination with chemical sealant, has a higher success rate than other techniques. However, due to the limited number of cases reported for implementing many kinds of techniques, an ideal endoscopic interventional technique has yet to be devised, often necessitating more treatment applications and close follow-up.

Esophageal atresia (EA) with or without trachea-esophageal fistula is relatively common congenital malformation with most patients living into adulthood. As a result, care of the adult patient with EA is becoming more common. Although surgical repair has changed EA from a fatal to a livable condition, the residual effects of the anomaly may lead to a lifetime of complications. These include effects related to the underlying deformity such as atonicity of the esophageal segment, fistula recurrence, and esophageal cancer to complications of the surgery including anastomotic stricture, gastroesophageal reflux, and coping with an organ transposition. This review discusses the occurrence and management of these conditions in adulthood and the role of an effective transition from pediatric to adult care to optimize adult care treatment.

The operative management of patients born with long-gap esophageal atresia (LGEA) remains a major challenge for most pediatric surgeons, due to the rarity and complex nature of this malformation. In LGEA, the distance between the proximal and distal esophageal end is too wide, making a primary anastomosis often impossible. Still, every effort should be made to preserve the native esophagus as no other conduit can replace its function in transporting food from the oral cavity to the stomach satisfactorily. In 1981, Puri et al. observed that in newborns with LGEA spontaneous growth and hypertrophy of the two segments occur at a rate faster than overall somatic growth in the absence of any form of mechanical stretching, traction or bouginage. They further noted that maximal natural growth arises in the first 8-12 weeks of life, stimulated by the swallowing reflex and reflux of gastric contents into the lower esophageal pouch. Since then, creation of an initial gastrostomy and continuous suction of the upper esophageal pouch followed by delayed primary anastomosis at approximately 3 months of age has been widely accepted as the preferred treatment option in most LGEA cases, generally providing good functional results. The current article offers a comprehensive update on the various aspects and challenges of this technique including initial preoperative management and subsequent gap assessment, while also discussing potential postoperative complications and long-term outcome.

Esophageal atresia is a major anomaly of varying severity. The complexity of surgical correction depends on the presence of a distal fistula.

This study aimed to determine the feasibility and accuracy of prenatal ultrasound detection of the distal fistula in fetuses diagnosed with esophageal atresia.

This was an observational study conducted at a single tertiary care center between 2019 and 2021. Included were pregnant patients carrying a fetus prenatally diagnosed with esophageal atresia that was confirmed postnatally during corrective surgery or at postmortem autopsy. During the scan, the performing investigator determined the presence or absence of a distal fistula by scanning the location of the lower esophagus during fetal breathing. Cases in which the lower esophagus was observed distending with amniotic fluid during breathing were deemed "fistula present," and the remaining cases "fistula absent." Test feasibility and performance indices, including sensitivity, specificity, and positive and negative predictive value were calculated. The offline clips and images were reviewed by 2 investigators for the assessment of interoperator agreement using Cohen's Kappa formula.

Included were 16 fetuses with esophageal atresia scanned between 2019 and 2021. All fetuses were successfully scanned with sufficient resolution of the area of interest during at least 3 cycles of breathing. It took a median of 8.5 minutes to determine the presence or absence of a distal fistula. The feasibility of the test was 100% (16/16). The test's sensitivity, specificity, and positive and negative predictive values were 80% (95% confidence interval, 55-100), 100% (95% confidence interval, 60-100), 100% (95% confidence interval, 65-100), and 75% (95% confidence interval, 45-100), respectively. The Cohen's Kappa for interoperator agreement was calculated to be 1, P<.001, corresponding to a "perfect" level of agreement.

Distal fistulas in esophageal atresia can be demonstrated prenatally by targeted scanning using appropriate technique. The method provided is feasible, reproducible, and has excellent performance indices. This novel technique and observations may improve the prenatal diagnosis and counseling of esophageal atresia.