Esophageal atresia (EA), with or without tracheoesophageal fistula (TEF), is a rare congenital anomaly. Long-gap esophageal atresia (LGEA) and failed primary repair of TEF often necessitate esophageal replacement in children. This study assesses the outcomes of staged isoperistaltic esophageal replacement using a gastric tube, particularly in resource-limited settings.

The aim of this study was to evaluate the outcomes of staged isoperistaltic esophageal replacement using a gastric tube in children with LGEA and failed primary repair of TEF, particularly in resource-limited settings.

The study was conducted tertiary care hospital, an observational design, spanning 10 years from January 2012 to January 2022.

This study, spanning 10 years (January 2012 to January 2022), focused on pure EA and LGEA with TEF. The three-stage approach included neonatal gastrostomy and esophagostomy, the creation of an isoperistaltic gastric tube at around 10 kg body weight, retrosternal tunneling, and subsequent closure of the cervical esophagostomy. Data on gender, weight, age, complications, gastric emptying, esophageal mucosal status, gastroesophageal reflux, and growth and nutritional status were analyzed.

The study primarily utilized descriptive statistics to analyze the data. This included reporting means with standard deviations for continuous variables such as weight and age at the time of the second surgery.

Twenty-seven cases were studied, with a male-to-female ratio of 3:1. At the second surgery, the mean weight was 9.52 ± 0.56 kg, and the mean age was 8.5 ± 1.25 months. Four cases had minor cervical anastomotic leaks, which were managed conservatively. No postoperative ventilator support was needed. Esophageal stenosis occurred in one case and was managed with endoscopic dilatation. No delayed gastric emptying or Barrett's esophagus was observed. Growth and nutritional assessments were normal. One mortality occurred due to postoperative bronchospasm.

This 10-year study shows that staged isoperistaltic esophageal replacement with a gastric tube is a feasible option for managing EA and LGEA with TEF in resource-limited settings. The technique had low complication rates and supported normal growth and nutrition during follow-up.

Survival rates in children born with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) have improved; however, morbidity associated with the disease remains high. This study aimed to assess the prevalence of gastroesophageal reflux disease (GERD), eosinophilic esophagitis (EoE), fungal esophagitis, esophageal strictures, and long-term outcomes in children with EA/TEF.

We conducted a retrospective chart review on patients with EA/TEF who were seen at Children's Wisconsin from January 2003 to January 2023. Patients born with EA/TEF were included if they underwent at least one endoscopy after 1 year of age. GERD was diagnosed based on abnormal findings on endoscopy, pH-metry, and/or history of fundoplication. EoE and fungal esophagitis were diagnosed based on abnormal endoscopy. Esophageal stricture diagnosis was based on findings on endoscopy and/or esophagram, and clinical symptoms necessitating esophageal dilation.

Eighty-five patients (64.7% males, mean age 7.5 years) were included, the majority had type C EA/TEF (90.6%). GERD was diagnosed in 61.1% (n = 52), 49.4% (n = 42) by macro and/or microscopic endoscopic findings, 22.3% (n = 19) by abnormal pH-metry, and 21.1% (n = 18) by the need for fundoplication for refractory reflux and/or esophageal stricture. Risk of GERD increased with lower gestational age (p = 0.0030), lower birth weight (p = 0.023), and long-gap EA (p = 0.034). In children diagnosed with GERD, only 13.4% of patients (n = 7/52) were able to be weaned off proton pump inhibitor (PPI) without disease recurrence. However, overall, at the completion of the study, 44.7% (n = 38) of patients were successfully weaned off PPI without evidence of GERD. EoE was diagnosed in 20% of the patients (n = 17). All patients diagnosed with EoE required escalation of therapy from PPI alone to swallowed corticosteroids in 52.9% (n = 9), dupilumab in 23.5% (n = 4), elemental formula in 17.6% (n = 3), and elemental formula and swallowed steroids in 5.8% (n = 1). Fungal esophagitis was diagnosed in 15.3% of patients (n = 13). An esophageal stricture requiring dilation was diagnosed in 77.6% (n = 66) of patients at a mean age of 28.5 months, with over 60% diagnosed by 24 months of age.

Children born with EA/TEF continue to be at high risk of developing GERD, EoE, fungal esophagitis, and esophageal stenosis. Diagnostic and therapeutic endoscopy remains a high-yield test to identify and treat these comorbidities.

Individuals with esophageal atresia (EA) have lifelong increased risk for mucosal and structural pathology of the esophagus. The use of surveillance endoscopy to detect clinically meaningful pathology has been underexplored in pediatric EA. We hypothesized that surveillance endoscopy in pediatric EA has high clinical yield, even in the absence of symptoms.

The medical records of all patients with EA who underwent at least 1 surveillance endoscopy between March 2004 and March 2023 at an international EA referral center were retrospectively reviewed. The primary outcomes were endoscopic identification of pathology leading to an escalation in medical, endoscopic, or surgical management. Logistic regression analysis examined predictors of actionable findings. Nelson-Aalen analysis estimated optimal endoscopic surveillance intervals.

Five hundred forty-six children with EA underwent 1,473 surveillance endoscopies spanning 3,687 person-years of follow-up time. A total of 770 endoscopies (52.2%) in 394 unique patients (72.2%) had actionable pathology. Esophagitis leading to escalation of therapy was the most frequently encountered finding (484 endoscopies, 32.9%), with most esophagitis attributed to acid reflux. Barrett's esophagus (intestinal metaplasia) was identified in 7 unique patients (1.3%) at a median age of 11.3 years. No dysplastic lesions were identified. Actionable findings leading to surgical intervention were found in 55 children (30 refractory reflux and 25 tracheoesophageal fistulas). Significant predictors of actionable pathology included increasing age, long gap atresia, and hiatal hernia. Symptoms were not predictive of actionable findings, except dysphagia, which was associated with stricture. Nelson-Aalen analysis predicted occurrence of an actionable finding every 5 years.

Surveillance endoscopy uncovers high rates of actionable pathology even in asymptomatic children with EA. Based on the findings of the current study, a pediatric EA surveillance endoscopy algorithm is proposed.

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.

A high prevalence of eosinophilic esophagitis (EoE) has been reported in children with repaired esophageal atresia (EA). Topical steroids proved to be an effective and safe therapy in EoE, although not approved in pediatrics. We report the results of the first clinical trial of oral viscous budesonide (OVB) performed in children with EoE after repaired esophageal atresia (EoE-EA).

This open-label, single-arm, phase 2 clinical trial with randomized pharmacokinetic sampling, was conducted at the Bambino Gesù Children's Hospital between September 2019 and June 2021. EoE-EA patients received an age-banded dose of OVB twice daily for 12 weeks and were endoscopically evaluated. The primary endpoint was the rate of patients achieving histological remission. Secondary endpoints included clinical and endoscopic benefit after treatment, and safety assessments.

Eight consecutive EA-EoE patients were enrolled (median age 9.1 years, interquartile range 5.5). Of these, 5 received 0.8 mg and 3 received 1.0 mg twice daily of OVB. Histological remission was obtained in all but 1 patient (87.5%). The clinical score showed significant improvement at the end of treatment in all patients. No endoscopic features of EoE were found after treatment. No treatment-emergent adverse event occurred.

OVB is an effective, safe, and well-tolerated formulation of budesonide for use in pediatric patients with EoE-EA.

To investigate potential early risk factors for anastomotic stricture formation and assess the predictive role of post-operative esophagrams.

A retrospective study of patients with esophageal atresia with distal fistula (EA/TEF) operated between 2011 and 2020. Fourteen predictive factors were tested for stricture development. Esophagrams were used to calculate early (SI1) and late (SI2) stricture index (SI = anastomosis diameter/upper pouch diameter).

Of 185 patients operated for EA/TEF in the 10-year period, 169 patients met the inclusion criteria. Primary anastomosis was performed in 130 patients and delayed anastomosis in 39 patients. Stricture formed in 55 patients (33%) within 1 year from anastomosis. Four risk factors showed strong association with stricture formation in unadjusted models: long gap (p = 0.007), delayed anastomosis (p = 0.042), SI1 (p = 0.013) and SI2 (p < 0.001). A multivariate analysis showed SI1 as significantly predictive of stricture formation (p = 0.035). Cut-off values using a receiver operating characteristic (ROC) curve were 0.275 for SI1 and 0.390 for SI2. The area under the ROC curve demonstrated increasing predictiveness from SI1 (AUC 0.641) to SI2 (AUC 0.877).

This study identified an association between long gap and delayed anastomosis with stricture formation. Early and late stricture indices were predictive of stricture formation.

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.

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 importance of multidisciplinary long-term follow-up for adults born with esophageal atresia (EA) is increasingly recognized. Hence, a valid, condition-specific instrument to measure health-related quality of life (HRQoL) becomes imperative. This study aimed to develop and validate such an instrument for adults with EA. The Specific Quality of life in Esophageal atresia Adults (SQEA) questionnaire was developed through focus group-based item generation, pilot testing, item reduction and a multicenter, nationwide field test to evaluate the feasibility, reliability (internal and retest) and validity (structural, construct, criterion and convergent), in compliance with the consensus-based standards for the selection of health measurement instruments guidelines. After pilot testing (n = 42), items were reduced from 144 to 36 questions. After field testing (n = 447), three items were discarded based on item-response theory results. The final SQEA questionnaire (33 items) forms a unidimensional scale generating an unweighted total score. Feasibility, internal reliability (Cronbach's alpha 0.94) and test-retest agreement (intra-class coefficient 0.92) were good. Construct validity was discriminative for esophageal replacement (P < 0.001), dysphagia (P < 0.001) and airway obstruction (P = 0.029). Criterion validity showed a good correlation with dysphagia (area under the receiver operating characteristic 0.736). SQEA scores correlated well with other validated disease-specific HRQoL scales such as the GIQLI and SGRQ, but poorly with the more generic RAND-36. Overall, this first condition-specific instrument for EA adults showed satisfactory feasibility, reliability and validity. Additionally, it shows discriminative ability to detect disease burden. Therefore, the SQEA questionnaire is both a valid instrument to assess the HRQoL in EA adults and an interesting signaling tool, enabling clinicians to recognize more severely affected patients.

The aim of this study was to evaluate the swallowing problems by fiberoptic endoscopic evaluation of swallowing (FEES) study in both short- and long-gap patients after esophageal atresia (EA) repair.

Hospital records of patients who had undergone surgery for EA were reviewed retrospectively. Patients were divided into two groups as short-gap (SG) group (n:16) and long-gap (LG) group (n:10) to compare the swallowing problems. FEES study was performed, and the results were discussed in detail.

There were twenty-six (16 M/10 F) patients with a mean age at evaluation was 7.52 ∓ 3.68 years. Mean follow-up period was 75.35 ∓ 44.48 months. In FEES study, pharyngeal phase abnormalities were detected in 10 patients (38.4%). Pharyngeal phase abnormalities were detected significantly higher in LG group (p:0.015). Laryngeal penetration/aspiration was seen in four patients on FEES study (15.3%). All of them was in LG group (40%). Laryngeal penetration/aspiration was seen significantly higher in LG group (p:0.014).

This is the first study to conduct FEES study in children after esophageal atresia repair to evaluate their swallowing conditions. Even though our sample is small, swallowing problems are more common than expected in the cases of LG when compared to SG.

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.

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.

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.

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.

Oesophageal atresia is a rare congenital malformation occurring in 1 : 3000/1 : 4000 neonates. Surgical correction is always required. Perioperative management concepts depend on the type of malformation. Postoperative results are closely related to postsurgical complications. Interdisciplinary management should extend from prenatal diagnosis, birth and perinatal care to neonatal intensive care and paediatric surgical therapy with specialised pediatric anaesthesia. Other areas that should be available are logopedia, paediatric gastroenterology and paediatric pulmonology. Long-term care should include systematic aftercare and transition programs to adult medicine.

Adults with esophageal atresia (EA) require a multidisciplinary follow-up approach, taking into account gastroesophageal problems, respiratory problems and psychosocial wellbeing. Too little is known about the full scope of these individuals' healthcare needs. We aimed to map all medical and psychosocial needs of adults with EA and their family members, and to formulate healthcare recommendations for daily practice.

A qualitative study was performed, using data from recorded semi-structured interviews with two focus groups, one consisting of adult patients with EA (n = 15) and one of their family members (n = 13). After verbatim transcription and computerized thematic analysis, results were organized according to the International Classification of Functioning, Disability and Health. Ethical approval had been obtained.

Healthcare needs were described through 74 codes, classified into 20 themes. Most important findings for patients included the impact of gastrointestinal and pulmonary problems on daily life, long-term emotional distress of patients and parents and the need of a standardized multidisciplinary follow-up program during both child- and adulthood.

The focus groups revealed numerous physical and mental health problems, as well as social difficulties, that require attention from different healthcare providers. We have formulated several healthcare recommendations that physicians may use in long-term follow-up.

Esophageal atresia (EA) is a life-threatening congenital condition, affecting one in 2600 newborns. Morbidity remains high, with many patients experiencing complications, including anastomotic leak/stricture, and gastro-esophageal reflux disease (GERD). Increased understanding of esophageal motility patterns may help explain the etiology of these complications.

We aimed to review knowledge regarding esophageal motility and related complications in children with EA, evaluate patients' symptomatology and relate this to esophageal motility.

We performed a systematic review (PROSPERO: CRD42018092277), according to the PRISMA protocol. Two investigators independently conducted search strategies (OvidMEDLINE, PubMed, Cochrane Review, BMJ BestPractice), identifying complications in patients following EA repair. Rates of esophageal dysmotility, GERD, dysphagia, anastomotic leak, anastomotic stricture, recurrent fistula formation, and esophagitis were sought.

A total of 65 publications met selection criteria (n = 4882). Rates of morbidity were high: esophageal dysmotility (78%), GERD (43%), dysphagia (44%), anastomotic leak (19%), anastomotic stricture (26%), recurrent fistula formation (7%), and esophagitis (47%). No correlation appeared to exist with severity of symptoms.

This systematic review identified high rates of complications in children with EA, with esophageal dysmotility present in the majority of patients. Increasing survival, with resultant longer timeframes to develop morbidities, makes standardized follow-up regimens crucial.

Prognosis study.

Level 3.

We investigated changes in anastomotic stricture indexes (SIs) and stricture diameter (SD) between before and 6 months after the first dilatation in children with anastomotic stricture after esophageal atresia (EA) repair and identified predictors of medium-term dilatation success (success for at least 3 months). We retrospectively reviewed the records and measurement indexes of patients who underwent post-EA repair endoscopic balloon dilatation between November 2017 and August 2019 in our hospital. We identified diagnostic and performance indicators that predicted medium-term dilatation success by univariate and multivariate analyses and receiver operator characteristic (ROC) curve analysis. Sixty patients (34 boys and 26 girls) showed post-EA repair anastomotic stricture. Paired sample t-tests showed that SD (P < 0.001), upper pouch SI (U-SI, P < 0.001), lower pouch SI (L-SI, P < 0.001), upper pouch esophageal anastomotic SI (U-EASI, P < 0.001) and lower pouch EASI (L-EASI, P < 0.001) were significantly better at 6 months after than before the first dilatation. Logistic regression analysis showed that dilatation number (P = 0.002) and U-SI at 6 months after the first dilatation (P = 0.019) significantly predicted medium-term dilatation success. ROC curve analysis revealed that combining U-SI (cut-off value = 55.6%) and dilatation number (cut-off value = 10) had good accuracy in predicting medium-term dilatation success 6 months after the first dilatation (area under the curve-ROC: 0.95). In conclusion, endoscopic balloon dilatation significantly improved SD and SIs in children with post-EA repair anastomotic stricture. Dilatation number and U-SI at 6 months after the first dilatation were useful in predicting medium-term dilatation success and could represent a supplementary method to improve judgment regarding whether further dilatation is needed 6 months after the first dilatation.

Esophageal biomechanical studies are important to understand structural changes resulting from stretches during repair of esophageal atresias as well as to obtain values to compare with the biomechanics of tissue-engineered esophagus in the future. This study aimed to investigate light microscopic changes after uniaxial stretching of the ovine esophagus.

In vitro uniaxial stretching was performed on esophagi (n = 20) of 1-month-old lambs within 4-6 h post-mortem. Esophagi were divided into 5 groups: control and stretched (1.1, 1.2, 1.3 and 1.4). Force and lengthening were measured with 5 cycles performed on every specimen using a PBS organ bath at 37 °C. Histological studies were performed on the 5 groups.

Low forces of ~ 2 N (N) were sufficient for a 1.2-1.25 stretch in the 1st cycle, whereas a three times higher force (~ 6 N) was needed for a stretch of 1.3. In the 2nd to 5th cycle, the tissue weakened and a force of ~ 3 N was sufficient for a stretch of 1.3. Histologically, in the 1.3-1.4 stretch groups, rupture of muscle fibers and capillaries were observed, respectively. Changes in mucosa and collagen fibers could not be observed.

These results offer norm values from the native esophagus to compare with the biomechanics of future tissue-engineered esophagus. Esophageal stretching > 1.3 leads to tears in muscle fibers and to rupture of capillaries. These findings can explain the decrease in microcirculation and scarring in mobilized tissue and possibly offer clues to impaired motility in esophagus atresias repaired under excessive tension.

Oesophageal atresia lacks sufficiently documented treatment approach, as guidelines are based rather on the opinion of experts than on systematic data. We aimed to answer the question if treatment of patients without major cardiovascular anomalies could be justified at a peripheral paediatric surgical institution, by evaluating the outcome of surgical correction.

Thirty-three neonates underwent surgery for correction of oesophageal atresia during a period of 20 years. They were categorised into two time-period groups, to follow-up the evolution of surgical intervention and complications through time. Evaluation of post-operative outcome and morbidity was performed. The results were related to those of our recent cross-sectional study on families having experienced oesophageal atresia performed years after repair, regarding the long-term quality of life.

A shift from staged to primary repair occurred throughout time in the patients with a marginal long gap between proximal and distal oesophagus (P = 0.008). Anastomotic stenosis was the major short-term complication encountered, treated with post-operative dilation sessions. Dysphagia and reflux were the most common long-term complications.

Oesophageal atresia without severe cardiovascular abnormalities could be treated at a peripheral paediatric surgical department with satisfactory outcomes. However, qualified paediatric surgeons, anaesthesiologists and neonatologists and the availability of neonatal intensive care unit should be definitively required.

Esophageal atresia with/without tracheoesophageal fistula (EA/TEF) is a congenital digestive tract anomaly that represents a major therapeutic challenge. Postoperative digestive morbidities such as gastroesophageal reflux disease (GERD) and esophageal stricture are common. The aim of this study was to identify the incidence of and potential risk factors for digestive morbidities after EA/TEF repair. We retrospectively reviewed all EA/TEF patients who underwent repair at a single institution between January 1999 and December 2018, excluding patients who died prior to discharge. Patient demographics, perioperative management, and postoperative GERD and esophageal stricture rates were collected. We performed univariate and multivariate analyses to examine risk factors associated with postoperative GERD and esophageal stricture. The study enrolled 58 infants (58.6% male, 17.2% with type A EA/TEF, 62.1% with associated anomalies). Postoperative GERD occurred in 67.2% of patients and was the most common digestive morbidity. Esophageal stricture occurred in 37.9% of patients after EA/TEF repair. Multivariate analysis showed that long-gap EA/TEF and postoperative GERD were independent risk factors for esophageal stricture after repair surgery.Conclusion: The incidence of postoperative GERD and esophageal stricture was 67.2% and 37.9%, respectively. The risk factors for postoperative esophageal stricture were long-gap EA/TEF and postoperative GERD. What is Known: • EA/TEF is a congenital digestive tract anomaly with a high postoperative survival rate but can be complicated by many long-term morbidities. What is New: • Long-gap EA/TEF and postoperative GERD are risk factors of anastomotic stricture after repair. • Surgeons and pediatricians should be highly experienced in managing anastomotic tension and the GERD.

Background: This study aims to identify the risk factors and reasons for treatment abandonment for patients with esophageal atresia (EA) in a tertiary care hospital in China. Methods: A retrospective study was conducted on 360 patients with EA admitted to Beijing Children's Hospital between January 1, 2007 and June 1, 2020. Medical records for treatment abandonment and non-treatment abandonment patients were compared. Univariate and multivariate logistic regression analyses were conducted to identify potential risk factors for treatment abandonment. Results: After the diagnosis of EA, parents of 107 patients refused surgical repair and discharged against medical advice, and 253 patients underwent surgical repair. Among these 253 patients, parents of 59 patients abandoned treatment after surgery; 52 patients were discharged in an unstable condition, and parents of seven patients abandoned resuscitation leading to death in the hospital. By comparing clinical characteristics between treatment abandonment before surgery (n = 107) and non-treatment abandonment (n = 253) groups, we found that mother's parity >1, unplanned admission to intensive care unit before surgery, associated anomalies, and Gross type A/B were significant independent risk factors for treatment abandonment before surgery. Furthermore, birth weight <2,545 g, being discharged from neonatal center/intensive care unit and other departments, unplanned admission to intensive care unit after surgery, operative time >133 min, admission before 2016, pneumothorax, and anastomotic leakage were significant independent risk factors for treatment abandonment after surgery. The reasons for treatment abandonment included financial difficulties, multiple malformations with poor prognosis, belief of incurability and concerns about the prognosis of the diseases, postoperative complications, and extensive length of intensive care unit stay. Conclusions: Treatment abandonment of children with EA/TEF is still a common and serious problem in China. This study showed that EA/TEF patients in critical conditions, with associated anomalies, Gross type A/B, and who had occurrence of complications had high-risk for treatment abandonment.

 Improvements in care of patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) have shifted the focus from mortality to morbidity and quality-of-life. Long-term follow-up is essential, but evidence is limited and standardized protocols are scarce. Nineteen representatives of the European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) from nine European countries conducted a consensus conference on the surgical management of EA/TEF.

 The conference was prepared by item generation (including items of surgical relevance from the European Society for Pediatric Gastroenterology Hepatology and Nutrition (ESPGHAN)-The North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) guidelines on follow-up after EA repair), item prioritization, formulation of a final list containing the domains Follow-up and Framework, and literature review. Anonymous voting was conducted via an internet-based system. Consensus was defined as ≥75% of those voting with scores of 6 to 9.

 Twenty-five items were generated in the domain Follow-up of which 17 (68%) matched with corresponding ESPGHAN-NASPGHAN statements. Complete consensus (100%) was achieved on seven items (28%), such as the necessity of an interdisciplinary follow-up program. Consensus ≥75% was achieved on 18 items (72%), such as potential indications for fundoplication. There was an 82% concordance with the ESPGHAN-NASPGHAN recommendations. Four items were generated in the domain Framework, and complete consensus was achieved on all these items.

 Participants of the first ERNICA conference reached significant consensus on the follow-up of patients with EA/TEF who undergo primary anastomosis. Fundamental statements regarding centralization, multidisciplinary approach, and involvement of patient organizations were formulated. These consensus statements will provide the cornerstone for uniform treatment protocols and resultant optimized patient care.

To evaluate the feasibility and efficacy of endoscopic stricture index (SI_EN) to define anastomotic strictures (ASs) and to predict the need of dilatations.

A retrospective longitudinal study was conducted on patients who underwent esophageal atresia repair from 1998-2020 (ethical committee approval CHPED-05-20-AS). SI_EN was calculated on the first endoscopy performed as follows: (D - d)/D, where D is the maximum diameter of lumen of the upper esophagus close to the AS and d is the diameter of lumen of the stricture. Nonparametric variables were examined using Wilcoxon-Mann-Whitney test, and continuous variables were analyzed using Spearman's test and regression analysis. A P value <0.05 was considered statistically significant. The sensitivity, specificity, and positive and negative predictive values of SI_EN were also calculated, and a receiver operating characteristic curve was designed.

A total of 46 patients were included in the study. A statistically significant correlation was found between SI_EN and number of dilations (Spearman's correlation rate, 0.7; P < 0.0005). A SI_EN threshold value ≥0.6 showed sensitivity of 100%, specificity of 80%, positive predictive value of 54%, negative predictive value of 100%, and the area under the curve of 84%.

SI_EN seems to be a good AS definer and prognostic tool; our study suggests that an AS could be defined by a SI_EN ≥0.6.

The role of intralesional steroid injection (ISI) in the treatment of anastomotic stricture in patients with esophageal atresia remains unclear. The aim of this study was to evaluate the efficacy and safety of ISI.

A total of 158 patients with esophageal atresia with at least 1 ISI for the treatment of esophageal anastomotic stricture between 2010 and 2017 were identified. The change in stricture diameter (ΔD) was compared between procedures with dilation alone (ISI-) and dilation with steroid injection (ISI+).

A total of 1055 balloon dilations were performed (452 ISI+). The median ΔD was significantly greater in the ISI+ group: 1 mm (interquartile range [IQR] 0, 3) versus 0 mm (IQR -1, 1.5) (P < 0.0001). The ISI+ group had greater percentage of improved diameter (P < 0.0001) and lesser percentages of unchanged and decreased diameters at subsequent endoscopy (P = 0.0009, P = 0.003). Multivariable logistic regression confirmed the significance of ISI on increasing the likelihood of improved stricture diameter with an adjusted odds ratio of 3.24 (95% confidence interval: 2.15-4.88) (P < 0.001). The ΔD for the first 3 ISI+ procedures was greater than the ΔD for subsequent ISI+ procedures: 1 mm (IQR 0, 3) versus 0.5 mm (IQR-1.25, 2) (P = 0.001). There was no difference in perforation incidence between ISI+ and ISI- groups (P = 0.82).

ISI with dilation was well tolerated and improved anastomotic stricture diameter more than dilation alone. The benefit of ISI over dilation alone was limited to the first 3 ISI procedures.

Poor growth is an under-recognised yet significant long-term sequelae of oesophageal atresia (OA) repair. Few studies have specifically explored the reasons for growth impairment in this complex cohort. The association between poor growth with younger age and fundoplication appears to have the strongest supportive evidence, highlighting the need for early involvement of a dietitian and speech pathologist, and consideration of optimal medical reflux management prior to referring for anti-reflux surgery. However, it remains difficult to reach conclusions regarding other factors which may negatively influence growth, due to conflicting findings, inconsistent definitions and lack of validated tool utilisation. While swallowing and feeding difficulties are particularly frequent in younger children, their relationship with growth remains unclear. It is possible that these morbidities impact on the diet of children with OA, but detailed analysis of dietary composition and quality, and its relationship with these complications and growth, has not yet been conducted. Another potential area of research in OA is the role of the microbiota in growth and nutrition. While the microbiota has been linked to growth impairment in other paediatric conditions, it is yet to be investigated in OA. Further research is needed to identify the most important contributory factors to poor growth, the role of the intestinal microbiota, and effective interventions to maximise growth and nutritional outcomes in this cohort.

To evaluate clinical, endoscopic, and pH-impedance measures in a cohort of children with esophageal atresia and concomitant eosinophilic esophagitis (EoE) and compared it with disease-matched controls, to identify predictive factors for the development of EoE and esophageal stricture.

We reviewed 63 patients with esophageal atresia assessed for refractory upper gastrointestinal symptoms between January 2015 and September 2017 at 2 tertiary referral centers. All patients underwent upper gastrointestinal endoscopy and pH-impedance monitoring. Based on esophageal histology, patients were classified as (1) esophageal atresia without evidence of esophagitis; (2) esophageal atresia with evidence of esophagitis (including esophageal eosinophilia not meeting the criteria for EoE); (3) esophageal atresia with concomitant EoE. Age and sex matched patients with gastroesophageal reflux disease were used as disease controls.

The presence of atopy and peripheral eosinophilia at baseline were significantly associated with EoE (P < .05). Although there was a tendency toward an increased number of strictures in patients with esophageal atresia-EoE, this did not reach statistical significance (P = .06). Higher esophageal acid exposure time and lower baseline impedance values were significantly associated with eosinophilic infiltration (P < .05 and P < .01, respectively). Using logistic regression analysis, the presence of mucosal eosinophilia was the most predictive factor for stricture formation (P < .05).

A history of atopy and the presence of peripheral eosinophilia in patients with esophageal atresia are predictive factors for the development of EoE, which in turn is a predictive factor for stricture occurrence. Higher esophageal acid exposure time and lower baseline impedance are associated with esophageal eosinophilic infiltration, suggesting their value in selecting which patients with esophageal atresia should undergo endoscopic examination.

Treatment of the neonate with long gap esophageal atresia (LGEA) is one of the most challenging scenarios facing pediatric surgeons today. Contributing to this challenge is the variability in case definition, multiple approaches to management, and heterogeneity of the reported outcomes. This necessitates a clear summary of existing evidence and delineation of treatment controversies.

The American Pediatric Surgical Association Outcomes and Evidence Based Practice Committee drafted four consensus-based questions regarding LGEA. These questions concerned the definition and determination of LGEA, the optimal method of surgical management, expected long-term outcomes, and novel therapeutic techniques. A comprehensive search strategy was crafted and the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were utilized to identify, review and report salient articles.

More than 3000 publications were reviewed, with 178 influencing final recommendations. In total, 18 recommendations are provided, primarily based on level 4-5 evidence. These recommendations provide detailed descriptions of the definition of LGEA, treatment techniques, outcomes and future directions of research.

Evidence supporting best practices for LGEA is currently low quality. This review provides best recommendations based on a critical evaluation of the available literature. Based on the lack of strong evidence, prospective and comparative research is clearly needed.

Treatment study, prognosis study and study of diagnostic test.

Level II-V.

This study is aimed to evaluate the effectiveness of osteopathic manipulative treatment (OMT) in dysphagia symptoms and aspiration risk. This is a clinical case report on a 40-day-old infant with a diagnosis of oesophageal atresia and tracheo-oesophageal fistula. The patient received one OMT every 15 days for 7 times. The clinical outcome was the clinical changes in dysphagia symptoms assessed by the Paediatric Eating Assessment Tool-10 (PEDI-EAT-10). At the first osteopathic evaluation (T0), the total score of the PEDI-EAT-10 was 7, at the fourth osteopathic evaluation (T1), the total score was 3, and at the seventh osteopathic evaluation (T2), the total score was 1. OMT was effective and safe in reducing dysphagia symptoms and aspiration risk.

Esophageal atresia is rare, but improved surgical and intensive care techniques have increased rates of survival in children, so there are now many adults with this disorder. Many patients with esophageal atresia develop gastroesophageal reflux (GER), raising concerns about increased risk of Barrett's esophagus (BE; prevalence of 1.3%-1.6% in general population) and esophageal carcinoma. We assessed the prevalence of BE and esophageal carcinoma in this population.

We performed a prospective study of 289 patients with esophageal atresia at the Department of Gastroenterology and Hepatology at Erasmus MC University Medical Center in The Netherlands, from May 2012 through March 2017. A total of 151 (median age, 25.4 y; age range, 16.8-68.6 y) underwent upper endoscopies as part of a surveillance program for (pre)malignant esophageal lesions. Biopsies were collected and analyzed by histology. We collected data on patients' use of medications, tobacco, and alcohol; gastrointestinal symptoms; ability to swallow; complaints of GER; and type of atresia and surgeries. Prevalence of esophageal squamous cell carcinoma (ESCC) was determined using data from The Netherlands Cancer Registry. The number of persons alive on January 1, 2016, in the esophageal atresia cohort and in the general Dutch population were used to calculate the 10-year prevalence of ESCC per 100,000 persons in both populations.

Forty-seven percent of patients with esophageal atresia had a history of GER and 20.5% had undergone fundoplication surgery. Endoscopy revealed normal esophagus in 68.2% of patients, esophagitis in 7.3%, and columnar-lined esophagus in 24.5%. Histology revealed normal mucosa in 50.3% of patients, esophagitis in 23.2%, gastric metaplasia in 17.2%, and BE in 6.6% (at a median age of 31.6 years). A history of fundoplication surgery was associated with BE (P = .03). Three ESCCs developed, in 2 men, at ages 42, 44, and 60 years. This corresponded to a prevalence of 0.7% in patients with esophageal atresia-a value 108-fold higher than in the same age group in the general population.

The prevalence of BE is 4-fold higher in young adults with esophageal atresia, and the prevalence of ESCC is 108-fold higher than in the general population. This finding could have important implications for transition of young adults from pediatric care to adult gastroenterology departments to receive life-long endoscopic follow-up evaluation to facilitate early diagnosis of relevant lesions.

Cases of esophageal carcinoma have been documented in survivors of esophageal atresia (EA). Children with EA undergo considerable amounts of diagnostic imaging and consequent radiation exposure potentially increasing their lifetime cancer mortality risk. This study evaluates the radiological procedures performed on patients with EA and estimates their cumulative radiation exposure and attributable lifetime cancer mortality risk.

Medical records of patients with EA managed at a tertiary care center were reviewed for demographics, EA subtype, and number and type of radiological investigations. Existing normative data were used to estimate the cumulative radiation exposure and lifetime cancer risk per patient.

The present study included 53 patients with a mean follow-up of 5.7 years. The overall median and maximum estimated effective radiation dose in the neonatal period was 5521.4 μSv/patient and 66638.6 μSv/patient, respectively. This correlates to a median and maximum estimated cumulative lifetime cancer mortality risk of 1:1530 and 1:130, respectively. Hence, radiation exposure in the neonatal period increased the cumulative cancer mortality risk a median of 130-fold and a maximum of 1575-fold in EA survivors.

Children with EA are exposed to significant amounts of radiation and an increased estimated cumulative cancer mortality risk. Efforts should be made to eliminate superfluous imaging.

We assessed the quality of life (QOL) of postoperative esophageal atresia (EA) with tracheoesophageal fistula (TEF) cases, comparing open with thoracoscopic repair.

A retrospective review of consecutive EA/TEF repairs (2001-2014) was performed, excluding cases with birth weight less than 2000 g and severe cardiac/chromosomal anomalies. Of 37 cases, 13 had thoracoscopic repair (TR) and 24 had open repair (OR) according to the operating surgeon's preference. QOL was determined regularly by scoring responses to a standard questionnaire about oral intake, vomiting, bougienage, coughing, growth retardation, learning ability, and thoracic deformity. Lower scores reflected poorer outcome. QOL after TR and OR was compared 1 year postoperatively (POQ) and after starting school (ScQ).

Subject demographics were similar. Apart from two anastomotic leaks that resolved spontaneously after TR, there were no intraoperative complications or recurrence of TEF. Laparoscopic fundoplication was required for gastroesophageal reflux in four cases (OR 1; TR 3) (p = ns). QOL scores went from 6.5 → 11.5 in OR and 4.6 → 11.3 in TR, respectively. Final ScQ scores were similar, but POQ was significantly higher after OR (p < 0.05).

Initial QOL scores were significantly lower after TR, but by school age QOL scores were similar.

Recent developments in paediatric gastrointestinal surgery have focused on minimally invasive surgery, the accumulation of high-quality clinical evidence, and scientific research. The benefits of minimally invasive surgery for common disorders like appendicitis and hypertrophic pyloric stenosis are all supported by good clinical evidence. Although minimally invasive surgery has been extended to neonatal surgery, it is difficult to establish its role for neonatal disorders such as oesophageal atresia and biliary atresia through clinical trials because of the rarity of these disorders. Advances in treatments for biliary atresia and necrotising enterocolitis have been achieved through specialisation, multidisciplinary management, and multicentre collaboration in research; similarly robust clinical evidence for other rare gastrointestinal disorders is needed. As more neonates with gastrointestinal diseases survive into adulthood, their long-term sequelae will also need evidence-based multidisciplinary care. Identifying cures for long-term problems of a complex developmental anomaly such as Hirschsprung's disease will rely on unravelling its pathogenesis through genetics and the development of stem-cell therapy.

Esophageal atresia (EA) and tracheoesophageal fistula (TEF) represent major therapeutic challenges, frequently associated with serious morbidities following surgical repair. The aim of this longitudinal study was to assess temporal changes in morbidity and mortality of patients with EA/TEF treated in a tertiary-level center, focusing on postoperative complications and their impact on long-term gastroesophageal function.

One hundred nine consecutive patients with EA/TEF born between 1975 and 2011 were followed for a median of 9.6 years (range, 3-27 years). Comparative statistics were used to evaluate temporal changes between an early (1975-1989) and late (1990-2011) study period.

Gross types of EA were A (n = 6), B (n = 5), C (n = 89), D (n = 7), and E (n = 2). Seventy (64.2%) patients had coexisting anomalies, 13 (11.9%) of whom died before EA correction was completed. In the remaining 96 infants, surgical repair was primary (n = 66) or delayed (n = 25) anastomosis, closure of TEF in EA type E (n = 2), and esophageal replacement with colon interposition (n=2) or gastric transposition (n=1). Long-gap EA was diagnosed in 23 (24.0%) cases. Postoperative mortality was 4/96 (4.2%). Overall survival increased significantly between the two study periods (42/55 vs. 50/54; P = 0.03). Sixty-nine (71.9%) patients presented postoperatively with anastomotic strictures requiring a median of 3 (range, 1-15) dilatations. Revisional surgery was required for anastomotic leakage (n = 5), recurrent TEF with (n = 1) or without (n=9) anastomotic stricture, undetected proximal TEF (n = 4), and refractory anastomotic strictures with (n = 1) or without (n = 2) fistula. Normal dietary intake was achieved in 89 (96.7%) patients, while 3 (3.3%) remained dependent on gastrostomy feedings. Manometry showed esophageal dysmotility in 78 (84.8%) infants at 1 year of age, increasing to 100% at 10-year follow-up. Fifty-six (60.9%) patients suffered from dysphagia with need for endoscopic foreign body removal in 12 (13.0%) cases. Anti-reflux medication was required in 43 (46.7%) children and 30 (32.6%) underwent fundoplication. The rate of gastroesophageal reflux increased significantly between the two study periods (29/42 vs. 44/50; P = 0.04). Twenty-two (23.9%) cases of endoscopic esophagitis and one Barrett's esophagus were identified.

Postoperative complications after EA/TEF repair are common and should be expertly managed to reduce the risk of long-term morbidity. Regular multidisciplinary surveillance with transitional care into adulthood is recommended in all patients with EA/TEF.

Improved surgical techniques, as well as preoperative and postoperative care, have dramatically changed survival of children with esophageal atresia (EA) over the last decades. Nowadays, we are increasingly seeing EA patients experiencing significant short- and long-term gastrointestinal morbidities. Anastomotic stricture (AS) is the most common complication following operative repair. An esophageal stricture is defined as an intrinsic luminal narrowing in a clinically symptomatic patient, but no symptoms are sensitive or specific enough to diagnose an AS. This review aims to provide a comprehensive view of AS in EA children. Given the lack of evidence-based data, we critically analyzed significant studies on children and adults, including comments on benign strictures with other etiologies. Despite there is no consensus about the goal of the luminal diameter based on the patient's age, esophageal contrast study, and/or endoscopy are recommended to assess the degree of the narrowing. A high variability in incidence of ASs is reported in literature, depending on different definitions of AS and on a great number of pre-, intra-, and postoperative risk factor influencing the anastomosis outcome. The presence of a long gap between the two esophageal ends, with consequent anastomotic tension, is determinant for stricture formation and its response to treatment. The cornerstone of treatment is endoscopic dilation, whose primary aims are to achieve symptom relief, allow age-appropriate capacity for oral feeding, and reduce the risk of pulmonary aspiration. No clear advantage of either balloon or bougie dilator has been demonstrated; therefore, the choice is based on operator experience and comfort with the equipment. Retrospective evidences suggest that selective dilatations (performed only in symptomatic patients) results in significantly less number of dilatation sessions than routine dilations (performed to prevent symptoms) with equal long-term outcomes. The response to dilation treatment is variable, and some patients may experience recurrent and refractory ASs. Adjunctive treatments have been used, including local injection of steroids, topical application of mitomycin C, and esophageal stenting, but long-term studies are needed to prove their efficacy and safety. Stricture resection or esophageal replacement with an interposition graft remains options for AS refractory to conservative treatments.

Neonatal surgery is recognized as an independent discipline in general surgery, requiring the expertise of pediatric surgeons to optimize outcomes in infants with surgical conditions. Survival following neonatal surgery has improved dramatically in the past 60 years. Improvements in pediatric surgical outcomes are in part attributable to improved understanding of neonatal physiology, specialized pediatric anesthesia, neonatal critical care including sophisticated cardiopulmonary support, utilization of parenteral nutrition and adjustments in fluid management, refinement of surgical technique, and advances in surgical technology including minimally invasive options. Nevertheless, short and long-term complications following neonatal surgery continue to have profound and sometimes lasting effects on individual patients, families, and society.

Esophageal atresia (EA) is one of the most common congenital digestive anomalies. With improvements in surgical techniques and intensive care treatments, the focus of care of these patients has shifted from mortality to morbidity and quality-of-life issues. These children face gastrointestinal (GI) problems not only in early childhood but also through adolescence and adulthood. There is, however, currently a lack of a systematic approach to the care of these patients. The GI working group of International Network on Esophageal Atresia comprises members from ESPGHAN/NASPGHAN and was charged with the task of developing uniform evidence-based guidelines for the management of GI complications in children with EA.

Thirty-six clinical questions addressing the diagnosis, treatment, and prognosis of the common GI complications in patients with EA were formulated. Questions on the diagnosis, and treatment of gastroesophageal reflux, management of "cyanotic spells," etiology, investigation and management of dysphagia, feeding difficulties, anastomotic strictures, congenital esophageal stenosis in EA patients were addressed. The importance of excluding eosinophilic esophagitis and associated GI anomalies in symptomatic patients with EA is discussed as is the quality of life of these patients and the importance of a systematic transition of care to adulthood. A systematic literature search was performed from inception to March 2014 using Embase, MEDLINE, the Cochrane Database of Systematic Reviews, Cochrane Central Register of Controlled Clinical Trials, and PsychInfo databases. The approach of the Grading of Recommendations Assessment, Development and Evaluation was applied to evaluate outcomes. During 2 consensus meetings, all recommendations were discussed and finalized. The group members voted on each recommendation, using the nominal voting technique. Expert opinion was used where no randomized controlled trials were available to support the recommendation.

The treatment of esophageal atresia is not centralized in Germany. Therefore, high numbers of departments are involved. Data on the results of esophageal atresia repair from Germany are lacking. The aim of this study was to evaluate the early postoperative results after repair of esophageal atresia based on unbiased data of a German health insurance. We aimed to determine whether characteristics of the departments had an impact on outcome and compared the results from this study with the literature data from centers with a high caseload. Data of a German health insurance covering ∼10% of the population were analyzed. All patients who had undergone esophageal atresia repair from January 2007 to August 2012 were included. Follow-up data of 1 year postoperatively were analyzed. The potential impact of various characteristics of the treating surgical institutions was assessed. Results were compared with the latest international literature. Seventy-five patients with esophageal atresia underwent reconstructive surgery in 37 departments. The incidences of anastomotic leak (3%) and recurrent tracheoesophageal fistula (7%) were comparable with the literature (both 2-8%). Anastomotic stricture required dilatation in 57% of patients (mean 5.1 ± 5.6 dilatations) comparing unfavorably to most, but not all international reports. During 1-year follow-up, 93% of the patients were readmitted at least once (mean 3.9 ± 3.1 admissions). The incidence of complications did not correlate with any of the characteristics of the treating institutions such as academic affiliation, the number of consultants, beds, and preterm infants treated per year (all P > 0.05). Based on unbiased data, postoperative results after repair of esophageal atresia in Germany are comparable with recently published reports from international single centers. A correlation between the complication rate and characteristics of the treating institutions was not identified.

Esophagectomy and subsequent reconstruction represent major physiological insults to the upper gastrointestinal (GI) tract, which as a consequence can lead to malnutrition, dysphagia and reflux. From a technical perspective, operative reconstruction involving gastric pull-up with a 2-3 cm wide tube and an anastomosis cranial to the azygos vein may minimize the symptoms. Overall, the problems tend to improve approximately 6 months after the operation. Newly occurring delayed physical functional impairments with previously known underlying malignant disease may be indicative of cancer relapse. Interventional techniques, such as stent placement or brachytherapy may be better suited for treatment of recurrent disease.

After repair of oesophageal atresia (OA), the need for endoscopic follow-up (EFU) remains unclear. To end this, we assessed the trends of oesophageal mucosal changes in successive follow-up biopsies.

EFU records of 264 patients including histological grades of oesophagitis (from 0 to III), gastric (GM) or intestinal (IM) metaplasia and dysplasia (mild to severe) at 1, 3, 5 10, 15, and >15 years after repair of OA were reviewed.

Included were 209 patients with 616 biopsies. A total of 60 patients had undergone antireflux surgery and 24 had long-gap OA (LG). Median follow-up was 12 (range 1-17) years with 3 (1-6) endoscopies per patient. Highest grade of oesophagitis was Gr 0 (no oesophagitis) in 47%, Gr I in 37%, and Gr II or III in 16%. Metaplasia, GM (n = 31), IM (n = 4), occurred in 17% of patients and reached 15% prevalence by 15 years. Dysplasia and cancer were not found. From 1 to 15 years after repair grade of histological oesophagitis often fluctuated between Gr 0 and Gr I, but further progression was unlikely, hazard ratio = 0.2-3.4 (95% confidence interval 0.0-29), P = 0.06-0.87. LG and antireflux surgery predicted early detection of metaplasia (P < 0.001). Only 9% of patients with metaplasia and 32% with Gr II oesophagitis were symptomatic. A total of 6 (3%) patients had a symptomatic anastomotic stenosis at 1 year.

EFU revealed frequent oesophagitis and metaplasia, but no dysplasia or cancer. Routine endoscopic surveillance had limited benefit and seems unnecessary during childhood after repair of OA.

Dysphagia is a common problem in children with repaired oesophageal atresia (OA). Abnormalities in the oropharyngeal and oesophageal phase have hardly been studied. The aims of this study were to assess the prevalence of dysphagia in children with repaired OA and to identify and differentiate oral and pharyngeal dysphagia based on videofluoroscopic swallow study (VFSS) findings in a limited number of children in this cohort. Medical records of 111 patients, born between January 1996 and July 2013 and treated at the Radboudumc Amalia Children's Hospital, were retrospectively reviewed. The prevalence of dysphagia was determined by the objective and modified Functional Oral Intake Scale (FOIS) in four age groups. The first performed VFSS of 12 children was structurally assessed. The prevalence of dysphagia was 61 of 111 patients (55 %) in age group <1 year. In age group 1-4, 5-11 and 12-18 years, the prevalence of dysphagia decreased from 54 of 106 (51 %) patients to 11 of 64 (17 %) and 5 of 24 (21 %) patients. The 12 VFSS's reviews revealed oral dysphagia in 36 % and pharyngeal dysphagia in 75 %.

This study highlights dysphagia as an important problem in different age groups of children with repaired OA. Furthermore, our study shows the presence of oropharyngeal dysphagia in this population. This study emphasizes the need to standardize the use of objective dysphagia scales, like the modified FOIS, to provide a careful follow-up of children with repaired OA.

• Prevalence of dysphagia in children with repaired oesophageal atresia varies widely (ranges from 45 to 70 %) in literature. • Oral, pharyngeal and oesophageal dysphagia require different treatment approaches. What is New: • We determined dysphagia based on functional oral intake and provide an overview of change in dysphagia prevalence and severity over time in children with repaired OA. • Our study shows that dysphagia, including oropharyngeal dysphagia, is highly prevalent in young children with repaired OA and improves with time.

The reported incidence of anastomotic stricture after esophageal atresia repair has varied in case series from as low as 9% to as high as 80%. The cornerstone of esophageal stricture treatment is dilation with either balloon or bougie. The goal of esophageal dilation is to increase the luminal diameter of the esophagus while also improving dysphagia symptoms. Once a stricture becomes refractory to esophageal dilation, there are several treatment therapies available as adjuncts to dilation therapy. These therapies include intralesional steroid injection, mitomycin C, esophageal stent placement, and endoscopic incisional therapy.

Esophageal atresia (EA) with or without tracheo-esophageal fistula (TEF) is a relatively rare congenital anomaly. Despite the advances in the management techniques and neonatal intensive care, esophageal dysmotility remains a very common problem following EA/TEF repair. Our current study aimed to describe the most significant ultrastructural changes of the smooth muscle cells (SMCs) trying to highlight some of the underlying mechanisms of esophageal dysmotility following EA/TEF repair. Twenty-three biopsies were obtained from the tip of the lower esophageal pouch (LEP) of 23 patients during primary repair of EA/TEF. Light microscopic examination was performed with hematoxylin and eosin (HE), and Van Gieson's stains. Ultrastructural examination was done using transmission electron microscopy (TEM). Histopathological examination showed distortion of smooth muscle layer and deposition of an abundant amount of fibrous tissue in-between smooth muscles. Using TEM, SMCs exhibited loss of the cell-to-cell adhesion, mitochondrial vacuolation, formation of myelin figures, and apoptotic fragmentation. There were also plasmalemmal projections and formation of ghost bodies. Interestingly, SMCs were found extending pseudopodia-like projections around adjacent collagen fibers. Engulfed collagen fibers by SMCs underwent degradation within autophagic vacuoles. Degeneration of SMCs and deposition of abundant extracellular collagen fibers are prominent pathological changes in LEP of EA/TEF. These changes might contribute to the pathogenesis of esophageal dysmotility in patients who have survived EA/TEF.

More than half of all congenital deformities can be detected in utero. The initial surgical correction is of paramount importance for the achievement of good long-term results with low surgical morbidity and mortality.

Selective literature review and expert opinion.

Congenital deformities are rare, and no controlled trials have been performed to determine their optimal treatment. In this article, we present the prenatal assessment, treatment, and long-term results of selected types of congenital deformity. Congenital diaphragmatic hernia (CDH) affects one in 3500 live-born infants, while esophageal atresia affects one in 3000 and small-bowel atresia one in 5000 to 10,000. If a congenital deformity is detected and its prognosis can be reliably inferred from a prenatal assessment, the child should be delivered at a specialized center (level 1 perinatal center). The associated survival rates are 60-80% after treatment for CDH and well over 90% after treatment for esophageal or small-bowel atresia. Despite improvements in surgical correction over the years, complications and comorbidities still affect 20-40% of the treated children. These are not limited to surgical complications in the narrow sense, such as recurrence, postoperative adhesions and obstruction, stenoses, strictures, and recurrent fistulae, but also include pulmonary problems (chronic lung disease, obstructive and restrictive pulmonary dysfunction), gastrointestinal problems (dysphagia, gastro-esophageal reflux, impaired intestinal motility), and failure to thrive. Moreover, the affected children can develop emotional and behavioral disturbances. Minimally invasive surgery in experienced hands yields results as good as those of conventional surgery, as long as proper selection criteria are observed.

Congenital deformities should be treated in recognized centers with highly experienced interdisciplinary teams. As no randomized trials of surgery for congenital deformities are available, longitudinal studies and registries will be very important in the future.

Like all modern medical therapy, neonatal surgery is founded on clinical research, well-tried clinical practice and basic scientific research. Likewise, modern neonatal surgery strives increasingly for evidence-based management and practice. The very nature of neonatal and pediatric surgery renders associated research challenging because of the rarity and small numbers of surgical disorders and varying resources in different countries and institutions and consequently only a few well-designed trials on truly important issues in neonatal surgical treatment have been performed. This article highlights the research methods by which valid evidence-based research data is obtained in observational studies, randomized controlled trials, and meta-analyses. The problem of small numbers of patients may be overcome by multi-center trials, meta-analyses, and networking. Consideration is also given on the quality and the validity of the study data as well as ethical issues in neonatal surgical research.

Health-related quality of life (HRQoL) after esophageal atresia (EA) repair is postulated to be good. However, little is known about the long-term results after repair of complex and/or complicated EA regarding HRQoL. We investigated long-term HRQoL after delayed anastomosis, esophageal replacement, major revisions, or multiple dilatations in patients registered in a support group.

Patients registered in the German patient support group database (KEKS) were enrolled and allocated to subgroups according to surgical treatment and age. HRQoL was evaluated using validated questionnaires (GIQLI, WHO-5, KIDSCREEN27).

Complete follow-up (mean 14.5 ± 9.8 years) was available for 90/92 patients. Patients were allocated to subgroups delayed anastomosis (n=28), esophageal replacement (n=27), major revisions (n=15), and multiple dilatations (n=20). Adult patients presented with impaired well-being according to WHO-score and gastrointestinal function (GIQLI). In contrast, HRQoL of children was comparable to controls in most KIDSCREEN27-dimensions. Delayed anastomosis was associated with most-favourable HRQoL. Regarding physical well-being, these children scored significantly better than controls [64.01 ± 10.40 vs. 52.36 ± 8.73;p=0.0011], children after replacement [51.40 ± 5.70;p=0.008], revisions [52.04 ± 6.97;p=0.026], and multiple dilatations [50.22 ± 9.67,p=0.04].

HRQoL after complex and/or complicated EA is excellent in children registered in a patient support group. In adults, disease-specific symptoms negatively affect HRQoL. Our data indicate that saving the esophagus may achieve the best HRQoL.