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.

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.

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.

There is currently no validated diagnostic modality to characterize the anatomy and predict outcomes of tracheal esophageal defects, such as esophageal atresia (EA) and tracheal esophageal fistulas (TEFs). We hypothesized that ultra-short echo-time MRI would provide enhanced anatomic information allowing for evaluation of specific EA/TEF anatomy and identification of risk factors that predict outcome in infants with EA/TEF.

In this observational study, 11 infants had pre-repair ultra-short echo-time MRI of the chest completed. Esophageal size was measured at the widest point distal to the epiglottis and proximal to the carina. Angle of tracheal deviation was measured by identifying the initial point of deviation and the farthest lateral point proximal to the carina.

Infants without a proximal TEF had a larger proximal esophageal diameter (13.5 ± 5.1 mm vs. 6.8 ± 2.1 mm, p = 0.07) when compared to infants with a proximal TEF. The angle of tracheal deviation in infants without a proximal TEF was larger than infants with a proximal TEF (16.1 ± 6.1° vs. 8.2 ± 5.4°, p = 0.09) and controls (16.1 ± 6.1° vs. 8.0 ± 3.1°, p = 0.005). An increase in the angle of tracheal deviation was positively correlated with duration of post-operative mechanical ventilation (Pearson r = 0.83, p < 0.002) and total duration of post-operative respiratory support (Pearson r = 0.80, p = 0.004).

These results demonstrate that infants without a proximal TEF have a larger proximal esophagus and a greater angle of tracheal deviation which is directly correlated with the need for longer post-operative respiratory support. Additionally, these results demonstrate that MRI is a useful tool to assess the anatomy of EA/TEF.

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.

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.

To describe the burden of esophageal dilatations in patients following esophageal atresia (EA) repair.

A retrospective review was performed at The Royal Children's Hospital, Melbourne, of all neonates undergoing operative repair for EA over a 17-year period (1999-2015). Stricture was defined by radiological and/or intra-operative findings of narrowing at the esophageal anastomosis. Data recorded included EA type, perinatal details, operative approach, esophageal anastomosis outcome, dilatation requirement, and survival. Key endpoints were anastomotic leakage and tension, esophageal dilatation technique, dilatation frequency, fundoplication, and complications.

During the study period, 287 newborn EA patients were admitted, of which 258 underwent operative repair and survived to primary discharge. Excluding 11 patients with isolated tracheoesophageal fistula, 247 patients were included in the final analysis. Intra-operative anastomotic tension was documented in 41/247 (16.6%), anastomotic leak occurred in 48/247 (19.4%), and fundoplication was performed in 37/247 (15.0%). Dilatations were performed in 149/247 (60.3%). Techniques included bougie-alone (92/149, 61.7%), combination of bougie and balloon (51/149, 34.2%), and balloon-alone (6/149, 4.0%). These patients underwent 1128 dilatations; median number of dilatations per patient was 4 (interquartile range 2-8). Long-gap EA and anastomotic tension were risk factors (p < 0.01) for multiple dilatations. Complications occurred in 13/1128 (1.2%) dilatation episodes: 11/13 esophageal perforation, 2/13 clinically significant aspiration. Perforations were rare events in both balloon (6/287, 2.1%) and bougie dilatations (4/841, 0.5%); one patient had a perforation from guidewire insertion.

Esophageal dilatation occurred in a majority of EA patients. Long-gap EA was associated with an increased burden of esophageal dilatation. Perforations were rare events in balloon and bougie dilatations.

Original article - retrospective review.

II.

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.

The present study aimed to evaluate whether the detailed observation of pH/MII waveforms and the analysis of baseline impedance (BI) values could detect esophageal dysmotility in pediatric patients with esophageal disorders.

Eleven patients with congenital esophageal disorder in whom pH/MII was conducted from April 2011 to June 2015, were enrolled in this study. The diagnoses of the patients were as follows: postoperative esophageal atresia (EA), n = 6; esophageal achalasia (EAch), n = 4; and congenital esophageal stenosis (CES), n = 1. The characteristics of the pH/MII waveform, pathological GERD, BI value, and the average BI value of the 2 distal channels (distal BI; DBI) were analyzed in each disorder.

Two EA (33%) patients and one EAch (25%) patient were diagnosed with GERD. The mean DBI values of the EA, EAch and CES patients was 912 ± 550, 2153 ± 915 and 1392 Ω, respectively. The EA patients showed consistently low DBI values. One CES patient and two infantile EAch patients showed postprandial prolonged low DBI values. Whereas, the pH/MII waveforms of the adolescent EAch patients were difficult to interpret due to their extremely low BI values.

The present study demonstrated that the detailed observation of the pH/MII waveforms in all channels and the analysis of BI were useful for evaluating esophageal motility in children with congenital esophageal disorders. In particular, infantile patients with EAch showed DBI findings that were distinct from those of adult EAch patients. Considering the difficulty of performing esophageal manometry in young children, the detailed observation of the pH/MII waveform may help in the diagnosis of esophageal dysmotility in children.

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.

We describe our experience using a modified suture fistula technique for addressing tension in longer gap esophageal atresia (EA). Esophagoesophagopexy (EEP) is the tacking of the proximal and distal ends of esophageal pouches without formal anastomosis. In this retrospective cohort, we review the outcomes of patients with EA after EEP.

We reviewed the operative reports of EA cases treated at our institution from 1997 to 2016 and identified all patients described as having EEP.

Of 129 EA cases, five patients underwent EEP. Formal anastomosis was not done due to patient's instability, prematurity, or long gap. Median birth weight was 1.4 kg (0.6-2.2 kg), and median gestational age at birth was 29 weeks (25-34 weeks). Age at time of EEP ranged 0-5 months. Esophagoesophageal fistula was confirmed in three patients. All three had strictures requiring weekly dilations. One of these patients died. The two surviving patients underwent fundoplication.

We describe an alternative technique for esophageal anastomosis in patients for whom a standard anastomosis is not possible. EEP can lead to a functional anastomosis through fistulization and avoid the morbidity of multiple thoracotomies and lengthening procedures. Families should be educated on the potential need for dilations and antireflux procedures.

Data on long-term outcome of exercise capacity in school-aged children with esophageal atresia (EA) are scarce. We evaluated maximal exercise capacity and its relation to lung function. Moreover, we studied other possible determinants of exercise capacity and lung function.

Exercise capacity of 63 children with EA born 1999-2007 was evaluated at the age of 8 years with the Bruce-protocol. Dynamic and static lung volumes, bronchodilator response and diffusion capacity were measured. Furthermore, perinatal characteristics, hospital admissions for lower respiratory tract infections (RTIs), RTIs treated with antibiotics in the past year, symptoms of gastroesophageal reflux, weight-for-height, and sports participation were evaluated as other potential determinants.

Exercise capacity was significantly below normal: mean (SD) SDS -0.91 (0.97); P < 0.001. All spirometric parameters were significantly below normal with significant reversibility of airflow obstruction in 13.5% of patients. Static lung volumes were significantly decreased (mean (SD) SDS TLC_he -1.06 (1.29); P < 0.001). Diffusion capacity corrected for alveolar volume was normal (mean (SD) SDS K_CO -0.12 (1.04)). Exercise capacity was positively associated with total lung capacity and negatively with SDS weight-for-height. Spirometric parameters were negatively associated with congenital cardiac malformation, duration of ventilation, and persistent respiratory morbidity.

Eight-year-old children with EA had reduced exercise capacity which was only associated with the reduction in TLC_he and higher SDS weight-for-height. We speculate that diminished physical activity with recurrent respiratory tract infections may also play a role in reduced exercise capacity. This should be subject to further research to optimize appropriate intervention.

The surgical management of oesophageal atresia (OA) differs between pediatric surgical teams without consensus. We aimed to describe the current practice of OA treatment in Belgium and Luxembourg and compare this to the literature.

A questionnaire was created and sent to all 18 hospitals (14 pediatric surgical units) performing OA surgery in Belgium and Luxembourg. The results were compared to the literature.

Most units treat an average of 2-5 OA+TOF (71%) and ≤1 pure OA (pOA) per year (86%). The preferred surgical approach for OA+TOF is thoracotomy (86%), mostly extra-pleural (75%). Thoracoscopic OA repair is performed in 21%. All centers perform an end-to-end anastomosis (interrupted sutures), and all leave a transanastomotic tube. A chest drain is routinely used in 8units (57%). In pOA the preferred surgical approach is gastrostomy formation with delayed primary anastomosis (77%). The timing for delayed anastomosis is 2 to 24months. Intra-operative lengthening is mostly attempted with Foker technique (46%). If oesophageal replacement is needed, gastric interposition is mostly used (75%). A postoperative contrast study is routinely performed in 86% for OA+TOF and in 100% for pOA. Anti-reflux medication is routinely prescribed by all units but one.

There are still many differences and controversies in the perioperative management of OA. Part of this is based on habits and is difficult to change without scientific evidence. There is a need for prospective (inter)national registries to further identify the existing differences, leading to a more widely accepted consensus.

Level III.

Esophageal dysmotility is almost universal after esophageal atresia (EA) repair and is mainly related to the developmental anomaly of the esophagus. Esophageal dysmotility is involved in the pathophysiology of numerous symptoms and comorbidities associated with EA such as gastroesophageal reflux disease, aspiration and respiratory complications, and symptoms of dysphagia and feeding disorders. High-resolution esophageal manometry (HREM) has facilitated the characterization of the dysmotility, but there is an incomplete correlation between symptoms and manometrical patterns. Impedance coupled to HREM should help to predict the clinical outcome and therefore personalize patient management. Nowadays, the management of esophageal dysmotility in patients with EA is essentially based on treatment of associated inflammation related to peptic or eosinophilic esophagitis.

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.

Oesophageal atresia and tracheo-oesophageal fistula are congenital anomalies of the oesophagus requiring surgical repair in infancy, either by open or thoracoscopic approach. Although mortality rates associated with this procedure are low, children may go on to have complications throughout childhood and into adulthood, most commonly related to ongoing gastrointestinal and respiratory symptoms. This review outlines the early, mid and long-term outcomes for these children in terms of quality of life and incidence of symptoms.

Impaired growth has been reported in children born with esophageal atresia (EA). Their nutritional fate at adulthood remains uncertain though. Our objectives were to determine the body mass index (BMI) of adult patients with EA followed up from 2009 to 2011 in the EA clinic of a university-affiliated hospital in Quebec (Canada), and investigate characteristics associated with underweight.

The 40 adult patients with EA attending the clinic were invited to participate. Height and weight were measured and BMI calculated. Patients with BMI < 18.5 kg/m were deemed underweight. Patients' characteristics were obtained, including digestive symptoms and compensatory eating behaviors. Nonparametric tests were used to compare the proportion of underweight among patients with EA with that found in the Quebec population and the characteristics between patients with EA deemed underweight and those with BMI ≥ 18.5 kg/m.

The final sample included 16 women and 21 men, ages 18 to 44 years. Mean BMI was 21.3 ± 4.9 kg/m and 24.3% had BMI < 18.5 kg/m, which is higher than in the Quebec adult population (2.5%, P < 0.001). Compared with patients with EA having BMI ≥ 18.5 kg/m, underweight patients had more often experienced failure to thrive during their adolescence (55.6% vs 7.4%, P = 0.006), and reported severe postprandial fullness (62.5% vs 21.4%, P = 0.040), the need to eat slowly (87.5% vs 46.4%; P = 0.045), and severe difficulties to swallow dry solid foods (50.0% vs 14.3%, P = 0.054).

Insufficient body weight is prevalent in this sample of adult patients with EA and could result from digestive symptoms. Follow-up with a gastroenterologist and nutritional counseling should be considered for adult patients with EA.

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.

Few studies have evaluated the long-term complications and outcomes of esophageal atresia with or without tracheoesophageal fistula (EA/TEF) beyond childhood. The aim of our study was to characterize the esophageal and respiratory morbidity of EA/TEF through evaluation of clinical symptoms, diagnostic testing and therapeutic intervention at a tertiary care center.

Patients with congenital EA/TEF evaluated from 2011 to 2014 were included. Demographic characteristics, type and mode of repair of EA/TEF, clinical symptoms, radiographic, endoscopic, bronchoscopic and medication use data were obtained.

A total of 43 patients were identified. The median age of this predominantly Caucasian population was 8 years (interquartile range: 3, 20). Twenty (62.5%) had type C (EA with distal TEF) abnormality. Twenty-one (48.8%) patients had heartburn, 19 (44.1%) had acid regurgitation, and 31 (72.1%) had dysphagia to solids. Barium swallow in 26 patients revealed strictures in 17 (65.4%), dysmotility in 20 (76.9%) and recurrent fistulas in four patients (15.4%). Thirty patients underwent upper endoscopy, of which 21 (70.0%) had a stricture, and six (20.0%) had recurrent fistula requiring surgical intervention. Eight (18.6%) patients underwent fundoplication. Pulmonary evaluation showed cough and choking in 31 (72.1%) patients and dyspnea and wheezing in 32 (53.4%) patients. Recurrent respiratory infections were reported in 19 (44.2%).

Other findings included tracheomalacia in 86.7% and restrictive lung disease in 54.5% of patients.

There is a high burden of residual esophageal and pulmonary pathology in patients with EA/TEF. Ongoing follow-up is required to monitor both the clinical symptoms and treatment responses.

Clinical experiences of adults who underwent surgery for esophageal atresia at birth is limited. There is some evidence that suggests considerable long-term morbidity, partly because of dysphagia, which has been reported in up to 85% of adult patients who undergo surgery for esophageal atresia. The authors hypothesized that dysphagia in this population is caused by dysmotility and⁄or anatomical anomalies.

To determine the motor and anatomical causes of dysphagia.

A total of 41 adults, followed at the Esophageal Atresia Clinic at Hôpital Saint-Luc (Montreal, Quebec), were approached to particpate in the present prospective study. Evaluation was completed using upper endoscopy, manometry and barium swallow for the participants who consented. The medical charts of respondents were systematically reviewed from the neonatal period to 18 years of age to assess medical and surgical history.

All 41 patients followed at the clinic consented and were included in the study. Dysphagia was present in 73% of patients. Esophagogastroduodenoscopy was performed in 32 patients: hiatal hernia was present in 62% (n=20); esophageal diverticulum in 13% (n=4); macroscopic Barrett esophagus in 31% (n=10); and esophagitis in 19% (n=6). Histological esophagitis was present in 20% and intestinal metaplasia in 10%. There were no cases of dysplagia or adenocarcinoma. Esophageal manometry was performed on 56% of the patients (n=23). Manometry revealed hypomotility in 100% of patients and included an insufficient number of peristaltic waves in 96%, nonpropagating peristalsis in 78% and low-wave amplitude in 95%. Complete aperistalsis was present in 78%. The lower esophageal sphincter was abnormal in 12 (52%) patients, with incomplete relaxation the most common anomaly. Of the 41 patients, 29 (71%) consented to a barium swallow, which was abnormal in 13 (45%). The anomalies found were short esophageal dilation in 28%, delay in esophageal emptying in 14%, diverticula in 14% and stenosis in 7% of patients. There was more than one anomaly in 14% of patients.

Dysphagia was a highly prevalent symptom in adults who underwent surgery for esophageal atresia. The present study is the first to demonstrate that motor and anatomical abnormalities may be implicated in causes of dysphagia in this population. Furthermore, these anomalies may be demonstrated with simple investigations such as endoscopy, manometry and barium swallow.

Esophageal atresia (EA) is a rare congenital anomaly with high infantile survival rates. The aim of this study was to outline the prevalence of common long-term problems associated with EA repair in patients older than 10 years of age.

Original papers were identified by systematic searching of MEDLINE and EMBASE databases from January 1993 to July 2014. Fifteen articles (907 EA patients) met inclusion criteria.

This is the first systematic review aiming to quantify the prevalence of the long-term problems associated with EA. The main active medical conditions (pooled estimated prevalence) identified were the following: dysphagia (50.3%), gastroesophageal reflux disease with (40.2%) or without (56.5%) histological esophagitis, recurrent respiratory tract infections (24.1%), doctor-diagnosed asthma (22.3%), persistent cough (14.6%), and wheeze (34.7%). The prevalence of Barrett's esophagus (6.4%) was 4 and 26 times higher than the adult (1.6%) and pediatric (.25%) general populations. Adult and pediatric practitioners should focus on how to develop effective long-term follow-up and transitional care for these patients.

Deglutition, or swallowing, refers to the process of propulsion of a food bolus from the mouth into the stomach and involves the highly coordinated interplay of swallowing and breathing. At 34 weeks gestational age most neonates are capable of successful oral feeding if born at this time; however, the maturation of respiration is still in progress at this stage. Infants can experience congenital and developmental pharyngeal and/or gastrointestinal motility disorders, which might manifest clinically as gastro-oesophageal reflux (GER) symptoms, feeding difficulties and/or refusal, choking episodes and airway changes secondary to micro or overt aspiration. These problems might lead to impaired nutritional intake and failure to thrive. These gastrointestinal motility disorders are mostly classified according to the phase of swallowing in which they occur, that is, the oral preparatory, oral, pharyngeal and oesophageal phases. GER is a common phenomenon in infancy and is referred to as GERD when it causes troublesome complications. GER is predominantly caused by transient relaxation of the lower oesophageal sphincter. In oesophageal atresia, oesophageal motility disorders develop in almost all patients after surgery; however, a congenital origin of disordered motility has also been proposed. This Review highlights the prenatal development of upper gastrointestinal motility and describes the most common motility disorders that occur in early infancy.

Esophageal atresia (EA), with or without tracheoesophageal fistula (TEF), is commonly associated with gastroesophageal reflux (GER) after surgical repair. One risk factor for anastomotic stricture is post-operative GER. This survey assessed practice patterns among attendees at the Canadian Association of Pediatric Surgeons (CAPS) annual meeting with respect to management of GER post EA-TEF repair.

A pre-piloted survey was handed out and collected at the 2012 CAPS annual meeting. Data were entered and coded, and descriptive statistics were calculated.

We distributed 70 surveys, and 57 (81.4%) surveys were returned. On average, the incidence of EA-TEF is 8-10 cases per institution, per year. Anti-reflux medication is started immediately post-operatively in 74% of patients at institution of feeds (11%), or if symptoms of reflux develop (14%). Proton pump inhibitors and H2-receptor antagonists are used in approximately equal proportion. Patients are typically kept on anti-reflux medication for 3-6 months (37%) or 6-12 months (35%).

Most CAPS attendees treat postoperative GER prophylactically. However, there is no consistency in management strategy regarding which anti-reflux agent to use or for how long. A multi-centered study is required to establish a standardized protocol for the post-operative management of EA-TEF to prevent reflux and its effect on anastomotic strictures.

A clinical demand exists for alternatives to repair the esophagus in case of congenital defects, cancer, or trauma. A seamless biocompatible off-the-shelf large-diameter tubular scaffold, which is accessible for vascularization, could set the stage for regenerative medicine of the esophagus. The use of seamless scaffolds eliminates the error-prone tubularization step, which is necessary when emanating from flat scaffolds. In this study, we developed and characterized three different types of seamless tubular scaffolds, and evaluated in vivo tissue compatibility, including vascularization by omental wrapping. Scaffolds (luminal Ø ∼ 1.5 cm) were constructed using freezing, lyophilizing, and cross-linking techniques and included (1) single-layered porous collagen scaffold, (2) dual-layered (porous+dense) collagen scaffold, and (3) hybrid scaffold (collagen+incorporated polycaprolacton knitting). The latter had an ultimate tensile strength comparable to a porcine esophagus. To induce rapid vascularization, scaffolds were implanted in the omentum of sheep using a wrapping technique. After 6 weeks of biocompatibility, vascularization, calcification, and hypoxia were evaluated using immunohistochemistry. Scaffolds were biocompatible, and cellular influx and ingrowth of blood vessels were observed throughout the whole scaffold. No calcification was observed, and slight hypoxic conditions were detected only in the direct vicinity of the polymer knitting. It is concluded that seamless large-diameter tubular collagen-based scaffolds can be constructed and vascularized in vivo. Such scaffolds provide novel tools for esophageal reconstruction.

To evaluate the mechanisms underlying gastroesophageal reflux (GER) following esophageal atresia (EA) repair and gastroesophageal function in infants and adults born with EA.

Ten consecutive infants born with EA as well as 10 randomly selected adult EA patients were studied during their first postoperative follow-up visit and a purposely planned visit, respectively. A (13)C-octanoate breath test and esophageal pH-impedance-manometry study were performed. Mechanisms underlying GER and esophageal function were evaluated.

Transient lower esophageal sphincter relaxation (TLESR) was the most common mechanism underlying GER in infants and adults (66% and 62%, respectively). In 66% of all GER episodes, no clearing mechanism was initiated. On EFT, normal motility patterns were seen in six patients (four infants, two adults). One of these adults had normal motility overall (>80% of swallows). Most swallows (78.8%) were accompanied by abnormal motility patterns. Despite this observation, impedance showed normal bolus transit in 40.9% of swallows. Gastric emptying was delayed in 57.1% of infants and 22.2% of adults.

TLESR is the main mechanism underlying GER events in patients with EA. Most infants and adults have impaired motility, delayed bolus clearance, and delayed gastric emptying. However, normal motility patterns were seen in a minority of patients.

Esophageal atresia (EA) is one of the most frequent congenital alimentary tract anomalies with a considerable morbidity throughout childhood. This study evaluates the gastroesophageal problems in 5-15 year old children with EA and aims to identify factors predisposing to esophagitis in EA.

Fifty-nine patients primarily operated at Odense University Hospital, Denmark, during 1993-2005 were included in this follow-up study. The patients underwent the following examinations: Interview, upper endoscopy, endoscopic ultrasonography, high-resolution esophageal manometry (HREM), and pH- and multichannel intraluminal impedance (MII) measurements. Twenty-five patients with suspected gastro-esophageal reflux disease (GERD) underwent the same investigations and served as controls.

Median age was 10.2 years (7.1-13.3). Thirty-three (55.9%) presented with GERD symptoms, 41 (69.5%) with dysphagia, and 33 (55.9%) with respiratory symptoms. Twenty-nine (49.2%) had endoscopic esophagitis, and 26 (44.1%) histological esophagitis. Median reflux index (RI) was 8.3 (4.8-14.9). In 32 (55.2%) RI was above 7. Ten percent had eosinophilic inflammation. HREM showed dysmotility in the esophagus in all EA patients, 83.3% had no propagating swallows. No predictive factors predisposing the development of endoscopic esophagitis were identified.

Gastroesophageal problems in children born with EA are common. Routine follow-up with endoscopy and pH-metry in EA patients is warranted.

Esophageal dysmotility, a considerable issue following esophageal atresia (EA) repair, has been reported but has not been precisely described and characterized. Using high-resolution esophageal manometry (HREM), we characterized the esophageal motility patterns in children with repaired EA and compared these patterns of dysmotility with symptomatology.

HREM was performed as an outpatient procedure in patients with repaired EA. The tracings were analyzed using the software provided by the company and were then reviewed visually. Charts were reviewed for medical/surgical histories and symptoms were assessed by a standardized questionnaire.

Forty patients (25 boys, 15 girls) with a median age of 8 years (11 months-18 years) underwent an HREM. Thirty-five patients had type C EA and 5 had type A EA. Only 7 patients were asymptomatic at the time of the examination. HREM results were abnormal in all of the patients. Three different esophageal motility patterns were derived from HREM tracing analysis: aperistalsis (15 patients, 38%), pressurization (6 patients, 15%), and distal contractions (19 patients, 47%). Distal contractions pattern was found exclusively in type C EA. Dysphagia was encountered in the 3 groups. Gastroesophageal reflux disease-related symptoms predominated in the aperistalsis group.

HREM improves our understanding and allows precise characterization of esophageal dysmotility in patients who have undergone EA repair.

Animal models are of great importance for medical research. They have enabled analysis of the aetiology and pathogenesis of complex congenital malformations and have also led to major advances in the surgical and therapeutic management of these conditions. Animal models allow us to comprehend the morphological and molecular basis of disease and consequently to discover novel approaches for both surgical and medical therapy. The anthracycline antibiotic adriamycin was incidentally found to have teratogenic effects on rats, producing a range of defects remarkably similar to the VACTERL association of congenital anomalies in humans, providing a reproducible animal model of this condition. VACTERL association is a spectrum of birth defects which includes vertebral, anal, cardiovascular, tracheo-oesophageal, renal and limb anomalies. In recent years, adriamycin rodent models of VACTERL have provided valuable insights into the pathogenesis of this complex association, particularly in relation to tracheo-oesophageal malformations. The adriamycin rat model and adriamycin mouse model are now well established in the investigation of the morphology of faulty organogenesis and the regulation of gene expression in tracheo-oesophageal anomalies.

Survival rates in esophageal atresia (EA) patients have reached 90%. In long-term follow-up studies the focus has shifted from purely surgical or gastrointestinal evaluation to a multidisciplinary approach. We evaluated the long-term morbidity in adolescent and adult EA patients and discussed mainly nonsurgical issues. Dysphagia is common and reported in up to 85% of patients. In young adults gastroesophageal reflux disease occurs frequently with development of Barrett esophagus in 6% reported in different series. It is difficult to estimate respiratory morbidity from the literature because many different definitions, questionnaires, and study designs have been used. However, many patients seem to suffer from respiratory problems even into adulthood. In conclusion, morbidity is not only restricted to surgical problems but many different domains are involved. These are all related and together determine to a large extent the quality of life of EA patients and also of their families. We assume that a multidisciplinary care approach seems best to address their special needs.

Esophageal dysmotility is a considerable long-term issue in patients born with esophageal atresia (EA). To better characterize it, the normal esophageal motility is briefly reviewed with emphasis on the specific defects in EA. Multiple studies attempted to describe the dysmotility seen in patients with operated EA using esophageal manometry. Recently, high-resolution manometry has improved our understanding of normal esophageal motility. Using this new technology, it is now possible to better characterize the esophageal motility of patients operated on for EA. Three different patterns are described and presented: aperistalsis, pressurization, and distal peristalsis. Up to now, it has not been possible to find a correlation between the dysmotility severity and the patient's symptomatology. Different pathophysiological hypotheses of esophageal dysmotility in that population are discussed. Developmental neuronal defects are certainly present from the beginning. Surgical trauma can also contribute to the dysmotility. Finally, defective esophageal acid clearance capacity is a cause of gastroesophageal reflux disease, but the resultant esophagitis can also impair the normal esophageal function. The evolution of esophageal dysmotility in patients with repaired EA is not known and further studies will be necessary to clarify it.

Surgery has dramatically improved survival of infants with esophageal atresia. However, early and late complications of these surgeries affect the future life of this population. A probable step toward minimizing such complications is through modifying the technique of surgery. We evaluated two groups of esophageal atresia undergoing surgery with two different techniques including preservation and ligation of the Azygos vein and compared early complications, duration of surgery, and hospital and neonatal intensive care unit (NICU) stay between them. A total number of 24 patients with mean age of 24 to 48 h, who were diagnosed with esophageal atresia, were included in the study. All cases were randomly allocated in two groups: group A (case group) in which patients underwent surgery with preservation of the Azygos vein and group B (control group) in which patients underwent the former surgical method with ligation of the Azygos vein. Incidence of early complications, duration of surgery, and NICU and hospital stay were compared between the two groups. None of the complications occurred in either group. Duration of surgery, NICU stay, and hospital stay were not statistically significant between the groups. Preserving the Azygos vein during esophageal atresia surgery is probably a good modification of the classic technique.

Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is the most common congenital anomaly of the esophagus. The improvement of survival observed over the previous two decades is multifactorial and largely attributable to advances in neonatal intensive care, neonatal anesthesia, ventilatory and nutritional support, antibiotics, early surgical intervention, surgical materials and techniques. Indeed, mortality is currently limited to those cases with coexisting severe life-threatening anomalies. The diagnosis of EA is most commonly made during the first 24 h of life but may occur either antenatally or may be delayed. The primary surgical correction for EA and TEF is the best option in the absence of severe malformations. There is no ideal replacement for the esophagus and the optimal surgical treatment for patients with long-gap EA is still controversial. The primary complications during the postoperative period are leak and stenosis of the anastomosis, gastro-esophageal reflux, esophageal dysmotility, fistula recurrence, respiratory disorders and deformities of the thoracic wall. Data regarding long-term outcomes and follow-ups are limited for patients following EA/TEF repair. The determination of the risk factors for the complicated evolution following EA/TEF repair may positively impact long-term prognoses. Much remains to be studied regarding this condition. This manuscript provides a literature review of the current knowledge regarding EA.

Esophageal atresia (EA) affects one in 2,840 newborns, and over half have associated anomalies that typically affect the midline. After EA repair in infancy, gastroesophageal reflux (GER) and esophageal dysmotility and respiratory problems are common. Significant esophageal morbidity associated with EA extends into adulthood. Surgical complications, increasing age, and impaired esophageal motility predict the development of epithelial metaplasia after repair of EA. To date, worldwide, six cases of esophageal cancer have been reported in young adults treated for EA. According to our data, the statistical risk for esophageal cancer is not higher than 500-fold that of the general population. However, the overall cancer incidence among adults with repaired EA does not differ from that of the general population. Adults with repaired EA have had significantly more respiratory symptoms and infections, as well as more asthma and allergies than does the general population. Nearly half the patients have bronchial hyperresponsiveness. Thoracotomy-induced rib fusion and gastroesophageal reflux-associated columnar epithelial metaplasia are the most significant risk factors for the restrictive ventilatory defect that occurs in over half the patients. Over half the patients with repaired EA are likely to develop scoliosis. Risk for scoliosis is 13-fold after repair of EA in relation to that of the general population. Nearly half of the patients have had vertebral anomalies predominating in the cervical spine, and of these, most were vertebral fusions. The natural history of spinal deformities seems, however, rather benign, with spinal surgery rarely indicated.

Gastroesophageal reflux (GER) and dismotility occur frequently after repair of esophageal atresia (EA). GER-associated complications can manifest either early or later; then precocious diagnosis and treatment are essential. The aim of the study was to evaluate characteristics of GER and esophageal clearance in children treated for EA with distal tracheoesophageal fistula, using pH-multichannel intraluminal impedance (pH-MII).

Twenty-two children (ages 3-40 months) treated for EA at birth, and 20 normal children of similar age with suspected GER disease were included in the study. Impedance parameters were analyzed according to age and symptoms.

Reflux events were mainly nonacidic. About bolus exposure index, mean acid clearing time (MACT), and mean bolus clearing time (MBCT), a significant difference was found between 2 groups: the median MACT and MBCT were longer, with values of 281 and 39 seconds, respectively, in the EA group and 110 and 15 seconds in the control group (P < 0.0005).

Our data show that in young patients the majority of refluxes are not acid. This implies that the incidence of GER may be underestimated if pH-metry is used. The pH-MII is an ideal test in children because it studies both GER with its characteristics and motility pattern. The quality of reflux does not seem to influence the onset of symptoms that are related to an impaired esophageal clearance. Hence, this technique could be useful to study patients treated for EA, avoiding the onset of complications.

Esophageal replacement by biological graft is associated with a high risk of anastomotic leak-related mediastinitis.

To determine whether a self-expanding plastic stent can help avoid anastomotic leak after full-thickness replacement of the esophagus in a porcine model.

Experimental feasibility study in a porcine model.

Twelve pigs were analyzed in the study.

Replacement of a 2-cm-long segment of the cervical esophagus by an aortic allograft was performed in 12 pigs, with 6 pigs used as graft donors. Animals were divided into 2 groups depending on whether a self-expanding removable plastic stent protecting the 2 aortoesophageal anastomoses was inserted (n = 7) or not (n = 5), and were allowed to eat 24 hours postoperatively.

The relative occurrence of mediastinitis caused by anastomotic leakage in stented and nonstented groups was assessed; endoscopic evaluation and histological analysis of the graft area were performed 1 month after esophageal replacement.

All animals (n = 5) without stent insertion died of anastomotic leakage within 20 days of surgery. Two of the 7 stented animals died at day 2, and 5 survived 1 month in good clinical condition. Two stent migrations were noted. Stent extraction was followed by the development of a fibrous stricture.

The use of a self-expanding plastic stent seems to allow leak-free healing after circumferential replacement of the esophagus by a biological graft in a porcine model.

Late complications of esophageal atresia (EA), particularly esophagitis and Barrett's esophagus, are increasingly being recognized. With the exception of patients with dysphagia associated with esophageal stricture, it is unknown whether patient symptomatology can predict endoscopic findings.

Data regarding the digestive symptoms of patients who were referred to the EA multidisciplinary clinic from October 2005 to October 2008, and underwent upper gastrointestinal endoscopic evaluation, were systematically collected. Macroscopic and histological findings were analyzed. Endoscopy was considered normal if no esophagitis, intestinal metaplasia or gastric metaplasia (GM) was discerned.

Sixty-three patients underwent endoscopy. Eighteen had dysphagia related to an esophageal stricture needing dilation and were subsequently excluded from the analysis. Forty-five patients (26 girls) with a median age of 7.3 years (range 0.4 to 17.9 years) were evaluated. Twenty-six patients (58%) were normal at endoscopy, 14 patients (31%) had esophagitis and 16 patients (36%) had GM. No intestinal metaplasia or adenocarcinoma was detected. Six patients with abnormal endoscopy results were asymptomatic. No correlation between digestive symptoms and endoscopy results was found.

The present cross-sectional study showed that symptomatology was not predictive of abnormal endoscopy in EA patients. Esophagitis or GM may be discovered, even in the absence of symptoms, suggesting that physicians cannot rely solely on symptomatology to accurately evaluate the extent of these esophageal complications in this population.

The aim of the study was to compare long-term respiratory morbidity in children after repair of esophageal atresia (EA) or congenital diaphragmatic hernia (CDH).

Children were seen at 6, 12, and 24 months and 5 years within a prospective longitudinal follow-up program in a tertiary children's hospital. Respiratory morbidity and physical condition were evaluated at all moments. At age 5 years, pulmonary function and maximal exercise performance were tested.

In 3 of 23 atresia patients and 10 of 20 hernia patients, bronchopulmonary dysplasia was developed. Seventeen atresia and 11 hernia patients had recurrent respiratory tract infections mainly in the first years of life. At age 5, 25% of EA and CDH patients measured showed reduced forced expiratory volume in 1 second (z-score < -2). Both atresia and hernia patients showed impaired growth, with catch-up growth at 5 years in patients with EA but not in those with hernia. Maximal exercise performance was significantly below normal for both groups.

Esophageal atresia and CDH are associated with equal risk of long-term respiratory morbidity, growth impairment, and disturbed maximal exercise performance. Prospective follow-up of EA patients aimed at identifying respiratory problems other than tracheomalacia should be an integral part of interdisciplinary follow-up programs.

Introduction and Aim. Disorders of esophageal motility causing dysphagia and gastroesophageal reflux are frequent in survivors to esophageal atresia (EA) and distal tracheoesophageal fistula (TEF). The aim of the present study was to investigate the histologic and immunohistochemical features in both esophageal atretic segments to further understand the nature of the motor disorders observed in these patients. Material and Methods. Esophageal specimens from 12 newborns with EA/TEF and 5 newborns dead of unrelated causes were examined. The specimens were fixed in 5% buffered formalin, included in paraffin and cut in 5 micron sections that were stained with hematoxilin and eosin (H and E), and immunohistochemical stainings for Actin, S-100 protein, Neurofilament, Neuron-Specific-Enolase, Chromogranin A and Peripherin were evaluated under the microscope. Results. In controls, the distribution of the neural elements was rather homogenous at both levels of the esophagus. In contrast, the atretic segments showed quantitative and qualitative differences between them with sparser nervous tissue in the distal one in comparison with the proximal one and with controls. Conclusions. These results further support the assumption that histomorphological alterations of the muscular and nervous elements within the esophageal wall might contribute to esophageal dysmotility in patients surviving neonatal operations for EA/TEF.

To determine the pattern of feeding milestones following primary repair of long-gap esophageal atresia (EA).

A questionnaire based upon well established feeding milestones was used. Children after long-gap EA repair, n=40, were compared from after primary repair to healthy children from birth, n=102.

The age when surveyed of the EA group and controls was different: 6.2+/-4.7 (mean+/-standard deviation) years, range 1.1-20.9, versus 2.5+/-2.4 years, range 0.0-12.1, p=0.00. The esophageal gap length in the EA group was 5.1+/-1.2 cm and age at repair was 5.5+/-5.0 months. There was no statistically significant difference between the atresia group and controls for feeding milestones; Self feeding finger foods approached significance. There was, however, greater variability in the timing of milestones in the atresia group compared to controls. Feeding milestones were negatively correlated with age at primary repair: drinking with a covered sippy cup, rho=-0.51, p=0.01 and self feeding finger foods, rho=-0.36, p=0.04 were statistically significant. Drinking from a cup correlated with gestational age, rho=0.38, p=0.04, and negatively correlated to esophageal gap length, rho=-0.45, p=0.01.

Despite delayed onset of feeding, major milestones after EA repair occurred in similar pattern to normal infants. An early referral for primary repair is beneficial for earlier acquisition of milestones for infants with long-gap EA.

Survivors of esophageal atresia are reaching their adulthood in large numbers for the first time enabling assessment of true long-term outcome among this group of patients. This review summarizes the current knowledge on the subject focusing on late symptoms and complications, esophageal pathology and pulmonary function. Relationships between esophageal dysmotility, gastroesophageal reflux, esophagitis and epithelial metaplastic changes including esophageal cancer are outlined. In addition to pertinent literature, institutional experience, and follow-up of patients with esophageal atresia for more than 60 years is included.

After repair of esophageal atresia with distal fistula (EADF), anastomotic dilatations are often required. We abandoned routine dilatations (RD), in 2002, for selective dilatations (SD) only when the symptoms arose. We compared the number of dilatations and long-term results after RD and SD. Eighty-one successive EADF patients from 1989 to 2007 (RD 46, SD 35), with primary anastomosis, native esophagus, and peroral feeding, were included. Spitz classification, birth weight, gestational age, incidence of gastroesphageal reflux, tracheomalacia, and postoperative complications did not differ statistically significantly between the groups whereas the total incidence of associated anomalies in RD group was higher than in SD (P < 0.05) In RD group, anastomotic dilatations were begun 3 weeks postoperatively and repeated until the anastomotic diameter was 10 mm. In SD group, dilatations were performed only in symptomatic patients. The number of dilatations, dilatation-related complications, nutritional status, and outcome up to 3 years after repair were compared. The median number of dilatations was seven (2-23) in RD and two (0-16) in SD group (P < 0.01). Sixteen (46%) patients in SD group had no dilatations during the first 6 months. The incidence of dysphagia, bolus obstructions, and development of nutritional status were similar between the groups. The incidence of complications/dilatation was 0.6% in RD and 1.0% in SD group. One patient in RD group underwent resection for a recalcitrant anastomotic stricture. After repair, EADF policy of SD resulted in significantly less dilatations than RD with equal long-term results.

The aim of this study was to evaluate oesophageal function after correction of oesophageal atresia in adults, and to investigate the association between complaints, oesophageal function and quality of life (QoL). Twenty-five adults were included who participated in previous follow-up studies, during which complaints of dysphagia and gastro-oesophageal reflux (GOR), results of upper gastrointestinal endoscopy, oesophageal biopsies and QoL had been collected. Manometry was performed in 20 patients, 24 h pH-measurements were performed in 21 patients. pH-values (sample time 5 s) were calculated using criteria of Johnson and DeMeester. Associations were tested with ANOVA and chi (2)-tests. Ten patients (48%) reported complaints of dysphagia, seven (33%) of GOR. The amplitude of oesophageal contractions was low (<15 mmHg) in four patients (20%). pH-measurements showed pathological reflux in three patients (14%). Patients reporting dysphagia more often had disturbed motility (P = 0.011), and lower scores on the domains "general health perceptions" (SF-36) (P = 0.026), "standardised physical component" (SF-36) (P = 0.013), and "physical well-being" (GIQLI) (0.047). No other associations were found. This study shows a high percentage of oesophageal motility disturbances and a moderate percentage of GOR after correction of oesophageal atresia. Patients reporting dysphagia, whom more often had disturbed motility, seemed to be affected by these symptoms in their QoL.

We evaluated the outcome in infants with esophageal atresia (EA) treated in our department over the last two decades. The medical records of 147 infants treated from 1986 to 2005 were reviewed. Patient characteristics, associated anomalies, surgery and complications were recorded. We divided the material into two time-periods: 1986-1995 and 1996-2005; 125 patients or parents were interviewed regarding gastrointestinal function, respiratory symptoms and education. The incidence of major cardiac defects increased from 23 to 29% and the overall survival increased from 87 to 94%. Using Spitz' classification survival increased from 93.5 to 100% in group I and from 68.4 to 77.8% in group II. In group III, during the second time period, survival was 100% in three patients. The incidence of anastomotic leakage and recurrent fistula did not change over time. The rate of anastomotic strictures increased from 53 to 59% between the two time-periods. A primary anastomosis could be done in 85% of the patients during the second period versus 78% of the patients during the first period. Anti-reflux surgery was done in only 11 and 9%, respectively, during the two time-periods. In patients who were 16-20 years old, 40-50% had gastrointestinal and respiratory symptoms. Ninety percent of the patients attended normal school. The major difference between the periods 1986-1995 and 1996-2005 was an increased survival despite an increased incidence of major cardiac defects. Gastrointestinal and respiratory symptoms were frequent. Long-term follow-up and treatment of complications of esophageal atresia is important for this patient group.

Esophageal atresia and tracheo-esophageal fistula (EA + TEF) are often associated with malformations of neural crest origin. Esophageal innervation is also derived from the neural crest and it is abnormal in EA + TEF in which there is motor dysfunction. Our aim was to examine the intrinsic esophageal innervation in children with isolated EA in which different embryogenic mechanisms might be involved. Specimens from the proximal and distal esophageal segments of 6/35 patients who had esophageal replacement for isolated EA between 1965 and 2006 were suitable for the study. They were sectioned and immunostained with anti-neurofilament (NF) and anti-S-100 antibodies. The muscle and neural surfaces on each section were measured with the assistance of image processing software. The surface of the ganglia and the number of neurons per ganglion were determined at high power microscopy. The findings were compared with those of six autopsy specimens from newborns dead of other diseases by means of standard statistical tests and a significance threshold of P < 0.05. Unmatched age/size of babies in isolated EA and control groups precluded comparison of the relative surfaces occupied by neural elements. Patients with pure EA had denser fibrilar network and larger ganglia than controls. The number of neurons/ganglion were similar in both groups although the cells from EA patients were larger. The findings were similar at both esophageal levels studied. In spite of methodologic biases, it seems that intrinsic esophageal fibrilar network is denser and the intramural ganglia larger with larger cells in patients with pure EA than in controls on both esophageal ends of the organ. These neural anomalies are only in part reminiscent of those described in regular EA/TEF but may as well explain esophageal dysfunction in patients with repaired isolated EA.

The objective of this study is to investigate the characteristics of esophageal motor activity responsible for the development of gastroesophageal reflux (GER) in patients with esophageal atresia (EA).

The subjects consisted of 29 patients with EA (1 month to 19 years). Computerized esophageal manometry was conducted to investigate esophageal contractions at swallow. A topographic esophageal manometric analysis was conducted in each subject, providing 3-dimensional displays to reveal the pressure continuum representing esophageal contractions.

Significant contractions in the middle esophagus just below the anastomosis were absent in all subjects. Contractions in the distal esophagus were conspicuously absent in 17 subjects. Of these 17, 6 had already undergone fundoplication, and 9 had symptomatic GER requiring fundoplication subsequent to this study. The remaining 12 patients had contractions in the distal esophagus and did not require medical/surgical intervention. A lack of distal esophageal contractions was significantly correlated with the development of GER (P < .001). There was a significant difference in esophageal acid exposure between the 2 groups (median, 38% vs 4%, P < .001).

Lack of distal esophageal contractions indicating an impaired clearing capacity is considered a potential key determinant of GER in patients with EA.