Esophageal dysmotility is common in patients with esophageal atresia (EA). High-resolution impedance manometry and pressure flow analysis (PFA) allow characterization of biomechanical events that drive bolus flow. The aims were to assess esophageal motility in children with EA, using PFA, and to test whether there is a correlation between PFA parameters and symptoms or endoscopic/histologic findings.

High-resolution impedance manometry was performed in 16 children with EA (median age 11 years), compared with 13 patient controls (median age 14 years; P = NS vs patients). Wet swallows were analyzed using PFA. Medical charts were reviewed for symptoms and pathology results of the attendant esophagoscopy. Patients with EA were arbitrarily subgrouped according to their motility pattern: group A with presence of distal contraction in ≥50% of the swallows and group B with presence of distal contractions in <50% of the swallows.

Esophageal peristaltic motor patterns were abnormal in all patients with EA. Bolus transport was impaired as shown by the higher impedance ratio in EA than in controls (0.47 vs 0.22; P < 0.001). Impedance ratio was also higher in group B (n = 8) versus group A (n = 8) (P < 0.001). Symptoms of dysphagia were not correlated with the PFA measures. Contractile segment impedance, a marker of mucosal integrity, was significantly lower in the EA group.

Bolus transport was severely altered in patients with EA but was not predictive of symptoms. The presence of residual distal contractions is associated with a more efficient bolus propulsion.

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.

This relationship between gastroesophageal reflux and airway disorders is complex, possibly bidirectional, and not clearly defined. The tools used to investigate gastroesophageal reflux are mostly informative about involvement of gastroesophageal reflux within the gastrointestinal tract, although they are often utilized to study the relationship between gastroesophageal reflux and airway issues with are suspected to occur in relation to reflux. These modalities often lack specificity for reflux-related airway disorders. Co-incidence of gastroesophageal reflux and airway disorders does not necessarily infer causality. While much of our focus has been on managing acidity, controlling refluxate is an area that has not been traditionally aggressively pursued. Our management approach is based on some of the evidence presented, but also often from a lack of adequate study to provide further guidance.

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.

Despite acute respiratory and chronic respiratory and gastro-intestinal complications, most infants and children with a history of oesophageal atresia / trachea-oesophageal fistula [OA/TOF] can expect to live a fairly normal life. Close multidisciplinary medical and surgical follow-up can identify important co-morbidities whose treatment can improve symptoms and optimize pulmonary and nutritional outcomes. This article will discuss the aetiology, classification, diagnosis and treatment of congenital TOF, with an emphasis on post-surgical respiratory management, recognition of early and late onset complications, and long-term clinical outcomes.

A retrospective study was performed to evaluate the functional disorders of deglutition with videofluoroscopy (VFS), in children operated for esophageal atresia (EA) and/or tracheoesophageal fistula (TEF).

Patients with the repair of EA-TEF were evaluated in respect to the type of malformation, operative procedure, postoperative complications, deglutitive and respiratory symptoms, and gastroesophageal reflux disease (GERD). The dysphagia score, VFS findings of oral, pharyngeal and esophageal phases, and penetration-aspiration scale (PAS) score were recorded in the evaluation of the deglutitive functions.

Thirty-two cases with a median age of 48months (2-120months), and male to female ratio of 14:18 were included in the study. Most of the cases had Gross type C anomaly (n=26, 81.3%), and the others were type A (n=3), D (n=2) and E (n=1). The incidence of associated anomalies was 71.8%. The patients underwent primary (n=26, 81.3%) or delayed (n=6, 18.7%) anastomosis. Postoperative complications including anastomotic stricture (n=12), leak (n=2) and recurrent fistula (n=2) were managed by dilatation, conservative approach and repair of the fistula, respectively. Recurrent pneumonia (n=13), cough with liquid intake (n=10) and food impaction (n=7) were recorded in the history. Management of GERD included medical (n=11) and surgical (n=7) treatment. The median dysphagia score was 3.5 (min: 0-max: 27). The oral phase of VFS was normal in most of the cases (n=29, 90.6%). Only three had mild or moderate impairment, and none had severe. The pharyngeal phase showed no impairment in 23 of the cases (71.8%), and severe impairment was observed only in 3 of all, for the parameters of hyolaryngeal elevation and airway closure. Opposite to the first two phases of the deglutition, the esophageal phase was normal in only 2 of the cases (6.3%). Among the other 30 cases with impairment, only two had mild, and the rest had moderate to severe problems. Esophageal backflow, motility and residue were the most severely impaired parameters of this phase. The PAS evaluation revealed no penetration and aspiration in 26 of the cases (81.3%), while 1 had penetration and five had aspiration.

The patients with repaired EA-TEF may reveal deglutitive and respiratory symptoms in follow-up, necessitating certain investigations. The deglutition is functionally evaluated with VFS. While the disorders of oral and pharyngeal phases are less frequent and prominent, the esophageal phase reveals disorders with higher incidence and severity.

There is variation in the management of postoperative gastroesophageal reflux (GER) in esophageal atresia-tracheoesophageal fistula (EA-TEF). Well-reported literature is important for clinical decision-making. We assessed the quality of reporting (QOR) of postoperative GER management in EA-TEF.

A comprehensive search of MEDLINE, EMBASE, CINHAL, CENTRAL databases and gray literature was conducted. Included articles reported a primary diagnosis of EA-TEF, a secondary diagnosis of postoperative GER, and primary treatment of GER with antireflux medications. The QOR was assessed using the STrengthening the Reporting of OBservational studies in Epidemiology (STROBE) checklist.

Retrieval of 2910 articles resulted in 48 relevant articles (N=2592 patients) with an overall quality percentage score of 48%-95% (median=65%). The best reported items were "participants" and "outcome data" (93.8% each), "generalisability" (91.7%) and "background/rationale" (89.6%). Less than 20% of studies provided detailed "main results"; less than 5% of studies reported adequately on "bias" or "funding." Sample size calculation and study limitations were included in 17 (35.4%) and 16 (33.3%) studies respectively. Follow-up time was inconsistently reported.

Although the overall QOR is moderate using STROBE, important areas are underreported. Inadequate methodological reporting may lead to inappropriate clinical decisions. Awareness of STROBE, emphasizing proper reporting is needed.

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.

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.

A wide spectrum of variations can occur in type IIIb esophageal atresia and distal tracheoesophageal fistula [EA-TEF] (Kluth D. Atlas of esophageal atresia. J Pediatr Surg 1976; 11 (6):901-19). The aim of this study was to evaluate the variant anatomy of subtype IIIb4 EA-TEF and its therapeutic implications.

We performed a retrospective review of 4 patients of this variant subtype within a series of 259 esophageal atresias, managed over a study period of three years, at our institution. The diagnosis was made at thoracotomy. The overlapping upper and lower esophageal pouches, initially, gave a false impression of esophageal continuity. The long TEF coursed parallel in proximity to the upper pouch (UP) and the trachea needing meticulous dissection. The TEF measured 8-10mm in diameter and was ligated and divided high on the trachea near the thoracic inlet. The patients were followed up by a contrast swallow, radioactive technetium-99m- sulfur colloid scan, echocardiography and bronchoscopy (if indicated).

The mean birth weight and gestational age was 2250 g and 38 weeks respectively. The mean follow up was eighteen months (range 5-42 months). An associated anorectal malformation was present in two patients while none had associated congenital heart disease. The Waterston grading was A and B in one patient each and C in two patients. All the patients had muscular UP extending up-to the level of arch of azygous vein (T(3-4)). The lower esophageal pouch (LP) and the fistula extended high, near the thoracic inlet. Tracheal injury occurred in one patient, with thin LP, warranting postoperative elective ventilation. A postoperative barium swallow did not reveal anastomotic leak or stricture in any patient. Follow-up barium swallow showed long LP extending high up in the chest, up to clavicles in all four patients and redundancy of UP in case 1. Severe gastroesophageal reflux was present in two patients. Bronchoscopy revealed tracheomalacia in case 1. Three patients were thriving well at the last follow-up. One patient died at five months of age due to aspiration pneumonia.

The knowledge of diagnostic and therapeutic implications of the variant subtype IIIb4 EA-TEF should be in the armamentarium of the treating surgeon to reduce pitfalls in its management.

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.

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.

Gastroesophageal reflux (GER) is almost constant in esophageal atresia and tracheoesophageal fistula (EA/TEF). These patients resist medical treatment and require antireflux surgery quite often. The present review examines why this happens, the long-term consequences of GER and the main indications and results of fundoplication in this particular group of patients. The esophagus of EA/TEF patients is malformed and has abnormal extrinsic and intrinsic innervation and, consequently, deficient sphincter function and dysmotility. These anomalies are permanent. Fifty percent of patients overall have GER, and one-fifth have Barrett's metaplasia. Close to 100%, GER of pure and long-gap cases require fundoplication. In the long run, these patients have 50-fold higher risk of carcinoma than the control population. GER in EA/TEF does not respond well to dietary, antacid, or prokinetic medication. Surgery is necessary in protracted anastomotic stenoses, in pure and long-gap cases, and when there is an associated duodenal atresia. It should be indicated as well in other symptomatic cases when conservative treatment fails. However, confection of a suitable wrap is anatomically difficult in this condition as shown by a failure rate of 30% that is also explained by the persistence for life of the conditions facilitating GER.

Gastroesophageal reflux (GER) is frequently observed in children treated for congenital diaphragmatic hernia (CDH) at birth, as well as esophageal dysmotility, that has been hypothesized to be caused by innervatory anomalies. The aim of this study is to evaluate GER and dysmotility in young patients with CDH using pH-multichannel intraluminal impedance.

Thirty children (17 boys and 13 girls) who underwent repair for CDH between 2002 and 2007 with a median age of 5.2 years (range, 3-10 years) were included in the study. All patients were operated on with a subcostal laparotomy incision and had a left-sided diaphragmatic defect. The defect repair required an artificial patch (Goretex, Gore Medical, Flagstaff, AZ) in 8 patients (27%) because of its size. We described impedance reflux parameters and some specific motility parameters studied on 10 standardized swallows.

The incidence of GER was 86%. Reflux was mainly nonacidic, postprandial, and short-term and reached only the distal esophagus. Esophageal dysmotility was observed only in the distal esophagus.

With the use of pH-multichannel intraluminal impedance, both GER and esophageal motility in patients with congenital malformations can be analyzed. In patients with CDH, impaired motility seems to involve only the distal esophagus. In this group, the specific pattern of reflux is probably caused by the involvement of gastroesophageal junction, without significant intrinsic innervation abnormalities as observed in patients with esophageal atresia.

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.

Gastroesophageal reflux (GER) and dysmotility are frequent in patients treated for esophageal atresia (EA). This aim of this study is to evaluate GER and dysmotility in young EA patients using pH-multichannel intraluminal impedance (pH-MII).

Fifteen patients with a mean age of 7.5 years (group 1) have been studied and compared with 15 children without congenital malformation, submitted to pH-MII for suspected GER (group 2). These latter patients serve as a control group of healthy subjects. The following impedance reflux and motility parameters have been studied on 10 standardized swallows: number of reflux episodes, mean acid clearing time, median bolus clearing time, bolus presence time, total bolus transit time, segmental transit time, and total propagation velocity.

In the group of EA patients, mean acid clearing time and median bolus clearing time were pathological. In the control group, all reflux parameters were normal. Patients with EA had significantly longer median bolus presence time at each measuring site, median total bolus transit time, and median segmental transit time and slower total propagation velocity (P < .001).

pH-multichannel intraluminal impedance evaluates both GER and motility patterns. Our report studies impedance parameters of esophageal motility in healthy children and in EA patients using only pH-MII.

Gastroesophageal reflux disease and impaired esophageal motility is a frequent problem after repair of esophageal atresia (EA). Combined multichannel intraluminal impedance (MII) and pH measurement identifies acidic and weakly acidic esophageal bolus movements. Our aim was to evaluate gastroesophageal reflux and its association with gastrointestinal or extraintestinal symptoms using combined MII-pH measurement.

In all, 24 patients (4 months-23 years; 17 male, 7 female) with repaired EA were included in this study. A single catheter with 6 impedance channels and 1 pH channel was used to perform the 24-hour MII-pH study. Symptoms were recorded during the study as events and with a standardized questionnaire. In a subgroup of patients, an impedance swallowing test evaluating liquid and viscous bolus movements was performed.

We detected a total of 911 episodes of retrograde bolus movement, 379 acidic and 532 weakly acidic bolus movements. In all, 201 symptom events were recorded. Of these events, 42% were associated with retrograde bolus movements. More symptom events were associated with weakly acidic reflux (26%) than with acid reflux episodes (16%). There was a poor correlation between symptoms and MII-pH study findings. In comparison with patients without esophageal surgery, EA patients showed significantly fewer complete swallows of liquid (42% vs 98%) and viscous material (18% vs 87%).

In patients with corrected EA, half of the reflux events could be detected only by MII. We also demonstrated that weakly acidic reflux can be responsible for the patients' symptoms. However, patients may have few or no symptoms despite poor esophageal function and extensive gastroesophageal reflux disease.

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.

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.

Fundoplication is the standard surgical approach to gastroesophageal reflux (GER) in a child. Although successful in many patients, there is a significant risk of complications and failure, especially in high-risk patients such as those with certain types of associated anomalies, diffuse motility disorders, chronic pulmonary disease, neurologic impairment, and young infants. Fundoplication failure can take the form of persistent reflux-related symptoms, symptoms that are caused by complications of the surgery, or anatomic problems such a para-esophageal hernia or migration of the wrap into the mediastinum. The most effective strategy for treatment of the child undergoing fundoplication is to prevent failure by careful patient selection, individualization of the operation based on the patient's anatomy and physiology, and meticulous attention to the technical details of the operation. Options for the child with a failed fundoplication include medical management, jejunal feeding using a percutaneous tube or a Roux-en-Y jejunostomy, revision of the fundoplication, or esophagogastric dissociation. If the fundoplication is to be revised, the same principles of patient selection, individualization of the operation, and attention to technique must be used to optimize the chance of success. The primary goal in the treatment of GER is to improve quality of life for the patient and the family.

Gastro-oesophageal reflux (GOR) is a major cause of morbidity in children who undergo surgical repair for oesophageal atresia with tracheo-oesophageal fistula (OA/TOF). We performed a retrospective analysis to determine the incidence of GOR on radionuclide scintigraphy in symptomatic and asymptomatic OA/TOF patients in the first post-operative year. A total of 124 patients (74 males, 50 females), with a mean age of 3.5 months (range, 20 days to 12 months), were studied. Of these 124 patients, 67 were symptomatic and 57 were asymptomatic. On radionuclide scintigraphy, 73 patients (48 symptomatic and 25 asymptomatic) had reflux. Of the 48 symptomatic patients with scintigraphic studies positive for reflux, 79.2% (38) had proximal reflux and 20.8% (10) had distal reflux, whereas, of the 57 asymptomatic patients, 48% (12) had proximal reflux and 52% (13) had distal reflux. There was a significantly higher incidence of GOR in symptomatic children than in asymptomatic children (P<0.01). In particular, there was a significantly higher incidence of proximal GOR in symptomatic children than in asymptomatic children (P<0.001). In conclusion, the severity and incidence of GOR were significantly higher in symptomatic than asymptomatic OA/TOF patients in their first post-operative year. Scintigraphic evidence of proximal reflux correlates with the presence of symptomatic GOR.