Esophageal Atresia Patients Research Articles

Application of magnetic compression anastomosis technique in pediatric surgery

Malformation of digestive tract is common in neonates. Esophageal atresia (EA) comprises a group of congenital abnormalities defined by discontinuity of the esophagus. EA with communication with the trachea is known as tracheoesophageal fistula (TEF). For the repair of EA, it is needed to establish an anastomosis between two structures that may be significantly spaced apart, such as in long-gap EA (with a gap >3 cm). The repair across a long-gap can induce tension on the anastomosis, leading to dehiscence, anastomotic leak, or procedure failure. In neonatal patients with long-gap EA with TEF, the complications of significant gastroesophageal reflux, or anastomotic leaks and severe refractory esophageal stricture after surgery are common, which remains challenging for pediatric surgery. There are multiple options for lengthening and approximating the ends in long-gap EA such as myotomies, Gough flap, or the Foker technique. The treatment of long gap EA usually requires thoracotomy or thoracoscopy to place traction sutures on each esophageal in multiple stage operations, the treatment period of which is long. Esophageal stricture is the most common complication following EA repair. Refractory esophageal anastomotic stricture after repair of EA in infants is a challenging surgical problem. In general, these strictures can be successfully managed using endoscopic techniques including bougie and balloon dilation, stenting, and chemotherapeutic agents. If these techniques are unsuccessful, treatment options are limited to surgical resection of the strictured segment and reanastomosis, or esophageal replacement. Magnetic compression anastomosis (MCA) has been introduced for several decades. Recently, rare-earth magnets have been employed to create solid anastomosis for long-gap EA and refractory esophageal stricture in children. In MCA, a sutureless anastomosis of the gastrointestinal tract can be formed using two magnetic Harrison rings. Magnetic compress has been shown to be effective for the treatment of long-gap EA in children. MCA can be conducted during the same stage surgery of ligating the TEF with customized magnetic rings. Under the magnetic force, the two magnetic rings pull along the gastric tube to achieve anastomosis. Once the postoperative permanent suction of these two pouches is instituted, spontaneous growth can be awaited. MCA has been shown to be effective in anastomosis of stomach, small bowel and colon in both experimental and clinical cases. The magnets are most commonly made of neodymium-boron or samarium-cobalt, which can display strengths significantly greater than those of ferrite. Magnetic compression has been used to treat biliary strictures in adult patients who have undergone liver transplantation, as magnetic connectors for coronary surgery, and for the functional undiversion of ileostomy in pediatric patients. Reports also describe the use of magnetic compression in restoring patency of anastomotic strictures of the alimentary tract in patients who have undergone surgery for esophageal, gastric, and rectal cancer. Yamanouchi and colleagues have published many papers on their experience with Samarium cobalt magnets. They even used them to treat recurrent anastomotic strictures after initial MCA. It has been reported that in conditions such as congenital oesophageal atresia or rectal atresia in children, and also is a safe, minimally invasive way to gradually refunctionalize the excluded distal bowel after previous diverting ostomy. MCA using mated magnets with their interposed compressed tissue may result in serosa-to-serosa apposition. At present, MCA for the treatment of EA patients is restricted to gross type A (without TEF) and anastomotic stenosis without thoracotomy. Magnamosis offers a minimally invasive technique for repair of this congenital anomaly. This is particularly helpful in those patients who cannot undergo initial or repeat thoracotomy or thoracoscopy due to anatomic considerations or overall severity of illness. Translumenal digestive tract anastomosis using the constant magnetic field is reliable and feasible as a minimally invasive technique to treat congenital esophageal and anorectal abnormalities. Magnamosis is technically feasible for esophago-esophageal anastomoses. In anorectal malformations with favorable anatomy, this procedure may avoid an operative repair such as posterior sagittal reconstruction. We propose that magnamosis can be considered as an alternative for the treatment of rectal atresia in certain children.

Fixed the gap, solved the problem? Eating skills in esophageal atresia patients at 3years.

Although eating problems have been described as long-term morbidities of esophageal atresia (EA), there have been few studies exploring eating outcomes in children born with EA as primary aim. Parents of children operated on for EA in our Institution from January 2012 to January 2016, answered a telephone structured interview developed specifically to conduct the present study, assessing eating skills at 3 years of age. Clinical data were collected from children's medical records. Parents (45 mothers and 6 fathers) of 51 children (male = 34; female = 17) with a median age of 3.5years form the object of the study. Considering eating problems, parents reported that 23 children (45%) still have episodes of choking during meals at 3 years of age, 9 (45%) of these have more than one episode a week, and 19 parents (39%) reported higher levels of anxiety during mealtimes. Forty-four children (86%) were described by their parents as able to eat alone, 32 (65%) accepted all food textures and 45 (90%) was described as curious about food (3 years). Forty-three (86%) parents let their children eat with other people. Correlations showed that weaning age was significantly associated with number of dilatations (rs = 0.35, P = 0.012), days of mechanical ventilation (rs =0.40, P < 0.001), and presence of gastrostomy tube at discharge (rs = 0.45, P < 0.001). Chewing age resulted associated with number of dilatations (rs = 0.34, P < 0.01) and days of mechanical ventilation (rs = 0.38, P < 0.01). Presence of choking episodes was associated with curiosity about food (rs = 0.29, P < 0.05), while frequent choking episodes were associated with higher parental anxiety during mealtimes (rs = 0.45, P < 0.05). In order to prevent delay in the achievement of eating developmental milestones in children operated on of EA, we advocate a dedicated preventive intervention from birth to follow-up.

First-Year follow-up of Newborns Operated for Esophageal Atresia in a Developing Country: Just Operating is not Enough!

Purpose:To identify complications, their incidence and risk factors for their occurrence in patients of esophageal atresia (EA) in the 1st year after discharge following surgery.Materials and Methods:Cases of EA discharged after surgical intervention in the period of July 2011–July 2013 were considered a cohort. All data regarding demographics, investigations, surgical procedure, outcome, and follow-up were recorded.Results:Seventy-six such patients were discharged in the study period, six of whom were lost to follow-up, and hence, seventy patients were included in the study. Of these 70, 48 (69%) had esophageal continuity restored (46 EA + tracheoesophageal fistula [TEF]; 2 pure EA), while 22 (31%) had been diverted (3 pure EA; 8 EA + TEF following major leak; 11 long gap EA + TEF). Risk of developing any complication (except death) was 48/70 (68%; 95% confidence interval [CI] = 57.4–79.7). Twenty-six of 48 patients with esophageal continuity restored, demonstrated narrowing on contrast study (54%; 95% CI = 39.5–68.7) but only 18 of these 48 (37.5%) had dysphagia. Thirty-one of seventy had an episode of lower respiratory tract infection (LRTI) (44.2%; 95% CI = 32.3%–56.2%). Poor weight gain was observed in 27/70 (37%), and this was significantly common in diverted patients (63% vs. 25%; P = 0.009). Twenty-one of total 70 (30%) patients died within the 1st year following discharge.Conclusions:Sixty-eight percent of cases developed some complication, while 30% succumbed within the 1st year of life following discharge. The common complications were stricture, LRTI, and poor weight gain. All of these were common in diverted patients.

Transhiatal isoperistaltic colon interposition without cervical oesophagostomy in long-gap oesophageal atresia.

Background:Oesophageal colonic interposition in oesophageal atresia (OA) patients is almost exclusively done as a staged operation with an initial oesophagostomy and gastrostomy followed by the definitive surgery months later. This study presents a series of patients in whom a cervical oesophagostomy was not performed before the substitution surgery.Patients and Methods:Records of EA patients were evaluated for those who underwent colon interposition without cervical oesophagostomy.Results:There were five patients: three with pure EA and two with proximal tracheo-oesophageal fistula. A delayed primary repair could not be performed because of intra-abdominally located distal pouch. The mean age at the time of definitive operation was 5.54 (±2.7) months and the mean weight was 6.24 (±1.3) kg. A right or a left colonic segment was used for interposition keeping the proximal anastomosis within the thorax. The post-operative results were quite satisfactory within a median follow-up period of 33.2 months.Conclusion:Avoiding cervical oesophagostomy and its inherent complications and drawbacks is possible in a subset of patients with long-gap EA who underwent colonic substitution surgery. This approach may be seen as an extension of the consensus that the native oesophagus should be preserved whenever possible, because it uses the native oesophagus in its entirety.

Quality of life assessment in esophageal atresia patients: a systematic review focusing on long-gap esophageal atresia

BackgroundChildren born with esophageal atresia (EA) have inherent abnormalities in esophageal motility which may impact upon patient and family Quality of Life (QoL). Currently, paucity of data exists for long-term outcomes of long-gap EA. We aimed to: (1) summarize QoL tools reported in the literature, focusing upon studies involving long-gap EA patients, and (2) compare QoL for long-gap versus non-long-gap EA patients. MethodWe performed a systematic review of Cochrane Register of Controlled Trials, PubMed, EMBASE, and Ovid databases (January 1980–May 2018) in accordance with the PRISMA protocol. ResultSix studies were identified (536 patients total), and 419/536 (78%) patients completed QoL assessment. Response rates ranged from 29% to 100%. Median study size was 86 (range 8–159). Esophageal atresia type was described in 477 patients, and 74/477 (16%) were long-gap. Common assessment tools were Gastrointestinal Quality of Life Index and 36-Item Short-Form Health Survey. Compared with healthy individuals, long-gap EA patients suffered more gastrointestinal symptoms. There were no significant differences in QoL outcomes between long-gap and non-long-gap EA patients. ConclusionCurrent literature suggests no significant difference in QoL outcomes between long-gap and non-long-gap EA patients. However, due to questionnaire variability and range of response rates, the data should be interpreted with care. Level of EvidenceLevel II.

A prospective study outcome of surgical intervention of EA and TOF in neonate coming to paediatric surgery division of general surgery at my hospital, Indore

Background: Esophageal atresia (EA) with or without Tracheoesophageal fistula (TEF) is most common congenital anomalies encountered in pediatric surgery and the management of these neonates require high degree of skill, manpower and tertiary care. This clinical study was undertaken to evaluate the outcome and postoperative complication of Esophageal atresia (EA) with or without Tracheoesophageal fistula (TEF) admitted in pediatrics surgery in M.Y. Hospital Indore.Objective: The aims of this study cases of EA and TEF regarding management and outcome.Methods: This prospective study included 220 patients of EA with TEF were enrolled in this study from Sep. 2016 to august 2018 who underwent surgical intervention. Their clinical profile, time of presentation, sex distribution, birth weight, type of EA and TEF and their effect on mortality and morbidity were observed.Results: Mortality was high (68.42%) among patients who reached the tertiary centre late i.e. >24 hours. The incidence of low birth weight babies is approximately 74% and mortality rate increased with low birth weight. EA with or without TEF More common in male then female but survival rate was high in female (71.60%). Type C was the most common (71.42) followed by type A. Septicemia and leak were most common complication. The mortality in septicemia patients is high 90.66%. Early surgical intervention improved the survival of EA and TEF. The overall survival rate was 48.63% and mortality was 51.36%. Conclusions: Esophageal atresia (EA) with Tracheoesophageal fistula (TEF) is the most common type of anomaly. Maturity, birth weight, early diagnosis are the most important factors that affect the outcome. Remarkable success has been achieved based on improvement in neonatal anesthesia & surgical technique, ventilator support & neonatal intensive care.

Use of Magnets as a Minimally Invasive Approach for Anastomosis in Esophageal Atresia: Long-Term Outcomes.

Introduction: The majority of esophageal atresia (EA) patients undergo surgical repair soon after birth. However, factors due to patient characteristics, esophageal length, or surgical complications can limit the ability to obtain esophageal continuity. A number of techniques have been described to treat these patients with "long-gap" EA. Magnets are a nonsurgical alternative for esophageal anastomosis. The purpose of this study was to report long-term outcomes for the use of magnets in EA. Materials and Methods: Between July 2001 and December 2017, 13 patients underwent placement of a magnetic catheter-based system under fluoroscopic guidance at six institutions. Daily chest radiographs were obtained until there was union of the magnets. Magnets were then removed and replaced with an oro- or nasogastric tube. Complications and outcomes were recorded. The average length of follow-up was 9.3 years (range 1.42-17.75). Results: A total of 85% of the patients had type A, pure EA, and 15% had type C with previous fistula ligation. The average length of time to achieve anastomosis was 6.3 days (range 3-13). No anastomotic leaks occurred, and all of the patients had an expected esophageal stenosis that required dilation given the 10F coupling surface of the magnets (average 9.8, range 3-22). Six patients (46%) had retrievable esophageal stents, and two underwent surgery; yet all maintained their native esophagus without interposition. A total of 92% were on full oral feeds at the time of follow-up. Conclusion: The use of magnets for treatment of long-gap EA is safe and feasible and accomplished good long-term outcomes. The main complication was esophageal stricture, although all patients maintained their native esophagus. A prospective observational study is currently enrolling patients to evaluate the safety and benefit of a catheter-based magnetic device for EA.

The repair of esophageal atresia and major complications—a systematic review and our experience in dealing with the tracheoesophageal fistula

Esophageal atresia (EA) is a rare and complicated congenital malformation. Since it was first reported, it has had a long history. Nowadays, standard treatment options consist of open procedure and thoracoscopic surgery. Each procedure has its benefits and defects, and the relative merits of both methods are still debated. We conducted a systematic literature review on the surgical repair of the EA, from the first descriptions of EA with tracheoesophageal fistula (TOF) appeared in the 19th century to 2018. The opening and the laparoscopic surgery are the two effective methods to deal with the EA, and they all have their benefits and some aspect to be improved. The main complications for the operation were anastomotic leakage, anastomotic stenosis and recurrence of TOF. Results indicate that the patients of opening surgery were more likely to have leakage, but these patients also showed a lower stenosis rate than the thoracoscopy patients. Meanwhile, the rate of recurrence for TOF had no statistical difference between the procedure types. Thoracoscopic surgery was beneficial and promoted a good prognosis in EA patients when compared to open surgery, although there is still more research to be done in this regard.

Great vessel anomalies and their impact on the surgical treatment of tracheobronchomalacia

BackgroundTracheobronchial compression (TBC) from great vessel anomalies (GVA) can contribute to tracheobronchomalacia (TBM) symptoms. The frequency, impact on symptoms and optimal management of GVA in these patients, with or without a history of esophageal atresia (EA), are still unclear. Study designPatients who underwent surgery for TBM/ TBC between 2001 and 2017 were reviewed. Demographics, type of GVA, and operative interventions were collected. The frequency and treatment modalities of GVA between EA and non-EA patients were compared. ResultsOverall, 209 patients met criteria; 120 (57.4%) patients had at least one GVA, including double aortic arches (n = 4, 1.9%), right aortic arches (n = 14, 6.7%), aberrant right subclavian arteries (n = 15, 7.2%), and innominate artery compression (n = 71, 34.0%). Non-EA patients were more likely to have surgery later in life (29.5 months versus 16 months, p = 0.0002), double aortic arch (p = 0.0174), right aortic arch (p < 0.0001), and undergo vascular reconstruction concurrently with their airway procedure (25% vs 8.4%, p = 0.002). Vessel reconstruction was performed in 25 patients; six required cardiac bypass. ConclusionThe frequency of GVA in patients with symptomatic airway collapse is substantial. Multidisciplinary evaluation is imperative for operative planning as many require complex reconstruction and collaboration with cardiac surgery, particularly patients without a history of EA. Level of evidenceLevel III.

Esophagitis in Pediatric Esophageal Atresia: Acid May Not Always Be the Issue.

Esophagitis is highly prevalent in patients with esophageal atresia (EA). Peptic esophagitis has long been assumed to be the primary cause of esophagitis in this population, and prolonged acid suppressive medication usage is common; such treatment is of unknown benefit and carries potential risk. To better understand the role of commonly used antireflux treatments in EA, we analyzed all patients with repaired EA who underwent endoscopy with biopsies at our institution between January 2016 and August 2018. Macroscopic erosive and histologic esophagitis on biopsy was graded per predefined criteria. Clinical characteristics including acid suppressive medication usage, type of EA and repair, presence of hiatal hernia, and history of fundoplication were reviewed. There were 310 unique patients (33.5% long gap EA) who underwent 576 endoscopies with biopsies during the study period. Median age at endoscopy was 3.7 years (interquartile range 21-78 months). Erosive esophagitis was found in 8.7% of patients (6.1% of endoscopies); any degree of histologic eosinophilia (≥1 eosinophil/high power field [HPF]) was seen in 56.8% of patients (48.8% of endoscopies), with >15 eosinophils/HPF seen in 15.2% of patients (12.3% of endoscopies). Acid suppression was common; 86.9% of endoscopies were preceded by acid suppressive medication use. Fundoplication had been performed in 78 patients (25.2%). Proton pump inhibitor (PPI) and/or H2 receptor antagonist (H2RA) use were the only significant predictors of reduced odds for abnormal esophageal biopsy (P = 0.011 for PPI, P = 0.048 for H2RA, and P = 0.001 for PPI combined with H2RA therapy). However, change in intensity of acid suppressive therapy by either dosage or frequency was not significantly associated with change in macroscopic erosive or histologic esophagitis (P > 0.437 and P > 0.13, respectively). Presence or integrity of a fundoplication was not significantly associated with esophagitis (P = 0.236). In EA patients, acid suppressive medication therapy is associated with reduced odds of abnormal esophageal biopsy, though histologic esophagitis is highly prevalent even with high rates of acid suppressive medication use. Esophagitis is likely multifactorial in EA patients, with peptic esophagitis as only one of multiple possible etiologies for esophageal inflammation. The clinical significance of histologic eosinophilia in this population warrants further investigation.

Should Proton Pump Inhibitors be Systematically Prescribed in Patients With Esophageal Atresia After Surgical Repair?

To evaluate outcomes of patients with esophageal atresia (EA) on systematic treatment with proton pump inhibitors (PPI) since the neonatal period and to determine factors associated with successful discontinuation of PPI. Longitudinal cohort study with prospective data collection of 73 EA patients, over 11 years systematically treated with PPI. Outcome and predictive factors for discontinuation of PPI treatment were evaluated at study end in February 2017. The incidence of anastomotic strictures was compared with a historical cohort of 134 EA patients followed in the same institution between 1990 and 2005 before the era of systematic PPI treatment. PPI treatment was discontinued definitively in 48% of patients during follow-up. Prematurity, longer initial hospitalization, moderate-to-severe tracheomalacia, anastomotic leak and anastomotic stricture had a significant negative association with PPI discontinuation on univariate analysis (P < 0.05). On adjusted multivariable Cox regression analysis, moderate-to-severe tracheomalacia and anastomotic leak were negatively associated with discontinuation of PPI treatment (hazard ratio 0.26 [95% CI 0.12-0.59]; P = 0.001 and hazard ratio 0.38 [95% CI 0.16-0.93]; P = 0.03, respectively). There was no significant difference in the incidence of anastomotic strictures in the present cohort compared with the historical cohort (44% vs 39%); (P > 0.05). PPI treatment does not prevent the formation of anastomotic strictures and appears to be over-prescribed in children with airway symptoms because of tracheomalacia. This suggests that PPI treatment could be prescribed more selectively. Close monitoring and long-term follow-up, however, of these vulnerable patients in specialized multidisciplinary clinics is imperative.

DOZ047.54: Fundoplication outcomes in children with esophageal atresia

Abstract Background In esophageal atresia (EA) patients, cumulative risk of having a fundoplication ranges from 0% to 45%, with long gap patients with recurrent strictures at even higher risk. However, there are no controlled trials evaluating the outcomes postfundoplication. We hypothesized that children with EA undergoing fundoplication will suffer from more postoperative dysphagia compared to non-EA patients as the fundoplication increases the resistance to esophageal bolus flow, which is already hampered by abnormal motility. Aims Our study aimed to compare outcomes in EA patients with age- and sex-matched control patients postfundoplication. Methods This was an international multicenter retrospective cohort study. All EA patients who had fundoplication between 2006 and 2017 during this period were included. Data were also collected from age- and sex-matched children without EA who underwent fundoplication. Results A total of 40 EA patients had fundoplication during this period. Of the EA patients 82.5% were type C, and 82.5% were long gap. Table 1 compares EA patients with controls. Significantly more EA patients were failing to thrive, on nasogastric/gastrostomy feeds at time of surgery and needed gastrostomy placement at time of fundoplication compared to controls. A total of 17.5% of EA patients developed infection and 22.5% a leak postfundoplication. EA patients had postoperative dysphagia and oral aversion significantly more often than controls. Of 90% of EA patients had recurrence of gastrointestinal or respiratory symptoms, 35% had recurrent strictures, 5% had a new diagnosis of eosinophilic esophagitis postfundoplication. Median time to symptom recurrence was 64 days (12–165 days), 90% were back on PPI and 7.5% had redofundoplication. Conclusions Our study is the first to examine postfundoplication outcomes in EA patients and compare these with a matched control group. EA patients had significantly more dysphagia and oral aversion postfundoplication and majority were back on PPI within two months of surgery. Based on these data, the role of fundoplication in EA patients should be reconsidered. Prospective data are urgently needed.

DOZ047.100: Impact of fundoplication on pH-MII tracings in pediatric patients with esophageal atresia

Abstract Introduction Gastroesophageal reflux disease (GERD) is the most common long-term complication in esophageal atresia (EA) patients. Fundoplication in EA is controversial but may be an option in those with maximum GER therapy failure, acute life-threatening event, failure to thrive, persistent esophagitis, and recurrent anastomotic stenosis. Aim The aim of this study was to compare GERD characteristics according pH-MII parameters in EA patients with and without fundoplication. Materials and Methods Retrospective review of pH-MII tracings performed at a Gastroenterology Unit between 2011 and 2018 was carried out. Inclusion criteria were children under 18 years with history of EA, with and without fundoplication, with both pH-MII and endoscopy performed, histology of esophageal biopsies, and off antireflux medications at the moment of examination. GER therapy failure was the indication for surgery. The total number of reflux episodes (acid, nonacidic), bolus and acid clearance time (ACT), and baseline values in distal channels (5–6) were evaluated. Group 1 (G1): patients with EA and fundoplication. Group 2 (G2): patients with EA without fundoplication. T-test was performed to compare results between G1 and G2. Results Fifteen tracings in EA patients were evaluated. Out of 15, 8 (53%) underwent fundoplication (G1), median age 7.4 years, 87.5% EA type C, 12.5% type A (long gap). Esophagitis was observed in 100% postfundoplication: 2 macroscopic, 1 eosinophilic, 5 microscopic esophagitis. Out of 15, 7 (47%) were EA without fundoplication (G2), median age 3.25 years, 57% EA type C, 43% type A–B. Esophagitis was observed in 85% in G2, all of them microscopic. No statistical differences were observed in pH-MII parameters between both groups. Number of refluxes, ACT, and distal channels (ch-6) were pathological in both groups. Nevertheless better acid clearance and more mucosal damage were observed in G1. Conclusion In this pediatric series of EA patients, pH-MII data show persistence of GERD even postfundoplication. This observation could be due to severe esophageal dismotility. Even asymptomatic EA patients should undergo monitoring of GER with pH-MII and endoscopy.

DOZ047.97: Anastomotic strictures in patients with oesophageal atresia systematically treated with PPI: prospective cohort study over 10 years

Abstract Objectives and Study Clinical course in oesophageal atresia (OA) patients is frequently complicated by gastroesophageal reflux disease, which is thought to be a risk factor for formation of anastomotic strictures. It is currently recommended that OA patients be treated systematically with proton pump inhibitors (PPI) after surgical repair. However, it is not clear if PPI treatment does really prevent the formation of anastomotic strictures and reduce the incidence of histological complications during long-term follow up. This study aimed to describe the outcomes of a cohort of OA patients with or without tracheoesophageal fistula (TOF) systematically treated with PPI since the neonatal period. Method Prospective longitudinal cohort study over 11 years of 73 children with OA-TOF, systematically treated with PPI, was carried out. Descriptive data is presented as median (interquartiles) for continuous variables and as frequency (%) for categorical variables. Pearson's χ2 test, Fisher's exact test, and Wilcoxon rank sum test were used for univariate analysis. SAS software, version 9.3 (SAS Institute, Cary, NC) was used; P-values &lt; 0.05 were considered statistically significant. Results Seventy-three patients (41 males) were included in the final analysis. The median age at study completion was 4.87 years (3.64, 7.97; range 1–11.4). According to Gross classification, 64 patients (88%) had OA type C, 8 (11%) had type A, and 1 (1%) had type D; long-gap OA was present in 16 patients (22%). Thoracotomy was performed in 56 patients and thoracoscopy in 17. Anastomotic strictures and recurrent strictures were diagnosed in 32 (44%) and 17 patients (23%), respectively. In all but one case, strictures occurred when the child was on PPI. Anastomotic strictures occurred significantly more frequently in long-gap OA, in patients with anastomotic leak after surgical repair and longer hospital stay (P = 0.02, 0.002, and 0.02 respectively). Histological complications, especially gastric metaplasia, appeared more frequently in OA patients with anastomotic strictures (P = 0.01). Conclusion Forty-two percent of patients developed anastomotic strictures on PPI treatment, therefrom 55% patients with recurrent anastomotic strictures, indicating that acid gastroesophageal reflux seems not to be the major trigger factor for developing anastomotic strictures. A more selective prescription of PPI should be discussed.

DOZ047.68: Intralesional steroid injection therapy for the treatment of esophageal anastomotic stricture in a pediatric esophageal atresia population

Abstract Background and Aims The role of intralesional steroid injection (ISI) in the treatment of anastomotic stricture in esophageal atresia (EA) patients remains unclear. The aim of this study is to evaluate the efficacy and safety of ISI in a large pediatric EA population. Methods One-hundred fifty eight EA patients who had undergone at least one ISI for the treatment of esophageal anastomotic stricture between 2010 and 2017 were identified. The change in stricture diameter (ΔD) was compared between procedures with balloon dilation alone (ISI-) and balloon dilation with steroid injection (ISI+). Assessment for change in efficacy with increasing numbers of ISI interventions was performed. Results A total of 1055 balloon dilations were performed (452 ISI+). The median ΔD was significantly greater in the group of steroid injection procedures: 1 mm (IQR 0, 3) versus 0 mm (IQR -1, 1.5) (P &lt; 0.0001). The ISI+ group had greater percentage of improved stricture diameter (P &lt; 0.0001) and lesser percentages of unchanged and decreased stricture diameters at subsequent endoscopy (P = 0.0009, P = 0.003). The ΔD for each of an anastomosis’ first 3 ISI+ procedures was greater than the ΔD for its subsequent ISI+ procedures: 1 mm (IQR 0, 3) versus 0.5 mm (IQR -1.25, 2) (P = 0.001). Multivariable logistic regression confirmed the significance of ISI on increasing the likelihood of improved stricture diameter. Conclusions This study demonstrates that intralesional steroid injection with dilation was well tolerated and improved anastomotic stricture diameter more than dilation alone. The benefit of ISI over dilation alone was limited to the first three ISI procedures.

DOZ047.26: Longitudinal motor development from infancy to adolescence in children with esophageal atresia

Abstract Introduction Motor performance may be affected in children with esophageal atresia (EA), but the development of motor performance from infancy to adolescence has not been properly studied. We have followed EA patients from one to 15 years of age and assessed development of motor performance. Methods Of 44 EA patients, born 1999–2002, 3 died, 6 with missing data, 4 excluded, and 10 refused to participate. Of 21 children were included. Perinatal data collected from medical records, and physical therapy was registered. The children were evaluated with The Bayley Scale of Infant Development-Second Edition at one year of age, and re-evaluated in adolescence with The Motor Assessment Battery for Children Second Edition. Both tests are well validated for fine and gross motor performance. The motor performances was categorized as normal, at risk or delayed at both evaluations. Results Of 21 EA children (GA 38.7, range: 34–40.7), 4 (19%) were premature, 18 (86%) had Gross type C, major cardiac anomaly in 1, VACTERL in 4 (19%). At 15.1 months, 19/21 (90%) had motor performance within normal range, 1 child had delayed motor performance, and 1 child at risk for motor delay. At 15 years 15/21 (71%) had motor performance within normal range, 3/21 (14%) children showed delayed motor performance and 3/21 (14%) at risk for motor delay. 5/21 (24%) EA patients had deteriorated and 1/21 (5%) remained delayed in motor performance, when grown up from 1 to 15 years of age. The gross motor function most often affected in adolescence was the balance skills. Impaired motor performance was correlated to prematurity (r = .499, P = .021), but not to other perinatal morbidity factors. The EA patients had not been offered systematic follow-up physical therapy program during childhood. Conclusion Motor performance was within normal range in 90% of the EA infants, but in adolescence, only 71% of the patients showed normal motor performance. However, at one year of age it is hard to distinguish the children who have mild motor delay. Close monitoring in childhood might help to diagnose them earlier and offer appropriate intervention.

DOZ047.27: Esophageal atresia and polyhydramnios

Abstract Background Prenatal diagnosis of esophageal atresia (EA) is difficult and the detection rate is only 20–35%. Ultrasound features of EA are nonspecific with polyhydramnios as the most reported finding. Polyhydramnios is reported in approximately 2% of all pregnancies and thus have low specificity. The aim of our retrospective study is to explore perinatal characteristics of EA patients with prenatal suspicion of EA. Methods Patients with EA born in the periods 1996–2002 and 2011–2017 were included after consent of the parents. Data regarding the pregnancy, birth, and perinatal treatment were obtained from medical records. Results We registered a total of 124 EA patients: 68 from 1996 to 2002, and 56 from 2011 to 2017. Among the 124 patients, 5(4%) had type Gross A or B, 108(87%) type C, 6(5%) type D, and 5(4%) type E. 73(59%) patients had an associated anomaly. 20/124(16%) patients had a prenatal suspicion of EA, and there were no significant differences between neonates with and without prenatal suspicion of EA in terms of birth weight, gestational age, and prematurity. Patients with prenatal suspicion of EA had more caesarean sections, were more frequently born at regional hospitals, and had more associated anomalies. Polyhydramnios was registered in 70 patients; in 18 of these (90% of all with prenatal diagnosis) a prenatal suspicion of EA had been raised. In 52 EA patients with polyhydramnios, no prenatal statement about anomaly was registered. Four of 5 patients (90%) with type A and B had a prenatal suspicion of EA. Corresponding number for type C was 15/108(14%) and 1/5(20%) with type E. Conclusion The detection rate for EA is low, and prenatal suspicion of EA implicates a more serious condition with higher morbidity than EA patients born without prenatal suspicion of malformation. Four of five patients without fistula to lower esophagus (type A and B) had a prenatal suspicion of EA, and polyhydramnios is the ‘signal sign’ for the possible presence of esophageal atresia.

DOZ047.114: Fundoplications in patients born with esophageal atresia: preoperative workup and evaluation of postoperative symptoms

Abstract Background The consensus esophageal atresia (EA) guideline recommends performing contrast esophagogram, esophagogastroduodenoscopy with biopsies and pH(+ /-impedance) measurement prior to fundoplication to assess upper gastrointestinal anatomy, severity of gastroesophageal reflux disease (GERD), and exclude eosinophilic esophagitis (EoE).(Krishnan et al. JPGN 2016) Our aim was to evaluate which of these investigations were performed prior to fundoplication and which in case of postoperative symptoms. Methods In this multicenter international retrospective cohort study, all EA patients who underwent fundoplication between 2006 and 2017 were included. Results Forty EA patients (52% male, median age 2.3 years [0.08–17 years], 82.5% type C) were included in three centers worldwide; 32(80%) underwent complete and 8 (20%) partial fundoplication. Preoperative investigations included contrast esophagogram (n = 35, 88%), esophagogastroduodenoscopy (n = 33, 83%), and pH(+/-impedance) measurement (n = 11, 28%). Only six patients (15%) underwent all three recommended preoperative investigations. Esophagogastroduodenoscopy prefundoplication revealed abnormalities in 33%, including esophageal strictures in 18%, EoE in 6%, hiatal hernia in 6%, and intestinal metaplasia in 3%. Patients with EoE underwent partial fundoplication; no associations between diagnostic testing outcomes and type of fundoplication were found. Nearly all patients had symptoms postfundoplication (n = 34, 85%). Symptoms included dysphagia (53%), gagging (33%), oral food aversion (23%), bloating (8%), and/or dumping (3%). Recurrent GERD symptoms were present in 34 patients (85%). Twenty-one patients (53%) had pH(+/-impedance) tests performed, which were abnormal in 14 patients (67%). Thirty-five patients (88%) also had EGD performed, which showed abnormalities in 5 patients (13%), of which 3 patients (8%) had strictures on EGD. One of these patients with postoperative strictures had EoE (3%). Abnormalities of 2 other patients on EGD were not further specified. Conclusion Preoperative diagnostic workup for fundoplication in EA patients varies greatly. Although the majority of patients had some preoperative evaluation, only 15% underwent all gastrointestinal diagnostic tests that are currently advocated by the consensus EA guidelines. Postoperatively, most patients (85%) had symptoms for which testing was indicated. A large percentage still had evidence of GERD. More research is needed to tailor (surgical) management of GERD in EA patients, develop a postoperative testing paradigm, and evaluate the efficacy and safety of fundoplication in this specific patient group.

DOZ047.15: Esophageal anastomosis diameter at initial endoscopy after surgery predicts treatment outcomes in patients with esophageal atresia or stricture

Abstract Background Children with esophageal atresia (EA) are at risk for anastomotic stricture that may ultimately need surgical resection. No studies have examined the relationship between anastomotic diameter at the time of initial postop endoscopy and treatment outcomes. Methods A retrospective chart review was performed of patients with EA who underwent a Foker procedure for repair of long-gap esophageal atresia (LGEA), primary repair of EA, or stricture resection for refractory stricture who were seen between January 2016 and May 2018. A refractory stricture was defined as one requiring ≥5 dilations ≤5 months after surgery. The anastomosis diameter was estimated by the endoscopist. We divided diameter sizes into the following groups: 1–2.9 mm, 3–4.9 mm, 5–6.9 mm, 7–8.9 mm, 9–10.9 mm, and 11–14 mm. The Wilcoxon rank sum test and Fisher's exact test were used. Results Forty-five patients who had a Foker procedure, 37 who had primary repair, and 58 who had stricture resection were identified. The first EGD occurred a median of 22 days (IQR 21–28) after surgery. Among all EA patients with initial diameter of &lt;3 mm, 82% developed refractory stricture and 41% required stricture resection. Of all patients with initial diameter &lt;5 mm, 74% developed refractory stricture and 33% required stricture resection. Of patients who underwent a Foker procedure with initial diameter &lt;3 mm, 56% required stricture resection; of these patients with initial diameter &lt;5 mm, 53% required stricture resection. Among all EA patients, refractory strictures were significantly more likely in patients with smaller initial diameters (P &lt; 0.001), from 18 patients (82%) with stricture 1–2 mm to 0 patients (0%) with stricture 11–14 mm. Patients with smaller anastomoses were more likely to need stricture resection (P = 0.020); 9 patients (41%) with size 1–2 mm underwent stricture resection, while none (0%) with size 11–14 mm needed stricture resection. These differences remained statistically significant for patients who underwent a Foker procedure (P ≤ 0.001 for stricture resection and refractory stricture). Conclusion Endoscopy performed shortly after EA repair or stricture resection can help predict which patients are more likely to develop a refractory stricture or require a stricture resection.

DOZ047.17: Characterization of esophageal motility in children with operated esophageal atresia using high-resolution impedance manometry and pressure flow analysis

Abstract Background Esophageal dysmotility is common in patients with esophageal atresia (EA). High-resolution impedance manometry (HRIM) and pressure flow analysis (PFA) allow characterization of biomechanical bolus flow and swallow properties. The aim of this study was to characterize esophageal motility and bolus flow propulsion in children who underwent surgical EA repair. Patients and Methods HRIM was used in 14 children with EA (type C, n = 13; type A, n = 1). Study was performed at a median (range) age of 11 years (5–17). The HRIM recordings were analyzed using conventional esophageal pressure topography and PFA (AIMplot software deployed via the open access Swallow Gateway application) and were compared with 13 patient controls (median age 14 years, range 5.75–17; P = NS vs patients) who underwent HRIM considered as normal according to Chicago classification and for whom the manometry results did not lead to treatment changes. Medical charts were reviewed for medical/surgical history and symptoms were assessed by standardized questionnaires. Results Esophageal peristaltic motor patterns were abnormal in all EA patients and were subdivided in two groups: Group A with presence of distal contraction in ≥ 50% of the swallows (n = 6) and Group B with presence of distal contractions in &lt; 50% of the swallows (n = 8). IRP4s was similar in EA and controls. Bolus transport was impaired as shown by the higher impedance ratio (IR) in EA than in controls (0.47 vs 0.22, P &lt; 0.001). In Group A, proximal and distal contractile integrals were lower than in controls (P &lt; 0.001) and distal contractile velocity was shorter in EA (P &lt; 0.01). IR was lower in Group A than in Group B (P &lt; 0.01). In this sample, symptoms of dysphagia and presence of gastric metaplasia or esophagitis were not correlated with any of the measures determined. Conclusions Bolus transport as measured by impedance ratio is severely altered in EA patients but is not predictive of symptoms. The presence of residual distal contractions is associated with a more efficient bolus propulsion. Whether this is associated with a better outcome warrants further studies.