Management Of Esophageal Atresia Research Articles

What Proportion of Children With Complex Oesophageal Atresia Require Oesophageal Lengthening Procedures?

IntroductionBabies with oesophageal atresia (OA) who cannot achieve a primary anastomosis (complex OA) may be treated by attempted oesophageal lengthening. We contrast reported outcomes of lengthening with our experience of managing complex OA. Patients and methodsA consecutive series treated in an English regional centre was completed. Outcomes of interest were the rate of retention of the native oesophagus, complications requiring thoracotomy, rates of anastomotic leak, stricture, fundoplication, and mortality. Possible explanatory variables were the surgical techniques applied. Results29/215 (13%) OA were complex, and 25/207 survived to repair. 14/25 (56%) had no distal fistula, pure OA, while 11/25 (44%) had a long gap with distal fistula. 18/25 (72%) had delayed primary anastomosis, while 7/25 (28%) required oesophageal replacement. However, 2 of the replacements were salvage procedures following failed traction. Only 4/207 (2%) of OA were potentially treatable by traction. Salvage surgery was required in 2/23 (9%) complex OA not subjected to lengthening. The native oesophagus was retained without utilising lengthening in 200/207 (97%). Amongst complex OA where traction techniques had not been attempted, the native oesophagus was retained in 18/23 (78%) of cases, with median time to oesophageal continuity of 77 days. There was no in hospital mortality following treatment of complex OA, and overall survival was identical to non-complex OA among cases surviving to anastomosis. DiscussionManagement of complex OA without lengthening procedures leads a similar rate of retention of the native oesophagus as reports describing lengthening, but with significantly less morbidity. We see little need for oesophageal lengthening in the management of complex OA. Level of EvidenceIV.

Arise Tube- A Novel Double-Lumen Suction Tube for Oesophageal Atresia

Background: Airway compromise is a major criterion that determines the outcome of care in oesophageal atresia (OA). The maintenance of dry throat through suction of the airway and throat is a vital step to the survival of neonates with OA. This prevents aspiration of saliva and mucus plugging of the airway which can compromise pulmonary function. Pre-operative aspiration pneumonitis increases the morbidity and possible mortality of neonatal OA. The Replogle® tube is usually not readily available and costly for caregivers in most centres in the low-income countries. Objective: To introduce a local adaptation of suction tubing for clearance of the airway in OA. Method and Case Series: The Abdur-Rahman Ilorin Suction oEsophageal (ARISE) suction tube is constructed with a size 6 Fr feeding tube which was tunnelled from a side opening into the lumen of a 10 Fr feeding tube. The distance of the pouch from the incisor is first measured and multiple fenestrations (1 cm apart) are made on the wall of the outer tubes sparing about 2 cm from the measured length near the incisor. Three side fenestrations are made 1.5 cm apart from the tip on the side of the inner tube. The ARISE tube is placed in the upper OA pouch to suck saliva and prevent aspiration in the neonate. The tip of tube is positioned about 0.5 cm above the blind end of the oesophageal pouch. The inner (small lumen) tube is connected to a low-pressure suction machine at 0.2KPa (−15 to −35 cm H2O) for continuous drainage of saliva and the outer (big lumen) tube functions as air vent and irrigation channel. This protects the mucosal from being sucked into the inner tube lumen. Irrigation of tube is done with 2–3 ml of 0.9% saline every 4 h and as necessary. Result: This ARISE suction tube allowed adequate and continuous suction of the pouch and throat with the baby being calm, and caregivers also stress free. The patient maintained good arterial oxygen saturation and there was no aspiration into the airway. Conclusion: The ARISE double-lumen suction tube can easily be constructed in many resource-poor health institutions where OA is managed. This will improve the pre-operative management of neonatal OA in such environment making the baby’s condition optimal for surgical intervention.

Predictors of enteral tube dependence in pediatric esophageal atresia.

Children with esophageal atresia (EA) may require enteral tube feedings in infancy and a subset experience ongoing feeding difficulties and enteral tube dependence. Predictors of enteral tube dependence have never been systematically explored in this population. We hypothesized that enteral tube dependence is multifactorial in nature, with likely important contributions from anastomotic stricture. Cross-sectional clinical, feeding, and endoscopic data were extracted from a prospectively collected database of endoscopies performed in EA patients between August 2019 and August 2021 at an international referral center for EA management. Clinical factors known or hypothesized to contribute to esophageal dysphagia, oropharyngeal dysphagia, or other difficulties in meeting caloric needs were incorporated into regression models for statistical analysis. Significant predictors of enteral tube dependence were statistically identified. Three-hundred thirty children with EA were eligible for analysis. Ninety-seven were dependent on enteral tube feeds. Younger age, lower weight Z scores, long gap atresia, neurodevelopmental risk factor(s), significant cardiac disease, vocal fold movement impairment, and smaller esophageal anastomotic diameter were significantly associated with enteral tube dependence in univariate analyses; only weight Z scores, vocal fold movement impairment, and anastomotic diameter retained significance in a multivariable logistic regression model. In the current study, anastomotic stricture is the only potentially modifiable significant predictor of enteral tube dependence that is identified.

Esophageal elongation by bougienage and delayed primary thoracoscopic anastomosis for pure esophageal atresia without tracheoesophageal fistula.

We aim to share our experience of esophageal elongation by bougienage and delayed primary thoracoscopic anastomosis for pure esophageal atresia (EA) without tracheoesophageal fistula (TEF). Fifteen patients with pure EA treated with delayed primary thoracoscopic anastomosis combined with or without esophageal elongation by bougienage were retrospectively analyzed. Four patients were managed without bougienage, and their surgical repair was performed thoracoscopically after natural esophageal growth. Among the remaining 11 patients, the average tension-free distance before elongation was 5 (4.5-6) vertebral bodies, and the mean age at the start and end of the bougienage period was 123 (63-280) days and 173 (106-350) days, respectively, with an average duration of 50 (29-82) days. The average age at the definitive operation in this series was 184 (107-385) days, with a mean operative duration of 186 (95-300) min. Neither anastomotic leakage nor TEF occurred, and oral feeding was partially or completely established in 13 patients during hospitalization. Among all patients, one was lost to follow-up, and others were followed up with an average duration of 47.7 (9.8-97.1) months. All patients had different degrees of anastomosis stricture, and 8 patients had gastroesophageal reflux. Oral feeding was completely established in 12 patients; however, tube feeding was required in 2 patients. The management of pure EA is complicated and inconclusive. Esophageal elongation by bougienage and delayed primary thoracoscopic anastomosis for long-gap pure EA without TEF is safe and effective.

Outcome of esophageal atresia: inborn versus outborn patients.

Esophageal atresia (EA) is a rare congenital disease which is usually not of the detected prenatally. Due to the lack of prenatal diagnosis, some newborns with EA are born outside of specialized centers. Nevertheless, centralized care of EA has been proposed, even if a clear volume-outcome association in EA management remains unconfirmed. Furthermore, whether outcomes differ between outborn and inborn patients with EA has not been systematically investigated. Therefore, this single-center, retrospective study aimed to investigate EA management and outcomes with a special focus on inborn versus outborn patients. The following data were extracted from the medical records of infants with EA from 2009 to 2019: EA type, associated anomalies, complications, and long-term outcome. Patients were allocated into inborn and outborn groups. Altogether, 57 patients were included. Five patients were excluded (referral before surgery, loss of data, death before surgery [n = 1], and incorrect diagnosis [diverticulum, n = 1]). Among all patients, the overall survival rate was 96%, with no mortalities among outborn patients. The overall hospitalization period was shorter for outborn patients. The median follow-up durations were 3.8years and 3.2years for inborn and outborn patients, respectively. Overall, 15% of patients underwent delayed primary anastomosis (long-gap atresia [n = 4] and other reasons [n = 4]). Early complications included three anastomotic leakages and one post-operative fistula; 28% of patients developed strictures, which required dilatation, and 38% of patients showed relevant gastroesophageal reflux, which required fundoplication, without any differences between the groups. The two groups had comparable low mortality and expected high morbidity with no significant differences in outcome. The outborn group showed nonsignificant trends toward lower morbidity and shorter hospitalization periods, which might be explained by the overall better clinical status.

Surgical treatment of esophageal atresia with lower tracheoesophageal fistula in an extremely preterm infant (510 g, 25 + 5 weeks): a case report

BackgroundThe surgical management of esophageal atresia in extreme-low-birth-weight infants (< 1000 g) is challenging. We report on an extreme-low-birth-weight infant who was extremely preterm (510 g, 25 + 5 weeks) and of prenatally unknown Gross type C esophageal atresia.Case presentationAfter resuscitation and intubation, the tracheoesophageal fistula was closed on the first day of life in the neonatal intensive care unit via an extrapleural approach using a titanium clip. On the sixth day of life, the Caucasian child was extubated. To minimize the operative trauma in the initial neonatal period, we prolonged gastrostomy placement until the 22nd day of life (weight 725 g). At the age of 3 months (weight 2510 g), thoracoscopic esophageal anastomosis was performed. The postoperative course was unremarkable. During the further clinical course, eight esophageal dilations were necessary. Currently, the patient swallows without difficulties at the age of 4 years and thrives well [15 kg (Percentile 28); 100 cm (Percentile 24)].ConclusionsOur case shows that minimized postnatal surgical trauma with primary tracheoesophageal fistula closure at the bedside, delayed gastrostomy, and minimally invasive esophageal repair after substantial weight gain (> 2.5 kg) is a good strategy for esophageal atresia/tracheoesophageal fistula in extreme-low-birth-weight infants.

Management Anesthesia of Esophagostomy in a Patient with a Double Outlet Right Ventricle

Background: Esophageal atresia is a congenital disorder in which there is no esophagus because the proximal and distal esophagus is not connected. Babies with esophageal atresia can show several symptoms: foamy mouth, cyanosis, coughing and tightness, flatulence, oliguria, or worse, pneumonia symptoms. Accompanying anomalies occur in greater than 50% of neonates with esophageal atresia. Esophageal atresia is identified by ultrasound at 18 weeks of gestation, ultrasound, and Magnetic resonance imaging (MRI) of the fetal neck, or examination of a nasogastric tube in the neck of a newborn. The management of esophageal atresia is challenging. The main choice remains the surgical procedure, which usually involves making a stoma on the proximal esophagus and gastrostomy. However, surgery has risky complications. Case: In this case, it was reported that a 22-day-old baby with tracheoesophageal fistula (TEF) type C with Ventricular Septum Defect and Atrial Septum Defect and Double Outlet Right Ventricle (DORV) underwent esophagostomy surgery with general anesthesia. Conclusion: Anesthesia management with general anesthesia, intubation using intravenous ketamine 3 mg, fentanyl 3µg, atracurium 1.5 mg gives stability for esophagostomy in a patient with a double outlet right ventricle.

Helen Rae Noblett

Helen Rae Noblett was a pioneering paediatric surgeon who came from Australia in 1976 to establish the specialty in Bristol. She trained in Brisbane and Melbourne. She had already made...

The Current Thoracoscopic Management of Esophageal Atresia.

The thoracoscopic repair of esophageal atresia with tracheoesophageal fistula is a complex neonatal minimally invasive procedure. The thoracoscopic approach is now nearing its third decade of experience and but is overall still not widely utilized, only in skilled centers and by experienced surgeons. This article will summarize the recent advancements in technique and knowledge in the thoracoscopic approach to this challenging neonatal congenital abnormality.

DOZ047.92: Oesophageal atresia: a multicenter retrospective study on surgical treatment and 1-year follow-up

Abstract Introduction No data are available on surgical management and outcomes of oesophageal atresia (OA) in Belgium. In collaboration with the Belgian Association of Paediatric Surgery (BELAPS) a retrospective study was organized to analyze data from children born with OA, with a 1-year follow-up. Materials and Methods Out of 17 centers in Belgium, 10 participated in this study. A novel checklist to collect data from OA patients born between the 1st of January 2010 and the 1st of January 2015 was created. Gross type A, B, C and D OA were included. Results One hundred children were included. Gross type C OA was seen in 86%. Associated malformations were present in 73%, most frequently cardiac malformations (52%). There was consensus regarding preoperative investigations, anastomotic technique, and transanastomotic tube placement. There was no consensus regarding genetic counseling, placement of a chest tube, and start of enteral feeding. There were six deaths in the first year of life. The majority of patients needed rehospitalization during the first year of life (85.9%) and there was a high occurrence of anastomotic strictures (51.6%). Conclusions This is the first study about management and outcome of OA in Belgium. Although the 1-year survival rate is high (93.8%), these data illustrate that OA has an important morbidity. Multidisciplinary follow-up and prospective studies/registries with longer follow-up are needed.

Management of esophageal atresia and early predictive factors of mortality and morbidity in a developing country.

The aim of this study is to investigate management and outcome in esophageal atresia (EA) and to identify early predictive factors of morbidity and mortality in a developing country. Charts of neonates with repaired EA from 2007 to 2016 were reviewed. Patients' characteristics, operative details, and postoperative outcomes were collected. Statistical analyses were performed to identify predictors of complicated evolution. Forty-two cases were collected. There were 14 girls and 28 boys. Only one patient had antenatal diagnosis (2.3%). The mean gestational age was 38 weeks. Nine patients (21.4%) weighed less than 2.5 kg. Seventeen (40.4%) patients had associated malformations most commonly cardiac (9/17). Thirteen patients had delayed diagnosis (30.9%). Thirty-nine (92.8%) patients underwent primary esophageal anastomosis. Overall survival was 76.2%. Nineteen patients (57% of survivals) had complicated evolution before the age of one year and 15 patients (46.8% of survivals) developed complications after the age of one year. Perinatal variables associated with mortality were prematurity (p=0.004, OR=5.4, IC95%=[1.13-25.80]), low birth weight (p=0.023, OR=7, IC95%=[1.38-35.47]), cardiac malformations (p=0.006, OR=10.5, IC95%=[2.03-54.27]) and delayed diagnosis (p=0.005, OR=10.11, IC95%=[2.005-50.980]). Variables associated with short-term and middle-term complications were duration of intubation (p=0.019, OR=0.118, IC95%=[0.019-0.713]) and the presence of short-term complications (p=0.016, OR=7.33, IC95%=[1.467-36.664]) respectively. These factors may be used to identify patients who will benefit from more intensive follow-up program.

Anaesthesia management of esophageal atresia repair surgery

Esophageal atresia (EA) is a congenital anomaly commonly found with tracheoesophageal fistula (TEF) of neonates in the first week of life. This anomaly can cause several complications including aspiration, reduction in respiration, and complication from other concomitant congenital anomalies, mostly from the heart origin. The treatment for this anomaly is a surgery. Intraoperatively, the patient may develop hypoxia due to lung retraction and hemodynamic instability from bleeding or hypothermia. Anaesthesiologists play an important role in the management of EA during the perioperative period. Careful examination of the preoperative period must be done to discover any other concomitant anomaly and complication. Good anticipation of any complication during surgery and continuous monitoring post surgery can elevate the prognosis of the patient.

Surgical and Anaesthesiology Management of Esophageal Atresia: What Are the Mortality Factors in a Developing Country?

Esophageal atresia is an extreme neonatal surgical emergency whose mortality remains high in our country. We report 8 cases collected over 7 years in a tertiary hospital in Abidjan, Cote d’Ivoire. The purpose of the study is to identify the elements of surgical and anaesthesiological man-agement in our department that influences mortality.

Importance of an International Registry for and Collaborative Research on Esophageal Atresia.

Esophageal atresia (EA) is a rare congenital defect. Data on EA prevalence, management, and long-term outcome are lacking because the available data come from small retrospective series from tertiary referral centers. An international multicenter registry would provide strong epidemiological data from large population-based cohorts on EA prevalence and incidence, treatment, long-term morbidity, and prognosis and would thus provide accurate data for evaluation of the current guidelines for EA management. The future challenge of the new international network on EA, which was created in 2013, is to promote the creation of a collaborative database and further studies.

Multicentre survey on the current surgical management of oesophageal atresia in Belgium and Luxembourg

IntroductionThe 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. Materials and methodsA 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. ResultsMost 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. ConclusionThere 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 of Evidence: Level III.

Impact of botulinum toxin A injection on esophageal anastomosis in a rabbit model

The management of esophageal atresia is established, but the rate of postoperative complications remains high. We focused on a new, recently reported method of esophageal elongation using botulinum toxin type A (BTX-A) and evaluated the efficacy of BTX-A injection around esophageal anastomoses with tension in a rabbit model. Twenty rabbits aged 8-10weeks and weighing 1.27-1.72kg underwent resections of the esophagus measuring 1.5cm long using an anterior cervical approach. Esophagoesophagostomies were performed after intramural administration of Xeomin™ (3U/body) in the BTX-A group and saline in the control group. Morphological and histological evaluations were examined on postoperative day 14. Six rabbits in each group survived. The BTX-A group showed significantly less postoperative anastomotic stricture and less fibrosis than the control group. Changes in wall thickness on both sides of the anastomotic areas were equivalent between the two groups, and no muscle fracturing was observed. Local administration of BTX-A for esophagoesophagostomy significantly reduced postoperative anastomotic stricture with less fibrosis than that observed in the control group. Reduced anastomotic tension with BTX-A presumably contributed to better anastomotic healing. Determining the optimum dose of BTX-A is necessary for clinical application.

Oesophageal atresia is correctable and survivable in infants less than 1kg.

IntroductionManagement of oesophageal atresia (OA) and trachea-oesophageal fistula (TOF) in babies of low birth weight is challenging especially when associated with other anomalies. Birth weight of <1500 g has previously formed part of a classification system designed to predict outcome, alongside the cardiac status of the patient. Improvements in neonatal care have led to increasing numbers of premature low birth weight infants surviving. The aim of this study was to look at the experience of our institution in the extremely low birth weight (ELBW) patients.MethodsA retrospective review of our institutions OA database was performed from 1993 to June 2015. Patients of birth weight less than 1000 g were included. A review of our OA/TOF clinical database and notes review established the following; gestation, birth weight, associated anomalies, operative procedures, morbidity and mortality.ResultsOf 349 patients with OA across the 22-year period, 9 ELBW patients were identified (<1000 g). Six males and three females. Gestational age ranged from 23 to 34 weeks and median birth weight was 815 g ranging from 630 to 950 g. Overall survival was 56 % (5/9). There were double the numbers of ELBW OA/TOF patients seen in the second half of the study period presumably the result of improving neonatal care. Seven patients had type C OA with TOF and underwent emergency TOF ligation, two had concomitant oesophageal repair. One of these patients died from NEC; the other survived. Of the five who had isolated TOF ligation three died—two from cardiac disease and one from prematurity. Both type A patients survived and after initial gastrostomy placement one had a primary delayed repair, the other a gastric transposition. All three babies under 800 g died—one from cardiac disease the others from conditions indicative of their prematurity—necrotising enterocolitis and intraventricular haemorrhage.Conclusions50 % survival is achievable in OA/TOF under 1 kg and the Spitz classification is still applicable in this group as a whole. However, none of the current classification systems are applicable in infants <800 g who in our study all had poor outcomes. We suggest these should be considered as separate group when predicting outcomes.

Outcome and management in infants with esophageal atresia – A single centre observational study

Background/PurposeA successful outcome in the repair of esophageal atresia (EA) is associated with a high quality pediatric surgical centre, however there are several controversies regarding the optimal management. The aim of this study was to investigate the outcome and management EA in a single pediatric surgical centre. MethodsMedical records of infants with repaired EA from 1994 to 2013 were reviewed. Results129 infants were included. Median follow-up was 5.3 (range 0.1–21) years. Overall survival was 94.6%, incidences of anastomotic leakage 7.0%, recurrent fistula 4.6% and anastomotic stricture 53.5% (36.2% within first year). In long gap EA (n=13), delayed primary anastomosis was performed in 9 (69.2%), gastric tube in 3 (23.1%) and gastric transposition in one (7.7%) infants. The incidences of anastomotic leakage and stricture in long gap EA were, 23.1% and 69.2%, respectively. Peroperative tracheobronchoscopy and postoperative esophagography were implemented as a routine during the study-period, but chest drains were routinely abandoned. ConclusionThe outcome in this study is fully comparable with recent international reports showing a low mortality but a significant morbidity, especially considering anastomotic strictures and LGEA. Multicenter EA registry with long-term follow up may help to establish best management of EA.

P-24: Neonatal Management of Esophageal Atresia (EA): A National Survey of Practice Variations in French Pediatric Surgery Departments

P-24: Neonatal Management of Esophageal Atresia (EA): A National Survey of Practice Variations in French Pediatric Surgery Departments

Management of oesophageal atresia in a developing country: Is primary repair forbidden?

The aim of the study was to evaluate the outcomes of initial surgical procedures for oesophageal atresia at our institution. Primary repair of oesophageal atresia at our centre was perceived to be associated with a high mortality rate. In view of this, almost all patients seen since January 2014 were offered initial surgery for staged repair. A retrospective review of records of infants with oesophageal atresia seen at the centre from January 2007 to December 2014 was used in this study. Eighty-five cases of oesophageal atresia were seen over the study period. Of these, 74 (87.1) had surgery performed. Surgical outcome was, however, indicated in 67 of the 74, with overall survival rate of 40.3%. Among the 67 patients, early primary repair was performed in 24 (35.8%) with a survival rate of 45.8% (11 patients). Totally, 12 (17.9%) of the 67 had initial procedure for delayed primary repair, with a survival rate of 16.7% (2 patients). The remaining 31 (46.3%) patients had initial surgery for staged repair, with survival rate of 45.2% (14 patients). There was no association between the type of surgery and the surgical outcome (χ2 = 3.396, df = 2, P = 0.183). The overall surgical survival rate of 40.3% for oesophageal atresia at our institution is low. This study did not show any difference in the survival rate of 45.8% and 45.2% associated with primary repair and staged repair respectively.