Management Of Long-gap Esophageal Atresia Research Articles

Thoracoscopic external traction suture elongation for the management of long-gap oesophageal atresia: a two-centre experience.

Van der Zee (VdZ) described a technique to elongate the oesophagus in long-gap oesophageal atresia (LGOA) by thoracoscopic placement of external traction sutures (TPETS). Here, we describe our experience of using this technique. Retrospective review of all LGOA + / - distal tracheo-oesophageal fistula (dTOF) cases where TPETS was used in our institutions. Data are given as medians (IQR). From 01/05/2019 to 01/03/2023, ten LGOA patients were treated by the VdZ technique. Five had oesophageal atresia (Gross type A or B, Group 1) and five had OA with a dTOF (type C, Group 2) but with a long gap precluding primary anastomosis. Age of first traction procedure was Group 1 = 53 (29-55) days and Group 2 = 3 (1-49) days. Median number of traction procedures = 3; time between first procedure and final anastomosis was 6days (4-7). Four cases were converted to thoracotomy at the third procedure. Three had anastomotic leaks managed conservatively. Follow-up was 12-52months. All patients achieved oesophageal continuity and were orally fed; no patient required an oesophagostomy. In this series, TPETS in LGOA facilitated delayed primary anastomoses and replicated the good results previously described but, in addition, was successful in cases with dTOF. We believe traction suture placement and tensioning benefit from being performed thoracoscopically because of excellent visualisation and the fact that the tension does not change when the chest is closed. Surgical and anaesthetic planning and expertise are crucial. It is now our management of choice in OA patients with a long gap with or without a distal TOF.

Management of long-gap esophageal atresia.

A uniquely challenging subset of infants diagnosed with esophageal atresia (EA) are those born with long-gap EA (LGEA). The common unifying feature in infants with LGEA is that the proximal and distal segments of the esophagus are too far apart to enable primary anastomosis via a single operation in the newborn period. Although any type of EA can technically result in a long gap, the Gross type A variant occurs in 8% of all EA cases and is most commonly associated with LGEA. In this review, we provide an evidence-based approach to the current challenges and management strategies employed in LGEA. There are fortunately a range of available surgical techniques for LGEA repair, including delayed primary repair, staged repair based on longitudinal traction strategies to lengthen both ends (e.g., Foker procedure, internal traction), and esophageal replacement using other portions of the gastrointestinal tract. The literature on the management of LGEA has long been dominated by single-center retrospective reviews, but the field has recently witnessed increased multi-center collaboration that has helped to increase our understanding on how to best manage this challenging patient population. Delayed primary repair is strongly preferred as the initial approach in management of LGEA in the United States as well as several European countries and is supported by the American Pediatric Surgery Association recommendations. Should esophageal replacement be required in cases where salvaging the native thoracic esophagus is not possible, gastric conduits are the preferred approach, based on the relative simplicity of the operation, low postoperative morbidity, and long-term durability. Long-term followup for monitoring of swallowing function, nutritional status, aspiration/respiratory illnesses, gastroesophageal reflux, and associated comorbidities is essential in the comprehensive care of these complex patients.

Isoperistaltic gastric tube for long gap esophageal atresia (LGEA) in newborn, infants, and toddlers: a case-control study from a tertiary center.

Limited evidence exists about outcomes after gastric tube formation as "rescue" technique to avoid esophageal replacement in long gap esophageal atresia (LGEA). The last ERNICA Consensus Conference on the Management of LGEA has placed the techniques of gastric tubulization among the priorities for future research. Evaluate personal experience with Isoperistaltic Gastric Tube (IGT) and compare its outcomes with other more popular techniques for LGEA. A case-control study has been conducted. A retrospective monocentric analysis of LGEA patients (period: 2010-19) has been conducted in all consecutive IGT patients and each of these has been type matched with two cases of LGEA treated with other techniques. The follow-up (FU) considered was 24-months. IGT and controls showed no statistically significant differences regarding preoperative variables like sex, gestational age, birth weight, syndromes, and EA type. However, IGT patients had a significantly longer esophageal GAP under boost pressure (4.5 vertebral bodies vs. 3.6, p = 0.019) at time of surgery. The analysis showed no statistical difference among the two groups about perioperative outcomes, ICU, or overall postoperative stay. No differences have been shown between IGT and controls during the follow-up regarding GERD, esophagitis, fundoplication, dysphagia, vocal cord paralysis, stenosis, and dilatations, auxologic data, need for anastomosis revision, oral aversion, and death. Isoperistaltic Gastric Tube is safe and effective even in LGEA patients with longer gaps, with good perioperative, post-operative and middle-term outcomes. This procedure may be considered as an alternative to avoid esophageal substitution when a primary anastomosis seems impossible for a residual gap after traction and growth techniques.

Delayed primary repair in 100 infants with isolated long-gap esophageal atresia: A nationwide analysis of children’s hospitals

BackgroundThis study aimed to evaluate the contemporary surgical management of long-gap esophageal atresia, a rare and challenging problem managed by pediatric general surgeons. MethodsA retrospective review of the Pediatric Health Information System database for infants who underwent neonatal gastrostomy, followed by surgical reconstruction for long-gap esophageal atresia (2014–2021). Patients with birthweight less than 1.5 kg and those who received neonatal cardiac surgery were excluded. Outcomes were analyzed, including the need for further procedures, length of stay, and mortality. ResultsOf 1,346 infants who underwent repair across 47 major children’s hospitals, 100 (7%) met the inclusion criteria for long-gap esophageal atresia. Cardiac anomalies were identified in 43% of patients. The median age at repair was 87 days (interquartile range, 62–133). Ten percent of patients had a planned or unplanned reoperation ≤30 days after index surgery, and 4% underwent reoperation at >30 days. The median time to reoperation was 9 days (interquartile range, 7–60). Mortality during index admission was 5%, and the median hospital length of stay was 143 days (interquartile range, 101–192). Length of stay was significantly longer in patients with cardiac anomalies (cardiac: 179 days, non-cardiac: 125 days; P < .001), and 52% of patients required at least 1 postoperative dilation. The median time to the first dilation was 70 days (interquartile range, 42–173). ConclusionThis large multicenter study highlights the challenges of infants with long-gap esophageal atresia but suggests a high rate of successful delayed primary reconstruction. Hospitalizations are prolonged, and anastomotic stricture rates remain high. These data are useful for pediatric surgeons in counseling families on surgical repair strategy, timing, and postoperative outcomes.

A new method of primary engineering of esophagus using orthotopic in-body tissue architecture

PurposeTissue engineering of esophagus is required for management of long-gap esophageal atresia (LGEA). Collagenous connective tissue membranes fabricated by in-body tissue architecture (iBTA), called biosheets, can repair esophageal defects and generate tissues similar to native esophagus. However, iBTA requires second-stage surgery because of heterotopic preparation of biosheets. Our aim was to develop orthotopic iBTA for primary engineering of the esophagus by interposing a tubular mold to the esophageal defect. MethodThe cervical esophagus of six rats was transected. An acrylic tube (internal diameter 2.6 mm, length 7.0 mm) was inserted and fixed between the ends of the upper and lower esophagus, and a 3 mm-long esophageal defect was created. Four weeks later, the rats were sacrificed for histological analysis. ResultsPostoperatively the rats could intake liquid food. After four weeks, the esophageal defects were filled with regenerated tissues. Histologically the new esophageal walls stained positive for collagen type I. The inner surfaces were covered with stratified squamous epithelium that expressed pan-cytokeratin. In only one of six rats, regeneration of muscular-like tissue was suggested by positive immunohistochemical staining for desmin. ConclusionOrthotopic iBTA can regenerate a substitute esophagus with esophageal epithelium and collagenous wall. This technique may be a novel treatment for esophageal atresia with gaps of various lengths including LGEA.

The Effect of Botulinum Toxin Type A Injections on Stricture Formation, Leakage Rates, Esophageal Elongation, and Anastomotic Healing Following Primary Anastomosis in a Long- and Short-Gap Esophageal Atresia Model - A Protocol for a Randomized, Controlled, Blinded Trial in Pigs.

Background:Esophageal atresia (EA) is a congenital malformation affecting 1:3000-4500 newborns. Approximately 15% have a long-gap EA (LGEA), in which case a primary anastomosis is often impossible to achieve. To create continuity of the esophagus patients instead have to undergo lengthening procedures or organ interpositions; methods associated with high morbidity and poor functional outcomes. Esophageal injections of Botulinum Toxin Type A (BTX-A) could enable primary anastomosis and mitigate stricture formation through decreased tissue tension.Methods and Analysis:In this randomized controlled blinded animal trial, 24 pigs are divided into a long- or short-gap EA group (LGEA and SGEA, respectively) and randomized to receive BTX-A or isotonic saline injections. In the LGEA group, injections are given endoscopically in the esophageal musculature. After seven days, a 3 cm esophageal resection and primary anastomosis is performed. In the SGEA group, a 1 cm esophageal resection and primary anastomosis is performed, followed by intraoperative injections of BTX-A or isotonic saline. After 14 days, stricture formation, presence of leakage, and esophageal compliance is assessed using endoscopic and manometric techniques, and in vivo and ex vivo contrast radiography. Tissue elongation is evaluated in a stretch-tension test, and the esophagus is assessed histologically to evaluate anastomotic healing.Ethics and Dissemination:The study complies with the ARRIVE guidelines for animal studies and has been approved by the Danish Animal Experimentation Council. Results will be published in peer-reviewed journals and presented at national and international conferences.Highlights:The optimal management of long-gap esophageal atresia remains controversialPrimary anastomosis could improve functional outcomes and reduce complicationsBotulinum Toxin Type A decreases tissue tension and could facilitate anastomosisReduced tension could further abate the risk for anastomotic stricture and leakageWe present a model to evaluate the method in long- and short-gap esophageal atresia

DOZ047.19: Outcomes in the management of long-gap esophageal atresia: is the Foker technique superior?

Abstract Background Long-gap esophageal atresia (LGEA) has traditionally been managed by delayed anastamosis. However, over the last 10 years the Foker technique of esophageal growth elongation has been used. There is limited data evaluating outcomes using both techniques. Aims The aim of this study was to compare the outcomes in LGEA repair using the traditional delayed repair technique versus the Foker technique. Methods A retrospective chart review was done of LGEA patients at Sydney Children's Hospital between 1997 and 2016, comparing the Foker technique with the delayed repair technique. Results There were 9 children repaired using the Foker technique, and 10 by delayed repair. There were 7 patients with Type A, 1 with Type B, and 1 with Type C in the Foker group and in the delayed repair cohort, 5 were Type A, 2 Type B, 2 Type C, and 1 Type D esophageal atresia/tracheoesophageal fistula. There were 4 males in the Foker group and 5 in the non-Foker group. Median time to join was 59 days (15–117 days) for Foker repair and 173 days (16–433 days) for delayed repair. There was no significant difference in the incidence of post-operative leak, incidence of strictures needing dilation, presence of reflux symptoms, presence of reflux esophagitis on endoscopy, proton pump inhibitor use, need for fundoplication, incidence of cyanotic spells, occurrence of recurrent fistula, and symptoms of dysphagia when comparing both groups. Data on time to oral feeds and incidence of malnutrition was incomplete. Details are shown in Table 1. Conclusions Although the time to anastamosis was shorter in the Foker group and the incidence of post-operative leak, strictures needing dilation, need for fundoplication, and dysphagia were lower in the Foker group, the difference was not significant probably secondary to the small sample size. These results require validation in larger cohorts.

DOZ047.64: Sham feeding promotes oral feeding success in long-gap esophageal atresia, even with traction sutures in situ

Abstract Introduction Sham feeding (SF) and staged repair using traction suture techniques (TST) were both introduced to the management of long-gap esophageal atresia (LGOA) at The Royal Children's Hospital, Melbourne (RCH) in January 2012. Previous studies report SF to reduce time to full oral feeding (FOF) postdefinitive repair. In our practice, SF is individually tailored, supervised, and involves offering 2–4 feeds daily. This single-center experience describes SF in newborns with LGOA, including those with staged TST prior to definitive repair, and the impact of SF on time to FOF. Methods Patients admitted 2000–2018 with LGOA were identified from the Nate Myers Oesophageal Atresia Database, RCH. Those with incomplete data or complications preventing oral feeds were excluded. Patients were grouped into two epochs: Group 1 (2000–2011, no SF, no TST) and Group 2 (2012–2018, all offered SF, some TST). Demographics and time to FOF were compared between groups. Parametric data (mean, SD) were analyzed with two sample t-test, and nonparametric data (median, IQR) with Mann–Whitney test; P &lt; 0.05 significant. Ethics approval was obtained (HREC#QA/51247/RCHM-2019). Results Of 37 LGOA patients, six met exclusion criteria leaving 31 for analysis; Group 1, n = 13; Group 2, n = 18. Groups did not differ in gestation (P = 0.63), birth weight (P = 0.91) or time to definitive repair (P = 0.85). In Group 2, 12/18 had successful SF, including 9 who underwent TST. Three additional patients with TST were unsuccessful with SF. Compared with Group 1, FOF was significantly reduced in those 12 patients with successful SF (median FOF 730 days, IQR 125–1100 vs. median FOF 75 days IQR 56–227; P = 0.03). Small sample size precluded meaningful subgroup analyses of successful versus unsuccessful SF patients. Importantly, no patient aspirated during SF, though one developed oral aversion post-TST and one ceased SF at parental request. Conclusions In LGOA, successful SF improves time to FOF postdefinitive repair, and was not associated with aspiration in our experience. Further, we report success with SF in patients undergoing staged repair with TST, including following traction suture placement. To our knowledge, this is the first report of SF in LGOA patients during the period between traction suture placement and definitive repair.

Bodyweight, not age, determines oesophageal length and breaking strength in rats

Background/PurposeDelayed primary repair is still the method of choice in the management of long-gap oesophageal atresia in many centres, but the timing of anastomoses varies. Some assume the infant's bodyweight to be an important factor, whereas others prefer age. We therefore aimed to clarify whether age or bodyweight determined oesophageal length in a rodent model. MethodsWe explanted the oesophagi of 20 Sprague–Dawley rats, aged 15 to 444 days (n = two per time point), measured bodyweight, oesophageal length, weight, and linear breaking strength to measure tissue resilience. Univariate and multivariate regression analyses were conducted to determine the influence of age and bodyweight on oesophageal length and linear breaking strength. ResultsAll parameters were highly correlated (R > 0.8), except for age and linear breaking strength (R = 0.65). Both age and bodyweight were univariate significant predictors of oesophageal length, weight, and linear breaking strength (p < 0.0001). Multivariate analyses showed bodyweight to be a significant predictor of oesophageal length (p < 0.0001), whereas age was not (p = 0.18) [adjusted R2 = 0.9031]. This was also true for linear breaking strength (p = 0.0007 and p = 0.97, respectively) [adjusted R2 = 0.71]. Moreover, the influence of age was negligible, as the adjusted R2 and the regression coefficient of bodyweight and its 95% confidence interval were almost identical between univariate und multinomial regressions. ConclusionsOnly weight determines oesophageal length and tissue resilience in rodents, whereas age is irrelevant. If a similar relationship exists in humans, it may facilitate choosing the optimum time point for delayed primary anastomosis. Level of evidenceIV – Experimental Paper.

Double Supercharged Jejunal Interposition for Late Salvage of Long-gap Esophageal Atresia.

A variety of surgical techniques exist to manage long-gap esophageal atresia (LGEA), including gastric pull-up (GPU), colonic interposition (CI), jejunal interposition (JI), and distraction lengthening. Salvage reconstruction for late failure of any conduit type is a complex surgical problem fraught with technical difficulty and significant risk. Jejunal interposition can be used as a salvage procedure in the management of LGEA. However, the opposing requirements of conduit length and adequate perfusion make the procedure technically challenging. Chronic comorbidities and abdominal and thoracic adhesions may further complicate these cases. We report a technique for the management of 3 late treatment failures of LGEA using pedicled JI in conjunction with 2 additional arterial and venous anastomoses, or double supercharging. For 2 patients who presented with failed CI, pedicled JI was performed and supercharged to internal mammary vessels as well as vasculature preserved from the prior colonic flap mesentery. The third patient presented with failed GPU and underwent pedicled JI that was supercharged caudally to the gastroepiploic vessels and cranially to the left common carotid artery. No flaps were lost in any patients. Median operation time was 16.5 hours. Patients were monitored postoperatively in the intensive care unit for a median of 23 days, extubated after 14 days, and discharged at 41 days. Postoperatively, all patients tolerated an oral diet by discharge and continue to enjoy oral intake of all food consistencies without dysphagia or aspiration. Follow-up time spanned 2 to 4 years (average, 3.3 years). One patient required dilatations and temporary stent for stricture, and another required removal of prominent sternal wires; otherwise, no additional procedures were performed. Although technically difficult, double supercharged JI should be considered as a salvage operation to restore esophageal continuity after CI or GPU failure for LGEA, when there are otherwise limited reconstructive options.

Surgical repair of long-gap esophageal atresia: A retrospective study comparing the management of long-gap esophageal atresia in the Nordic countries

BackgroundSeveral surgical procedures have been described in the reconstruction of long-gap esophageal atresia (LGEA). We reviewed the surgical methods used in children with LGEA in the Nordic countries over a 15-year period and the postoperative complications within the first postoperative year. MethodsRetrospective multicenter medical record review of all children born with Gross type A or B esophageal atresia between 01/01/2000 and 12/31/2014 reconstructed within their first year of life. ResultsWe included 71 children; 56 had Gross type A and 15 type B LGEA. Delayed primary anastomosis (DPA) was performed in 52.1% and an esophageal replacement procedure in 47.9%. Gastric pull-up (GPU) was the most frequent procedure (25.4%). The frequency of chromosomal abnormalities, congenital heart defects and other anomalies was significantly higher in patients who had a replacement procedure. The frequency of gastroesophageal reflux (GER) was significantly higher after DPA compared to esophageal replacement (p = 0.013). At 1-year follow-up the mean body weight was higher after DPA than after organ interposition (p = 0.043). ConclusionDPA and esophageal replacement procedures were equally applied. Postoperative complications and follow-up were similar except for the development of GER and the body weight at 1-year follow-up. Long-term results should be investigated. Type of studyTreatment study. Level of evidenceLevel III.

Long-gap esophageal atresia

The management of long-gap esophageal atresia remains challenging with limited consensus on the definition, evaluation, and surgical approach to treatment. Efforts to preserve the native esophagus have been successful with delayed primary anastomosis and tension-based esophageal growth induction processes. Esophageal replacement is necessary in a minority of cases, with the conduit of choice and patient outcomes largely dependent on institutional expertise. Given the complexity of this patient population with significant morbidity, treatment and long-term follow-up are best done in multidisciplinary esophageal and airway treatment centers.

Delayed primary anastomosis for management of long-gap esophageal atresia: a meta-analysis of complications and long-term outcome

Management of newborns with long-gap esophageal atresia (LGEA) remains a challenge for pediatric surgeons. Since spontaneous growth of the esophageal segments occurs without mechanical stretching, initial gastrostomy followed by delayed primary anastomosis (DPA) comprises treatment in most LGEA patients. This meta-analysis aimed to investigate the complications and long-term outcome in patients with LGEA managed by DPA. A systematic literature search was conducted for relevant articles published between 1981 and 2011, and a meta-analysis of complications and long-term outcome was performed. Forty-four articles presented data on 451 newborns with LGEA managed by DPA. Most common variants were pure LGEA (194/451) and LGEA with tracheoesophageal fistula (257/451). Initial gap lengths ranged from 1.9 to 7.0 cm. At the time of DPA, performed at a mean of 11.9 weeks (range 0.5-54.0), the gap had decreased to 0.5-3.0 cm. Mean follow-up was 5.5 years (range 0.5-27.0). Frequent complications were anastomotic leaks/strictures, gastroesophageal reflux (GER), esophagitis and dysphagia. Relative risk for strictures was significantly higher in patients who previously had a leak (p < 0.0001) or GER (p < 0.0001). Patients with GER also had a significantly higher risk for esophagitis (p = 0.0283) and dysphagia (p = 0.0174). The majority of patients could eat without swallowing difficulties at follow-up. DPA provides good long-term functional results. However, the high incidence of GER and associated strictures requires early intervention to prevent feeding problems.

Early primary repair of long gap esophageal atresia: The VATER operation

Despite the numerous approaches described for the management of neonates with “long gap” esophageal atresia, controversy still exists as to the preferred method. Delayed primary anastomosis is probably the most frequently adopted practice, but often the native esophagus is abandoned, and the long gap is bridged by some form of esophageal replacement. A case is reported of a 1.38-kg newborn with pure esophageal atresia and a long gap undergoing early primary repair. The technique used in this patient and the various options available for management of long-gap esophageal atresia are discussed. J Pediatr Surg 35:1830-1832. Copyright © 2000 by W.B. Saunders Company.

Long-gap oesophageal atresia

The various options for the management of long-gap oesophageal atresia are discussed. Of 89 infants treated, 27 had isolated atresia, 6 distal atresia with proximal fistula, and 56 atresia with distal fistula. The preferred approach for the former two groups was oesophageal replacement via gastric transposition. Recently, delayed primary anastomosis has been attempted after 6-12 weeks. For wide-gap atresia with distal fistula, primary anastomosis under marked or extreme tension with elective paralysis and mechanical ventilation for 5 days postoperatively achieved highly successful results in 39 infants. There were no major anastomotic disruptions and only 7 minor leaks. Strictures developed in 72% of cases and gastro-oesophageal reflux in 54%, 66% of whom required antireflux surgery. There was only 1 death in the 43 patients undergoing oesophageal replacement (none after gastric transposition, n = 34). A highly satisfactory outcome was achieved in 85%-90% of infants undergoing a replacement procedure.

Gastric tube interposition: a new technique for the management of long-gap oesophageal atresia

A new technique for the management of long-gap oesophageal atresia maximises the use of existing upper and lower oesophageal pouches and maintains the oesophageal hiatus. It provides a good-calibre neo-oesophagus whilst maintaining the patients' own anti-reflux mechanisms. Two cases are reported in which this technique was successfully used.