Pure Esophageal Atresia Research Articles

Our experience in repairs using the native esophagus such as the Foker and Gazi methods in the management of patients with long-gap esophageal atresia.

This study aimed to share our experience with infants with repaired long-gap esophageal atresia (LGEA) using the native esophagus and Foker and Gazi methods. We retrospectively analyzed the medical records of 10 patients with LGEA (six with pure esophageal atresia [EA], and four with distal trachea-esophageal fistula [TEF] + EA). The mean length between the esophageal pouches was 5.9 cm (4-9 cm). Five Foker methods, three Gazi methods, and two delayed primary repairs after a daily bougie were performed an average of 19.3 days after the first surgery and 26.4 days after the final esophageal anastomosis. For the Foker technique, it was 36.1 days. Their first oral intake was 10.2 days, and their transition to full enteral food was 26.2 days. An esophageal leak was detected in six patients. Fundoplication and dilatations were performed for three and four patients, respectively. For good results, LGEA patients should be operated on at least under the supervision of an experienced surgeon in specialized centers, and the team should be familiar with the techniques for using the native esophagus.

Non-VACTERL Association of Pure Esophageal Atresia with Lumbar Meningocele and White Forelock of Hair: A Case Report

Abstract Background: After the introduction of term VATER for the association of anomalies of vertebra, anorectum, tracheoesophageal, and renal systems by Quan and Smith in 1973, cardiac and limb defects were added by Nora and Nora in 1975, and the acronym was extended to VACTERL. However, other defects such as genitourinary, neural tube defects, and cleft lip or palate are even more common than the VACTERL association. A white forelock of hair is often associated with Hirschsprung’s disease and is considered a part of neurocristopathies but rarely seen with the esophageal atresia (EA) and meningocele. We are reporting such a rare case of EA associated with meningocele and white forelock. Case Report: A one-day-old male baby presented to the emergency room with complaints of respiratory distress, frothing of saliva, and swelling at the back with a white forelock. The baby was diagnosed as pure EA with meningocele, and a surgical repair of both anomalies was done at the same sitting. The baby could not survive due to prematurity, low-birth weight, and multiple congenital anomalies. Conclusion: Presence of white forelock along with this kind of associations should raise the suspicion of severe life-threatening congenital anomalies, and the baby should be kept under neonatal intensive care.

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.

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.

Outcomes of Gastric Transposition in Pure Esophageal Atresia

Outcomes of Gastric Transposition in Pure Esophageal Atresia

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.

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.

DOZ047.102: Previously undescribed association of esophageal atresia, congenital microgastria, and pyloric atresia: a difficult case of ‘pure’ atresia.

Abstract This study reports the case of a male infant born at full term with antenatal suspicion for esophageal atresia (EA). At birth inability to pass a feeding tube and a gasless abdomen on plain film X-ray led to the diagnosis of pure EA according to international guidelines. In the first week of life a stomach could not be identified at surgery for open gastrostomy and a feeding jejunostomy was formed. Subsequent investigations including CT scan did not detect the presence of a stomach. Cervical esophagostomy was performed at 8 weeks old as there was no expectation of achieving an esophageal anastomosis and a deviated septum had complicated the placement of nasal tubes for management of the proximal esophageal pouch. At 6 months of age esophageal replacement with a jejunal pull-up graft was undertaken as staged surgery. At mobilization of a large midline liver to expose the hiatus, a microstomach was revealed, in association with a type 2 pyloric atresia (PA) and a distal tracheoesophageal fistula (TEF). Following ligation of the TEF by lateral thoracotomy, the TEF and microstomach were resected and the jejunal pull-up graft was completed. Discharge home was achieved 1 month post-op. Oral intake was established for solids by 1 year of age. The patient is thriving at 4 years of age with additional overnight jejunal feeds. A unifying genetic diagnosis has not been made to date. Congenital microgastria or PA has been described to co-exist with EA but never previously have all these anomalies been reported occurring together. This unique scenario permitted the distal TEF to be clinically silent and present as a pure atresia. In turn congenital microgastria together with a diagnosis of pure EA obscured the presence of PA, which classically presents with vomiting of feeds and massive gastric distension. Investigations, and in particular cross-sectional imaging, were on the whole unhelpful. This case illustrates that correct diagnosis in this complex case ultimately could only be made at the time of definitive surgery.

Primary repair of long gap esophageal atresia in a neonate employing circular myotomy on upper pouch and a novel hemicircular myotomy on the distal pouch

Abstract Introduction Pure esophageal atresia (EA) with long gap between the pouches remains a challenge for primary repair. Several techniques to facilitate primary repair in long gap EA have been described: complete mobilization of proximal and/or distal esophageal pouch, single or double circular myotomies or spiral myotomy of upper pouch, circular myotomy of both upper and lower pouch, flaps raised from the upper pouch to bridge the gap and external traction sutures. Some of these techniques are not routinely performed for anatomical reasons or unfamiliarity with the technique. Complete mobilization of upper pouch with circular myotomy is standard and often favored approach. Complete mobilization of lower pouch with or without circular myotomy is described. It has a risk of impairing the blood supply to the lower pouch due to segmental nature of its blood supply. We present a novel case of primary repair of this defect using a circular myotomy on upper pouch along with a hemicircular myotomy of the lower pouch without complete circumferential mobilization of the lower esophageal pouch. This allowed adequate lengthening of the lower pouch without compromising its blood supply facilitating a minimal tension primary repair. Case We report the case of a 38 4/7 term infant with intra-uterine growth retardation (IUGR) and Tetralogy of Fallot (TOF) with a long 4.5 cm gap pure esophageal atresia who was repaired aged 16 weeks. Per-operative gap assessment revealed the gap to more than 4 vertebral bodies. Even after complete mobilization of the upper esophageal pouch, a 2.5 cm (cm) gap persisted. The upper pouch was narrow and hence unsuitable to facilitate flap reconstruction. A single circular myotomy was performed on the upper esophageal pouch approximately 1 cm proximal to its blind end. The lower pouch was then mobilized, taking care that its blood supply was not compromised. This still left a one cm gap that was overcome by performing a hemicircular (180°) myotomy on the lower esophageal pouch approximately 1 cm away from the blind end. A tension free primary esophageal anastomosis was then performed. Post operatively patient developed a stricture which was dilated successfully to 12 Fr (4 mm). No other complications are reported. Conclusion Hemicircular myotomy of the distal esophageal pouch can be employed successfully to achieve primary repair in cases of long gap esophageal atresia. This technique does not require full circumferential mobilization of lower pouch as required with full circular myotomy and has less risk of compromising blood supply to the distal esophageal pouch decreasing risks of anastomotic leak, failure and esophageal motility of lower pouch.

Prospective evaluation of clinical outcomes and quality of life after gastric tube interposition as esophageal reconstruction in children.

Few studies on gastric tube interposition for esophageal reconstruction in children have assessed the long-term outcomes and quality of life (QoL). The aim of this study is to evaluate the long-term outcomes and QoL after a gastric tube interposition by reviewing our experiences with esophageal reconstruction.Twenty-six patients were included who underwent gastric tube interposition from 1996 to 2011 at our institution. We reviewed the medical records and conducted telephone surveys, prospectively performed esophagography, endoscopy, 24-hour pH monitoring, and esophageal manometry. The median follow-up period of 12 (range, 3–18) years.Median age at the time of surgery and survey were 9 (range, 2–50) months and 12.4 (range, 3.1–19.0) years, respectively. There were 14 cases of reoperation of gross type C and B esophageal atresia (EA) and 10 cases of long gap pure EA. The z scores of anthropometric data at the survey did not increase after the operation. Severe stricture in esophagography was observed in 20% of patients, but improved with balloon dilation with intact passage. Gastroesophageal reflux was able to be treated with medications. Esophageal peristalsis was observed in 1 of 8 patients in manometry. No Barrett esophagus or metaplasia was not found from endoscopy. QoL was similar to the general population and did not differ between age groups.Gastric tube interposition could be considered for esophageal reconstruction in pediatric patients when native esophageal anastomosis is impossible. Nutritional evaluation and support with consecutive radiological evaluation to assess the anastomosis site stricture are advised.

Magnetic compression anastomosis (magnamosis) in a porcine esophagus: Proof of concept for potential application in esophageal atresia

BackgroundMagnetic compression anastomosis (magnamosis) is the process of forming a sutureless anastomosis of the gastrointestinal tract using two magnetic Harrison rings. It has been shown to be effective in stomach, small bowel and colon, but has not been implemented in the esophagus. A pure esophageal atresia porcine model was developed to test the concept. MethodsFive pigs weighing between 35 and 65 kg were used. In all pigs, a percutaneous endoscopic gastrostomy (PEG) tube was placed, and a right thoracotomy was performed. Esophageal atresia was simulated by transecting the esophagus with a cutting stapler, and magnets were placed endoscopically to approximate the two ends of the esophagus. In the first pig, the tissue within the magnetic ring was excised endoscopically to achieve immediate patency. In the second pig, approximation of the blind esophageal ends was reinforced with 3–4 externally-placed sutures but immediate patency was not performed. In the last three pigs, both external suture reinforcement and immediate patency were performed. The pigs survived for 10–14 days and received nutrition through PEG tube. At necropsy, an esophagram was performed, the specimen was explanted and a leak test was performed. ResultsThe first pig died in the early postoperative period from a leak owing to separation of the magnets. The second pig died from aspiration before the anastomosis formed. The last three pigs survived until the study endpoint. The third pig had a contained leak owing to the staple line being placed between the magnets; this was not clinically significant. The last two pigs had well-formed anastomoses. Burst tests showed no leak when injecting saline up to 30 mmHg. ConclusionMagnamosis is technically feasible for esophagoesophageal anastomoses. A survival model for pure esophageal atresia was developed and refined in pigs. Further work in this area may lead to clinical use in humans.

Surgical Surprise in a Case of Pure esophageal atresia: How High Can the Lower Pouch Be?

The gap between the upper and lower pouches in case of pure esophageal atresia (EA) is usually long and requires multistaged repair. However, in rare cases, the gap may be short and can be repaired primarily commonly through thoracotomy approach. We report an unusual case of a 2-day-old baby girl who presented with short gap EA where the lower esophageal pouch was found adjacent to the blind upper pouch in the neck and primary repair could be performed through the cervical route itself.

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A small proportion of infants with oesophageal atresia (OA) are thought to have a proximal tracheoesophageal fistula (TOF). Failure to recognize these can hamper mobilization of the upper pouch and lead to life-threatening episodes of aspiration once oral feeding starts. We reviewed our experience of upper pouch fistulae to identify characteristic features of proximal TOF.A retrospective review of TOF/OA patient notes and bronchoscopy photographs and videos, identified from our database from 01/01/2006 to 12/31/2015, was performed.Eight (6.1%) infants were identified (M:F 5:3) from a total population of 131 newly diagnosed TOF/OA infants during the period. Their median gestational age was 33 (range 28–39) weeks, and median birth weight was 1647g (range 1100–3400g). Five were initially diagnosed with pure OA and 3 with a distal TOF. All patients underwent rigid bronchoscopy at the initial surgery but only one proximal fistula was identified. The 7 missed proximal fistulae were subsequently found either during on-table oesophagograms for gap assessment (n = 2), at the time of thoracotomy when mobilizing the upper pouch (n = 3), or during subsequent bronchoscopy for symptoms post OA repair (n = 2). Two patients needed a further operation to divide the fistula. Review of the bronchoscopy videos identified four characteristic differences between upper and lower pouch fistulae. Proximal fistulae are found just distal to the vocal cords, are very small, often no more than a pit, do not open and close with ventilation, and are best identified by insufflation of the esophagus.Upper pouch fistulae are relatively easy to miss because of different characteristics compared with H-type or distal fistulae that have not previously been mentioned in the literature.Level of evidence: level IV.

Creation of an Esophageal Atresia Animal Model Using a Bifurcated Esophagus to Maintain Digestive Tract Continuity.

We previously developed a porcine model of long gap pure esophageal atresia (EA) to aid in the creation of novel devices and techniques for treatment of EA. Shortcomings of the model included difficulty decompressing the proximal esophageal pouch (leading to recurrent aspiration) and a requirement for gastrostomy tube (G-tube) feeds. Therefore, we sought to create a porcine model with a bifurcated esophagus wherein one portion of the esophageal lumen retained patency and the other part mimicked EA. After G-tube placement, thoracotomy was performed with subsequent partial stapled transection of the esophagus in a transverse manner, followed by longitudinal, partial stapled transection of the esophagus proximally and distally. Magnets were placed in the esophageal pouches. Proximal and distal esophageal pouches were created while preserving a parallel, narrower segment of continuous esophagus. G-tube feeds were used initially, but the animal ultimately tolerated full nutrition by mouth. The magnets successfully restored esophageal continuity. The animal regained much of the initial weight lost and survived to planned euthanasia. Necropsy revealed anastomosis formation without evidence of leak. A bifurcated porcine esophagus model was successfully devised, simulating EA while allowing the animal to receive oral feeds and clear oral secretions. This model is anticipated to promote animal well-being and ease of care during future investigations, as well as a platform for the safe development of new therapies for EA.

Fundoplication in Patients with Esophageal Atresia: Patient Selection, Indications, and Outcomes.

Patients with esophageal atresia (EA) suffer from abnormal and permanent esophageal intrinsic and extrinsic innervation that affects severely esophageal motility. The repair of EA also results in esophageal shortening that affects distal esophageal sphincter mechanism. Consequently, gastroesophageal reflux (GER) is common in these patients, overall approximately half of them suffer from symptomatic reflux. GER in EA patients often resists medical therapy and anti-reflux surgery in the form of fundoplication is required. In patients with pure and long gap EA, the barrier mechanisms against reflux are even more damaged, therefore, most of these patients undergo fundoplication during first year of life. Other indications for anti-reflux surgery include recalcitrant anastomotic stenoses and apparent life-threatening episodes. In short term, fundoplication alleviates symptoms in most patients but recurrences are common occurring in at least one third of the patients. Patients with fundoplication wrap failure often require redo surgery, which may be complicated and associated with significant morbidity. A safe option in a subset of patients with failed anti-reflux surgery appears to be long-term medical treatment with proton pump inhibitors.

13 ribs as a predictor of long gap esophageal atresia: myth or reality? Analysis of associated findings of esophageal atresia and abnormal rib count

BackgroundThe presence of 13 pairs of ribs on pre-operative chest x-ray has been postulated to be an indicator for long gap esophageal atresia (EA). This study sought to determine the validity of this theory and identify associated pathological conditions in patients with EA and abnormal rib count. MethodsBabies with EA from January 2005 – December 2012 were retrospectively analyzed. Information was gathered from neonatal health records and operation notes. Chest x-rays were reviewed to determine rib count. Long gap EA was defined as failure to achieve primary esophageal anastomosis. Statistical analysis performed with Fisher's exact test. ResultsSeventy-six patients were identified. Eight patients had long gap EA, with none of these patients having 13 pairs of ribs.Paradoxically, 10 patients with esophageal atresia +/− trachea-esophageal atresia (EA +/− TEF) and supernumerary ribs underwent primary repair.Nine patients had 11 pairs of ribs, of which 2 had pure EA and a long gap.Using Fisher's exact test to compare the groups of supernumary ribs and non-supernumary ribs there is a p value of 0.587.VACTERL association was identified in 40% of those with supernumerary ribs. Various associated syndromes and concomitant abnormalities were identified. ConclusionWe found no association between 13 pairs of ribs and long gap in esophageal atresia. Those with 13 pairs of ribs were more likely to have associated anomalies, although this was not statistically significant. Our cohort of patients was found to have a range of pathology related to genetic syndromes, further atresias, and malformations, which is well known to be associated with children born with EA +/− TEF. Level of evidencePrognosis study – level IV.

Twin pregnancy complicated by esophageal atresia, duodenal atresia, gastric perforation, and hypoplastic left heart structures in one twin: a case report and review of the literature

BackgroundThe antenatal diagnosis of a combined esophageal atresia without tracheoesophageal fistula and duodenal atresia with or without gastric perforation is a rare occurrence. These diagnoses are difficult and can be suspected on ultrasound by nonspecific findings including a small stomach and polyhydramnios. Fetal magnetic resonance imaging adds significant anatomical detail and can aid in the diagnosis of these complicated cases. Upon an extensive literature review, there are no reports documenting these combined findings in a twin pregnancy. Therefore we believe this is the first case report of an antenatal diagnosis of combined pure esophageal and duodenal atresia in a twin gestation.Case presentationWe present a case of a 30-year-old G1P0 white woman at 22-week gestation with a monochorionic-diamniotic twin pregnancy discordant for esophageal atresia, duodenal atresia with gastric perforation, hypoplastic left heart structures, and significant early gestation maternal polyhydramnios. In this case, fetal magnetic resonance imaging was able to depict additional findings including area of gastric wall rupture, hiatal hernia, dilation of the distal esophagus, and area of duodenal obstruction and thus facilitated the proper diagnosis. After extensive counseling at our multidisciplinary team meeting, the parents elected to proceed with radiofrequency ablation of the anomalous twin to maximize the survival of the normal co-twin. The procedure was performed successfully with complete cessation of flow in the umbilical artery and complete cardiac standstill in the anomalous twin with no detrimental effects on the healthy co-twin.ConclusionsPrenatal diagnosis of complex anomalies in twin pregnancies constitutes a multitude of ethical, religious, and cultural factors that come into play in the management of these cases. Fetal magnetic resonance imaging provides detailed valuable information that can assist in management options including possible prenatal intervention. The combination of a cystic structure with peristalsis-like movement above the diaphragm (for example, “the upper thoracic pouch sign”), polyhydramnios, and progressive distention of the stomach and duodenum should increase suspicion for a combined pure esophageal and duodenal atresia.

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.

Characterization of the upper pouch tracheo–oesophageal fistula in oesophageal atresia

AimA small proportion of infants with oesophageal atresia (OA) are thought to have a proximal tracheoesophageal fistula (TOF). Failure to recognize these can hamper mobilization of the upper pouch and lead to life-threatening episodes of aspiration once oral feeding starts. We reviewed our experience of upper pouch fistulae to identify characteristic features of proximal TOF. MethodsA retrospective review of TOF/OA patient notes and bronchoscopy photographs and videos, identified from our database from 01/01/2006 to 12/31/2015, was performed. ResultsEight (6.1%) infants were identified (M:F 5:3) from a total population of 131 newly diagnosed TOF/OA infants during the period. Their median gestational age was 33 (range 28–39) weeks, and median birth weight was 1647g (range 1100–3400g). Five were initially diagnosed with pure OA and 3 with a distal TOF. All patients underwent rigid bronchoscopy at the initial surgery but only one proximal fistula was identified. The 7 missed proximal fistulae were subsequently found either during on-table oesophagograms for gap assessment (n=2), at the time of thoracotomy when mobilizing the upper pouch (n=3), or during subsequent bronchoscopy for symptoms post OA repair (n=2). Two patients needed a further operation to divide the fistula. Review of the bronchoscopy videos identified four characteristic differences between upper and lower pouch fistulae. Proximal fistulae are found just distal to the vocal cords, are very small, often no more than a pit, do not open and close with ventilation, and are best identified by insufflation of the esophagus. ConclusionUpper pouch fistulae are relatively easy to miss because of different characteristics compared with H-type or distal fistulae that have not previously been mentioned in the literature.Level of evidence: level IV.

Creation of an animal model for long gap pure esophageal atresia.

Long gap pure esophageal atresia (LGPEA) is a congenital disorder in which the esophagus is in discontinuity, and the proximal and distal ends cannot be anastomosed in a primary fashion. No animal model for pure esophageal atresia exists. Here we describe a survival animal model for LGPEA, which will ultimately serve to test novel devices and techniques to restore continuity. A non-survival study was first conducted in six rabbits to refine a protocol for the survival model. An open gastrostomy tube was placed, followed by a partial esophagectomy. Next, a survival study was performed with seven rabbits in which the same procedures were performed. Finally, the procedure was optimized in domestic swine. Despite developing the techniques and gaining valuable information in the non-survival study, none of the rabbits in the survival portion of the study lived beyond post-operative day four. Due to this complication with the rabbit, the LGPEA model was attempted in a porcine model. The pig survived to post-operative day ten, and was healthy enough to be used for further study. A porcine model of long gap pure esophageal atresia was developed which is effective and feasible to be used for testing new methods of treatment of LGPEA.