Long-gap Esophageal Atresia Research Articles

Impact of serial clinical swallow evaluations and feeding interventions on growth and feeding outcomes in children with long-gap esophageal atresia after anastomosis: a retrospective cohort study.

Children undergoing surgical anastomosis for long-gap esophageal atresia (LGEA) often suffer from complications related to delayed oral feeding, which may impair their early development. Clinical swallow evaluation (CSE) is an effective technique to improve feeding outcomes. However, there are limited evidences on the application of CSE in these children. Since 2020, serial CSEs have been consistently implemented for children undergoing anastomosis for LGEA in our hospital. We conducted a retrospective study comparing 19 children who received CSE with 31 historical controls who did not. Inverse probability of treatment weighting (IPTW) was applied to balance preoperative characteristics. We compared the time from surgery to full oral feeding and the rate of postoperative complications between the two groups. Growth curves for length-for-age Z score (LAZ) and weight-for-age Z score (WAZ) up to age 3 were fitted using generalized additive mixed models. The median time to full oral feeding was 1.1months [interquartile range (IQR), 0.8-2.4] in the CSE group and 1.5months (IQR, 0.6-5.7) for controls. After IPTW, CSE was associated with a shorter time to full oral feeding, with a weighted hazard ratio of 2.26 [95% confidence interval (CI), 1.21 to 4.24]. LAZ growth curves significantly differed between groups (P = 0.001). CSE was associated with the expedited achievement of full oral feeding and a more favorable growth pattern before 3years of age.

Clinical translation of tissue-engineered oesophageal grafts: are patients ready for us?

PurposeWe sought to engage with expert patient/carers to understand attitudes towards use of tissue engineering (TE) for long-gap oesophageal atresia (OA).MethodsAn in-person engagement event for 70 patients/parents was held by the OA patient group, TOFS. Attitudes towards TE were assessed before and after a talk on use of TE oesophagi in a pre-clinical OA model. Perceptions were assessed using a 5-point Likert scale (median [range]) and compared using Mann–Whitney test.Results43 attendees responded; 56% parents/caregivers, 21% patients, 7% healthcare workers, 16% unreported. Most (85%) had some awareness of TE but for 15%, it was a new concept. Attendees were receptive to TE; 89% reported no concerns about growth of their/child(s) cells in a lab and 61% reported no concerns about using animal products. Perceptions of TE significantly improved after the presentation from 4 (2–5, n = 32) to 5 (3–5, n = 28) p < 0.0001, and 96% would like to be involved in focus groups on development of a TE product for use in OA.ConclusionInput from key stakeholders is essential to introduction of TE constructs clinically. The overall response to TE constructs was positive, and informs development of an OA-specific focus group to guide translation.

Esophagitis, treatment outcomes, and long-term follow-up in children with esophageal atresia.

Survival rates in children born with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) have improved; however, morbidity associated with the disease remains high. This study aimed to assess the prevalence of gastroesophageal reflux disease (GERD), eosinophilic esophagitis (EoE), fungal esophagitis, esophageal strictures, and long-term outcomes in children with EA/TEF. We conducted a retrospective chart review on patients with EA/TEF who were seen at Children's Wisconsin from January 2003 to January 2023. Patients born with EA/TEF were included if they underwent at least one endoscopy after 1 year of age. GERD was diagnosed based on abnormal findings on endoscopy, pH-metry, and/or history of fundoplication. EoE and fungal esophagitis were diagnosed based on abnormal endoscopy. Esophageal stricture diagnosis was based on findings on endoscopy and/or esophagram, and clinical symptoms necessitating esophageal dilation. Eighty-five patients (64.7% males, mean age 7.5 years) were included, the majority had type C EA/TEF (90.6%). GERD was diagnosed in 61.1% (n = 52), 49.4% (n = 42) by macro and/or microscopic endoscopic findings, 22.3% (n = 19) by abnormal pH-metry, and 21.1% (n = 18) by the need for fundoplication for refractory reflux and/or esophageal stricture. Risk of GERD increased with lower gestational age (p = 0.0030), lower birth weight (p = 0.023), and long-gap EA (p = 0.034). In children diagnosed with GERD, only 13.4% of patients (n = 7/52) were able to be weaned off proton pump inhibitor (PPI) without disease recurrence. However, overall, at the completion of the study, 44.7% (n = 38) of patients were successfully weaned off PPI without evidence of GERD. EoE was diagnosed in 20% of the patients (n = 17). All patients diagnosed with EoE required escalation of therapy from PPI alone to swallowed corticosteroids in 52.9% (n = 9), dupilumab in 23.5% (n = 4), elemental formula in 17.6% (n = 3), and elemental formula and swallowed steroids in 5.8% (n = 1). Fungal esophagitis was diagnosed in 15.3% of patients (n = 13). An esophageal stricture requiring dilation was diagnosed in 77.6% (n = 66) of patients at a mean age of 28.5 months, with over 60% diagnosed by 24 months of age. Children born with EA/TEF continue to be at high risk of developing GERD, EoE, fungal esophagitis, and esophageal stenosis. Diagnostic and therapeutic endoscopy remains a high-yield test to identify and treat these comorbidities.

Clinical outcomes of endoscopic balloon dilation for refractory esophageal strictures after esophageal atresia repair

BackgroundManaging refractory esophageal strictures (RES) presents formidable challenges. Although endoscopic balloon dilation (EBD) is the first step for esophageal stricture, the clinical outcomes of EBD for RES after esophageal atresia (EA) repair are not established. MethodsAll EA patients with esophageal balloon strictures (EBS) from October 2016 to October 2022 treated by EBD in our institution were retrospectively reviewed. The primary endpoint was to evaluate the clinical outcomes and the risk factors for poor outcomes of EBD for RES. The secondary endpoint was to evaluate the risk factors for pathological weight in RES patients. Results87 patients with RES were included in our study. After the first session of EBDs, 40.2 % experienced a recurrence of esophageal strictures. The median number (IQR) of the first session of EBD was 13.0 (8.0, 16.0), and the median number (IQR) of total dilations of achieving long-term clinical success was 14.0 (10.0, 19.0) with 81.6 % achieving long-term clinical success with less than 20 EBDs. In follow-up, all patients achieved a total oral diet. On multivariable analysis, the presence of GERD (OR 4.17, 95%Cl 1.29–13.51, p = 0.017), LGEA (long-gap esophageal atresia) (OR 5.19, 95 % Cl 1.15–23.52, p = 0.033), eccentric stricture shape (OR 3.34, 95%Cl 1.06–10.53, p = 0.040), and longer stricture length (OR 10.22, 95%Cl 1.14–92.01, p = 0.038) were statistically significant associated with increased endoscopic dilations. The presence of LGEA (OR 3.25, 95%Cl 1.03–10.20, p = 0.044) was significantly associated with recurrence after short-term clinical success. Additionally, Older age at first dilation after LEAP, stricture level at 1/3 upper (ref = 1/3 middle), and LGEA were identified as risk factors for developing pathological weight. ConclusionEndoscopic balloon dilation is an effective method for treating RES after EA repair. GERD, LGEA, eccentric stricture shape, and longer stricture length are the risk factors for increased dilation times. Older age at first dilation after LEAP, stricture level at 1/3 upper, and LGEA were identified as risk factors for developing pathological weight.

Critical Role of Pleural Wrap and Post-operative Neonatal Protocol in Long-gap Oesophageal Atresia: A Team Effort.

The objectives of this study were to bring out the results of application of pleural wrap in primary repair of tracheo - esophageal fistula (TEF) with long-gap oesophageal atresia (LGEA) and also define the role of neonatologists and paediatric intensivists in post-operative management in these patients by laying down standard neonatal management protocol. This was a retrospective descriptive observational study conducted between March 2011 and April 2019 on 23 cases of LGEA with TEF. The study was conducted at two tertiary care paediatric surgery centres in Northern part of India wherein these newborn babies were operated by two paediatric surgeons with variable experience of 8-12 years. It also describes the neonatal management protocol used in post-operative period. Out of 23 patients, 15 were boys and 8 were girls, with a mean age of 32.25 weeks and a mean birth weight of 2.02 kg. The mean hospital stay was 23.5 ± 8 days. Eleven cases had gap between 3 and 3.5 cm, 8 cases between 3.5 and 4 cm and 4 cases had gap more than 4 cm. The incidence of associated anomalies was 52%. Anastomotic leak rate was 8.69%, and 3 (13.04%) patients died in the post-operative period. All the operated patients were managed postoperatively as per strict neonatal management protocol exclusively by the team of neonatologists and neonatal intensivists. Application of pleural wrap over anastomosis following primary repair of LGEA with TEF significantly reduced the incidence of anastomotic leak in our study. Apart from the pleural wrap, the key to successful outcome also is contributed by the little prolonged, controlled ventilation and patience and perseverance in post-operative feeds. This post-operative management protocol that has been followed by us in our study is easily reproducible and can be adopted by paediatric surgeons working alongside neonatologists as a team.

Management Strategies and Outcomes of Distal Congenital Esophageal Strictures in the Setting of Long-gap Esophageal Atresia

BackgroundThe management of neonates with long-gap esophageal atresia (LGEA) combined with distal congenital esophageal strictures (CES) is challenging. We sought to review our approach for this rare set of anomalies. MethodsWe reviewed children with LGEA + CES surgically treated at two institutions (2018–2024). LGEA repair was performed using the Foker technique (traction-induced esophageal lengthening). A CES strategy was chosen based on preoperative evaluations and intraoperative findings. The configuration and length of the CES were assessed using retrograde flexible esophagoscopy via gastrostomy with contrast fluoroscopy. ResultsEight patients (75% male) with LGEA + CES were treated: Four had type A and four had type B EA. Median gap length was 3.5 cm. Three underwent thoracoscopic esophageal lengthening. After a median follow-up of 18 months (IQR: 9–25), all retained their native esophagus. However, those who had CES resection concurrent with the lengthening process or at the time of EA anastomosis had more challenging perioperative courses: one required additional time on traction and another required esophageal anastomotic stricture resection. ConclusionsOur experience with LGEA and distal CES emphasizes tailoring surgical approaches to each patient's unique condition, avoiding a one-size-fits-all strategy. However, if the esophageal tissue above the distal CES is in good condition, our preference has shifted towards retaining the CES during traction, performing gentle dilation at anastomosis time, and conducting definitive endoscopic management subsequently. We would caution against making the assumption that salvage of the native esophagus is not possible or that resection of the CES is always needed. Level of EvidenceLevel III.

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.

Botulinum Toxin Enhanced Foker Process for Long Gap Esophageal Atresia

BackgroundThe traction-induced esophageal growth (Foker) process for the treatment of long gap esophageal atresia (LGEA) relies on applying progressive tension to the esophagus to induce growth. Due to its anti-fibrotic and muscle-relaxing properties, we hypothesize that Botulinum Toxin A (BTX) can enhance traction-induced esophageal growth. MethodsA retrospective two-center cohort study was conducted on children who underwent a BTX-enhanced Foker process for LGEA repair from 2021 to 2023. BTX (10 units/ml, 2 units/kg, per esophageal pouch) was applied at the time of traction initiation. Time on traction, complications, and anastomotic outcomes were compared against historical controls (Foker process without BTX) from 2014 to 2021. ResultsTwenty infants (LGEA type A:12, B:4, C:4; 35% reoperative; median [IQR] age 3 [2–5] months), underwent BTX-enhanced Foker process (thoracotomy with external traction: 9; minimally invasive [MIS] multi-staged internal traction: 11). Mean gap lengths were similar between BTX-enhanced external and external traction control patients (mean [SD], 50.6 mm [12.6] vs. 44.5 mm [11.9], p = 0.21). When compared to controls, the BTX-enhanced external traction process was significantly faster (mean [SD], 12.1 [1.6] days vs. 16.6 [13.2] without BTX, p = 0.04) despite similar preoperative gap lengths. There was no difference in time on traction for those undergoing a minimally invasive process. There were no significant differences in complications or anastomotic outcomes in either cohort. ConclusionBotulinum toxin may play a role in accelerating the traction-induced esophageal growth process for LGEA repair. Minimizing time on traction can decrease sedation and paralysis burden while on external traction. Further studies are needed to elucidate the effects of BTX on the esophagus. Level of EvidenceLevel III. Type of StudyRetrospective, Two-center, Cohort study.

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.

Analysis of gap length as a predictor of surgical outcomes in esophageal atresia with distal fistula: a single center experience.

Long-gap esophageal atresia (LGEA) is still a challenge for pediatric surgery. No consensus exists as to what constitutes a long gap, and few studies have investigated the maximum gap length safely repairable by primary anastomosis. Based on surgical outcomes at a single institution, we aimed to determine the gap length in LGEA with a high risk of complications. The medical records of 51, consecutive patients with esophageal atresia (EA) with primary repair in the early neonatal period between 2001 and 2021 were retrospectively reviewed. Three, major complications were found in the surgical outcomes: (1) anastomotic leakage, (2) esophageal stricture requiring dilatation, and (3) GERD requiring fundoplication. The predictive power of the postsurgical complications was assessed using receiver operating characteristic analysis, and the area under the curve (AUC) and the cutoff value with a specificity of > 90% were calculated. Sixteen patients (31.4%) experienced a complication. The AUC of gap length was0.90 (p < 0.001), and the gap length cutoff value was ≥ 2.0cm for predicting any complication (sensitivity: 62.5%, specificity: 91.4%). A gap length ≥ 2.0cm was considered as defining LGEA and was associated with an extremely high complication rate after primary repair.

Experience in the treatment of long-gap esophageal atresia by intraluminal esophageal stretching elongation.

To summarize the experience with intraluminal esophageal stretching elongation (ILESE) in the successful treatment of long-gap esophageal atresia (LGEA) at a single center. Clinical data of 68 neonates who underwent LGEA between February 2015 and January 2022 were retrospectively analyzed. Four patients died of multiple associated severe malformations and did not undergo ILESE. Esophageal anastomosis was successfully performed in 60 cases (93.75%) and failed in 4 cases (6.25%) treated with ILESE. The ILESE techniques, esophageal reconstruction, results, postoperative complications, and follow-up treatment were analyzed. The beginning time of performing ILESE preoperation was 53.4 ± 39.4 days after birth, and the age of esophageal reconstruction was 122.2 ± 70.3 days after birth in 60 cases. The gap length of proximal and distal esophageal segments which were evaluated the first time at admission was 4.8 ± 1.3 vertebral bodies, whereas the gap before anastomosis was -0.46 ± 0.90 vertebral bodies. Among the patients with esophageal primary-anastomosis, 55 received thoracoscopic surgery, and 5 underwent thoracotomy in the early stage. Of the 60 children with ILESE, 58 underwent end-to-end esophagostomy, of which 17 cases were combined with circular esophagotomy (livaditis), and 2 cases of esophageal lengthening were combined with the reversal of the ligulate loop of the proximal esophagus (flap). Overall, 59 cases were cured (98.3%), and 1 patient died of respiratory failure postoperatively. All patients were followed up for 7-96 months. Postoperative anastomotic leakage occurred in 16 patients (27.6%), all of whom were successfully treated conservatively. Anastomotic stenosis occurred in 49 cases (83.1%), all of which were successfully managed by non-surgical treatment, including 12.7 ± 9.3 times of esophageal balloon dilatation and 2 cases of stent dilatation. Gastroesophageal reflux occurred in 44 patients (74.6%), including associated or acquired esophageal hiatal hernia in 22 patients, and Nissen fundoplication was performed in 17 patients. ILESE is an effective method for prolonging the proximal and distal esophagus of the LGEA to reconstruct esophageal continuity using its esophageal tissue, with an efficacy rate of 93.75%. Postoperative anastomotic stricture and gastroesophageal reflux are common and require long-term, standardized follow-up and treatment.

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.

Thoracoscopic Stage Internal Traction Repair Reduces Time to Achieve Esophageal Continuity in Long Gap Esophageal Atresia.

Management of long gap esophageal atresia (LGOA) is controversial. This study aims at comparing the management of LGOA between two high-volume centers. We included patients with LGOA (type A and B) between 2008 and 2022. Demographics, surgical methods, and outcomes were collected and compared. The study population involved 28 patients in center A and 24 patients in center B. A surgical approach was thoracoscopic in center A, only for one patient was open for final procedure. In center B, 3 patients were treated only thoracoscopically, 2 converted to open, and 19 as open surgery. In center A primary esophageal anastomosis concerned 1 case, two-staged esophageal lengthening using external traction 1 patient, and 26 were treated with the multistaged internal traction technique. In 24 patients a full anastomosis was achieved: in 23 patients only the internal traction technique was used, while 1 patient required open Collis-Nissen procedure as final management. In center B primary anastomosis was performed in 7 patients, delayed esophageal anastomosis in 8 patients, esophageal lengthening using external traction in 1 case, and 9 infants required esophageal replacement with gastric tube. Analyzed postoperative complications included: early mortality, 2/28 due to accompanied malformations (center A) and 0/24 (center B); anastomotic leakage, 4/26 (center A) treated conservatively-all patients had a contrast study-and 0/24 (center B), 1 case of pleural effusion, but no routine contrast study; recurrent strictures, 13/26 (center A) and 7/15 (center B); and need for fundoplication, 5/26 (center A) and 2/15 (center B). Age at esophageal continuity was as a median of 31 days in center A and 110 days in center B. Median time between initial procedure and esophageal anastomosis was 11 days in center A and 92 days in center B. Thoracoscopic internal traction technique reduces time to achieve esophageal continuity and the need for esophageal substitution while maintaining a similar early complication rate.

Long-gap esophageal atresia: is native esophagus preservation always possible?

Esophageal atresia (EA) is a congenital defect that causes esophageal discontinuity, often with an associated tracheo-esophageal fistula (TEF) in 70%-90% of cases. When the distance between esophageal ends precludes primary anastomosis, it results in long gap esophageal atresia (LGEA), complicating the surgical management. This study retrospectively reviewed LGEA cases from the past decade, treated with the goal of preserving the native esophagus, comparing surgical techniques and outcomes with current literature. The data of patients treated for LGEA between 2013 and 2024 were collected from medical charts, focusing on patients treated with the preservation of their native esophagus. Ten patients were enrolled for this study. All of them had a gap between the esophageal ends equal to or greater than three vertebral bodies. Four patients (40%) underwent a delayed primary anastomosis (DPA) procedure, while the remaining six (60%) underwent a traction staged repair. All patients were treated with open surgery. The follow-up period extended from 3 months to 10 years. Preserving the native esophagus in patients with LGEA is a challenging but feasible goal, with delayed primary anastomosis and traction techniques playing key roles. We advocate for the preservation of the native esophagus as the preferred approach for ensuring a high quality of life for patients, as it helps to avoid severe long-term complications associated with esophageal substitution.

The History and Legacy of the Foker Process for the Treatment of Long Gap Esophageal Atresia

Historically, children afflicted with long gap esophageal atresia (LGEA) had few options, either esophageal replacement or a life of gastrostomy feeds. In 1997, John Foker from Minnesota revolutionized the treatment of LGEA. His new procedure focused on “traction-induced growth” when the proximal and distal esophageal segments were too far apart for primary repair. Foker's approach involved placement of pledgeted sutures on both esophageal pouches connected to an externalized traction system which could be serially tightened, allowing for tension-induced esophageal growth and a delayed primary repair.Despite its potential, the Foker process was received with criticism and disbelief, and to this day, controversy remains regarding its mechanism of action - esophageal growth versus stretch. Nonetheless, early adopters such as Rusty Jennings of Boston embraced Foker's central principle that “one's own esophagus is best” and was instrumental to the implementation and rise in popularity of the Foker process. The downstream effects of this emphasis on esophageal preservation would uncover the need for a focused yet multidisciplinary approach to the many challenges that EA children face beyond “just the esophagus”, leading to the first Esophageal and Airway Treatment Center for children. Consequently, the development of new techniques for the multidimensional care of the LGEA child evolved such as the posterior tracheopexy for associated tracheomalacia, the supercharged jejunal interposition, as well as minimally invasive internalized esophageal traction systems. We recognize the work of Foker and Jennings as key catalysts of an era of esophageal preservation and multidisciplinary care of children with EA.

Long-term follow-up after retrosternal ileocolic esophagoplasty in two cases of long-gap esophageal atresia: why it is still a valid option as a rescue strategy

IntroductionEsophageal replacement surgery in children is sometimes necessary for long-gap esophageal atresia. Ileocolic esophagoplasty in the retrosternal space can serve as a good alternative technique in case of hostile posterior mediastinum. We present two cases of successful ileocolic transposition performed at 6 months of age.MethodsEsophageal replacement was performed through a midline laparotomy incision associated with a left cervical approach. The ileocolic transplant was pediculized on the right superior colic artery after ligating the right colic and ileocolic vessels. A retrosternal tunnel was created, and the ileocolic transplant pulled through it to reach the cervical region. Proximally, esophageal-ileal anastomosis and, distally, colonic–gastric anastomosis were performed. Ileocolic continuity was repaired.ResultsThere were no early postoperative complications. In both cases, the patients presented oral feeding difficulties during the first 6 postoperative months. Thereafter, full oral feeding was achieved, and both patients were clinically asymptomatic during the following 18 and 20 years, respectively, with satisfactory oral radiological assessments, showing no redundancy or inappropriate growth of the graft and no anastomotic stricture. Currently, these patients do not complain of dysphagia, pathological reflux, or respiratory symptoms.ConclusionWhen native esophagus preservation in long-gap esophageal atresia is estimated unfeasible, ileocolic transposition in the retrosternal space might be considered a good and safe option, particularly in those difficult cases after multiple previous surgical attempts and mediastinitis. This technique is putatively associated with a beneficial anti-reflux effect, thanks to the presence of the ileocecal valve, in preventing cervical peptic esophagitis. Long-term follow-up confirms that the transposed colon in the retrosternal space did not suffer any abnormal modification in size and growth.

Kimura extrathoracic esophageal elongation for the treatment of complex esophageal atresia: Primary versus secondary treatment when other strategies failed

IntroductionThe Kimura´s extrathoracic esophageal elongation (KEEE) procedure uses upper esophageal pouch traction to induce its growth, as an alternative to repair long gap esophageal atresia (LGEA). It can be used as a first option in the neonatal period (primary Kimura) or as a rescue procedure when another strategy fails (secondary Kimura).The aim of our study is to compare the results of the primary versus secondary Kimura procedure after unsuccessful Puri procedure or end-to-end esophageal anastomosis. MethodsThe present work is a retrospective review of patients with complex esophageal atresia (CEA) who underwent Kimura procedure at our institution from 1997 to 2021. We divided the patients into two groups, primary group: patients who underwent primary Kimura extrathoracic esophageal elongation (KEEE), esophagostomized in the first surgical intervention; and secondary group: those who underwent secondary KEEE, esophagostomized due to complications of the primary end-to-end anastomosis (EEA), or due to failure of preservation of native esophagus (PNE) techniques. Demographic variables (gestational age, birth weight, type of esophageal atresia (EA) and associated malformations) and evolution variables related to the elongations and reconstruction (days of mechanical ventilation (MV), days of hospitalization and complications of the anastomosis, feeding/oral aversion, need for nutritional support, gastroesophageal reflux disease (GERD), and mortality) were evaluated. ResultsDuring the 24-year period, 31 patients were evaluated, nine patients belonged to the primary group (of which 7 were reconstructed) and the remaining twenty-two to the secondary group (of which 12 completed the procedure).Regarding demographic variables, there was a statistical difference in gestational age (p = 0.016). Regarding the variables associated with the elongations, there was a statistically significant lower number of elongations (p = 0.0547), longer hospitalization time (p = 0.045) and more complications (p = 0.027) for the secondary group. Regarding the final reconstruction, the primary group had a significantly shorter postoperative hospital stay (p = 0.03), and also fewer days on MV, although these variables did not reach a statistically significant difference.Two patients died before the final reconstruction (one from each group). DiscussionWe believe that KEEE is a useful tool for both primary treatment and rescue in case of failure of other esophageal preservation strategies. In addition, it allows preserving the native esophagus and also performing esophageal replacement strategies with other organs if it fails. Level of evidenceAccording to the Journal of Pediatric Surgery this research corresponds to type of study level III for retrospective comparative study.

Primary Posterior Tracheopexy at Time of Esophageal Atresia Repair Significantly Reduces Respiratory Morbidity

PurposeEsophageal atresia with tracheoesophageal fistula (EA/TEF) is often associated with tracheobronchomalacia (TBM), which contributes to respiratory morbidity. Posterior tracheopexy (PT) is an established technique to treat TBM that develops after EA/TEF repair. This study evaluates the impact of primary PT at the time of initial EA/TEF repair. MethodsReview of all newborn primary EA/TEF repairs (2016–2021) at two institutions. Long-gap EA and reoperative cases were excluded. Based on surgeon preference and preoperative bronchoscopy, neonates underwent primary PT (EA + PT Group) or not (EA Group). Perioperative, respiratory and nutritional outcomes within the first year of life were evaluated. ResultsAmong 63 neonates, 21 (33%) underwent PT during EA/TEF repair. Groups were similar in terms of demographics, approach, and complications. Neonates in the EA + PT Group were significantly less likely to have respiratory infections requiring hospitalization within the first year of life (0% vs 26%, p = 0.01) or blue spells (0% vs 19%, p = 0.04). Also, they demonstrated improved weight-for-age z scores at 12 months of age (0.24 vs −1.02, p < 0.001). Of the infants who did not undergo primary PT, 10 (24%) developed severe TBM symptoms and underwent tracheopexy during the first year of life, whereas no infant in the EA + PT Group needed additional airway surgery (p = 0.01). ConclusionIncorporation of posterior tracheopexy during newborn EA/TEF repair is associated with significantly reduced respiratory morbidity within the first year of life. Level of evidenceLevel III.

Graft dilatation and Barrett's esophagus in adults after gastric pull-up and jejunal interposition for long-gap esophageal atresia.

Esophageal replacement (ER) with gastric pull-up (GPU) or jejunal interposition (JI) used to be the standard treatment for long-gap esophageal atresia (LGEA). Changes of the ER grafts on a macro- and microscopic level however, are unknown. To evaluate long-term clinical symptoms and anatomical and mucosal changes in adolescents and adults after ER for LGEA. A cohort study was conducted including all LGEA patients ≥ 16 years who had undergone GPU or JI between 1985-2003 at two tertiary referral centers in the Netherlands. Patients underwent clinical assessment, contrast study and endoscopy with biopsy. Data was collected prospectively. Group differences between JI and GPU patients, and associations between different outcome measures were assessed using the Fisher's exact test for bivariate variables and the Mann-Whitney U-test for continuous variables. Differences with a P-value < 0.05 were considered statistically significant. Nine GPU patients and eleven JI patients were included. Median age at follow-up was 21.5 years and 24.4 years, respectively. Reflux was reported in six GPU patients (67%) vs four JI patients (36%) (P = 0.37). Dysphagia symptoms were reported in 64% of JI patients, compared to 22% of GPU patients (P = 0.09). Contrast studies showed dilatation of the jejunal graft in six patients (55%) and graft lengthening in four of these six patients. Endoscopy revealed columnar-lined esophagus in three GPU patients (33%) and intestinal metaplasia was histologically confirmed in two patients (22%). No association was found between reflux symptoms and macroscopic anomalies or intestinal metaplasia. Three GPU patients (33%) experienced severe feeding problems vs none in the JI group. The median body mass index of JI patients was 20.9 kg/m2 vs 19.5 kg/m2 in GPU patients (P = 0.08). The majority of GPU patients had reflux and intestinal metaplasia in 22%. The majority of JI patients had dysphagia and a dilated graft. Follow-up after ER for LGEA is essential.

Extrathoracic esophageal elongation with Foker technique in patients with Type-A esophageal atresia. A case series

Background: Long-gap esophageal atresia (EA) is not amenable to primary anastomosis though the goal of surgical treatment is to restore the patency in the native esophagus; the Foker technique is one of the methods that can accomplish it. Case Presentation: This case series consists of three patients who presented with a type-A long-gap EA. Extrathoracic esophageal elongation was achieved using the Foker technique. During the first stage, we utilized pledgeted sutures reinforced with titanium clips for elongation that began five days after the surgery. As the elongation progressed, and the gap between esophageal pouches decreased, we restored the continuity of the esophagus. On follow-up, all patients needed pneumatic dilatation of the esophagus. Conclusion: The Foker technique successfully restored the esophageal continuity using the native esophagus in our patients with esophageal atresia.