Tracheoesophageal Fistula Research Articles

VACTERL association with non-rotated bowel: A rare case report

VACTERL is an acronym for a group of congenital anomalies where V stands for vertebral defects or vascular anomalies(single umbilical artery), A stands for anal atresia, C stands for cardiac defects, TE denotes trachea-esophageal fistula, R stands for renal abnormalities and L stands for limb defects such as polydactyly or syndactyly. Atleast, three of above features are considered necessary for diagnosis of the condition [1].It may or may not be associated with other bowel pathologies (most commonly duodenal obstruction).If there is accompanying hydrocephalus, then the term VACTERL-H is used.The estimated incidence is 1 in 10,000 – 40,000 births. Few familial cases have been identified and maternal diabetes and maternal diabetes is only environmental influence implicated. It is called an association, rather than a syndrome because the complications are not pathogenetically related, tend to occur more frequently than expected and are thought to be linked to embryonic mesodermal defects.It is thought to be autosomal recessive in nature [2].It has been statistically associated with maternal exposure to exogenous sex hormones during the first trimester of pregnancy [3].It has also been associated with Fanconi Anemia [4].

Left Nostril Agenesis in a Fetus with VACTERL Association: A Case Report

VACTERL association represents a rare condition with a broad spectrum of coexisting congenital abnormalities. Although a multifactorial origin has been described, the etiology is still unclear. Prenatal diagnosis can be challenging, as specific characteristics may be difficult to be determined before birth. This case report describes the finding of multiple congenital abnormalities in a male fetus at 30 weeks of gestation. The most notable findings were the presence of duodenal stenosis, esophageal atresia with distal tracheoesophageal fistula (type 3), interatrial septal communication foramen ovale, and left nostril and choana agenesis. After birth and making a broad differential diagnosis, all these characteristics oriented the diagnosis towards the VACTERL association. To the best of our knowledge, no cases of VACTERL association and unilateral nostril and choana agenesis have been previously described.

К вопросу диагностики задней расщелины гортани у новорожденного с множественными врожденными пороками развития

The article describes a rare congenital anomaly — laryngeal cleft in a newborn. Laryngeal anomaly occurs with a frequency of 1 per 10–20 thousand newborns. There are 4 types depending on the lesion depth and tracheoesophageal fistula presence. Clinical manifestations are characterized by diverse nonspecific symptoms in the form of dysphagia, cough, aspiration, stridor. Condition severity is determined by the anomaly depth and degree, as well as concomitant somatic and neurological child pathology. There is no prenatal diagnosis. The gold standard of diagnostics is videolaryngoscopy, in which a doctor can study in detail the structure of the laryngeal cartilage and vocal cords from any angle, and, if necessary, take photos and videos. Medical and surgical treatment of the laryngeal cleft is quite complicated and depends on the cleft type and size, concomitant pathology, as well as the condition of the child. The article presents a clinical case of a type III laryngeal cleft in a newborn with multiple congenital anomalies, and a diagnostic search for a cleft. An infant at the age of 7 months underwent the endoscopic suturing of this defect, which allowed restoring enteral nutrition and normalizing the child nutritional status. KEYWORDS: dysphagia, laryngeal cleft, congenital anomalies, newborn, diagnosis, endoscopic correction. FOR CITATION: Petrova V.I., Dmitriev A.V., Fedina N.V., Zaplatnikov A.L. On the issue concerning diagnosis of the posterior laryngeal cleft in a newborn with multiple congenital anomalies. Russian Journal of Woman and Child Health. 2023;6(4):433–437 (in Russ.). DOI: 10.32364/2618-8430-2023-6-4-17.

Retracted: Analysis of the Application Value of Different Esophagography Techniques in the Diagnosis of H-Type Tracheoesophageal Fistula in Neonates.

[This retracts the article DOI: 10.1155/2022/7264343.].

Atresia de esôfago com história familiar positiva: um relato de caso

INTRODUCTION: Esophageal atresia is a malformation marked by discontinuity of the esophagus, where the proximal part ends in a cul-de-sac, with or without the presence of a fistula with the trachea. An incidence pathology of 1 in every 2500 to 4500 live births, predominantly in individuals of white race and males, whose diagnosis can be made in the prenatal period by ultrasound or in the post-natal period when the clinical suspicion is present. The diagnosis must be early for treatment success, avoiding complications such as the evolution from pneumonia to respiratory failure. In view of the investigation, family history can be an alert for early suspicion. OBJECTIVES: To describe a case of esophageal atresia with distal tracheoesophageal fistula associated with heart disease, diagnosed after birth, in a female child, who had a positive family history for this condition, in the absence of a diagnosis of known genetic syndromes. METHOD: This is a descriptive observational study with data collection from the patients record at the Maternity Hospital. Literature reviews were carried out on platforms such as Lilacs, Scielo, Pubmed and research in Pediatric and Pediatric Surgery books on the subject. CONCLUSIONS: In the reported case, the diagnosis occurred after birth, with the newborn being referred early for surgical evaluation, which presented a successful approach, without complications. We hope that with the report, it will be possible to establish the family history as a point of the anamnesis to be valued in the investigation of the newborn.

Anesthetic consideration of a patient with acquired tracheoesophageal fistula undergoing radical left colectomy for sigmoid cancer.

Anesthetic consideration of a patient with acquired tracheoesophageal fistula undergoing radical left colectomy for sigmoid cancer.

A systematic review of the thoracoscopic treatment of H-type congenital tracheoesophageal fistulas

A systematic review of the thoracoscopic treatment of H-type congenital tracheoesophageal fistulas

Surgical Management of Post-Intubation Tracheal Injuries: A Single-Center Experience from Nepal

Introduction: Despite various improvements in technology and patient care, tracheal intubation and tracheostomy still result in significant tracheal injuries like stenosis and airway fistula. Pressure necrosis by the inflated balloon is the commonest culprit. Post-intubation tracheal stenosis is a major indication for tracheal resection and anastomosis. This study was done to find out the indications and results of surgery for post-intubation tracheal lesions. Methods: It was a descriptive observational study of patients with post- intubation tracheal lesions managed in Manmohan Cardiothoracic Vascular and Transplant Center over a period of 20 years (2001 to 2021). Results: Twenty-five patients were treated for post-intubation tracheal lesions. Four had isolated tracheo-esophageal fistula (TEF); eighteen had an isolated tracheal stenosis and three had both. Most had a tracheal resection and end-end anastomosis (REEA) via a cervical approach, resecting an average of 2.7 cm of tracheal length. The average size of stenotic lumen was 4.32 mm. There were no operative deaths. Two patients expired within a month of surgery: one due to fatal restenosis and the other due to sepsis. There were a few immediate and a few long-term complications. The average follow-up period was 5 years. Conclusion: Among the various post-intubation lesions, tracheal stenosis is the commonest indications for surgery. The management is challenging but safe and feasible, with a multidisciplinary team approach.

Congenital heart defects in children with Gastro-intestinal malformations

Background. Congenital malformations of the gastrointestinal (GI) tract are common birth defects detected in the neonatal period and usually present with signs of GI obstruction which at times can be life threatening. Anorectal malformations are among the more frequent congenital anomalies. The co-occurrence of congenital heart defect (CHD) along with GI malformation can significantly affect the natural history of either defect. Purpose - to study the prevalence of GI malformations in children with CHD and study the risk factors. Materials and methods. A total 100 patients of GI malformations were enrolled out of which 66 (66%) were males and 34 (34%) were females. All patients with any GI malformations (anorectal malformation, tracheoesophageal fistulae, anterior abdominal wall defects) presenting to Neonatal Intensive Care Unit (NICU), Paediatric Cardiac unit, Pediatric and Pediatric surgery outpatient department (OPD) as well as In-patient department (IPD) between October 2019 to October 2021 were included in study. A detailed history and examination was done followed by echocardiography and the prevalence of CHD among GI malformation was observed and risk factors were studied. Results. Most common GI anomaly was anorectal malformation (71%) followed by tracheoesophageal fistula (17%), CHD was seen in 14 children. Most common heart defect was ventricular septal defect (VSD) (43%) followed by patent ductus arteriosus (PDA) (36%) and atrial septal defect (ASD) (14%). Among cases with anorectal malformation, CHDs was seen in 10% and the most common defect was VSD (43%). Forty one percent of cases with tracheoesophageal fistula had CHDs with PDA (57%) being the most common underlying defect. Functional heart defects were present in 28 children of GI malformations. Conclusions. The coexistence and severity of CHD in patients with GI malformation can have prognostic implications. Thus, early cardiac evaluation should be performed in every case of GI malformation, preferably with echocardiography. This is likely to help in the risk stratification as well as management of such children. The study was performed in accordance with the principles of the Declaration of Helsinki. Study is approved by Ethical Committee of the Institution. Informed consent of patients was obtained for the study. No conflict of interests was declared by the authors.

Staged surgical approach of neonates with esophageal atresia and tracheoesophageal fistula from low- and middle-income countries.

Neonates born in low- and middle-income countries (LMICs) with esophageal atresia (EA) and tracheoesophageal fistula (TEF) often do not have access to adequate surgical care. We have partnered with the non-profit organization World Pediatric Project (WPP) to facilitate care for such patients. Our protocol included placement of a gastrostomy tube by local surgeons before definitive repair at the Children's Hospital of Richmond at VCU (CHoR). The outcomes of WPP-sponsored patients with EA and TEF in this program (n = 9) were compared to patients born in the U.S. with the same condition at CHoR (n = 9). The baseline characteristics of the groups aside from the age at admission to CHoR and at day of surgery were comparable (0.5 ± 1.3days vs. 15.3 ± 11.1days, p < 0.0001; 1.1 ± 3.9days vs. 37.3 ± 8.3days, p < 0.0001, respectively). All patients received definitive repair without mortality; the average length of stay after surgery was lower in the WPP group. There were 4 surgical complications in the U.S. group and 1 in WPP group. All patients were weaned off of TPN and all WPP patients are now on a regular PO diet. Every WPP patient has been seen in follow-up in the U.S. and in their home country. We provided successful multidisciplinary care for neonates with EA and TEF from LMICs with outcomes similar to the neonates with the same abnormality in the U.S. The partnership with WPP has been invaluable as it has provided essential support to identify and manage these patients. IV.

Feeding and Swallowing Characteristics of Children With Esophageal Atresia and Tracheoesophageal Fistula.

This study aimed to characterize feeding/swallowing difficulties in children with esophageal atresia and/or tracheoesophageal fistula (EA/TEF) and evaluate associations among feeding difficulties, pharyngeal dysphagia (PD), and other aerodigestive evaluation findings. This was a retrospective cohort study of feeding/swallowing characteristics of 44 patients with EA/TEF treated in the aerodigestive program of a single academic medical institution from 2010 to 2015. Demographics, comorbidities, presence and characteristics of feeding/swallowing difficulties, and results of relevant diagnostic tests [videofluoroscopic swallow studies (VFSS), clinical feeding evaluations (CFEs), chest computerized tomography (CT) scans, pulmonary bronchoscopies, and upper GI (UGI)/esophagrams] were reviewed. Fifty percent of the cohort had PD and 88.6% had feeding difficulties. Across 118 encounters (87 VFSS and 31 CFEs), feeding difficulties suggestive of esophageal dysphagia were most frequently seen in children over 48 months and feeding difficulties suggestive of developmental feeding problems were most frequently seen in children from 24 to 48 months. Abnormal findings were present in 59.8% of VFSS, with aspiration (34.5%) and pharyngeal residue (26.4%) the most frequently observed signs of dysphagia. Abnormal UGI/esophagram findings were not associated with significantly increased risk of feeding difficulties during visits within 3 months (risk ratio, RR = 1.33). Presence of dysphagia was associated with increased risk for some abnormal CT findings (RR= 3.0 for airspace and 3.0 for bronchiectasis). Feeding/swallowing difficulties are common in EA/TEF, and types of feeding difficulties vary by patient age. The presence of abnormal findings on UGI/esophagram did not increase the risk of feeding complaints; however, the presence of dysphagia increased the risk of abnormal chest CT.

A rare case of VACTERL association with a genito-urinary anomaly

VACTERL association is a cipher used to describe a cluster of conditions wherein there is a seemingly unexplainable non-random association of specific birth defects of multiple organ systems. It includes vertebral anomalies (V), anal anomalies (A), cardiac anomalies (C), tracheoesophageal fistula (TEF), renal anomalies (R) or radial bone anomalies (R), and limb defects (L). At least three of these seven malformations need to be present to constitute the VACTERL association. Other congenital anomalies of different systems may additionally be present with this association but are classified separately and the rarity of this occurrence is greater than even the VACTERL association. We report a neonate with congenital abnormalities of the cardiac system atrial septal defect and ventricular septal defect, tracheoesophageal fistula H type, and a multicystic dysplastic kidney. Additionally, the baby was also found to have a micropenis, a genito-urinary anomaly. With optimal surgical correction in the post-natal period and vigilant periodic follow-up, the prognosis may be relatively positive. Medical management can be used as therapy for treating long-term sequelae of the anomalies if any. Therefore, morbidity and mortality can be prevented to a great extent with early diagnosis and immediate intervention.

Prenatal diagnosis of distal 13q deletion syndrome in a fetus with esophageal atresia: a case report and review of the literature

BackgroundChromosome 13q deletion syndrome shows variable clinical features related to the different potential breakpoints in chromosome 13q. The severely malformed phenotype is known to be associated with the deletion of a critical region in 13q32. However, esophageal atresia is a rare symptom and the relevant region is unknown. Thus, determining the association between accurate breakpoints and new clinical features is essential.Case presentationA 28-year-old Japanese primigravid woman was referred for fetal growth restriction, absence of a gastric bubble, cerebellar hypoplasia, overlapping fingers, and polyhydramnios at 31 weeks gestation. At 38 + 0 weeks, she delivered a 1774 g female infant. The infant presented with isolated esophageal atresia (Gross type A), Dandy–Walker malformation, right microphthalmia, left coloboma, overlapping fingers, pleurocentrum in the thoracic vertebrae, reduced anogenital distance, and hearing loss. Her karyotype was diagnosed as 46,XX,del(13)(q32.1–qter) by amniocentesis, but array comparative genomic hybridization after birth revealed the deletion of 13q31.3–qter. At 48 days after birth, the infant underwent surgery for esophageal atresia and was later discharged from the hospital at 7 months of age.ConclusionThis case report and the literature reviews supports the previous findings on the pathological roles of haploinsufficiency of the ZIC2/ZIC5 in Dandy–Walker malformation and the EFBN2 haploinsufficiency in eye malformation and hearing loss. Furthermore, the possible involvement of IRS2, COLA1, and COLA2 in eye malformation were identified. This is the first case of 13q deletion syndrome with esophageal atresia (Gross A), but it may be a symptom of VATER/VACTER association (vertebral defects, anorectal malformations, cardiac defects, tracheoesophageal fistula with or without esophageal atresia, renal malformations, and limb defects), as in the previous cases. These symptoms might also be associated with EFBN2 haploinsufficiency, although further research is required.

The Role of Postoperative Mechanical Ventilation in Neonates with Tracheoesophageal Fistula

Background: Many advances have been made in managing neonates with esophageal atresia/tracheoesophageal fistula (EA/TEF). This resulted in a progressive decrease in mortality as a result of improved neonatal intensive care and neonatal mechanical ventilation; however, this is not the case in developing countries. Objectives: We aimed to evaluate the efficacy of postoperative ventilation in neonates with EA/TEF in Iraq as a developing country. Materials and Methods: It is a prospective study for all neonates with EA and/or TEF admitted to the pediatric intensive care unit (PICU) in Alkhansaa Teaching Hospital between December 2012 to December 2014. Forty operated neonates were randomly divided into two groups according to the record number of presentations (even = G1, odd = G2): Twenty patients (group l) had been assigned to be post-operatively on a ventilator as a routine protocol. While group 2 planned to be extubated in the theater. We evaluated the complications and mortality rates in two groups. Results: For 24 months, 40 neonates with EA/TEF were admitted to PICU; Thirty-two were operated on within 24 hours of life. There were no significant differences between the two groups regarding fetal maturity (p-value 0.490), gender, and weight. The mean period for mechanical ventilation was four days. Six neonates had tube mal-positioning complications, leading to lung collapse, hypoxia, and pneumonia (p-value 0.250). Anastomotic leaks occur in 10 neonates (five in each group) (p-value 0.642), which sometimes leads to sepsis (p-value 0.347). Trachea-malacia was recorded after weaning in 5 intubated patients (p-value 0.078). The mortality rate in G1 was 35%, while in group 2 was 25% (p-value 0.366). Conclusions: Although the study’s sample is small, it is recommended to avoid mechanical ventilation in the postoperative care of neonates with EA and/or TEF.

Exploration of the thoracoscopic treatment of esophageal atresia under high-frequency ventilation.

Explore the feasibility and safety of thoracoscopy in the treatment of esophageal atresia under high-frequency oscillatory ventilation (HFOV) mode. This was a single-center retrospective analysis. A total of 24 children were divided into the HFOV and the No-HFOV group. The demographic information, surgical results and relevant experience were analyzed. All patients in the HFOV group underwent thoracoscopic esophageal atreplasty with a mean operation duration of 165.8 ± 33.9 min. Two patients had postoperative anastomotic leakage, which was cured after conservative treatment. One child had a recurrent tracheoesophageal fistula, which was closed after endoscopic cauterization. The mean postoperative mechanical ventilation time was 8.83 ± 8.02 days. There was no return of anastomotic leakage or r-TEF after oral feeding. Furthermore, there was no significant difference between the NO-HFOV and the HFOV groups except for the operation time where the HFOV group was shorter than that of the NO-HFOV group. Thoracoscopic esophageal atresia anastomosis under HFOV ventilation is feasible for patients with severe pulmonary infection, heart malformation, such as patent ductus arteriosus, ventricular septal defect, and poor anesthesia tolerance, but the long-term prognosis still needs further study in a large sample size.

Evolution of left-sided thoracoscopic approach for long gap esophageal atresia repair

BackgroundLeft-sided repair for long gap esophageal atresia (LGEA) has been described for patients with a large leftward upper pouch, no thoracic tracheoesophageal fistula (TEF) nor tracheobronchomalacia (TBM), or as salvage plan after prior failed right-sided repair. We describe our experience with left-sided MIS traction induced growth process. MethodsWe retrospectively reviewed patients who underwent Foker process for LGEA at two institutions between December 2016 and November 2021. Patient characteristics, surgical techniques, and outcomes were reviewed. Results71 patients underwent Foker process. Of 34 MIS cases, 28 patients (82%) underwent left-sided repair (median gap length 5 cm) at median age 4 months with median 3 (range 2–8) operations and median 13.5 (IQR 11–21) days on traction until esophageal anastomosis. 9 patients (32%) underwent completely MIS approach, whereas 5 patients (18%) converted to open at first operation and 14 patients (50%) converted to open later in the traction process. Traction was internal in 68%, external in 11%, and combination in 21%. Median follow-up was 15.4 (IQR 7.5–31.7) months after anastomosis. 14% had anastomotic leak managed with antibiotics and/or esophageal vacuum therapy. Median number of esophageal dilations was 3.5 (range 0–13). 18% required stricture resection. 39% underwent Nissen fundoplication. None have needed esophageal replacement. ConclusionsFor multiple reasons including the tendency of both esophageal pouches to have a leftward bias, less tracheal compression by upper pouch, and clean field of surgery for reoperative cases, we now more commonly use left-sided approach for MIS LGEA repair compared to right side, regardless of left aortic arch. Level of evidenceLevel IV Treatment Study.

Proton Therapy With Concurrent Chemotherapy for Thoracic Esophageal Cancer: Toxicity, Disease Control, and Survival Outcomes.

When treating esophageal cancer with radiation therapy, it is critical to limit the dose to surrounding structures, such as the lung and/or heart, as much as possible. Proton radiation therapy allows a reduced radiation dose to both the heart and lungs, potentially reducing the risk of cardiopulmonary toxicity. Here, we report disease control, survival, and toxicity outcomes among patients with esophageal cancer treated with proton radiation therapy and concurrent chemotherapy (chemoradiation therapy; CRT) with or without surgery. We enrolled 17 patients with thoracic esophageal carcinoma on a prospective registry between 2010 and 2021. Patients received proton therapy to a median dose of 50.4-GyRBE (range, 50.4-64.8) in 1.8-Gy fractions.Acute and late toxicities were graded per the Common Terminology Criteria for Adverse Events, version 4.0 (US National Cancer Institute, Bethesda, Maryland). In addition, disease control, patterns of failure, and survival outcomes were collected. Nine patients received preoperative CRT, and 8 received definitive CRT. Overall, 88% of patients had adenocarcinoma, and 12% had squamous cell carcinoma. With a median follow-up of 2.1 years (range, 0.5-9.4), the 3-year local progression-free, disease-free, and overall survival rates were 85%, 66%, and 55%, respectively. Two patients (1 with adenocarcinoma and 1 with squamous cell carcinoma) recurred at the primary site after refusing surgery after a complete clinical response to CRT. The most common acute nonhematologic and hematologic toxicities, respectively, were grades 1 to 3 esophagitis and grades 1 to 4 leukopenia, both affecting 82% of patients. No acute cardiopulmonary toxicities were observed in the absence of surgical resection. Reagarding surgical complications, 3 postoperative cardiopulmonary complications occurred as follows: 1 grade 1 pleural effusion, 1 grade 3 pleural effusion, and 1 grade 2 anastomotic leak. Two severe late CRT toxicities occurred: 1 grade 5 tracheoesophageal fistula and 1 grade 3 esophageal stenosis requiring a feeding tube. Proton radiation therapy is a safe, effective treatment for esophageal cancer with increasing evidence supporting its role in reducing cardiopulmonary toxicity.

Supporting Breastfeeding in Complex Pediatric Otolaryngology Dyads: Clinical Pathway With Illustrative Case Series.

The benefits of breastfeeding are well established, yet complex infants are less frequently supported to feed direct or pumped breastmilk. This article aims to provide an algorithm to support complex infants in meeting breastfeeding goals. Two cases are presented to illustrate the breastfeeding support pathway. Each involves early identification of motivated caregivers, consultation of allied health, and assessment of swallowing safety with modification of position or route as appropriate. Two infants presented herein successfully continued to receive breastmilk despite airway complexity-1 with oral aversion secondary to prolonged nil per os period in the setting of tracheoesophageal fistula, and 1 with a tracheostomy secondary to subglottic stenosis. It is both possible and imperative to support complex infants in continuing to breastfeed, either directly or via the safest possible route, for the benefits to both members of the dyad.

Our experiences of percutaneous tracheostomy in intensive care unit

Aim We aimed to evaluate the indications for tracheostomy and early complications in patients who underwent percutaneous tracheostomy with the Griggs method in our clinic. Material and Method This study is retrospective and observational. Demographic data, hospitalization diagnoses, days of opening tracheostomy, days of intensive care hospitalization, indications for tracheosotomy and early complications of tracheostomy were recorded and evaluated in a total of 78 patients between the ages of 23-93 who underwent percutaneous tracheostomy with the Griggs method between January 2021 and September 2022. Results A total of 78 patients were included in the study. Of the patients, 42 (54%) were female and 36 (46%) were male. In the patients included in the study, the earliest tracheostomy day was the 7th day of intubation and the latest tracheostomy day was the 32nd day of intubation. The mean day in terms of tracheostomy day was 23rd day of intubation. In terms of early complications, bleeding was observed in 5 (6.5%) patients, tracheoesophageal fistula in 1 (1.3%) patient, and pneumothorax in 1 (1.3%) patient. No complications were encountered in 71 (90.9%) patients.

Distal Recurrent Tracheoesophageal Fistula after Repair of Esophageal Atresia-Incidence, Risk Factors, and Outcome.

The aim of this study was to assess incidence, risk factors, and outcomes of distal recurrent tracheoesophageal fistula (RTEF) after repair of esophageal atresia (EA). Ethical consent was obtained. Data collection was done by review of hospital records of 286 patients (268 in-house and 18 referred) who underwent repair of type C or D EA from 1980 to 2021. Spitz class, long-gap (tracheoesophageal fistula at carina), fundoplication, leakage, and stricture were assessed as RTEF risk factors. Outcome measures were long-term closure of RTEF, retainment of native esophagus, and survival. RTEF occurred in 23 patients (19 in-house) with type C (n = 22) or type D (n = 1) EA with median 4.4 (interquartile range [IQR]: 1.7-13) months after repair. Five patients had late RTEF 3.5 to 16 years after repair. Nineteen (7.3%) in-house patients developed RTEF. Presenting symptoms, age at diagnosis, and presence of anastomotic stricture (AS) are listed. No statistically significant risk factors were found, risk ratio of 0.5 to 2.7 (IQR: 0.1-8.7), p-value of 0.25 to 0.75. Detached closure clip and esophageal foreign body contributed to RTEF in two patients. Sixteen patients underwent rethoracotomy for closure of RTEF, 5 (22%) with AS eventually underwent esophageal reconstruction after a period with cervical esophagostomy. Two patients with late-manifested RTEFs underwent closure with laser cauterization. In 22 patients, treatment of RTEF succeeded, whereas 1 (4%) premature patient died of instant re-RTEF. RTEF had an incidence of 7% with diverse patterns of manifestation and predicting factors. Closure rate and patient survival were excellent, but RTEF with AS predicted loss of native esophagus.