Treatment Of Esophageal Atresia Research Articles

Using a Miniature Stapler to Divide a Mucosal Bridge at the Anastomosis after Gastric Pull-up for Iatrogenic Tracheoesophageal Fistula.

We report the first use of a miniature stapler to divide a mucosal bridge at the gastroesophageal junction after complex esophageal atresia (EA) repair. A 4-year-old girl was referred to our center after treatment of EA elsewhere. On our initial enodoscopy, a large iatrogenic tracheoesophageal fistula had formed, prompting us to perform a tracheoplasty and gastric interposition. One year after recovery, she had dysphagia with solid food. Upon endoscopy, a mucosal bridge was noted at the gastroesophageal anastomosis. This bridge was divided under endoscopy using a 5 mm miniature stapler. No complications were noted. Upon follow-up, she had no more complaints with solid food. Our report shows that the mucosal bridges may cause dysphagia after EA repair and can be safely divided using a miniature stapler.

Торакоскопическое лечение атрезии пищевода: результаты мультицентрового исследования

Торакоскопическое лечение атрезии пищевода: результаты мультицентрового исследования

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.

No Tissue Expression of KRAS or BRAF Mutations in 61 Adult Patients Treated for Esophageal Atresia in Early Childhood.

Previous studies have reported an association among esophageal atresia (EA), Barrett's esophagus, and esophageal adenocarcinoma later in life. The objective of the article is to evaluate KRAS and BRAF mutations as potential genetic markers for early detection of malignant transformation, we used an ultrasensitive technique to detect tissue expression of KRAS and BRAF mutations in endoscopic biopsies from 61 adult patients under follow-up after treatment for EA. RNA was extracted from 112 fresh-frozen endoscopic tissue biopsies from 61 adult patients treated for EA in early childhood. RNA was reverse transcribed using the extendable blocking probe reverse transcription method. KRAS codons 12 and 13, as well as BRAF mutations were detected by quantitative polymerase chain reaction. No mutations of KRAS codon 12, KRAS codon 13, or BRAF were found in 112 endoscopic biopsy samples from 61 patients. Despite the presence of histological findings indicating long-standing gastroesophageal reflux in 25%, as well as symptomatic gastroesophageal reflux in more than 40%, there was no detectable tissue expression of KRAS or BRAF mutations in this cohort of patients.

Improvement of surgical treatment of esophageal atresia in newborns

Improvement of surgical treatment of esophageal atresia in newborns

One Stage Reconstruction of Esophageal Atresia and Distal Tracheoesophageal Fistula in a 3250-gm Neonate: A Case Report

Esophageal atresia (EA) occurs when the upper part of the esophagus does not connect with the lower part of esophagus and stomach. Tracheoesophageal fistula (TEF) is an abnormal connection between the upper part of the esophagus and the trachea. Treatment for esophageal atresia has advanced over several decades due to improvements in surgical techniques and neonatal intensive care. The aim is to share our experience regarding the treatment of esophageal atresia with tracheoesophageal fistula. A 4-day-old neonate suffering from esophageal atresia with type IIIB tracheoesophageal fistula underwent one stage esophageal reconstruction and obtained good outcome without any complications. In this paper, a simple intra-operative technique for tracheal fistula repair and end to end esophageal anastomosis is discussed. We used a simple technique that we have found useful for ligation of tracheal fistula. Anastomosis of lower and upper esophagus without any complication like anastomotic leakage or stricture/stenosis of the neonate with EA/TEF (type IIIB), was proved to be safe and effective.

Balloon or Bougienage Dilation for Esophageal Stenosis in Children?

Purpose: Esophageal stenosis (ES) in children is defined as a fixed intrinsic narrowing of the esophagus caused by numerous etiologies. Treatment mainly consists of intraluminal dilation using balloon or Savary-Gilliard bougies. Methods: We retrospectively reviewed all medical records of children with ES treated by balloon or bougies. Our aim of this study was to compare both method of esophageal dilation in terms of safety, short- and long-term outcome. Results: Forty-two children were included in this study, 22 males (52%), mean age at diagnosis was 3.9 ± 5.7 years. This group underwent 190 dilation procedures in our institution between 1994-2013. The median treatment period was 5.5 months and the median follow-up after the last dilation was 2.25 years. The average number of dilations was 4.5 (range 1-22). Twenty-four patients had anastomotic stricture after surgical treatment of esophageal atresia (57.1%), 8 had stenosis following caustic ingestion (19%), 3 had functional stenosis due to esophageal motility disorders (achalasia) (7.1%) and the others (16.7%) had congenital ES, eosinophilic esophagitis, foreign body ingestion, or were post-fundoplication. Dilations were defined as failures in 11 children (26.2%). The success rate was 87% for the bougienage group (13 children) and 67% for the balloon group (18 children) (p=NS). The success rate was 75% (24 children) after caustic ingestion and surgical correction of esophageal atresia. There were 6 (3.1%) procedure-related complications that included 2 cases of aspiration pneumonia and 4 esophageal perforations. Conclusions: Esophageal dilation in children is a safe procedure with a high rate of long-term success. Longterm success of dilation among children with ES depends primarily on the etiology of stenosis and less on the method of dilation.

Thoracoscopic repair of esophageal atresia with and without tracheoesophageal fistula

Esophageal atresia (EA) is the interruption of the continuity of the esophagus, with or without persistent communication with the trachea. Recent advances in surgical techniques have made possible correction with minimally invasive surgery (MIS). To evaluate the management of thoracoscopic technique in the treatment of EA. Retrospective analysis of medical records in two centers was carried out between 2007 and 2012. Variables such as gestational age, gender, weight, type of esophageal atresia, malformations, surgery and postoperative complications were recorded. Twenty patients, 15 of them with type III EA, 4 with type I and one unclassifiable, were part of the study. 13 patients underwent ligation, cut of tracheoesophageal fistula and end to end anastomosis. Two underwent laparoscopic gastrostomy and fistula ligation. One patient required conversion and underwent esophagostomy plus gastrostomy. 4 patients without fistula required laparoscopic gastrostomy and aspiration of the proximal esophagus to be able to perform esophageal anastomosis. In the postoperative period, pleural drainage and a transanastomotic feeding tube were installed. Seven patients required esophageal dilations and 4 patients developed stenosis due to reflux. Two had recurrent fistula, one with spontaneous resolution and another with endoscopic resolution. Three children died: two of them due to pathologies not related to surgery and one as a result of limitation of therapeutic effort after fistula patency. Esophageal atresia repair with MIS is a safe and effective option with excellent exposure and visualization of anatomic landmarks and little associated morbidity.

Clinical characteristics and treatment of esophageal atresia: a single institutional experience

PurposeTreatment for esophageal atresia has advanced over several decades due to improvements in surgical techniques and neonatal intensive care. Subsequent to increased survival, postoperative morbidity has become an important issue in this disease. The aim of our study was to analyze our experience regarding the treatment of esophageal atresia.MethodsWe reviewed and analyzed the clinical data of patients who underwent surgery for esophageal atresia at Severance Children's Hospital from 1995 to 2010 regarding demographics, surgical procedures, and postoperative outcomes.ResultsSeventy-two patients had surgery for esophageal atresia. The most common gross type was C (81.9%), followed by type A (15.3%). Primary repair was performed in 52 patients. Staged operation was performed in 17 patients. Postoperative esophageal strictures developed in 43.1% of patients. Anastomotic leakages occurred in 23.6% of patients, and recurrence of tracheoesophageal fistula was reported in 8.3% of patients. Esophageal stricture was significantly associated with long-gap (≥3 cm or three vertebral bodies) atresia (P = 0.042). The overall mortality rate was 15.3%. The mortality in patients weighing less than 2.5 kg was higher than in patients weighing at least 2.5 kg (P = 0.001). During the later period of this study, anastomotic leakage and mortality both significantly decreased compared to the earlier study period (P = 0.009 and 0.023, respectively).ConclusionThe survival of patients with esophageal atresia has improved over the years and the rate of anastomotic leakage has been significantly reduced. However, overall morbidities related to surgical treatment of esophageal atresia still exists with high incidence.

Thoracoscopic treatment of esophageal atresia with distal fistula and of tracheomalacia

Single center experience with thoracoscopic repair of esophageal atresia with distal fistula and of tracheomalacia. Between May 2000 and December 2006, 51 neonates with an esophageal atresia were presented for thoracoscopic repair. Gestational age varied from 31 3/7 to 42 2/7 weeks (M = 37 2/7). Birth weight was between 1025 g and 4030 g (mean 2620 g). Concomitant anomalies or VACTERL association were encoutered in 31 patients (61%). Duration of the operation was from 90 minutes to 390 minutes (mean 178 minutes). All but 1 patient had an esophageal atresia with a distal fistula. Six patients had tracheomalacia requiring aortopexia, which was performed thoracoscopically. In 2 patients the thoracoscopic procedure had to be converted to a thoracotomy. All other patients underwent a successful thoracoscopic repair. One patient died in the postoperative period because of sepsis. A total of 22 patients (45%) developed a stenosis in the postoperative follow up (1 month-7y 7 month) requiring 1 to 18 dilatations (mean 1.5). Postoperative leakage occurred in 9 patients (18%). Recurrent fistula was encountered in 2 patients. A total of 11 patients (22%) underwent a laparoscopic antireflux procedure for either recurring stenosis (8) or ALTES (3). Six children (12%) underwent thoracoscopic aortopexy for tracheomalacia. In 2 children symptoms recurred for which a successful repeat thoracoscopic aortopexy was undertaken. The thoracoscopic approach to the treatment of esophageal atresia and tracheomalacia is becoming increasingly accepted. The cosmesis is undoubtedly better. The secundary effects like thoracic cage deformities, winged scapula, or scoliosis have not yet been described and are expected to be reduced in comparison to the open technique. Sequelae like leakage, stenosis, recurrent fistulae, and GERD and ALTES will probably remain the same. Whether thoracoscopic dissection has less detrimental effect on disturbed motility remains to be proven. Thoracoscopic aortopexy for severely symptomatic tracheomalacia is relatively simple. Even repeat thoracoscopic aortopexy is not that difficult. It was immediately effective in 2/3 of the patients.

Use of a bioprosthesis to relieve tension in oesophageal anastomosis: an experimental study.

Collagen-coated Vicryl mesh was assessed as a prosthetic repair material in the oesophagus of growing piglets. In a group of six piglets (group A), a full thickness defect was created in the oesophagus without fully transecting the organ. The removed tissue was subsequently replaced with a patch of collagen-coated Vicryl mesh. Animals were killed at 4 weeks and in the five surviving piglets the prosthesis had been replaced by host scar tissue with a normal epithelial lining. Two further groups of six piglets (groups B and C) were used as controls. In group B the oesophagus was either partly transected (four animals) or completely transected (two animals) and an anastomosis was performed, whereas in group C a 2.5-cm length of oesophagus was removed so that the anastomosis was performed under tension. In group B, all six piglets survived, whereas in group C, where the anastomosis was performed under tension, only two piglets survived. Microscopic and macroscopic examination of all groups showed that the collagen-coated Vicryl prosthesis was replaced by scar tissue; moderate stenosis was observed in only one animal in group A. Collagen-coated Vicryl mesh may be used either to reinforce an anastomosis performed under tension or may find use in tubular form to bridge the gap between the two ends of the oesophagus to be joined. It is envisaged that such a material may find applications in the treatment of oesophageal atresia.

Impact of preoperative diagnosis of congenital heart disease on the treatment of esophageal atresia

Congenital heart disease (CHD) has a major impact on the survival of babies with esophageal atresia (EA). The present study assesses whether early diagnosis influences the management strategies in a large series of EA. Cases of EA treated between 1982 and 2002 were retrospectively divided into groups according to the presence or absence of CHD and to whether this was diagnosed or not prior to tracheo-esophageal fistula repair. Patients were also staged according to Spitz's classification in which major congenital heart disease (MCHD) are those associated with cyanosis and/or heart failure requiring surgery. Comparisons between groups of patients were made by standard statistical tests. Among 195 babies with EA (99 boys and 96 girls), 82 (42%) had CHD (31 boys, 39% and 51 girls, 61%, chi2 P < 0.05) and 43 out of these 82 (52%) had MCHD. Six children died without treatment. CHD was diagnosed prenatally in six cases and before EA repair in 26 cases. The diagnoses were missed in 12 instances (in five it was a MCHD). Regardless of preoperative diagnosis of right aortic arch in 3/6 cases, the oesophagus was approached from the right thorax in all cases and only in one of them the operation was not completed due to hemodynamic instability. There were 145/195 (75%) Spitz group I patients; 44/195 (22%) were group II and 6/195 (3%) group III. The mortality was respectively 9.5, 59 and 83%. We lost 15/113 (13%) babies with EA without CHD, 4/39 (10.2%) with EA and CHD and 26/43 (60%) with EA and MCHD. In children with CHD diagnosed prior to the esophageal operation 8/26 (30.7%) died and among those without diagnoses 3/12 (25%) died, (chi2 P > 0.1). 1. CHD was diagnosed after EA repair in one-third of cases. 2. Preoperative diagnosis of CHD changed the operative indication only in one case. 3. Survival after treatment for EA was not influenced by the moment or the accuracy of the diagnosis of CHD in this series. 4. With only a few exceptions, associated CHD should not change the strategies of EA repair.

Cameron Haight: Pioneer in the Treatment of Esophageal Atresia

e o u i n p g A Cameron Haight is best known for being the first surgeon to uccessfully perform a 1-stage repair of the congenital anomaly of tresia of the esophagus with distal tracheoesophageal fistula (TEF) n 1941. There are 5 variants of esophageal atresia and tracheosophageal fistula, but the most common type is a blind esophageal ouch associated with a distal TEF. Newborns with esophageal tresias present with copious oral secretions, and the diagnosis is uspected when attempts to pass a nasogastric tube into the stomch fail because of the blind esophageal pouch. X-ray examination evealing air in the gastrointestinal tract provides confirmation of he associated TEF. Thomas Gibson first clearly described this ongenital anomaly in 1697, and for nearly 2 1/2 centuries, this ondition carried an unequivocally fatal prognosis. Infants with his condition inevitably died of pulmonary complications from spiration and pneumonitis. In the late nineteenth century, feeding gastrostomies were atempted to address the issues of dehydration and malnutrition esulting from the esophageal atresia. However, the success of these ttempts was foiled by gastro-esophageal reflux of the gastric conents through the fistula into the trachea and lungs, resulting in neumonia and death. Recognizing the critical importance of separating the commuication between the pulmonary and gastrointestinal tracts, Harry ichter made the first attempts at TEF ligation on 2 infants in 913, performing a transpleural ligation of the fistula coupled with feeding gastrostomy. Unfortunately, Richter’s success was hwarted by limitations in neonatal perioperative care of the time. is second patient expired 20 hours after surgery but might have urvived if it were not for the bronchial pneumonia the child had eveloped preoperatively. From 1936 to 1937, Thomas Lanman performed an extrapleural igation of the fistula with a primary anastomosis of the 2 esophageal egments in 4 separate cases. Although none of Lanman’s patients urvived, his fourth patient was found postmortem to have an intact nastomosis, and findings suggested that death was caused by overhyration rather than by infection, pneumonia, or mediastinitis. Lan-

Advances in the treatment of oesophageal atresia over three decades: the 1970s and the 1990s.

Oesophageal atresia management has evolved alongside the development of paediatric surgery. An analysis of a 30-year prospective collection of oesophageal atresia cases treated at the Royal Alexandra Hospital for Children, NSW, Australia is presented. There has been a dramatic change in the surgical approach. Fewer infants are undergoing operations of cervical oesophagostomy, gastrostomy and oesophageal replacement. More, including fragile infants, are surviving with a repaired native oesophagus. Fewer infants are suffering morbidity secondary to anastomotic leak. The progression in treatment has been enabled by improved neonatal support due to advances in neonatology, neonatal anaesthesia, nutritional support and antimicrobial therapy. Oesophageal atresia treatment and outcome has changed markedly over three decades. Cooperative multi-centre database development is now required to provide data in order to further refine treatment for clinical challenges that remain.

Oesophageal atresia in premature infants: an analysis of morbidity and mortality over a period of 20 years

To determine the morbidity and mortality of premature infants born with oesophageal atresia (OA) and to evaluate historical changes in morbidity and mortality over time. Retrospective analysis of morbidity and mortality of all patients admitted for OA, with or without tracheo-oesophageal fistula, between 1982 and 2002. The study group consisted of 197 consecutive patients, of whom 55 (28%) were premature and 21 (11%) very premature. Type A atresia was found more often in very premature and premature infants than in those born at term (p = 0.02). Type E atresia was not found in the premature group (p = 0.004). At least one associated congenital anomaly was also present in 121 patients (61%). Postoperative complications developed more often in very premature and premature infants than in those born at term (p < 0.001). Gastro-oesophageal reflux was diagnosed in 32/76 premature infants and in 41/121 term infants (p = 0.001). Mortality among very premature and premature infants was higher than among those born at term (p = 0.003). Withdrawal of treatment was the most frequent cause of death. Premature infants with OA have a higher morbidity and mortality than term infants with OA. The complications of prematurity contribute significantly to morbidity and mortality in premature infants with OA. There is no reason to refrain from the standard treatment of OA in premature infants with no severe associated congenital anomalies.

Esophageal atresia in infants with very low birth weight.

Infants with esophageal atresia (EA), with or without a tracheoesophageal fistula (TEF) frequently are of low birth weight. With advances in neonatal, respiratory, surgical, and anesthetic care, more infants with very low birth weight (VLBW; birth weight less than 1.5 kg) are surviving. The therapy of the VLBW neonate with EA is not longer automatically staged. Primary or delayed primary anastomosis can be performed safely if the patient is stable. This report will review the epidemiology, pathophysiology, treatment, and prognosis of EA in VLBW infants. The authors present their own experience in dealing with the VLBW with EA and review the world literature.

Treatment of recurrent tracheo-oesophageal fistula in an infant using bronchoscopic I application of fibrin glue

SummaryRecurrent tracheo-oesophageal fistula (TEF) is the most common and serious complication of the treatment of oesophageal atresia with TEF. Following this complication, the patient might need several operations. In this study, the authors report on the treatment of recurrent TEF using bronchoscopic application of fibrin glue (FG) to the fistula tract.

Factors contributing to poor results of treatment of esophageal atresia in developing countries

The results of treatment of 341 consecutive cases of esophageal atresia/tracheoesophageal fistula over an 11-year period have been analyzed to determine the factors resulting in a poorer prognosis of these cases in a developing country; 121 neonates in the first 6-year period are compared with 220 in the last 5 years. In the latter group only 8% were in Waterston's group A; 46% reached the hospital within 24 h of birth, 13% were normothermic on presentation, 70% had a chest infection, and 28% had major associated malformations. This was similar to the preceding 6-year period. In the last 5 years the overall mortality was 58%, compared to 67% in the preceding 6 years. Despite improvement in survival in the last 5 years, the overall survival is far inferior to that reported from the developed countries. The factors resulting in poorer results have been analyzed and remedial actions suggested.

Use of a Collagen Coated Vicryl Tube in Reconstruction of the Porcine Esophagus

This experimental study assessed the use of a collagen-coated vicryl mesh tube to reconstruct the esophagus in growing piglets. Initial experiments, in which a segment of thoracic esophagus was excised and replaced by this prosthetic tube, resulted in all the animals succumbing to mediastinitis within the first 3-4 days. This was shown, at post-mortem, to be due to leakage of the prosthesis, secondary to acid reflux, resulting in dissolution of the prosthesis. The collagen-coated vicryl mesh was thereafter treated with glutaraldehyde and in-vitro studies showed that the glutaraldehyde-treated material exhibited a higher resistance to 0.01 M HCl at pH 2.0, when compared with untreated material. In addition, the glutaraldehyde-treated material also showed an increased resistance to digestion by bacterial collagenase. Further experiments to reconstruct the cervical esophagus in growing piglets were performed using the revised glutaraldehyde cross-linked collagen coated vicryl mesh tube. All the animals survived the procedure and the prosthesis were leakproof immediately post-operatively. The animals, however, developed severe stenosis at a mean of 11 days post-operatively. Attempts at neo-epithelialization of the graft were seen histologically. There was considerable granulation tissue and scar tissue formation on the mediastinal aspect of the graft. It is suggested that collagen coated vicryl mesh tube may find applications in the treatment of esophageal atresia only if the problem of stenosis of the prosthesis can be solved.

Esophageal atresia: Primary results of 500 consecutively treated patients

During 1947-1978, 500 patients with esophageal atresia and/or tracheoesophageal fistula were treated at the Children's Hospital, University of Helsinki. The proportion of different types of anomaly followed the usual distribution, the commonest being the type with distal fistula (88.2%). For analysis, the patients were divided into five phases, each consisting of 100 consecutively treated patients. The hospital mortality decreased from 81% in the first phase to 15% in the last phase. No patients were excluded, not even those cases with lethal associated anomalies. Many more severe cases were treated in the last two phases than in the first three. Patients with distal fistula were examined separately as were the patients without a fistula and those with only a tracheoesophageal fistula. With time, early diagnosis and early referral for treatment became a rule. This led to the policy that nearly all patients in the last phase had an early operation without staging. Gastrostomy was not considered necessary when early anastomosis was possible. In the last phase, only the transpleural approach was used and single-layer end-to-end anastomosis was favored. As a whole, the type of approach or anastomosis did not have significant effect on the results, except that the Sulamaa-type end-to-side anastomosis had the highest frequency for refistula. With time there was only a slight decrease in the incidence of anastomotic leak, but significant improvement in its management. Refistula as an early complication was seen only once in the last phase. As all anastomoses were routinely dilated, severe strictures were uncommon; there were only six in the series. The factor that probably improved the prognosis the most was better pulmonary care. The improved prognosis was also due to earlier referral, modern anesthesia, and intensive care. Postoperative pulmonary complications dropped from 92% in the first phase to 40% in the last. They were the most common single cause of death in the early series but caused no deaths among the last 100 patients with no associated anomalies. Low birth weight was an important prognostic factor early in the series but in the last phase the survival rate of under-2500-gm infants with no associated anomalies was 88%. The presence of severe associated anomalies remains the most important single cause of death of an esophageal atresia patient today. Some patients (eg, trisomy 18) are beyond the possibilities of surgical treatment. To improve the prognosis of the others, efforts in the treatment of their associated (especially cardiovascular) anomalies must be made. In the treatment of esophageal atresia itself, improvement may still be achieved in the number of patients using their own esophagus without replacement procedures. Finally, a new prognostic classification of esophageal atresia patients is suggested, excluding pneumonia as an index factor in the Waterston classification.