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Purpose: Congenital esophageal stenosis (CES) associated with esophageal atresia (EA) is rare, and no standard treatment has been established. We reviewed cases of EA-associated CES to assess the clinical characteristics and treatment outcomes, especially the feasibility of endoscopic dilatation., Methods: We retrospectively examined patients with EA-associated CES. We also compared treatment outcomes of EA-associated CES with those of EA patients without CES who developed postoperative anastomotic stricture., Results: Among 44 patients with EA, ten had CES (23%). Postoperative complications were not significantly different between EA patients with CES and those without CES but with anastomotic stricture. All CES patients underwent balloon dilatation as initial treatment. Eight of nine patients (89%) were successfully treated by dilatation only, and one patient underwent surgical resection. The median number of balloon dilatations for CES was five (2-17), which was higher than that for anastomotic stricture in patients without CES (p = 0.012). Esophageal perforation occurred in five patients with CES (5/9, 56%) after dilatation, but all perforations were successfully managed conservatively with an uneventful post-dilatation course., Conclusions: Twenty-three percent of patients with EA had CES. Although balloon dilatation for EA-associated CES required multiple treatments and carried a risk of perforation, balloon dilatation showed an 89% success rate and all perforations could be managed conservatively., (© 2024. The Author(s).)

Congenital esophageal atresia, in which the proximal esophagus forms a blind end, results in respiratory failure due to aspiration of saliva. When accompanied by a distal tracheoesophageal fistula, increased intragastric pressure causes gastric juice to reflux into the trachea, resulting in severe pneumonia. Because of the above conditions, surgery should be performed early in life once the diagnosis is made. Conventionally, a two-stage radical operation was performed after gastrostomy. Recently, if the patient is in good general condition, a one-stage radical operation is preferred without gastrostomy. The patient is positioned in the left lateral recumbent position. The tracheoesophageal fistula is double ligatured near the tracheal junction by extra-pleural approach. After dissection of the upper esophageal blind end, the upper and lower esophagus are anastomosed using a single-layer intermittent suture. In cases of a long gap in which one-stage anastomosis is difficult, two-stage esophageal anastomosis is performed. As the long-term prognosis of esophageal reconstruction using the stomach, small intestine, or large intestine in children is not always good, it is desirable to reconstruct the esophagus using autologous esophagus. Recently, thoracoscopic repair has been performed for esophageal atresia, and its results have been reported to be comparable to those of open repair.

Thoracoscopic repair (TR) of esophageal atresia (EA) has been performed with increasing frequency over the last two decades, with the expectation of improved outcomes by avoiding thoracotomy. To understand the current practice and outcomes of TR of EA, we reviewed the relevant literature, including 15 case series, 7 comparative studies, and 3 meta-analysis comparing TR with conventional open repair (COR). Most of the studies had a retrospective design and small numbers of patients. Although the evidence level is low because of the lack of prospective studies, this review found that TR is as safe as COR, with comparative outcomes. Moreover, there were several advantages of TR over COR, such as less blood loss and a shorter hospital stay. The long-term outcomes of TR remain unclear because of limited data. Moreover, there is a significant learning curve over the first 10-20 TRs performed. We conclude that TR of EA, when conducted by experienced surgeons, is a safe and minimally invasive alternative to COR and may yield better results than COR in appropriately selected patients.

Aim: Thoracoscopic repair (TR) of esophageal atresia with tracheoesophageal fistula (EA/TEF) remains a considerable challenge, even for the most experienced pediatric surgeons. The aim of this study is to report the outcomes of our experience with TR of EA/TEF and to determine the learning curve for this procedure., Methods: Eleven consecutive cases that had undergone TR of EA/TEF at our institutes were included in this study. The medical charts were reviewed retrospectively. To determine the learning curve for TR of EA/TEF, a logarithmic curve-fitting analysis was performed. The data were expressed as medians with ranges., Results: The median age and birth weight were 1 day (range, 1-3 days) and 2.8 kg (range, 2.5-3.7 kg), respectively. TR was completed in all cases without any complications. The median operative time was 230 min (range, 164-383 min). There were no cases of anastomotic leakage. One patient with a long gap required repeated balloon dilatation for refractory anastomotic stricture. No mortality or recurrence of tracheoesophageal fistula occurred. The operative time was significantly longer in patients with a long gap (>20 mm) than in those with a shorter gap. Once the three cases with a long gap had been excluded, the operative time decreased as the number of treated cases increased. The relationship between the operative time and case number fit a logarithmic function curve well (operative time in minutes = 300 - 62 × log (case number), R 2  = 0.8359, P = 0.0015)., Conclusions: Our results suggest that TR of EA/TEF is a safe procedure. It has a considerable learning curve, but requires advanced endoscopic surgical skills., (© 2017 Japan Society for Endoscopic Surgery, Asia Endosurgery Task Force and John Wiley & Sons Australia, Ltd.)

Purpose: To investigate the long-term morbidity of surgically treated esophageal atresia (EA) in adolescents and young adults and establish whether these long-term morbidities are affected by the type of EA., Patients and Methods: We reviewed the medical records, including backgrounds and associated conditions, of 69 long-term survivors of EA, aged >15 years. The long-term morbidities included neurodevelopmental abnormality, nutritional impairment (short height <-2SD, low BMI <18.5), subjective symptoms, and musculoskeletal deformities. Comparisons of the results were made between Gross A-type EA (n = 6) and Gross C-type EA (n = 63)., Results: All patients underwent esophageal anastomosis without esophageal replacement. Cardiac anomalies and long gap were present in 26 and 18%, respectively. Esophageal dilatation, fundoplication, and aortopexy were performed in 40, 34, and 18%, respectively. The incidence of long gap and esophageal stenosis was higher in Gross A-type EA than in Gross C-type EA. The long-term morbidities included neurodevelopmental abnormality (13%), nutritional impairment (62%: as short height in 34% and as low BMI in 46%), subjective symptoms (14%), and musculoskeletal deformities (59%). There were no differences in the long-term morbidities between Gross A and Gross C., Conclusions: The incidence of the long-term morbidities is high in adolescents and young adults, regardless of the type of EA. Early detection of morbidity is important to improve the long-term outcomes of EA.

Background: In order to better understand the current practice and outcomes of thoracoscopic repair of esophageal atresia (EA)/tracheoesophageal fistula (TEF), a multi-institutional analysis was conducted among seven Japanese institutes., Materials and Methods: A survey was sent to the seven institutes regarding the surgical technique, postoperative management, and outcomes of thoracoscopic repair of EA/TEF., Results: The operation was uniformly performed via an intrapleural approach in the 0-45° prone position. The TEF was occluded with suture ligature in four (57.1%) institutes and clips in the remaining three (42.9%) institutes. Anastomosis was performed using the extracorporeal knot-tying technique in four institutes and the intracorporeal technique in three institutes. Patients were routinely left intubated and paralyzed for 3-7 days postoperatively in four institutes. In total, 58 patients underwent thoracoscopic repair of EA/TEF. Fifty-two (89.7%) of the patients underwent successful thoracoscopic repair. Six (10.3%) operations were converted to open thoracotomy because of a long gap (n=4), right aortic arch (n=1), and intraoperative instability (n=1). The body weight at operation ranged from 1.2 to 4.6 kg, and the operative time ranged from 115 to 428 minutes. There were no major intraoperative complications. Eleven patients (19.0%) suffered from anastomotic leakage. Twenty-eight patients (48.3%) developed anastomotic stricture. One patient died during the postoperative period because of an unrelated disease. Recurrent TEF developed in three patients (5.2%). Thirteen patients (22.4%) later required fundoplication., Conclusions: The outcome of thoracoscopic repair of EA/TEF was comparable to that of the open procedure. As considerable variability was observed among the seven institutes with respect to the surgical technique and management, standardizing the surgical management may improve the outcome.

Introduction: We report four cases of long-gap esophageal atresia (LGEA) treated with thoracoscopic esophagoesophagostomy (TEE) after sequential extrathoracic esophageal elongation (SEEE: Kimura's technique)., Methods: All initially had gastrostomy, then SEEE. The proximal end of the esophagus was introduced into the apex of the thorax under direct vision during TEE., Results: Mean birth weight was 1.9 kg. Mean gestational age was 35.0 weeks (range 30-39); mean age at initial esophagostomy was 43.2 days (range 15-110); SEEE was performed for a mean of 3 times (range 2-4) at mean intervals of 5.3 months (range 2-10), with the upper esophageal segment lengthened by a mean of 2.1 cm each time (range 1.1-3.5). Mean age and weight at TEE were 22.3 months and 9.8 kg, respectively. Gap was initially 4.5 vertebrae (range 4-5). Mean operating time was 9.6 h. TEE was successful in 3; 1 required thoracotomy, then re-anastomosis after 11 months of anastomosis leakage; 2 cases with post-TEE anastomosis leakage were treated conservatively. Postoperatively, all cases required fundoplication and esophageal dilatation (2, 3, 5, 8 times, respectively). At mean follow-up of 3.6 years, 2 eat normally and 2 eat minced food., Conclusions: TEE after SEEE appears to be a feasible option for treating LGEA.

Background/purpose: Thoracoscopic repair has recently been attempted in newborns with esophageal atresia (EA), but it remains unclear whether thoracoscopic dissection reduces pathological gastroesophageal reflux. We investigated the influence of a thoracoscopic approach on esophageal motor function in patients with EA., Methods: Clinical and gastrointestinal data of 10 patients with EA with open repair (group A) and 7 with thoracoscopic repair (group B) were analyzed retrospectively. Videomanometry was conducted to investigate esophageal motor patterns. Esophageal acid exposure was evaluated with 24-hour esophageal pH monitoring. Data are expressed as medians and ranges., Results: Contractions in the distal esophagus were conspicuously absent in 1 and 3 patients in groups A and B, respectively (P = .26). There were no significant differences in esophageal acid exposure (5.5% [0.7%-24.6%] vs 3.7% [0.3%-56.8%]; P = .71) or mean esophageal acid reflux time (0.5 minutes [0.1-1.4 minutes] vs 0.5 minutes [0.1-1.3 minutes]; P = .87) between the 2 groups. Fundoplication was conducted in 2 patients in each group (P = .60), all of whom had conspicuously absent distal esophageal contractions. Those contractions were preserved in the remaining patients with the exception of 1 group B patient., Conclusion: There are unlikely to be benefits from thoracoscopic repair of EA in terms of postoperative esophageal motor function.

Background/purpose: The objective of this study is to investigate the characteristics of esophageal motor activity responsible for the development of gastroesophageal reflux (GER) in patients with esophageal atresia (EA)., Methods: The subjects consisted of 29 patients with EA (1 month to 19 years). Computerized esophageal manometry was conducted to investigate esophageal contractions at swallow. A topographic esophageal manometric analysis was conducted in each subject, providing 3-dimensional displays to reveal the pressure continuum representing esophageal contractions., Results: Significant contractions in the middle esophagus just below the anastomosis were absent in all subjects. Contractions in the distal esophagus were conspicuously absent in 17 subjects. Of these 17, 6 had already undergone fundoplication, and 9 had symptomatic GER requiring fundoplication subsequent to this study. The remaining 12 patients had contractions in the distal esophagus and did not require medical/surgical intervention. A lack of distal esophageal contractions was significantly correlated with the development of GER (P < .001). There was a significant difference in esophageal acid exposure between the 2 groups (median, 38% vs 4%, P < .001)., Conclusion: Lack of distal esophageal contractions indicating an impaired clearing capacity is considered a potential key determinant of GER in patients with EA.

Congenital laryngeal atresia (LA) is a life-threatening anomaly in which appropriate perinatal management is essential for survival. The authors report a neonate with LA associated with esophageal atresia (EA) and tracheoesophageal fistula (TEF) who was successfully resuscitated by emergent tracheostomy. Before birth, the patient had a diagnosis of EA based on the findings of polyhydramnios and absent stomach bubble. Immediately after birth, severe respiratory distress, cyanosis, and sternal retraction were evident. Because either endotracheal or esophageal intubation was unsuccessful, an emergent tracheostomy was performed. A direct laryngoscope revealed a subglottic atresia with normal appearance of the vocal cords. Repair of EA with TEF was performed on the third day of life, and the postoperative course was uneventful. In 2 years follow-up, the patient has no mental retardation nor central nervous impairment. Because of the presence of TEF, the antenatal ultrasonogram did not demonstrate the characteristic findings of the congenital high airway obstruction syndrome. This case represents one of the very few reported cases of successful resuscitation of a neonate with an unanticipated LA in which emergent airway management is required immediately after birth.

Aim Although many approaches to esophageal replacement have been investigated, these efforts have thus far only met limited success. In-body-tissue-engineered connective tissue tubes have been reported to be effective as vascular replacement grafts. The aim of this study was to investigate the usefulness of an In-body-tissue-engineered collagenous connective tissue membrane, “Biosheet”, as a novel esophageal scaffold in a beagle model. Methods We prepared Biosheets by embedding specially designed molds into subcutaneous pouches in beagles. After 1–2 months, the molds, which were filled with ingrown connective tissues, were harvested. Rectangular-shaped Biosheets (10 × 20 mm) were then implanted to replace defects of the same size that had been created in the cervical esophagus of the beagle. An endoscopic evaluation was performed at 4 and 12 weeks after implantation. The esophagus was harvested and subjected to a histological evaluation at 4 (n = 2) and 12 weeks (n = 2) after implantation. The animal study protocols were approved by the National Cerebral and Cardiovascular Centre Research Institute Committee (No. 16048). Results The Biosheets showed sufficient strength and flexibility to replace the esophagus defect. All animals survived with full oral feeding during the study period. No anastomotic leakage was observed. An endoscopic study at 4 and 12 weeks after implantation revealed that the anastomotic sites and the internal surface of the Biosheets were smooth, without stenosis. A histological analysis at 4 weeks after implantation demonstrated that stratified squamous epithelium was regenerated on the internal surface of the Biosheets. A histological analysis at 12 weeks after implantation showed the regeneration of muscle tissue in the implanted Biosheets. Conclusion The long-term results of patch esophagoplasty using Biosheets showed regeneration of stratified squamous epithelium and muscular tissues in the implanted sheets. These results suggest that Biosheets may be useful as a novel esophageal scaffold. [ABSTRACT FROM AUTHOR]