Congenital Esophageal Atresia Research Articles

Enhanced estimation strategy for determining the location of tracheoesophageal fistula in a preterm, low-birth-weight infant with congenital esophageal atresia type C and duodenal atresia: a case report

BackgroundIn esophageal atresia type C, identifying the tracheoesophageal fistula (TEF) location is crucial for airway management. However, a thin bronchoscope may not always be available.Case presentationWe report on a low-birth-weight neonate with esophageal atresia type C who required immediate gastrostomy after birth. With no suitable thin bronchoscope available, alternative methods were utilized to estimate the TEF location post-gastrostomy. Submerging the gastrostomy tube tip in water and applying positive pressure ventilation via a tracheal tube allowed for observation of air bubbles emerging from the gastrostomy tube. As the tracheal tube was advanced, the cessation of bubbles indicated that the TEF was sealed by the tracheal tube. The location of the tracheal tube tip, confirmed by chest radiographs, was consistent with the TEF location identified during corrective surgery for TEF.ConclusionsThis innovative technique facilitated successful estimation of the TEF location without bronchoscopy, demonstrating its efficacy in resource-limited settings.

ZNF717 or PABPC1 Gene Variants Contribute to Congenital Esophageal Atresia by Interfering with Normal Esophageal Growth

Congenital esophageal atresia (EA) is an abnormality induced by the incomplete differentiation of the foregut in infants, and is frequently accompanied by tracheoesophageal fistula (TEF). Our understanding of the pathogenesis of EA-TEF is limited, additionally, there is still a lack of standard animal or cell models for in vitro EA-TEF investigation. Therefore, we analyzed esophageal tissue samples from 10 children with EA-TEF via Exome sequencing (ES) to identify gene variants. And esophageal organoid units (EOUs) were established as an in vitro model of EA by culturing esophageal tissues from Adriamycin-challenged rats. The ES results indicated 11 mutated genes, including the frameshift variants of ZNF717 and PABPC1. The EA organoids expressed the esophageal marker proteins CK13 and CK4 and showed a significantly slower rate of growth and dysplasia of cell development. In EA organoids, the transcription of SOX2, ZNF717, and PABPC1 was downregulated at varying levels, while NOGGIN transcription was markedly upregulated. Furthermore, when siRNA-ZNF717 or siRNA-PABPC1 was transfected into normal esophageal organoids, the proliferation of esophageal cells was significantly decreased. In conclusion, we found that normal ZNF717 and PABPC1 expressions are essential to the esophageal development, whereas the variant or deficiency of these genes might lead to EA-TEF.

Developing a core outcome set for the health outcomes for children and adults with congenital oesophageal atresia and/or tracheo-oesophageal fistula: OCELOT task group study protocol

IntroductionHeterogeneity in reported outcomes of infants with oesophageal atresia (OA) with or without tracheo-oesophageal fistula (TOF) prevents effective data pooling. Core outcome sets (COS) have been developed for many conditions...

Diagnose and treatment for Type D congenital esophageal atresia with tracheoesophageal fistula.

Type D esophageal atresia (EA) with tracheoesophageal fistula (TEF) is characterized by EA with both proximal and distal TEFs. It is a rare congenital anomaly with a very low incidence. To investigate diagnostic and treatment strategies for this rare condition. We retrospectively reviewed the clinicopathological features of patients with EA/TEF treated at our institution between January 2007 and September 2021. Among 386 patients with EA/TEF, 14 (3.6%) had type D EA/TEF. Only two patients were diagnosed with proximal TEF preoperatively. Seven patients were diagnosed intraoperatively. Five patients were missed for diagnosis during the initial surgery but was later confirmed by bronchoscopy. During the neonatal period, seven patients underwent a one-stage repair of proximal and distal TEF via thoracoscopy or thoracotomy. Due to missed diagnosis and other reasons, the other 7 patients underwent two-stage surgery for repair of the proximal TEF, including cervical incision and thoracoscopy. Ten of the 14 patients experienced postoperative complications including anastomotic leakage, pneumothorax, esophageal stricture, and recurrence. Patients who underwent one-stage repair of distal and proximal TEF during the neonatal period showed a higher incidence of anastomotic leak (4/7). In contrast, only one of seven patients with two-stage repair of the proximal TEF developed an anastomotic leak. Type D EA/TEF is a rare condition, and proximal TEFs are easily missed. Bronchoscopy may aim to diagnose and determine the correct surgical approach. A cervical approach may be more suitable for repairing the proximal TEF.

Innovative regenerative therapy for refractory postoperative esophageal anastomotic stenosis in children

Autologous oral mucosa-derived epithelial cell sheet transplantation was devised as a new treatment for refractory postoperative anastomotic stenosis in patients with congenital esophageal atresia (CEA) and congenital esophageal stenosis (CES). Transplantation therapy was performed on three patients with refractory postoperative anastomotic stenosis who had undergone endoscopic balloon dilatation (EBD) at least twice in one year and at least five times in total. Epithelial cell sheets were prepared from the patients' oral mucosa and transplanted over the esophageal wounds caused by EBD using a specially developed transplantation device. After transplantation, the patients were followed for 48 weeks. Subject 1 underwent two transplantations and showed temporary improvement in stenosis, but required periodic EBD and eventually had the stenotic area removed. Subject 2 did not require EBD for more than two years post-transplantation; Subject 3, until one year after transplantation. Both Subject 2 and Subject 3 experienced improvement in the passage of food and drink and were able to consume a regular diet as opposed to a full liquid diet. These results suggest that cell sheet transplantation therapy is effective in some cases.Trial registration: UMIN, UMIN000034566, registered 19 October 2018, https://upload.umin.ac.jp/cgi-open-bin/ctr_e/ctr_view.cgi?recptno=R000039393.

Retrospective analysis of endoscopic dilatation for pediatric patients with esophageal stricture: Bangladesh perspective

Background and objectives: Esophageal stricture in children is an intrinsic narrowing of the esophagus due to different etiologies, including congenital anomalies, corrosive substance or foreign body ingestion, and post-esophageal surgeries. The purpose of this study is to retrospectively evaluate the results and procedures of endoscopic dilatation (ED) performed on children with esophageal strictures in a specialized center in Bangladesh. Methods: Between September 2018 and October 2022, 21 children aged 0.2 to 15 years at the time of the first procedure who underwent 48 ED sessions were included at Bangladesh Specialized Hospital (Dhaka, Bangladesh). We documented the basic characteristics of patients, indications for ED, therapeutic procedures, and outcomes. Outcome parameters were the frequency of dilatations, complications (if any), and clinical success rates. Clinical success was defined as no necessity of ED for a minimum of one year or increasing intervals among repeated dilatations. Results: Among the studied patients, the most common causes of stricture were congenital esophageal atresia and ingestion of corrosive substances. More than half of the studied patients required multiple ED sessions, with one patient requiring eight sessions who had congenital esophageal atresia with a postsurgical stricture. Dilatation was achieved mostly using Savary-Gilliard or controlled radial expansion balloons varying in sizes between 8.0 mm to 30 mm. More than 76% of the studied patients had clinical success, while the rest had clinical failures or unfinished treatment. Conclusion: ED attained good clinical success if performed by skilled gastroenterologists. However, repeated dilatation is frequent, especially in patients with a corrosive-caused stricture.

Esophageal Atresia Associated with Congenital Duodenal Obstruction: Turkish Esophageal Atresia Registry (TEAR) Evaluation.

Coexistent congenital duodenal obstruction and esophageal atresia (EA) is known to have significant morbidity and mortality. Management strategies are not well-defined for this association. The data from the Turkish EA registry is evaluated. A database search was done for the years 2015 to 2022. Among 857 EA patients, 31 (3.6%) had congenital duodenal obstruction. The mean birth weight was 2,104 (± 457) g with 6 babies weighing less than 1,500 g. Twenty-six (84%) had type C EA. The duodenal obstruction was complete in 15 patients and partial in 16. Other anomalies were detected in 27 (87%) patients. VACTERL-H was present in 15 (48%), anorectal malformation in 10 (32%), a major cardiac malformation in 6 (19%), and trisomy-21 in 3 (10%). Duodenal obstruction diagnosis was delayed in 10 (32%) babies for a median of 7.5 (1-109) days. Diagnosis for esophageal pathologies was delayed in 2. Among 19 babies with a simultaneous diagnosis, 1 died without surgery, 6 underwent triple repair for tracheoesophageal fistula (TEF), EA, and duodenal obstruction, and 3 for TEF and duodenal obstruction in the same session. A staged repair was planned in the remaining 9 patients. In total, 15 (48%) patients received a gastrostomy, the indication was long-gap EA in 8. Twenty-five (77%) patients survived. The cause of mortality was sepsis (n = 3) and major cardiac malformations (n = 3). Congenital duodenal obstruction associated with EA is a complex problem. Delayed diagnosis is common. Management strategies regarding single-stage repairs or gastrostomy insertions vary notably depending on the patient characteristics and institutional preferences.

Safety, efficacy and pharmacokinetics of palivizumab in off-label neonates, infants, and young children at risk for serious respiratory syncytial virus infection: a multicenter phase II clinical trial

Pediatric patients with certain rare diseases are at increased risk of severe respiratory syncytial virus (RSV) infection. However, the prophylactic use of anti-RSV antibody (palivizumab) in these patients is not indicated at present in Japan. This first-in-the-world multicenter, uncontrolled, open-label, phase II clinical trial was carried out between 28 July 2019 and 24 September 2021at seven medical institutions in Japan to investigate the efficacy, safety, and pharmacokinetics of palivizumab in 23 subjects recruited from among neonates, infants, or children aged 24 months or younger who had any of the following conditions: pulmonary hypoplasia, airway stenosis, congenital esophageal atresia, inherited metabolic disease, or neuromuscular disease. At least four continuous doses of palivizumab were administered intramuscularly at 15mg/kg at intervals of 30 days. Twenty-three enrolled subjects completed the study. No subject required hospitalization for RSV. Adverse events (AE) did not notably differ from the event terms described in the latest interview form. Five severe AEs required unplanned hospitalization, but resolved without RSV infection. Therapeutically effective concentrations of palivizumab were maintained throughout the study period. Palivizumab might be well tolerated and effective in preventing serious respiratory symptoms and hospitalization due to severe RSV infection, indicating the prophylactic use in the pediatric patients included in this study. Japan Agency for Medical Research and Development (AMED), grant numbers 19lk0201097h0001 (to MM), 20lk0201097h0002 (to MM), 21lk0201097h0003 (to MM), and 22lk0201097h0004 (to MM). AMED did not have any role in the execution of this study, analysis and interpretation of the data, or the decision to submit the results.

Association between birth location and short-term outcomes for babies with gastroschisis, congenital diaphragmatic hernia and oesophageal fistula: a systematic review

BackgroundNeonatal care is commonly regionalised, meaning specialist services are only available at certain units. Consequently, infants with surgical conditions needing specialist care who are born in non-surgical centres require postnatal...

Routine Fogarty Catheter Occlusion of Fistula in Esophageal Atresia with Tracheoesophageal Fistula Surgery: A Retrospective Study

Objective: We aimed to analyze the outcomes of patients who underwent surgical repair of congenital esophageal atresia (EA) with a distal tracheoesophageal fistula (EA/TEF) or a Gross type C with successful routine Fogarty catheter occlusion of TEF. Materials and Methods: We retrospectively reviewed the medical records of patients who underwent surgical repair of Gross type C with successful routine Fogarty catheter occlusion of fistula between April 2010 and November 2016. Results: Nineteen patients were enrolled and included for analysis. Mean gestational age was 38.7 (1.9) weeks with 2 (10.5%) neonates born prematurely. Mean birthweight was 2569.3 (425.3) g. Five (26.3%) patients required mechanical ventilation (MV) before surgical repair of TEF. Median post-operative required MV after TEF surgery was 4 (3-6) days. The most common of post-operative complications were wound dehiscence (10.5%) and pneumothorax (10.5%). Long-term complications were gastroesophageal reflux disease (36.8%) and tracheomalacia (31.6%). Conclusion: The success rate of routine TEF occlusion with a Fogarty catheter was 86.4%. Routine Fogarty catheter occlusion of TEF can be used safely with experienced personnel, low incidence of aspiration and satisfied ventilation. There was no serious complication associated with placement of Fogarty catheter or catheter dislodgement, and it did not occur during any of the procedures.

Factors Predicting Postoperative Esophageal Stricture after Repaired Tracheoesophageal Malformation in Children with Esophageal Atresia

Background: An esophageal stricture is the most frequent complication after surgical correction for esophageal atresia (EA) patient, resulting in hospitalization or surgical intervention. Objective: To describe the characteristics of EA with a stricture and assess the factors influencing the postoperative esophageal stricture. Materials and Methods: All children after surgical correction between 1999 and 2014 were included. All complications at one and five years were recorded and predicting factors for esophageal stricture were analyzed. Results: Forty-seven patients were recorded. Thirty-one EA patients were divided into two groups as stricture and non-stricture. Stricture had 18 patients and non-stricture had 13 patients. Median follow-up time of EA with esophageal stricture was 10 years (3,690.5 days), and 5.7 years (2082.0 days) for EA without stricture. Vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities (VACTERL) association was found in 38% and 58.1% had anastomotic stricture. The waiting time from birth to surgical repair was significantly longer in the stricture group at 15 versus 3 days (p=0.009). The median esophageal stricture time was about five years (1,829 days). The most common clinical presentation of EA with stricture was dysphagia following with recurrent pneumonia. Respiratory pneumonia was more common in the stricture group (p=0.033). Conclusion: Primary repairs should not be delayed in EA patients. Anastomotic strictures can be found beyond the fifth year after surgery. A multidisciplinary follow-up plan should be continued for longer than five years to enable the detection of further late stricture formation. Keywords: Esophageal atresia; Tracheoesophageal fistula; Congenital esophageal atresia; Postoperative esophageal stricture

Evaluation of safety and efficacy of autologous oral mucosa-derived epithelial cell sheet transplantation for prevention of anastomotic restenosis in congenital esophageal atresia and congenital esophageal stenosis

BackgroundWe performed the first autologous oral mucosa-derived epithelial cell sheet transplantation therapy in a patient with refractory postoperative anastomotic stricture in congenital esophageal atresia (CEA) and confirmed its safety. In this study, patients with CEA and congenital esophageal stenosis were newly added as subjects to further evaluate the safety and efficacy of cell sheet transplantation therapy.MethodsEpithelial cell sheets were prepared from the oral mucosa of the subjects and transplanted into esophageal tears created by endoscopic balloon dilatation (EBD). The safety of the cell sheets was confirmed by quality control testing, and the safety of the transplantation treatment was confirmed by 48-week follow-up examinations.ResultsSubject 1 had a stenosis resected because the frequency of EBD did not decrease after the second transplantation. Histopathological examination of the resected stenosis revealed marked thickening of the submucosal layer. Subjects 2 and 3 did not require EBD for 48 weeks after transplantation, during which time they were able to maintain a normal diet by mouth.ConclusionsSubjects 2 and 3 were free of EBD for a long period of time after transplantation, confirming that cell sheet transplantation therapy is clearly effective in some cases. In the future, it is necessary to study more cases; develop new technologies such as an objective index to evaluate the efficacy of cell sheet transplantation therapy and a device to achieve more accurate transplantation; identify cases in which the current therapy is effective; and find the optimal timing of transplantation; and clarify the mechanism by which the current therapy improves stenosis.Trial registration: UMIN, UMIN000034566, registered 19 October 2018, https://upload.umin.ac.jp/cgi-open-bin/ctr_e/ctr_view.cgi?recptno=R000039393.

Epidemiology of congenital diaphragmatic hernia, esophageal atresia, and gastroschisis in South Brazil

Background: Birth defects remain a major contributor to infant mortality and lifelong disabilities. The epidemiology of congenital abnormalities varies around the world and little information is available from Latin America. Methods: This is an epidemiological, descriptive, cross-sectional study with data from the Department of Informatics of the Unified Health System (DATASUS) on Congenital diaphragmatic hernia (CDH), esophageal atresia (EA) and gastroschisis (GS) in South Brazil from 2009 to 2019. Results: The incidence of CDH is 0.93 cases, while EA is 0.47 and GS is 2.87, all per 10,000 live births. There is an association between all the malformations and premature birth, cesarean delivery, low birth weight, and low Apgar scores. Both EA and GS are associated with maternal age, EA with older, and GS with younger mothers. While EA is associated with multiple pregnancies, GS is associated with fewer years of maternal formal education, single parenting, and a lower number of prenatal consultations. CDH is associated with the male sex and black ethnicity. Conclusion: This large population-based study estimates the prevalence and demographic factors associated with CDH, EA, and GS, and extends the limited descriptive epidemiologic information available.

Retracted: Study of Nursing Models by Machine Learning in Children with Congenital Esophageal Atresia.

[This retracts the article DOI: 10.1155/2022/4147217.].

Factors Influencing Performance in Laparoscopic Suturing and Knot Tying: A Cohort Study.

Laparoscopic suturing and knot tying are regarded as some of the most difficult laparoscopic skills to learn. Training is essential to reach proficiency, but available training opportunities are limited. Various techniques to improve training have been evaluated. It appears that individual-related factors affect initial performance and response to training. Thus, the current study aimed to assess factors influencing laparoscopic-suturing and knot-tying performances. All patients were trained one-on-one (teacher-student) for 3 hours. Patients were tested before training (bowel anastomosis model) and directly after training (congenital diaphragmatic hernia or esophageal atresia model) to evaluate transferability. Primary endpoints were time, knot quality, precision, knot strength, and overall laparoscopic knotting performance. Moreover, factors such as (1) age, (2) gender, (3) handedness, (4) previous training or operative experience, (5) playing an instrument, (6) sportive activities, and (7) computer gaming which may influence the primary endpoints were assessed. In total, 172 medical students or novice surgical residents were included. Training significantly improved all outcome parameters assessed in the current study. More than 50% of the patients reached proficiency after 3 hours of training. Personal factors like operative experience, playing music instruments, sportive activities, and computer gaming affected some outcome parameters. Handedness and gender affected initial performance but differences partially subsided after training. Younger participants showed a much better response to training. In conclusion, several factors influence initial performance and response to the training of laparoscopic suturing and knot tying. Surgeons who want to improve their laparoscopic performance, should concentrate on playing a musical instrument and performing sports rather than playing videos gaming. It appears that training is a key and more practice opportunities should be incorporated into medical school and surgical curricula. Moreover, handedness may affect the outcome but only if the training concepts are not tailored to the dominant hand of the surgeon. Future training and surgical curricula should adapt to this and tailor their concepts accordingly.

Esophageal magnetic compression anastomosis in dogs.

BACKGROUNDMagnetic compression anastomosis (MCA) is a novel suture-free reconstruction of the digestive tract. It has been used in gastrointestinal anastomosis, jejunal anastomosis, cholangioenteric anastomosis and so on. The traditional operative outcomes of congenital esophageal atresia and benign esophageal stricture are poor, and there are too many complications postoperatively.AIMTo test MCA technology to reconstruct the esophagus in dogs, prior to studying the feasibility and safety of MCA in humans.METHODSThirty-six dogs were randomized into either the study or control group (n = 18 per group). The dogs in the study group were subjected to end-to-end esophageal anastomosis with the magnetic compression device, while those in the control group underwent hand-sewn anastomosis with 4-0 absorbable multifilament Vicryl. We used interrupted single-layer inverting sutures. The anastomosis time, gross appearance, weight and pathology of the anastomosis were evaluated at one month, three months and six months postoperatively.RESULTSThe anastomosis time of the MCA group was shorter than that of the hand-sewn group (7.5 ± 1.0 min vs 12.5 ± 1.8 min, P < 0.01). In the MCA group, X-ray examination was performed every day to locate the magnetic device in the esophagus before the magnetic device fell off from the esophagus. In the hand-sewn group, dogs did not undergo X-ray examination. One month after the surgeries, the mean weight of the dogs in the hand-sewn group had decreased more than that of the dogs in the MCA group (11.63 ± 0.71 kg vs 12.73 ± 0.80 kg, P < 0.05). At 3 mo and 6 mo after the operation, the dogs’ weights were similar between the two groups (13.75 ± 0.84 kg vs 14.03 ± 0.82 kg, 14.93 ± 0.80 kg vs 15.44 ± 0.47 kg). The number of inflammatory cells in MCA group was lower than that in hand-sewn group on 1 mo after operation.CONCLUSIONMCA is an effective and safe method for esophageal reconstruction. The anastomosis time of the MCA group was less than that of the hand-sewn group. This study shows that MCA technology may be applied to human esophageal reconstruction, provided these favorable results are confirmed by more publications.

A retrospective review of the perioperative management of patients with congenital oesophageal atresia and tracheo-oesophageal fistula at a South African third level hospital

A retrospective review of the perioperative management of patients with congenital oesophageal atresia and tracheo-oesophageal fistula at a South African third level hospital

Study of Nursing Models by Machine Learning in Children with Congenital Esophageal Atresia.

This research paper elucidates the clinical effect of an integrated nursing model of medical care and patient in the diagnosis and treatment of congenital esophageal atresia (CEA). For this purpose, a total of 120 children with CEA were selected as study subjects who were admitted to our hospital (January 2017 to April 2020). They were randomly divided into the control group and observation group. Each group had 60 cases. The control group was given routine nursing, while the observation group adopted the integrated nursing model of medical care. The integrated nursing model had the characteristics of recognizing and managing the CEA quickly and efficiently. Thus, it can help increase the survival rate of infants. This model works along with the parents to provide specialized services to the child. They were tasked to carefully observe the infants as well as calm the parents. They were also given the additional task of keeping track of patients who were currently admitted in the hospital and those who were already discharged. The tracking and communication were done with the help of a communication platform which is WeChat. The rehospitalization rate, 1-hour visit rate, accuracy rate of children with suspected postoperative complications, psychological status of children's parents, medical compliance, and satisfaction were compared between the two groups. The rehospitalization rate in the observation group was lower than that in the control group (P < 0.05). The 1-hour visit rate and accuracy of children with suspected postoperative complications in the observation group were higher than those in the control group (P < 0.05). The anxiety and depression scores of the parents in the observation group were lower than those in the control group (P < 0.05). The compliance and satisfaction of parents in the observation group were higher than those in the control group (P < 0.05). The clinical effect of the integrated nursing model of medical care and patient in CEA was highly satisfactory. It reduces the rehospitalization rate and enables timely diagnosis and treatment of suspicious complications effectively. It also improved parents' negative psychological emotions, compliance, and satisfaction.

Ultrasound-guided thoracic paravertebral block in a premature neonate with congenital oesophageal atresia with a tracheo-oesophageal fistula: a case report

Ultrasound-guided thoracic paravertebral block (TPVB) is a promising new technique for use in children of various ages. In this case report, a successful ultrasound (USG) guided TPVB procedure in premature neonate is described. A 3-day-old 1600-g neonate, born at 32 weeks of gestation, was underwent thoracostomy for esophageal atresia with esophageal fistula repair. At the end of the surgery, USG-guided TPVB were successfully performed using a linear probe in the paramedian position. USG-guided TPVB could be an alternative method for providing effective analgesia in a premature neonates in the future.

Congenital Esophageal Atresia Long-Term Follow-Up—The Pediatric Surgeon’s Duty to Focus on Quality of Life

Esophageal atresia (EA) is the most common congenital esophageal malformation. An improvement in survival led to a focus on functional outcomes and quality of life (QoL). We analyzed the long-term outcomes and QoL of patients submitted to surgery for EA. Perinatal characteristics, surgical procedures, gastrointestinal and respiratory current symptoms and QoL were investigated. Thirty-nine patients were included. Long Gap patients had a higher rate of prematurity and low birth weight. The prevalent surgical procedure was primary esophageal anastomosis, followed by gastric pull-up. Twenty-four patients had post-operative stenosis, while gastroesophageal reflux (GER) required fundoplication in eleven cases. Auxological parameters were lower in Long Gap patients. The lowest scores of QoL were in the Long Gap group, especially in younger patients, which was the group with the highest number of symptoms. In the long term, the QoL appeared to be more dependent on the type of esophageal atresia rather than on associated malformations. Surgical management of GER was indicated in all patients with Long Gap EA, supposedly due to the prevalence of gastric pull-up for this type of EA. The assessment of QoL is part of surgeon’s management and needs to be performed in each phase of a child’s development.