Management of long-gap esophageal atresia
Ontology highlight
ABSTRACT:
SUBMITTER:
PROVIDER: S-EPMC10915436 | biostudies-literature | 2024 Feb
REPOSITORIES: biostudies-literature
Similar Datasets
Project description:BackgroundElongation and repair of long gap esophageal atresia (LGEA) can be performed thoracoscopically, even directly after birth. The effect of thoracoscopic CO2-insufflation on cerebral oxygenation (rScO2) during the consecutive thoracoscopic procedures in repair of LGEA was evaluated.MethodsProspective case series of five infants, with in total 16 repetitive thoracoscopic procedures. A CO2-pneumothorax was installed with a pressure of maximum 5 mmHg and flow of 1 L/min. Parameters influencing rScO2 were monitored. For analysis 10 time periods of 10' during surgery and in the perioperative period were selected.ResultsMedian gestational age was 35+3 [range 33+4 to 39+6] weeks; postnatal age at time of first procedure 4 [2-53] days and time of insufflation 127[22-425] min. Median rScO2 varied between 55 and 90%. Transient outliers in cerebral oxygenation were observed in three patients. In Patient 2 oxygenation values below 55% occurred during a low MABP and Hb < 6 mmol/L. The rScO2 increased after erythrocytes transfusion. Patient 5 also showed a rScO2 of 50% with a Hb <6 mmol/L during all procedures, except for a substantial increase during a high paCO2 of 60 mmHg. Patient 4 had a rScO2 > 85% during the first procedure with a concomitant high FiO2 > 45%. All parameters recovered during the surgical course.ConclusionsThis prospective case series of NIRS during consecutive thoracoscopic repair of LGEA showed that cerebral oxygenation remained stable. Transient outliers in rScO2 occurred during changes in hemodynamic or respiratory parameters and normalized after interventions of the anesthesiologist. This study underlines the importance of perioperative neuromonitoring and the close collaboration between pediatric surgeon, anesthesiologist and neonatologist.
| S-EPMC5394154 | biostudies-literature
Project description:The ideal approach to long gap esophageal atresia is still controversial. On one hand, preserving a patient's native esophagus may require several steps and can be fraught with complications. On the other hand, most replacement procedures are irreversible and disrupt gastrointestinal physiology. The purpose of this study was to evaluate the short- and medium-term outcome of electively delayed esophageal elongation procedures before esophageal reconstruction in patients with long-gap esophageal atresia. Since the neonatal esophagus grows over-proportionally and can increase its wall thickness in the first few months of life, we hypothesized that postponing the elongation steps until 3 months of age would lead to a lower complication rate. We thus retrospectively recorded complications such as mediastinitis, anastomotic leakage, stricture formation, or gastroesophageal reflux requiring surgery, and compared it to reported outcomes. In our treatment protocol, patients born with long-gap esophageal atresia underwent gastrostomy placement and were sham fed until 3 months of age. We then assessed the gap between the esophageal ends and started serial elongation procedures. We only proceeded to the reconstruction of the esophagus when its length allowed a tension-free anastomosis. From April 2013 to April 2019, we treated 13 Patients with long-gap esophageal atresia. Nine patients without prior surgical procedures underwent Foker procedures. Four patients arrived with a pre-existing cervical esophagostomy and thus underwent Kimura's procedure, two of them with a concomitant Foker elongation of the lower pouch. Esophageal reconstruction was feasible in all patients, while none of them developed mediastinitis at any point in their treatment. We managed the only anastomotic leak conservatively. Almost half of the patients did not require any further intervention following reconstruction, while three patients required multiple (≥5) anastomotic dilatations. All but one patient achieved full oral nutrition. Only one child required a fundoplication to manage gastroesophageal reflux symptoms. Electively delayed esophageal elongation procedures in patients with long-gap esophageal atresia allowed preservation of the native esophagus in all patients. The approach had low peri-procedural morbidity, and patients enjoy favorable functional outcomes. Therefore, we suggest considering this method in the management of patients with long-gap esophageal atresia.
| S-EPMC8290357 | biostudies-literature
Project description:BackgroundChildren with long-gap esophageal atresia (LGEA) risk living with aerodigestive morbidity and mental health difficulties. No previous study has investigated their experiences of schooling, despite the importance of schools in children's development, learning and social relationships. We aimed to describe experiences of schooling in children with LGEA in Sweden in comparison with children with EA who had primary anastomosis.MethodChildren with LGEA aged 3-17 were recruited nationwide in Sweden. One parent completed a survey on their child's school-based supports (according to definitions from the Swedish National Agency for Education), school absence, school satisfaction, school functioning (PedsQL 4.0), mental health (Strength and Difficulties Questionnaire) and current symptomatology. School data were compared between 26 children with LGEA to that from 95 children with EA who had PA, a hypothesized milder affected group. Mental health level was determined using validated norms; abnormal ≥ 90 percentile. Data were analyzed using descriptives, correlation and Mann-Whitney-U test. Significance level was p < 0.05.ResultsFormal school-based support was reported in 17 (65.4%) children with LGEA and concerned support with nutritional intake (60%), education (50%) and medical/special health needs (35%). The prevalence of school-based support was significantly higher compared to children with PA overall (36.8%, p = 0.013) and regarding nutritional intake support (20%, p < 0.001). In children with LGEA, school-based support was related to low birth weight (p = 0.036), young child age (p = 0.014), height ≤ -2SD for age/sex (p = 0.024) and an increased number of aerodigestive symptoms (p < 0.05). All children with LGEA who had abnormal mental health scores had school-based support, except for one child. Nine children with LGEA (36%) had school absence ≥ 1times/month the past year, more frequently because of colds/airway infections (p = 0.045) and GI-specific problems compared to PA (p = 0.003). School functioning scores were not significantly different from children with PA (p = 0.34) but correlated negatively with school-based support (< 0.001) and school absence (p = 0.002). One parent out of 26 reported their child's school satisfaction as "not good".ConclusionsChildren with LGEA commonly receive school-based support, reflecting multifaceted daily needs and disease severity. School absence is frequent and related to poorer school functioning. Future research focusing on academic achievement in children with EA is needed.
| S-EPMC10408199 | biostudies-literature
Project description:Esophageal atresia and tracheoesophageal fistula (EA/TEF) are major congenital malformations affecting 1:3500 live births. Current research efforts are focused on understanding the etiology of these defects. We describe well-known animal models, human syndromes, and associations involving EA/TEF, indicating its etiologically heterogeneous nature. Recent advances in genotyping technology and in knowledge of human genetic variation will improve clinical counseling on etiologic factors. This review provides a clinical summary of environmental and genetic factors involved in EA/TEF.
| S-EPMC2874026 | biostudies-literature
Project description:The survival of the patients with esophageal atresia an tracheo esophageal fistula is believed to be an epitome of the success of the neonatal surgery. Restoring the continuty of the food pipe by esophagus to esophagus anastomosis is the best option. Preservation of natural esophagus by delayed repair in a wide gap esophageal atresia is a preferred technique worldwide, however such a management required prolonged hospitalization and dedicated nursing care, which is often not available in most of the centres in India. Esophageal substitutes in wide gap requires multiple operations and have long term problems, so remains the last option. I use the technique of oblique anastomosis which had distrinct advantage over circular anastomosis in the management of esophageal atresia1. This techniqe helps in bridging wide gap to some extent & minimal stricture formation.
| S-EPMC5473312 | biostudies-other
Project description: Not available
| S-EPMC10514492 | biostudies-literature
Project description:IntroductionPediatric surgeons have yet to reach a consensus whether a gastric sleeve pull-up or delayed primary anastomosis for the treatment of esophageal atresia (EA), especially of the long-gap type (LGEA) should be performed. Thus, the aim of this study was to evaluate clinical outcome, quality of life (QoL), and mental health of patients with EA and their parents.MethodsClinical outcomes of all children treated with EA from 2007 to 2021 were collected and parents of affected children were asked to participate in questionnaires regarding their Quality of Life (QoL) and their child's Health-Related Quality of Life (HRQoL), as well as mental health.ResultsA total of 98 EA patients were included in the study. For analysis, the cohort was divided into two groups: (1) primary versus (2) secondary anastomosis, while the secondary anastomosis group was subdivided into (a) delayed primary anastomosis and (b) gastric sleeve pull-up and compared with each other. When comparing the secondary anastomosis group, significant differences were found between the delayed primary anastomosis and gastric sleeve pull-up group; the duration of anesthesia during anastomosis surgery (478.54 vs 328.82 min, p < 0.001), endoscopic dilatation rate (100% vs 69%, p = 0.03), cumulative time spent in intensive care (42.31 vs 94.75 days, p = 0.03) and the mortality rate (0% vs 31%, p = 0.03). HRQoL and mental health did not differ between any of the groups.ConclusionDelayed primary anastomosis or gastric sleeve pull-up appear to be similar in patients with long-gap esophageal atresia in many key aspects like leakage rate, strictures, re-fistula, tracheomalacia, recurrent infections, thrive or reflux. Moreover, HrQoL was comparable in patients with (a) gastric sleeve pull-up and (b) delayed primary anastomosis. Future studies should focus on the long-term results of either preservation or replacement of the esophagus in children.
| S-EPMC10073059 | biostudies-literature
Project description:BackgroundChildren undergoing surgical anastomosis for long-gap esophageal atresia (LGEA) often suffer from complications related to delayed oral feeding, which may impair their early development. Clinical swallow evaluation (CSE) is an effective technique to improve feeding outcomes. However, there are limited evidences on the application of CSE in these children.MethodsSince 2020, serial CSEs have been consistently implemented for children undergoing anastomosis for LGEA in our hospital. We conducted a retrospective study comparing 19 children who received CSE with 31 historical controls who did not. Inverse probability of treatment weighting (IPTW) was applied to balance preoperative characteristics. We compared the time from surgery to full oral feeding and the rate of postoperative complications between the two groups. Growth curves for length-for-age Z score (LAZ) and weight-for-age Z score (WAZ) up to age 3 were fitted using generalized additive mixed models.ResultsThe median time to full oral feeding was 1.1 months [interquartile range (IQR), 0.8-2.4] in the CSE group and 1.5 months (IQR, 0.6-5.7) for controls. After IPTW, CSE was associated with a shorter time to full oral feeding, with a weighted hazard ratio of 2.26 [95% confidence interval (CI), 1.21 to 4.24]. LAZ growth curves significantly differed between groups (P = 0.001).ConclusionCSE was associated with the expedited achievement of full oral feeding and a more favorable growth pattern before 3 years of age.
| S-EPMC11634968 | biostudies-literature
Project description:BackgroundEndoscopic dilatation is the first-line treatment of stricture formation after esophageal atresia (EA) repair. However, there is no consensus on how to perform these dilatation procedures which may lead to a large variation between centers, countries and doctor's experience. This is the first cross-sectional study to provide an overview on differences in endoscopic dilatation treatment of pediatric anastomotic strictures worldwide.MethodsAn online questionnaire was sent to members of five pediatric medical networks, experienced in treating anastomotic strictures in children with EA. The main outcome was the difference in endoscopic dilatation procedures in various centers worldwide, including technical details, dilatation approach (routine or only in symptomatic patients), and adjuvant treatment options. Descriptive statistics were performed with SPSS.ResultsResponses from 115 centers from 32 countries worldwide were analyzed. The preferred approach was balloon dilatation (68%) with a guidewire (66%), performed by a pediatric gastroenterologist (n = 103) or pediatric surgeon (n = 48) in symptomatic patients (68%). In most centers, hydrostatic pressure was used for balloon dilatation. The insufflation duration was standardized in 59 centers with a median duration of 60 (range 5-300) seconds. The preferred first-line adjunctive treatments in case of recurrent strictures were intralesional steroids and topical mitomycin C, in respectively 47% and 31% of the centers.ConclusionsWe found a large variation in stricture management in children with EA, which confirms the current lack of consensus. International networks for rare diseases are required for harmonizing and comparing the procedures, for which we give several suggestions.
| S-EPMC8195894 | biostudies-literature
Project description:ObjectivesAfter surgical repair, up to 70% of esophageal atresia (EA) patients suffer from gastroesophageal reflux disease (GERD). The ESPGHAN/NASPGHAN guidelines on management of gastrointestinal complications in EA patients were published in 2016. Yet, the implementation of recommendations on GERD management remains poor.We aimed to assess GERD management in EA patients in more detail, to identify management inconsistencies, gaps in current knowledge, and future directions for research.MethodsA digital questionnaire on GERD management in EA patients was sent to all members of the ESPGHAN EA working group and members of the International network of esophageal atresia (INoEA).ResultsForty responses were received. Thirty-five (87.5%) clinicians routinely prescribed acid suppressive therapy for 1-24 (median 12) months. A fundoplication was considered by 90.0% of clinicians in case of refractory GERD with persistent symptoms despite maximal acid suppressive therapy and in 92.5% of clinicians in case of GERD with presence of esophagitis on EGD. Half of clinicians referred patients with recurrent strictures or dependence on transpyloric feeds. Up to 25.0% of clinicians also referred all long-gap EA patients for fundoplication, those with long-term need of acid suppressants, recurrent chest infections and feedings difficulties.ConclusionsRespondents' opinions on the optimal duration for routine acid suppressive therapy and indications for fundoplication in EA patients varied widely. To improve evidence-based care for EA patients, future prospective multicenter outcome studies should compare different diagnostic and treatment regimes for GERD in patients with EA. Complications of therapy should be one of the main outcome measures in such trials.
| S-EPMC9278714 | biostudies-literature