Impacto del manejo multidisciplinar digestivo-quirúrgico en los pacientes con atresia de esófago / Impact of digestive-surgical cross-disciplinary management in patients with esophageal atresia

Objetivos: El objetivo de este estudio es analizar si los pacientesintervenidos de atresia de esófago (AE) se benefician de un programade seguimiento multidisciplinar, basado en las guías clínicas actuales,implantado en nuestro centro. Material y métodos: Estudio retrospectivo, observacional y analí-tico incluyendo los pacientes intervenidos de AE entre 2012 y 2022. Seanalizaron los resultados de la implantación en 2018 de un programa deconsultas conjuntas de gastroenterología y cirugía pediátrica aplicandoun protocolo basado en las nuevas guías ESPGHAN-NASPGHAN. Sedividieron a los pacientes tratados antes y después de 2018 y se compararon las variables cuantitativas: pérdidas de seguimiento, inicio y duracióndel tratamiento antirreflujo e inicio de nutrición enteral, y cualitativas:prevalencia de reflujo gastroesfoágico, realización de cirugía antirreflujo,infecciones respiratorias, estenosis de la anastomosis, refistulizaciones,disfagia, episodios de impactación, necesidad de gastrostomía y resul-tados de las endoscopias. Resultados: Se incluyeron 38 pacientes. Un 63,2% presentaronreflujo gastroesofágico. El 97,4% tomaron tratamiento antirreflujo el primer año de vida que posteriormente se retiró en el 47,4%. El tiempo deretirada se redujo una media de 24 meses tras la aplicación del programa(p< 0,05). Se realizaron 4,6 veces más pHmetrías tras la implantacióndel programa. El protocolo estandarizó la realización de endoscopiasen pacientes asintomáticos al cumplir 5 y 10 años. Se realizaron 25endoscopias con tomas de biopsia después de 2018, detectando alteraciones histológicas en un 28%. El número de pérdidas de seguimiento seredujo de forma significativa tras la implantación del protocolo (p< 0,05). Conclusiones: El seguimiento multidisciplinar digestivo-quirúrgicode los pacientes con AE genera un impacto positivo en su evolución.(AU)

Objective: The objective of this study was to analyze whether patients undergoing esophageal atresia (EA) surgery benefit from a cross-disciplinary follow-up program, based on current clinical guidelines,implemented in our institution. Materials and methods: An observational, analytical, retrospectivestudy of patients undergoing EA surgery from 2012 to 2022 was carriedout. The results of a joint pediatric surgery and gastroenterology consultation program –which was implemented in 2018 and applies a protocolbased on the new ESPGHAN-NASPGHAN guidelines– were analyzed.Patients were divided according to whether they had been treated before or after 2018. Quantitative variables –follow-up losses, anti-refluxtreatment initiation and duration, and enteral nutrition initiation– andqualitative variables –prevalence of gastroesophageal reflux, anti-refluxsurgery, respiratory infections, anastomotic stenosis, re-fistulizations,dysphagia, impaction episodes, need for gastrostomy, and endoscopicresults– were compared. Results: 38 patients were included. 63.2% had gastroesophagealreflux. 97.4% received anti-reflux treatment in the first year of life, withtreatment being subsequently discontinued in 47.4%. Discontinuationtime decreased by a mean of 24 months following program implementation (p< 0.05). A 4.6-fold increase in the frequency of pH-metries wasnoted following program implementation. The protocol standardizedendoscopies in asymptomatic patients when they turn 5 and 10 years old. 25 endoscopies with biopsy were carried out after 2018, with histologicaldisorders being detected in 28% of them. The number of follow-up lossessignificantly decreased following protocol implementation (p< 0.05). Conclusions: Digestive-surgical cross-disciplinary follow-up of EApatients has a positive impact on patient progression. Applying the guidelines helps optimize treatment and early diagnosis of complications.(AU)

Oral Viscous Budesonide in Children With Eosinophilic Esophagitis After Repaired Esophageal Atresia: A Clinical Trial.

OBJECTIVES: A high prevalence of eosinophilic esophagitis (EoE) has been reported in children with repaired esophageal atresia (EA). Topical steroids proved to be an effective and safe therapy in EoE, although not approved in pediatrics. We report the results of the first clinical trial of oral viscous budesonide (OVB) performed in children with EoE after repaired esophageal atresia (EoE-EA). METHODS: This open-label, single-arm, phase 2 clinical trial with randomized pharmacokinetic sampling, was conducted at the Bambino Gesù Children's Hospital between September 2019 and June 2021. EoE-EA patients received an age-banded dose of OVB twice daily for 12 weeks and were endoscopically evaluated. The primary endpoint was the rate of patients achieving histological remission. Secondary endpoints included clinical and endoscopic benefit after treatment, and safety assessments. RESULTS: Eight consecutive EA-EoE patients were enrolled (median age 9.1 years, interquartile range 5.5). Of these, 5 received 0.8 mg and 3 received 1.0 mg twice daily of OVB. Histological remission was obtained in all but 1 patient (87.5%). The clinical score showed significant improvement at the end of treatment in all patients. No endoscopic features of EoE were found after treatment. No treatment-emergent adverse event occurred. CONCLUSION: OVB is an effective, safe, and well-tolerated formulation of budesonide for use in pediatric patients with EoE-EA.

Endoscopic Injections of Botulinum Toxin Type A in the Piglet Esophagus Is Safe and Feasible but Did Not Result in any Significant Structural Changes 3 Days after Injection.

INTRODUCTION: Treatment for long-gap esophageal atresia (LGEA) aims at achieving primary anastomosis with minimal tension. Previous studies have shown that intramural injections with botulinum toxin type-A (BTX-A) from the adventitial side can increase the elongation of the piglet and rat esophagus before bursting, and that this effect is dose and time dependent. Our aim was to determine if endoscopic injections would be feasible, safe, and with an effect on the mechanical properties of the esophagus. METHODS: Twenty-two male piglets (5.15 kg) were randomized into two groups, one receiving 2 units/kg BTX-A, the other equal volume 0.9% NaCl. On day 3, the esophagus was harvested and tested in a stretch-tension machine to evaluate elongation and maximum load, followed by histological examination. RESULTS: No adverse effects to the procedure were observed. No statistically significant difference in elongation or maximum load before bursting between the treatment and placebo group was found. In histopathological analysis, inflammation and abscess formation were observed with no statistically significant difference between the two groups. CONCLUSION: Endoscopic placement of BTX-A injections in the piglet esophagus was safe and feasible but did not result in any difference in the mechanical properties or histology of the esophagus.

Pharmacogenomics fail to explain proton pump inhibitor refractory esophagitis in pediatric esophageal atresia.

BACKGROUND: Esophagitis is prevalent in patients with esophageal dysmotility despite acid suppression, likely related to poor esophageal clearance. Esophageal atresia (EA) is a classic model of dysmotility where this observation holds true. In adult non-dysmotility populations, failure of esophagitis to respond to proton pump inhibitors (PPI) has been linked to variants in CYP2C19 that influence the activity of the encoded enzyme. It is unknown if CYP2C19 metabolizer phenotype contributes to PPI-refractory, non-allergic esophagitis in EA. METHODS: We performed a cross-sectional study of 314 children with (N = 188) and without (N = 126) EA who were on PPI therapy at the time of endoscopy to evaluate for possible gastroesophageal reflux disease. Patients with eosinophilic esophagitis and/or fundoplication were excluded. Clinical and histology data were collected. Genomic DNA from biopsy samples was genotyped for polymorphisms in CYP2C19. RESULTS: CYP2C19 metabolizer phenotypes were not associated with presence or severity of esophagitis (P = 0.994). In a multivariate logistic regression adjusted for potential confounders, EA was the strongest and only significant predictor of esophagitis (odds ratio 2.72, P = 0.023). Using negative binomial regression, we found that CYP2C19 phenotype was not a significant predictor of eosinophil count in children with PPI-refractory esophagitis. CONCLUSIONS: Patients with EA are significantly more likely to experience PPI-refractory, non-allergic esophagitis than controls regardless of CYP2C19 metabolizer phenotype, suggesting that factors other than CYP2C19 genetics, including dysmotility, are the primary drivers of esophagitis in EA. CYP2C19 genotype failed to predict PPI-refractory, non-allergic esophagitis in both EA and non-EA children.

Prolonged Use of Proton Pump Inhibitors as Stricture Prophylaxis in Infants with Reconstructed Esophageal Atresia.

Introduction Proton pump inhibitors (PPIs) are used as prophylaxis, guarding against anastomotic stricture (AS) in the aftermath of reconstructed esophageal atresia (EA). The incidence of stricture formation was studied in this setting, comparing outcomes of 3- and 12-month PPI prophylactic regimens. Patients and Methods Patient characteristics (gestational age, birth weight, prevalence of chromosomal aberrations, and other malformations), as well as rates of survival, AS formation, and required balloon dilation, were recorded in the following therapeutic subsets: (1) all infants undergoing primary surgical anastomosis for EA in years 2010-2014 and given postoperative PPI prophylaxis for 12 months and (2) all infants similarly treated for EA in years 2001-2009 but given postoperative PPI prophylaxis for 3 months only. Duration of follow-up was 1 year in each group. Results Patient characteristics and survival rates in 12-month (n = 33) and in 3-month (n = 30) treatment groups did not differ significantly. The prevalence of AS was 42%/43% in each group (12 months, 14/33; 3 months, 13/30; p = 1). Median number of dilations required was 3 (range, 1-9) per patient in each group (p = 0.69). Median age at initial dilation was 163 days and 63 days in 12- and 3-month groups, respectively (p = 0.04). Conclusion Development of AS in the first year after reconstruction of EA was not reduced by prolonged PPI prophylaxis (12 vs. 3 months), but initial balloon dilation procedures were performed later in infants who were treated longer.

The Effect of Intramural Botulinum Toxin Injections on the Elongation of the Piglet Oesophagus Is Time Dependent.

Introduction One in 4,000 infants is born with oesophageal atresia. Approximately 15% of these have a long gap oesophageal atresia, where primary anastomosis is difficult or impossible. Previous studies have shown an effect of intramural botulinum toxin type A (BTX-A) injections on the elongation and max load of the esophagus 1 hour after injection. We hypothesized that a longer waiting period of 2 hours could increase this effect. Methods Forty-five piglets were randomized into three groups. Two treatment groups received 2 units/kg of BTX-A and one group received saline. After 1 or 2 hours, a segment of the esophagus was harvested and put in a stretch-tension device to assess elongation and max load. Results Elongation from preload to max load and percentage elongation in the BTX-A 2h group (17.09 mm, 46.46%) was significantly higher compared with the BTX-A 1h group (13.59 mm, 40.16%) and the placebo group (13.77 mm, 39.92%). Conclusion Elongation of the piglet esophagus was significantly improved with a 2-hour waiting period after BTX-A injection. Injections with BTX-A could be useful in oesophageal atresia, where primary anastomosis is not possible.

Intramural Injection with Botulinum Toxin Type A in Piglet Esophagus. The Influencer on Maximum Load and Elongation: A Dose Response Study.

Introduction The treatment of esophageal atresia (OA) is challenging. The main goal is to achieve primary anastomosis. We have previously demonstrated in a pig model that intramural injection of botulinum toxin type A (BTX-A) resulted in significant elongation of the esophagus during tensioning until bursting point. The objectives of the present study were to investigate the influence of different amounts of intramural BTX-A on the stretch-tension characteristics and histological changes of the esophagus in piglets. Materials and Methods A total of 52 piglets were randomized to four groups receiving 2, 4, or 8 units/kg of BTX-A or isotonic saline (placebo). After a 1-hour of rest the esophagus was harvested and subjected to a stretch-tension test and histological examination to assess changes in the density of presynaptic vesicles in the nerve cells. Results Overall, 9 of the 52 animals were excluded from analysis due to problems with the stretch-tension test or death from anesthesia. The maximum loads were higher in the BTX-A groups (2 units/kg: +2.1 N; 4 units/kg: +1.3 N; 8 units/kg: +1.9 N) than the placebo (p = 0.046). There were no significant differences in percentage elongation, or histology. Conclusions This study demonstrated that injection of 2 units/kg BTX-A into a nonanastomosed esophageal wall resulted in a modest increase in the maximum load achieved before bursting; this may be due to the muscle-relaxant effect of BTX-A. BTX-A injection produced no significant effects on elongation or esophageal histology. The clinical usefulness of BTX-A in treatment of OA is still unclear.

Tratamiento anestésico del neonato con atresia de esófago asociada a fístula traqueoesofágica y ano imperforado / Anesthetic management of a neonate with esophageal atresia, tracheoesophageal fistula and imperforate anus

La atresia de esófago es una malformación infrecuente (1:2.500-4.500 recién nacidos vivos), incompatible con la vida y una urgencia quirúrgica neonatal. El 30% de los pacientes son prematuros o presentan bajo peso al nacer y el 50% presentan anomalías asociadas, principalmente cardíacas. Las cardiopatías congénitas de orden mayor o el bajo peso al nacer son predictores independientes de mortalidad y eventos críticos perioperatorios. Presentamos el caso de un paciente intervenido de urgencia de atresia de esófago, fístula traqueoesofágica tipo iii b/C e imperforación anal. El objetivo de este artículo es la exposición de las consideraciones anestésicas en pacientes con esta afección, cuyo complejo manejo perioperatorio supone un importante reto y debe realizarse por equipos multidisciplinares con experiencia en neonatología. Establecer una vía aérea segura y obtener una ventilación pulmonar efectiva que minimice la fuga de aire al tracto digestivo debe ser uno de los objetivos prioritarios del manejo anestésico (AU)

Esophageal atresia is a rare condition (1:2,500-4,500), incompatible with life, and a surgical emergency in the neonatal period. It is associated with prematurity in 30% of cases, and to congenital abnormalities in 50% of cases, especially cardiac anomalies. Major congenital heart diseases and low weight are independent predictors of mortality and critical perioperative events. The aim of this article is to describe the most significant anaesthetic challenges presented in a case of a term neonate undergoing emergency surgery after being diagnosed with esophageal atresia, tracheoesophageal fistula type iiib/C, and imperforate anus. The major priorities during the anaesthetic management consist of establishing a safe airway and effective pulmonary ventilation that minimises air leakage to the upper digestive tract (AU)

Esophageal atresia: data from a national cohort.

PURPOSE: A prospective national register was established in 2008 to record all new cases of live-birth newborns with esophageal atresia (EA). This epidemiological survey was recommended as part of a national rare diseases plan. METHODS: All 38 national centers treating EA participated by completing for each patient at first discharge a questionnaire validated by a national committee of experts. Data were centralized by the national reference center for esophageal anomalies. Quantitative and qualitative analyses were performed, with P-values of less than 0.05 considered statistically significant. Results of the 2008-2009 data collection are presented in this report. RESULTS: Three hundred seven new living cases of EA were recorded between January 1, 2008, and December 31, 2009. The male/female sex ratio was 1.3, and the live-birth prevalence of EA was 1.8 per 10,000 births. Major characteristics were comparable to those reported in the literature. Survival was 95%, and no correlation with caseload was noted. CONCLUSIONS: Epidemiologic surveys of congenital anomalies such as EA, which is a rare disease, provide valuable data for public health authorities and fulfill one important mission of reference centers. When compared with previous epidemiological data, this national population-based registry suggests that the incidence of EA remains stable.

Quilotórax tras reparación de atresia de esófago: tratamiento conservador con octreotida / Chylothorax following repair of oesophageal atresia: conservative treatment with octreotide

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Prostaglandin therapy for ductal patency: how long is too long?

UNLABELLED: The management of neonates born with duct-dependent congenital heart defects in association with prematurity or low birth weight is challenging. We describe two preterm and low birth weight infants with duct-dependent congenital heart disease where cardiac surgery was successfully delayed by maintaining ductal patency using a prolonged prostaglandin infusion. This allowed time for growth and maturation and therefore reduced the risks associated with surgery and cardiopulmonary bypass. CONCLUSION: Maintenance of ductal patency by a prolonged prostaglandin infusion in low birth weight or preterm infants with duct-dependent congenital heart disease is a viable option that allows cardiac surgery to be delayed whilst awaiting further growth and development.

Long-term administration of prostaglandin E1: report of two cases with tetralogy of Fallot and esophageal atresia.

The authors continuously administered prostaglandin E1 (PGE1) iv to 2 infants with tetralogy of Fallot (TOF) and esophageal atresia with tracheoesophageal fistula (TEF) for more than 30 days, and observed side effects which could be attributed to the long-term administration of PGE1. After division of the TEF and anastomosis of the esophagus, leakage from the anastomosis developed in both cases. Because of the infectious foci in the thorax, Blalock's procedure was postponed and PGE1 was continued for 49 and 37 days. The authors believe radiographs of long bones and ribs demonstrated cortical hyperosteosis in both cases. Bone abnormalities became apparent approximately 30 days after the start of PGE1 and were associated with increases in serum alkaline phosphatase (peak value of about 2000 IU/L). Roentgenographic changes reverted toward normal and alkaline phosphatase values decreased after the cessation of PGE1 in both cases.

Use of cimetidine in esophageal atresia with lower tracheoesophageal fistula.

Pneumonia, in infants suffering from esophageal atresia with lower esophageal fistula, is usually caused by gastric reflux through the fistula, In order to abolish the acidity of the gastric content, Cimetidine i.v. infusion was used during the first 12--48 hr after diagnosis, while treating the pneumonia. The amount of Cimetidine required to induce achlorhydria was established by serial aspiration of gastric content, in infants and children with gastrostomies performed for various causes.