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)

Pharmacologic treatment of hyperthyroidism during pregnancy.

Clinical hyperthyroidism has been associated with an increased risk of maternal, fetal, and neonatal complications. The available antithyroid drugs are methimazole/carbimazole and propylthiouracil. Several case reports and some epidemiologic studies suggest that methimazole/carbimazole exposure during the first trimester of pregnancy is associated with an increased risk of congenital malformations, including ectodermal anomalies, choanal atresia, esophageal atresia, and omphalocele. However, the absolute risk appears to be very small, and it remains unclear whether the association is driven by the maternal disease, the medication, or the combination of both factors. Propylthiouracil exposure has not been associated with an increased risk of congenital malformations and is the recommended drug during the first trimester of pregnancy. Since propylthiouracil-induced hepatotoxicity has been reported in approximately 0.1% of exposed adults and the number of case-reports of severe liver injury is increasing, treatment with low dose methimazole during the second and third trimesters should be considered. Until now, there has been no evidence that children prenatally exposed to methimazole/carbimazole or propylthiouracil have an increased risk of neurodevelopmental delay.

Long term respiratory outcomes of congenital diaphragmatic hernia, esophageal atresia, and cardiovascular anomalies.

Intrathoracic congenital malformations may be associated with long-term pulmonary morbidity. This certainly is the case for congenital diaphragmatic hernia, esophageal atresia and cardiac and aortic arch abnormalities. These conditions have variable degrees of impaired development of both the airways and lung vasculature, with a postnatal impact on lung function and bronchial reactivity. Pulmonary complications are themselves frequently associated to non-pulmonary morbidities, including gastrointestinal and orthopaedic complications. These are best recognized in a structured multidisciplinary follow-up clinic so that they can be actively managed.

In vitro analysis of esophageal atresia using a whole-embryo culture system.

BACKGROUND/PURPOSE: Administration of Adriamycin to pregnant rats leads to the development of esophageal atresia with tracheo-esophageal fistula. This defect arises from failure of the trachea to develop normally from the primitive foregut; instead,the upper foregut differentiates into trachea, then continues to the lower esophageal segment as a tracheo-esophageal fistula. Our aim was to explore the possibility of growing Adriamycin-exposed embryos using a whole-embryo culture technique and to determine whether or not esophageal atresia with tracheo-esophageal fistula could be prevented in an Adriamycin-treated rat model. METHODS: Rat embryos were exposed to Adriamycin in utero on days 6 - 9 of gestation, removed on day 10 and grown in vitro as described by New (11) for 48 hours using 100% serum from animals not exposed to Adriamycin. RESULTS: Thirty Adriamycin-exposed embryos were grown in vitro using normal serum. Histologic assessment of tracheo-esophageal development showed that 14 embryos had normal development, while 16 developed esophageal atresia. CONCLUSIONS: Growth of Adriamycin-exposed embryos was successful using "whole-embryo culture technique"; abnormal tracheo-esophageal development could in some cases be altered by removing the embryos at day 10 and exposing them to normal serum for 48 hours.

Congenital tracheoesophageal fistula: preventing recurrence.

Recurrent tracheoesophageal fistula is a major complication of surgical therapy among children with congenital esophageal atresia and tracheoesophageal fistula. In a consecutive series of 153 patients operated on during a 20-year period in the same institution, only one patient had this complication. The authors believe that adherence to sound surgical principles can lower the risk of fistula recurrence considerably.

Failure of short-term luminal IGF-I to protect against atrophy in a model of fetal esophageal atresia.

Short-term luminal infusion in utero (3 days) of insulin-like growth factor I (IGF-I) failed to protect the fetal small intestine against atrophy induced by ablation of swallowing. Human recombinant IGF-1 (or vehicle) was infused into the duodenum of fetal sheep at 125 days' gestation for 3 days (day 1, 0.025 mg; day 2, 0.25 mg: day 3, 2.5 mg). Fetal swallowing was prevented by esophageal ligation, and a carotid catheter was implanted for blood sampling. There were no changes in body growth of in major organ growth. Small intestinal (SI) weight (corrected for body weight) was significantly lower for IGF-I treated fetuses. Villus height decreased significantly in proximal regions. Villus enterocyte cellularity was reduced significantly in the proximal regions. The percentage of crypt cells labeled with a 4-hour pulse of tritiated thymidine (as assessed by autoradiography) decreased significantly in the proximal SI only, from 16.14% (1.06% SEM) to 13.28% (1.05% SEM) (P < .05). Plasma levels of IGF-1 increased in the treated fetuses by an average of 76%. IGF-1 immunoreactivity was detected in the apical endocytic complex of enterocytes from proximal SI. This study shows that wasting of fetal intestinal tissues in the absence of enteral input cannot be prevented by IGF-1 delivered luminally.