Introduction: Infants with esophageal atresia and tracheoesophageal fistula (EA/TEF) are at increased risk for respiratory compromise and gastric perforation until fistula ligation. We sought to describe current practice regarding the timing of EA/TEF repair and hypothesized that age at repair is a predictor of adverse outcomes.
Methods: The Pediatric Health Information System (PHIS) database was used to identify patients with EA/TEF who underwent fistula ligation and esophago-esophagostomy at US children's hospitals from July 2016-June 2021. Patients with a repair >10 days of age, a long-gap atresia, or H-type fistula were excluded. Comorbidities including prematurity and operative congenital heart disease were noted. Outcomes including anastomotic leak, gastric perforation, and post-operative respiratory failure were assessed for association with age and day of the week of operation.
Results: Among 863 patients that were evaluated, the plurality of operations was on DOL 2 (36%) and 83% were on a weekday (random rate = 71%). Later operations had shorter LOS (p = 0.04) and more recurrent nerve injuries (p = 0.01). Weekend repairs were associated with equivalent outcomes. Gastric perforations occurred in 18 (2.0%) patients; 11 (61%) of these occurred after DOL 2.
Conclusions: We found no significant differences in outcomes other than more recurrent nerve injury and decreased LOS with EA/TEF repair at older ages. Although repair beyond DOL 2 was safe from a respiratory standpoint, most gastric perforations occurred after this point. In the absence of contraindications or significantly reduced weekend capabilities, we recommend repair of EA/TEF by DOL 2.
Level Of Evidence: III.
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http://dx.doi.org/10.1016/j.jpedsurg.2024.08.020 | DOI Listing |
J Community Genet
December 2024
EURORDIS - Rare Diseases Europe, Paris, France.
The European Reference Networks (ERNs) for rare and complex diseases offer significant potential for building, maintaining and evaluating patient partnership, for which the recently developed ERN Patient Partnership Framework may serve as guidance. This scoping review aims to identify and describe relevant frameworks published in scientific literature, capturing key learning points to inform future updates of the ERN Patient Partnership Framework and promote its use in practice. MEDLINE, Embase, and the Web of Science Core Collection were searched to identify recently published frameworks (2013-2023) focused on patient partnership and aligned with at least one core ERN activity.
View Article and Find Full Text PDFJ Indian Assoc Pediatr Surg
November 2024
Department of Pediatric Surgery, IMS-BHU, Varanasi, Uttar Pradesh, India.
Background: Elective ventilation and paralysis have been shown to decrease the anastomosis-related complications following primary repair of esophageal atresia (EA). Repeated endotracheal tube (ETT) block and replacement can increase these complications. We evaluated the results of our strategy of electively changing the ETT just before shifting the patient to the postoperative ward for elective ventilation.
View Article and Find Full Text PDFJ Infect Chemother
December 2024
Japanese Society for Inherited Metabolic Diseases.
Background: In March 2024, children with pulmonary hypoplasia, airway stenosis, congenital esophageal atresia, inborn errors of metabolism, and neuromuscular diseases became eligible for palivizumab in Japan. Despite limited epidemiological data, expert consensus guided the recommendation for palivizumab use in these children to ensure its proper application.
Objectives: This article outlines the proper use of palivizumab for RSV infection in infants and children with the specified conditions, aiming to enhance understanding of the medical basis for its clinical guidance.
Asian J Endosc Surg
December 2024
Department of Pediatric Surgery, Research Field in Medical and Health Sciences, Medical and Dental Area, Research and Education Assembly, Kagoshima University, Kagoshima, Japan.
We herein report a neonatal case showing specific findings of blood perfusion in the anastomosed esophagus of esophageal atresia (EA) and tracheoesophageal fistula (TEF) using indocyanine green (ICG) fluorescence during thoracoscopic surgery. The patient was a 3054 g, 0-day neonatal boy diagnosed with EA-TEF based on a coil-up sign of the nasogastric tube. Thoracoscopic surgery was performed on Day 4 after birth.
View Article and Find Full Text PDFBMJ Open
December 2024
Congenital Oesophageal and Airway Team Utrecht, Departments of Paediatric Surgery and Otorhinolaryngology and Head and Neck Surgery, Wilhelmina Children's Hospital University Medical Center, Utrecht, The Netherlands.
Introduction: Tracheomalacia (TM) often occurs in children with oesophageal atresia (OA), leading to recurrent respiratory symptoms and in severe cases to blue spells or ultimately respiratory arrest. In some patients, a secondary posterior tracheopexy may then be indicated. This secondary surgery, as well as respiratory morbidity, may be prevented by performing a primary posterior tracheopexy (PPT) concurrent with primary OA correction.
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