Search results for "ESOPHAGEAL ATRESIA"

Gastroesophageal reflux in young children treated for esophageal atresia: evaluation with pH-multichannel intraluminal impedance

2011

Objectives: Gastroesophageal reflux (GER) and dismotility occur frequently after repair of esophageal atresia (EA). GER-associated complications can manifest either early or later; then precocious diagnosis and treatment are essential. The aim of the study was to evaluate characteristics of GER and esophageal clearance in children treated for EA with distal tracheoesophageal fistula, using pH-multichannel intraluminal impedance (pH-MII). Patients and Methods: Twenty-two children (ages 3‐40 months) treated for EA at birth, and 20 normal children of similar age with suspected GER disease were included in the study. Impedance parameters were analyzed according to age and symptoms. Results: Ref…

Type IV Laryngotracheoesophageal Cleft Associated with Type III Esophageal Atresia in 1p36 Deletions Containing the RERE Gene: Is There a Causal Role…

2018

The causes of embryological developmental anomalies leading to laryngotracheoesophageal clefts (LTECs) are not known, but are proposed to be multifactorial, including genetic and environmental factors. Haploinsufficiency of the RERE gene might contribute to different phenotypes seen in individuals with 1p36 deletions. We describe a neonate of an obese mother, diagnosed with type IV LTEC and type III esophageal atresia (EA), in which a 1p36 deletion including the RERE gene was detected. On the second day of life, a right thoracotomy and extrapleural esophagus atresia repair were attempted. One week later, a right cervical approach was performed to separate the cervical esophagus from the tra…

Novel Device for Endoluminal Esophageal Atresia Repair: First-in-Human Experience

2021

Thoracoscopic esophageal atresia (EA) repair affords many benefits to the patient; however, intracorporeal suturing of the anastomosis is technically challenging. Esophageal magnetic compression anastomosis (EMCA) is a compelling option for endoluminal EA repair, but available EMCA devices have prohibitive rates of recalcitrant stricture. Connect-EA is a new endoluminal EMCA device system that employs 2 magnetic anchors with a unique mating geometry designed to reliably create a robust anastomosis and decrease rates of leak and stricture. We describe our first-in-human experience with this novel endoluminal device for staged EA repair in 3 patients (Gross type A, B, and C) at high risk for …

Genetic syndrome suspicion: examples of clinical approach in the neonatal unit.

2010

Overgrowth syndromes: the practical clinical approach. Excessive growth can be present in a variety of medical conditions as result of abnormal fetal metabolism (i.e., maternal gestational diabetes) or of an overgrowth syndrome. Within this latter group of diseases, a LGA newborn requires a complex differential diagnosis encompassing several syndromes, such as Beckwith-Wiedemman, Sotos, Weaver, Simpson-Golabi-Behmel, Perlman, and Bannayan-Riley-Ruvalcaba. Partial or global overgrowth, other dysmorphisms, abdominal organs anomalies, as well as benign and malignant tumors are the common issues to examine for the diagnosis and the monitoring of all these disorders. The molecular bases of these…

Second study on the recurrence risk of isolated esophageal atresia with or without trachea-esophageal fistula among first-degree relatives: no eviden…

2013

BACKGROUND Esophageal atresia with/without trachea-esophageal fistula (EA/TEF) denotes a spectrum of severe congenital malformations. The aim of this systematic study was to determine both the recurrence risk for EA/TEF, and the risk for malformations of the VATER/VACTERL association spectrum, in first-degree relatives of patients with isolated EA/TEF. METHODS A total of 108 unrelated patients with isolated EA/TEF were included. These individuals had 410 first-degree relatives including 194 siblings. The presence of EA/TEF and malformations of the VATER/VACTERL association spectrum in relatives was systematically assessed. Data from the EUROCAT network were used for comparison. RESULTS None…

Dilated azygos arch mimicking an aortic arch anomaly during thoracic surgery

2017

Cardiovascular malformations are frequently associated in patients with esophageal atresia (EA). We observed azygos continuation mimicking an aortic arch anomaly in four newborns with type III EA. They presented concomitant rib anomalies indicating a common developmental defect. Foreknowledge is important for planning thoracotomy or interventional cardiac catheterization in this population.

An experimental study on magnetic esophageal compression anastomosis in piglets.

2019

Abstract Introduction Fashioning a patent, watertight anastomosis in patients with esophageal atresia is a challenging task in pediatric surgery, particularly when performed under tension. A reproducible suture-less alternative would decrease operative time. We evaluated magnetic esophageal compression anastomoses in a novel bypass-loop swine model. Methods Eight-week-old piglets underwent thoracotomy to mobilize the esophagus at the carina to create a U-shaped loop. Custom-made 8 mm diameter Neodymium Magnets were inserted into the esophagus proximal and distal to the loop, then mated side-to-side at the future anastomosis site. Pigs were observed for 8 (n = 4), 10 (n = 6), and 12 (n = 2) …

Evaluation of esophageal motility using multichannel intraluminal impedance in healthy children and children with gastroesophageal reflux: comments

2011

Oesophageal pH/impedance monitoring in children treated for oesophageal atresia

2008

Quality of Life after Surgical Treatment for Esophageal Atresia: Long-Term Outcome of 154 Patients.

2017

Background The short- and long-term surgical results in patients with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) have been described in depth from a physician's perspective. Contrarily, the perception and coping strategies of affected patients and their parents have rarely been reported. The aim of this study was to generate data on this matter. Patients and Methods A total of 154 patients who had operative reconstruction for EA between 1971 and 2012 were evaluated for demographic data, surgical technique, affection of daily life, and coping strategies. Results Gastroesophageal reflux (GER) symptoms were reported in 59% of cases with 33% requiring fundoplicatio…