Abstract

BackgroundChildren with congenital esophageal atresia (EA) and tracheoesophageal fistula (TEF) have chronic respiratory symptoms including recurrent pneumonia, wheezing and persistent cough. The aim of this study is to describe the clinical findings of a large group of children with EA and TEF surgically corrected and the instrumental investigation to which they have undergone in order to better understand the patient’s needs and harmonize the care.MethodsA retrospective data collection was performed on 105 children with EA and TEF followed at Department of Pediatric Medicine of Bambino Gesù Children’s Hospital (Rome, Italy) between 2010 and 2015.Results69/105 (66%) children reported lower respiratory symptoms with a mean age onset of 2.2 ± 2.5 years and only 63/69 (91%) performed specialist assessment at Respiratory Unit. Recurrent pneumonia (33%) and wheezing (31%) were the most reported symptoms. The first respiratory evaluation was performed after surgically correction of gastroesophageal reflux (GER) at mean age of 3.9 ± 4.2 years. Twenty nine patients have undergone to chest CT with contrast enhancement detecting localized atelectasis (41%), residual tracheal diverticulum (34%), bronchiectasis (31%), tracheal vascular compression (21%), tracheomalacia (17%) and esophageal diverticulum (14%). Fifty three patients have undergone to airways endoscopy detecting tracheomalacia (66%), residual tracheal diverticulum (26%), recurrent tracheoesophageal fistula (19%) and vocal cord paralysis (11%).ConclusionsOur study confirms that respiratory symptoms often complicate EA and TEF; their persistence despite medical and surgical treatment of GER means that other etiological hypothesis must be examined and that a complete respiratory diagnostic work up must be considered.

Highlights

  • Children with congenital esophageal atresia (EA) and tracheoesophageal fistula (TEF) have chronic respiratory symptoms including recurrent pneumonia, wheezing and persistent cough

  • One hundred five Caucasian children with esophageal atresia and tracheoesophageal fistula followed at the Department of Pediatric Medicine of Bambino Gesù Children’s Hospital for a period of 6 years were included in the study

  • Data on mean gestational age and mean neonatal weight were unavailable respectively for 16 and 21 patients. 55/105 patients (52%) had associated malformations: heart disease and vascular anomalies were the most described defects and affected respectively 46/105 (43%) and 9/105 (9%) children. 20/105 children (19%) had syndromic picture and Vertebral defects (VACTERL) association was diagnosed in 17/105 patients (16%)

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Summary

Introduction

Children with congenital esophageal atresia (EA) and tracheoesophageal fistula (TEF) have chronic respiratory symptoms including recurrent pneumonia, wheezing and persistent cough. The aim of this study is to describe the clinical findings of a large group of children with EA and TEF surgically corrected and the instrumental investigation to which they have undergone in order to better understand the patient’s needs and harmonize the care. Children with congenital esophageal atresia (EA) and tracheoesophageal fistula (TEF) have chronic respiratory and digestive symptoms due to abnormal development of trachea and esophagus during intrauterine life. The main goals of treatment are timely recognition of the underlying malformation, reduction of morbidity and provision of the best possible quality of life for patients and their parents. Surgical treatment is the only therapeutic option and prognosis is good in 95 – 99% of children [2].

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