Longitudinal Follow-up of Chronic Pulmonary Manifestations in Esophageal Atresia: A Clinical Algorithm and Review of the Literature

Abstract

In the past decades improved surgical techniques and better neonatal supportive care have resulted in reduced mortality of patients with esophageal atresia (EA), with or without tracheoesophageal fistula, and in increased prevalence of long-term complications, especially respiratory manifestations. This integrative review describes the techniques currently used in the pediatric clinical practice for assessing EA-related respiratory disease. We also present a novel algorithm for the evaluation and surveillance of lung disease in EA. A total of 2813 articles were identified, of which 1451 duplicates were removed, and 1330 were excluded based on review of titles and abstracts. A total of 32 articles were assessed for eligibility. Six reviews were excluded, and 26 original studies were assessed. Lower respiratory tract infection seems frequent, especially in the first years of life. Chronic asthma, productive cough, and recurrent bronchitis are the most common respiratory complaints. Restrictive lung disease is generally reported to prevail over the obstructive or mixed patterns, and, overall, bronchial hyperresponsiveness can affect up to 78% of patients. At lung imaging, few studies detected bronchiectasis and irregular cross-sectional shape of the trachea, whereas diffuse bronchial thickening, consolidations, and pleural abnormalities were the main chest X-ray findings. Airway endoscopy is seldom included in the available studies, with tracheomalacia and tracheobronchial inflammation being described in a variable proportion of cases. A complete diagnostic approach to long-term respiratory complications after EA is mandatory. In the presence of moderate-to-severe airway disease, patients should undergo regular tertiary care follow-up with functional assessment and advanced chest imaging.

Recurrent Pneumonia in Children: A Reasoned Diagnostic Approach and a Single Centre Experience

“…The assessment of BAL pepsin has a higher specificity [55], but this test can only be performed in a few centers. A tube esophagram is the usual investigation performed if late-presenting congenital H-type fistula is suspected, but a normal test does not exclude this diagnosis, and direct visualization with bronchoscopy may be needed [36,56]. Sophisticated manometry should be considered if esophageal dysmotility defects are suspected.…”

How to Care for Patients with EA-TEF: The Known and the Unknown

“…EA patients are at high risk for this, given their high rates of lower respiratory tract infections in the first years of life, with more than 5 such infections reported in the 1 st year of life. [120]Increased bronchial responsiveness: This has been described in 22-65% of EA patients, and is thought to reflect damaged airway epithelium from recurrent acidic aspiration. [121]…”

“…EA patients are at high risk for this, given their high rates of lower respiratory tract infections in the first years of life, with more than 5 such infections reported in the 1 st year of life. [120]…”

Thoracoscopic Posterior Tracheopexie in a Newborn With Tracheomalacia and Esophageal Atresia

“…Ранняя диагностика трахеомаляции очень важна, поскольку эти дети обычно имеют признаки обструкции дыхательных путей, располагающие к развитию респираторных инфекций. Инфекции могут в конечном итоге привести к тяжелому повреждению легких у 27% пациентов [1,12,20,21]. В раннем грудном возрасте коллапс дыхательных путей может вызвать жизнеугрожающие состояния -очевидное опасное для жизни событие (от англ -apparent life-threatening event, ALTE) или краткие разрешенные необъяснимые события (от англ -brief, resolved, unexplained event, BRUE) [19].…”