Respiratory Morbidity in Children with Repaired Congenital Esophageal Atresia with or without Tracheoesophageal Fistula

Abstract

Congenital esophageal atresia with or without tracheoesophageal fistula (CEA ± TEF) is a relatively common malformation that occurs in 1 of 2500–4500 live births. Despite the refinement of surgical techniques, a considerable proportion of children experience short- and long-term respiratory complications, which can significantly affect their health through adulthood. This review focuses on the underlying mechanisms and clinical presentation of respiratory morbidity in children with repaired CEA ± TEF. The reasons for the short-term pulmonary impairments are multifactorial and related to the surgical complications, such as anastomotic leaks, stenosis, and recurrence of fistula. Long-term respiratory morbidity is grouped into four categories according to the body section or function mainly involved: upper respiratory tract, lower respiratory tract, gastrointestinal tract, and aspiration and dysphagia. The reasons for the persistence of respiratory morbidity to adulthood are not univocal. The malformation itself, the acquired damage after the surgical repair, various co-morbidities, and the recurrence of lower respiratory tract infections at an early age can contribute to pulmonary impairment. Nevertheless, other conditions, including smoking habits and, in particular, atopy can play a role in the recurrence of infections. In conclusion, our manuscript shows that most children born with CEA ± TEF survive into adulthood, but many comorbidities, mainly esophageal and respiratory issues, may persist. The pulmonary impairment involves many underlying mechanisms, which begin in the first years of life. Therefore, early detection and management of pulmonary morbidity may be important to prevent impairment in pulmonary function and serious long-term complications. To obtain a successful outcome, it is fundamental to ensure a standardized follow-up that must continue until adulthood.

Care recommendations for the respiratory complications of esophageal atresia‐tracheoesophageal fistula

“…or "brassy") is the trademark symptom of TM especially in infancy and childhood; this cough tends to decrease somewhat with age. 8,18,38 A chronic wet cough (often described as "smoker's cough") may develop in early childhood and can remain across the lifespan. 39,40 3.…”

Pneumonia after Repair of Esophageal Atresia–Incidence and Main Risk Factors

“…In some patients, repair of EA is complicated by manifest or insidious vocal cord paralysis or dysfunction which further increases the risk of aspiration and poor airway clearance. 10 In a newborn with EA tracheomalacia may pose significant respiratory problems and even lifethreatening spells and surgical measures to eliminate tracheal collapse are needed. In the present study, the occurrence of tracheomalacia did not increase susceptibility to pneumonia.…”

Comorbidities and course of lung function in patients with congenital esophageal atresia

“…Therefore, respiratory tract abnormalities and early complications conditioned the course of lung function in these patients, which frequently led to abnormal spirometry patterns. 4 Some mechanisms that accounted for such variability in lung function patterns were rib cage deformities secondary to surgery, bronchial hyperresponsiveness, persistent inflammation, and tracheomalacia. 6 In our cohort of patients, the restrictive pattern was more common than the obstructive and mixed patterns.…”

“…Likewise, it is usually present in trisomy 18 (Edwards syndrome) and 21 (Down syndrome), Di George syndrome, and Pierre-Robin syndrome. 4 Follow-up of these patients is clinical but also functional, based on complementary tests, including forced spirometry. Respiratory complications occur in the short term, as determined by surgery itself, and in the long term, due to respiratory tract and gastrointestinal involvement, which facilitates aspirations or dysphagia.…”