Pulmonary Problems in Children with Esophageal Atresia

Abstract

Most children with esophageal atresia (EA) have pulmonary problems. The causes include associated malformations of the central airways, tracheomalacia, consequences of intubation and mechanical ventilation, as well as prematurity. This leads to an increased frequency of pulmonary infections with bacterial involvement. Long-term consequences are bronchiectasis and other structural damage to the lungs, resulting in a restrictive ventilatory defect, reduced physical resilience, and increased mortality. To prevent these late pulmonary complications, early and consistent multidisciplinary follow-up care and treatment for children with EA are essential, with continuation into adulthood. Only a few large centers can maintain such structures and acquire the necessary expertise.

Keywords

oesophageal atresia/esophageal atresia - pulmonary complications - bronchiectasis - tracheomalacia - pulmonary destruction

Introduction

Esophageal atresia (EA) is primarily a condition requiring pediatric surgical treatment. However, it often becomes clear very early that the airways and lungs play a critical role in a childʼs subsequent development. To date, there is a lack of prospective controlled studies on airway problems in children with EA. This is certainly due to the absence of structured care and, in particular, follow-up. As long as 111 hospitals in Germany perform surgery on children with EA–29 of them only once every five years–this situation is unlikely to change. In some countries (France, Australia, Scandinavia), there are initiatives toward centralization, which has led to more available data. In the literature, reports on pulmonary sequelae after esophageal atresia are found primarily in the form of case reports or small case series. Nevertheless, an initiative by INoEA (International Network of Esophageal Atresia) successfully established a working group (Respiratory Complications Working Group), which has published recommendations for the detection and treatment of these problems [1].

Prevalence of Pulmonary Problems after EA surgery

Data from the French registry, including 1,287 children with EA, show a 12-month mortality rate of 7%. Nearly one-third of the children were hospitalized within the first year of life due to respiratory problems. Significant risk factors include: an initial hospital stay of more than 90 days, tube feeding, inhalation therapy at the time of first discharge, recurrent fistula, aortopexy, reflux, anti-reflux surgery, dilatations, and insufficient weight gain [2].

Most studies do not specifically assess respiratory symptoms. In a meta-analysis, case series that did assess respiratory symptoms reported chronic respiratory problems in up to one-third of children. However, these figures are difficult to compare and interpret. The main problems are chronic cough, recurrent respiratory infections, and chronic lung disease [3].

In a study conducted approximately ten years ago, 80 of 110 surviving adults who had undergone EA surgery were invited to participate, and 28 responded. Almost 80% had pulmonary problems, predominantly restrictive ventilatory disorders. In most cases, the lung disease had not been diagnosed before the study [4].

One difficulty in assessing pulmonary problems in adulthood is that predominantly the healthier patients survived [5]. This leads to data bias, especially considering that the main cause of mortality in children with EA beyond the first year of life has pulmonary causes. In some countries, structured care has led to a significant improvement in this regard.

Symptoms

Dry or barking cough is a typical symptom in children with EA. This is due to tracheomalacia, which also changes the resonance characteristics of the airways, in addition to altering respiratory mechanics. Even without excessive mucus production, children with EA very frequently have chronic cough that is difficult to suppress. This occurs independently of infections and is significantly more frequent than in their peers [6].

Chronic cough with secretions is a strong indicator of a relevant functional or anatomical problem in the central airways and/or peripheral lungs. The underlying cause must be identified to prevent further damage to the airways.

Recurrent (viral) respiratory infections are normal in early childhood. However, when such infections are regularly prolonged or complicated, this is also indicative of a relevant pulmonary problem.

Hilar “central” bilateral bronchopneumonia occurs in toddlers with a frequency of around 4% per year, usually in the context of viral infections, and are relatively harmless. In children with EA, pneumonias occur very frequently in the first years of life, almost always as complications during or after viral respiratory infections, and typically with localized infiltrates (middle lobe, basal unilateral or bilateral, among others). About half of EA children have had at least one pneumonia requiring hospitalization, and many have had three or more pneumonias in the first years of life [6], [7], [8].

Typical Complex Problems in Children with EA

Aspiration

Children aspirate with EA very frequently, usually recurrently and often in small amounts. Causes include anatomical or functional abnormalities in the larynx, esophagus, and trachea. Typical anatomical examples are unrecognized laryngeal clefts, fistulas (recurrent or unrecognized), and vocal cord paralysis.

Functional swallowing disorders without anatomical stenosis are very common and often remain undiagnosed for a long time. Symptoms can be misinterpreted by both pediatric surgeons and pulmonologists. For instance, liquids can become trapped due to interrupted or chaotic peristalsis of the esophagus. A typical case history: drinking carbonated soft drinks from a glass leads to coughing (= aspiration due to a large gulp and foaming), whereas drinking through a straw is easier (= smaller sips that pass more easily and foam less). Children with EA usually eat more slowly because peristalsis is not functioning normally or is uncoordinated and “chaotic” in the distal segment.

The accumulation of secretions (saliva), reflux, or regurgitation after food impaction can also lead to aspiration. Protective laryngospasm against aspiration does not always work well in EA and, in itself, can also be an additional problem [9]. Asthma-like symptoms can be mimicked by microaspiration, leading many EA patients to receive inappropriate and ineffective asthma therapy. This is particularly detrimental when an inhaled steroid with high local absorption (e.g., beclomethasone) is used, as this may cause fungal infection and further impair laryngeal function.

Growth and physical performance

Although physical growth and performance are not primarily pulmonary symptoms, there are interrelationships. Severe lung impairment reduces physical growth. Conversely, for example, poor growth due to insufficient nutritional intake can limit physical performance, and lung growth is also affected.

EA patients tend to be below the age-matched average in height percentiles. They exhibit lower physical activity than their peers. Across all age groups–and most pronounced in adolescents–the sports index (minutes/week) is significantly lower in children with EA than in healthy comparison groups [10].

Orthopedic symptoms

Adolescents and young adults after EA have a several-fold increased risk of scoliosis: 12% have scoliosis > 20°, and an additional 22% have mild scoliosis. In the cohort studies, most patients did not have additional vertebral malformations. Many exhibited reduced physical endurance. The risk of scoliosis increases with age [11].

Since these are usually relatively rigid scolioses that are not comparable to classic adolescent idiopathic scoliosis (AIS), it can be assumed that there is additional impairment of cardiopulmonary capacity.

Typical Clinical Diagnoses or Complications

Tracheomalacia

Most children with EA have tracheomalacia, regardless of what type of fistula(s) exists or existed. With an expiratory residual lumen of more than 50%, there are usually no symptoms apart from barking cough. When the expiratory residual lumen is below 10%, children often have significant episodes of dyspnea, especially during infections. These cyanotic spells can be highly dramatic. This is further exacerbated by a food bolus–when food gets stuck–and particularly if there are also cardiac malformations, especially vascular anomalies or a right-to-left shunt.

Tracheomalacia not only causes the typical barking cough but also recurrent lower respiratory tract infections. Auscultatory findings with “wheezing” often lead to inappropriate treatment with beta-mimetics. In cases of severe tracheomalacia, approximately 50% of children show aspiration during contrast examination [7].

In pronounced tracheomalacia, symptoms often persist, so many surviving adults continue to have typical respiratory problems [8].

In addition, vocal cord disorders are very common (up to 30%), often as a result of (long-term) ventilation and/or surgical interventions, or due to reflux.

Impaired airway clearance

Tracheal narrowing causes not only mechanical problems. Due to chronic inflammation and mucosal abnormalities in the fistula region, there is a loss of cilia in the central airways [1]. This results in mucus retention with secondary infection and airway inflammation causing bacterial bronchitis. The long-term consequences are bronchiectasis and destruction of lung structure. These problems are particularly significant in young children but persist into adulthood for many patients [8]. Causes include the malformation itself, acquired surgical injury, inadequate infection management in childhood, comorbidities, recurrent lower respiratory tract infections, atopy, and smoking.

Bronchiectasis

Extremely few newborns are born with bronchiectasis. Bronchiectasis is a late symptom and, in most cases, a consequence of respiratory infections. It is most common in cystic fibrosis and ciliary dysfunction disorders and is not entirely avoidable in these conditions even with structured therapy.

Recurrent pneumonias in early childhood are an important risk factor for the development of non-CF bronchiectasis, regardless of the underlying disease or malformation. Consequences include progressive loss of lung function, frequent pulmonary exacerbations, reduced quality of life, and death in early adulthood [8], [12]. Immotile cilia (primary or secondary, as in EA) promote the development of bronchiectasis.

In children with EA (6 months to 12 years), bronchiectasis is found in 30% [13], often combined with atelectasis, e.g., in the right middle lobe. Only a few larger CT studies exist in EA children. In one series, in children with a mean age of 7.4 years, bronchiectasis was found in 31%. Parallel bronchoscopy showed no signs of this [14]. In 14%, tracheal diverticula were also present–mostly in children referred from smaller centers.

Bronchiectasis is so common that there is an explicit recommendation to exclude it via CT in adults after EA with chronic cough [9].

Associated malformations

Many EA patients have other associated malformations, most frequently cardiac defects or anomalies of the central vessels. EA can occur in various syndromes and chromosomal abnormalities such as trisomy 21.

The most well-known is the VACTERL association, much more rarely the CHARGE association.

Diagnostics

Children with EA are entitled to qualified airway diagnostics. Already during the initial hospital stay, it should be determined whether there is an associated laryngeal malformation, most often a dorsal cleft. This is not uncommon and is very often missed initially [9]. Furthermore, the extent of tracheomalacia should be known. It is also important to detect atypical bronchial branching patterns (e.g., of the right upper lobe bronchus), additional narrowing caused by aberrant major vessels/cardiac malformations, and other anomalies before pulmonary complications develop. Flexible bronchoscopy under sedation with spontaneous breathing, e.g., during induction of anesthesia for surgery, is suitable for this purpose. If a fistula is suspected, combined bronchoscopy and esophagoscopy should be performed.

Even beyond the neonatal period, there are indications for–if necessary invasive–evaluation, e.g., chronic (wet) cough or repeated pneumonia episodes.

Bronchoalveolar lavage (BAL) may be useful to rule out chronic aspiration.

A low-dose chest CT or, alternatively, lung MRI (as per cystic fibrosis standards) performed during a symptom-free interval after pneumonia is suitable for detecting long-term damage. A normal chest X-ray does not rule out bronchiectasis. Annual routine chest X-rays are not recommended [1], and certainly not annual CT scans.

Another method not yet generally available is Real-Time MRI. This can be used to document the interaction of the larynx, swallowing, and breathing.

Blood count and CRP are not suitable parameters for ruling out bacterial superinfection in the context of pulmonary symptoms [1]. This may delay necessary antibiotic therapy, with corresponding consequences.

Pulmonary function testing is of limited value in diagnosing bronchomalacia (misinterpretation as asthmatic obstruction). The reduced lung function in children with EA has long been known and was previously, at least in part, justifiably attributed to prematurity, intensive care, and ventilation [15].

However, the problem persists even with improved neonatal care. In a follow-up study of adolescents with EA, 63% had abnormal lung function [16]. The length of the EA gap correlates with the degree of restriction.

Registry data from Sweden show that lung function declines with age: mean forced expiratory volume in one second (FEV1) is 82% at age 8 and 76% at age 15. Many patients exhibit “obstruction,” restriction, or a combination of both [17]. In adults, vital capacity is significantly reduced at 74% compared to 104% in control groups. It should be noted that these data include only patients with fewer complications, since they survived. Despite these limitations, regular monitoring of pulmonary function parameters is important.

Therapy

In very severely ill infants, non-invasive ventilation with PEEP may be useful in the initial phase, and a small number of children benefit from a tracheostomy, possibly with a long cannula. These are usually EA children with multiple severe associated malformations.

In cases of very severe tracheomalacia with frequent cyanotic episodes and recurrent aspirations, aortopexy (with intraoperative bronchoscopic monitoring) can be helpful [1]. A characteristic clinical sign is frequent hyperextension, which children instinctively adopt to “stretch” the trachea. Highly successful and prophylactically effective is the technique of posterior tracheopexy, which can be performed in the context of minimally invasive primary surgery. This technique is not yet widely available–another argument for centralized care in a small number of highly specialized centers.

Recommendations for anti-reflux therapy are based only on “expert opinion” [1]; good clinical studies are lacking. Bronchodilators such as salbutamol should generally be avoided, as they may worsen bronchial collapse [1]. Mucolytics have a very limited effect. Inhaled steroids are only useful in exceptional cases (see above) and can lead to infectious complications.

It is crucial to detect and treat bacterial or bacterial/viral mixed infections at an early stage. In principle, every pneumonia must be prevented to avoid the development of bronchiectasis. A low threshold for prescribing antibiotics is explicitly recommended [1]. Early and consistent treatment of infections with beginning superinfection takes priority over acute diagnostics, as neither blood count, CRP, nor current chest X-rays are reliable decision-making tools [14]. The bacterial spectrum must be taken into account: staphylococci are the second most common pathogen in non-CF bronchiectasis. Therefore, an antibiotic should be chosen that reliably covers the triad of Haemophilus, S. pneumoniae, and S. aureus. Resistance development is very rare. Resistant pathogens (particularly MRSA) are practically only seen after long-term inpatient treatment of the EA patient or close contact to people harboring MRSA.

To enable timely therapy, a practical and responsible approach is to prescribe parents two antibiotic courses of 7 – 10 days each, to be used as needed. Parents are usually well able to decide when therapy is appropriate. Emergency antibiotic prescriptions outside regular office hours, upon parental request with reference to EA, work neither with general practitioners or pediatrician nor in emergency departments, and when given, often involve less suitable substances. Long-term antibiotic prophylaxis–e.g., during the infection season–should be reserved for exceptional cases.

Adults with EA differ only slightly from the general population with respect to the pulmonary microbiome [5], though the available numbers are small and include only relatively uncomplicated EA cases.

Based on a meta-analysis of available studies on pulmonary outcomes, an algorithm for management has been proposed, which is roughly consistent with the approach outlined here [18].

Airway clearance techniques, including respiratory physiotherapy and, if necessary, autogenic drainage, are helpful in many children [7], [9]. This can be learned in physiotherapy practices with cystic fibrosis experience.

Summary

The available data clearly demonstrate the urgent need to break the vicious cycle of early pulmonary complications and progressive, permanent destruction of lung structure [14]. It is crucial to identify children with pulmonary problems in EA in a timely manner, something that does not occur in many surgical centers. Approximately half of children with EA experience such pulmonary problems. Therefore, all children must undergo regular evaluation. In some cases, problems arise only during adolescence.

The risk factors for a problematic course are known. Children who receive primary care in smaller hospitals with low numbers of EA surgery have a higher risk of late pulmonary complications [14]. All available data support the need for multidisciplinary follow-up care, involving at least (pediatric) surgery, (pediatric) pulmonology, (pediatric) gastroenterology, orthopedics, and other specialties as required [1], [7]. Particularly in the first three years of life, short-interval monitoring every 3 – 6 months is essential [14].

This leads to the following recommendations:

• Pulmonology follow-up care from the neonatal stage should be actively recommended by the treating pediatricians.

• Regular follow-up by pediatric pulmonologists with specific expertise in bronchopulmonary malformations and in recognizing and treating bronchiectasis. If necessary, parents should be advised to seek a second opinion in a certified center.

• A transition concept aimed at continued pulmonary care in adulthood. Here too, the pediatrician can raise awareness of this need at an early stage.

Only by implementing these recommendations lung damage in EA patients can be avoided–damage that diminishes quality of life and ultimately shortens life expectancy.

Conflict of Interest

The authors declare that they have no conflict of interest.

• Literatur/References

• 1 Koumbourlis AC, Belessis Y, Cataletto M. et al. Care recommendations for the respiratory complications of esophageal atresia-tracheoesophageal fistula. Pediatr Pulmonol 2020; 55: 2713-2729
• 2 Lejeune S, Sfeir R, Rousseau V. et al. Esophageal atresia and respiratory morbidity. Pediatrics 2021; 148: e2020049778
• 3 Brooks G, Gazzaneo M, Bertozzi M. et al. Systematic review of long term follow-up and transitional care in adolescents and adults with esophageal atresia – why is transitional care mandatory?. Eur J Pediatr 2023; 182: 2057-2066
• 4 Gatzinsky V, Wennergren G, Jönsson L. et al. Impaired peripheral airway function in adults following repair of esophageal atresia. J Pediatr Surg 2014; 49: 1347-1352
• 5 Arneitz C, Windhaber J, Castellani C. et al. Cardiorespiratory performance capacity and airway microbiome in patients following primary repair of esophageal atresia. Pediatr Res 2021; 90: 66-73
• 6 Pedersen RN, Markøw S, Kruse-Andersen S. et al. Long-term pulmonary function in esophageal atresia – a case-control study. Pediatr Pulmonol 2017; 52: 98-106
• 7 DeBoer E, Prager JD, Ruiz AG. et al. Multidisciplinary care of children with repaired esophageal atresia and tracheoesophageal fistula. Pediatr Pulmonol 2016; 51: 576-581
• 8 Patria MF, Ghislanzoni S, Macchini F. et al. Respiratory morbidity in children with repaired congenital esophageal atresia with or without tracheoesophageal fistula. Int J Environ Res Public Health 2017; 14: 1136
• 9 Krishnan U, Dumont MW, Slater H. et al. The International Network on Oesophageal Atresia (INoEA) consensus guidelines on the transition of patients with oesophageal atresia – tracheoesophageal fistula. Nat Rev Gastroenterol Hepatol 2023; 20: 735-755
• 10 König TT, Frankenbach ML, Gianicolo E. et al. Habitual physical activity in patients born with oesophageal atresia: a multicenter cross-sectional study and comparison to a healthy reference cohort matched for gender and age. Eur J Pediatr 2023; 182: 2655-2663
• 11 Møinichen UI, Mikkelsen A, Gunderson R. et al. New insights in the prevalence of scoliosis and musculoskeletal asymmetries in adolescents with esophageal atresia. J Pediatr Surg 2023; 58: 412-419
• 12 Patria MF, Longhi B, Lelii M. et al. Children with recurrent pneumonia and non-cystic fibrosis bronchiectasis. Ital J Pediatr 2016; 42: 13
• 13 Tuğcu GD, Soyer T, Polat SE. et al. Evaluation of pulmonary complications and affecting factors in children for repaired esophageal atresia and tracheoesophageal fistula. Respir Med 2021; 181: 106376
• 14 Porcaro F, Valfré L, Aufiero LR. et al. Respiratory problems in children with esophageal atresia and tracheoesophageal fistula. Ital J Pediatr 2017; 43: 77
• 15 Beucher J, Wagnon J, Daniel V. et al. Long-Term evaluation of respiratory status after esophageal atresia repair. Pediatr Pulmonol 2013; 48: 188-194
• 16 Dittrich R, Stock P, Rothe K. et al. Pulmonary outcome of esophageal atresia patients and its potential causes in early childhood. J Pediatr Surg 2017; 52: 1255-1259
• 17 Donoso F, Hedenström H, Malinovschi A. et al. Pulmonary function in children and adolescents after esophageal atresia repair. Pediatr Pulmonol 2020; 55: 206-213
• 18 Mirra V, Maglione M, Di Micco LL. et al. Longitudinal follow-up of chronic pulmonary manifestations in esophageal atresia: a clinical algorithm and review of the literature. Pediatr Neonatol 2017; 58: 8-15

Publication History

Article published online:
10 October 2025