Esophageal atresia refers to a group of congenital anomalies in which the continuity of the esophagus is interrupted (1). Tracheoesophageal anomalies are divided into subtypes depending on anatomy and the most prominent (85%) subtype has a tracheoesophageal fistula to the distal esophageal segment (1). The prevalence of esophageal atresia varies according to country and time period (2–8). Observational studies from 1981 to 2018 have estimated the prevalence to span from 0.88 to 4.55 per 10,000 births in China and in Germany, respectively. Recent European studies suggest that the prevalence is relatively stable over time (9–11) and that males are most affected with a male:female ratio of 1:0.74 (9).

At birth, the neonate presents with typical drooling of saliva, inability to swallow, choking, coughing, cyanotic attacks, and distended abdomen if the subtype involves a fistula to the trachea (11). The diagnosis is confirmed by the inability to pass a feeding tube into the stomach (11, 12) and a plain X-ray showing the non-progression of the feeding tube located in a blind-ending pouch (11, 12). Prenatal diagnostics having improved from 26 to 36% during the last 30 years (9). Postnatal diagnosis occurs on the first day after birth in 83% of cases, the remaining 15% of cases within the first week, and only in 1.2% after the first week (9).

Most cases seem to occur sporadically, therefore the etiology is likely to be multifactorial involving multiple genes and complex gene-environment interactions (13, 14). Despite observational studies suggesting various maternal risk factors (10, 13, 15), the exact etiology is still unclear (13, 14). Since esophageal atresia is an early organogenesis defect, associated anomalies are frequency found (9, 16–19). Isolated esophageal atresia occurs in ~45–53% of the cases, whereas 32–47% have multiple anomalies, and 24–25% have an association or a syndrome, the most common being VACTERL association occurring in ~10% (9, 16–21). Among the most common associated anomalies are congenital heart defects (23–29% of cases), other gastrointestinal anomalies (16–21%), urinary tract anomalies (15–16%), and limb anomalies (13–14%) (9, 16–19).

Left untreated, the condition is fatal due to starvation, infection, and respiratory complications and survival therefore relies on early surgical correction (1, 11). The surgery aims to reconstruct the continuity of the esophagus and eliminate any possible fistulae (11, 14), which can be done either as a transpleural thorascopical procedure, or as open surgery, most commonly extrapleural (11, 22).

The mortality rate in isolated esophageal atresia range from 4.3 to 8.1% (7, 8, 17, 23–25), but varies with the type of atresia (with higher mortality and morbidity in the long gap esophageal atresia presentation), and mortality increases furthermore in case of prematurity and/or low birth weight, and with the presence of associated abnormalities, notably major cardiac and chromosomal anomalies (2, 6, 9, 26, 27). Even after hospital discharge, the children have increased mortality with post-discharge mortality is primarily due to respiratory compromise, including sudden infant death, aspiration, tracheomalacia, and reactive airway disease (23, 28, 29).

The most common postoperative complications are anastomosis leakage, fistula recurrence, anastomotic strictures, respiratory complications, and infections (25, 30–40). Anastomotic leakage is one of the most common serious complications occurring in about 5–17% of cases (25, 34, 35). Leakage into the mediastinum result mainly from anastomotic tension (particularly in cases with increased gap length) leading to ischemia of the esophageal ends, particularly in the small, friable lower segment and sub-optimal surgical technique; sepsis and even use of prosthetic materials can contribute (36–38). Major leaks are uncommon and tend to present with acute deterioration associated with pneumothorax and sepsis, and may require emergency decompression with placement of a postoperative chest tube (35, 39, 40). Most leaks heal spontaneously given proper drainage and antibiotics and only few require surgical intervention (35, 39, 40).

Long-term complications include strictures of the anastomotic region [incidence 25–75% (25, 34, 41, 42)], gastroesophageal reflux [incidence 22–63% (43)], esophagitis, tracheomalacia, feeding difficulties [incidence up to 80% (30, 44)], pulmonary symptoms, and developmental challenges (30–33, 45). These long-term complications have an impact on quality of life (28) in both patients and parents, especially in the case of tracheal and esophageal complications (6, 31, 46–50).

During the esophageal repair a prophylactic intraoperative chest tube (IOCT) has traditionally been placed close to the anastomosis to drain access fluid and air through a one-way system (51, 52). The routine use of prophylactic IOCTs is now debated (11, 39, 40, 52) and at the European Reference Network for rare Inherited and Congenital Anomalies (ERNICA) consensus conference, no consensus was found with only 21.4% of the members voting for the use of IOCTs (22). However, IOCTs are still used and reported as common as in 54% of the cases in the UK (53), 57% in Belgium (54), and 69% in an international survey, respectively (55).

IOCTs are not without drawback as they can cause insertion site infection (56) and when improperly placed, the tube can cause disruption of the site of anastomosis or penetration of proximal myotomy (57). IOCTs may also cause considerable postoperative pain, which would decrease inspiratory effort tand need for administration of more opoids, both leading to secondary effects such as atelectasis and pneumonia (58). Importantly, in some cases, IOCTs are insufficient to drain major leaks, necessitating the placement of a new chest tube (39, 40).

Whether the potential benefits of the prophylactic IOCT overweigh the potential harms is therefore unclear (22). Accordingly, the objective of this study was to examine the benefits and harms of prophylactic IOCT during primary surgical repair of esophageal atresia.

We conducted a systematic review of the existing literature according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines (PRISMA) and the Cochrane Handbook for Systematic Reviews of Intervention (59, 60). The predefined methodology, and method for this review in general, is described in our protocol, registered in June 2021 (61).

A systematic search done December 3rd, 2021, identified a total of 953 records from databases and registers. A total of 894 were excluded based on the title and abstract. We assessed 19 full-text original articles, of which following studies were included: three RCTs (64–66) and two case-control studies (51, 52) for narrative description in the discussion. See Figure 1: PRISMA flowchart and Table 1: Table of excluded studies regarding details on inclusion and exclusion of the studies.

We did not identify any studies advocating for the use of prophylactic IOCTs. Based on the limited amount of research on this topic and results from the included studies, we did not find sufficient evidence to support or discontinue the routine use of prophylactic IOCTs for neonates undergoing surgical repair of esophageal atresia, as all confidence intervals were compatible with no effect. Further trials, ideally multicentric, are warranted to explore the effects of the prophylactic IOCT for neonates undergoing surgical repair of esophageal atresia. Importantly, future trials should adhere to SPIRIT guidelines (84).

The original contributions presented in the study are included in the article/supplementary material, further inquiries can be directed to the corresponding author.

ML drafted the protocol and review, extracted data, co-ordinated the review, analyzed the data, and revised the review. SK drafted and revised the protocol, extracted data, analyzed the data, interpreted the data, commented on, revised the review, interpreted the data, provided supervision, and provided a methodological and statistical expertise. SH revised and commented on the protocol and review and provided supervision. JO and MF revised and commented on the protocol and review and provided clinical expertise. SP revised and commented on the review. UL-T drafted the protocol, conceived and designed the review, revised the protocol, commented on, revised the review, provided supervision, and clinical expertise. All authors contributed to the article and approved the submitted version.

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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