Esophageal morbidity in patients following repair of esophageal atresia: A systematic review

doi:10.1016/j.jpedsurg.2020.09.010

Journal of Pediatric Surgery

Volume 56, Issue 9, September 2021, Pages 1555-1563

https://doi.org/10.1016/j.jpedsurg.2020.09.010 Get rights and content

Abstract

Background

Esophageal atresia (EA) is a life-threatening congenital condition, affecting one in 2600 newborns. Morbidity remains high, with many patients experiencing complications, including anastomotic leak/stricture, and gastro-esophageal reflux disease (GERD). Increased understanding of esophageal motility patterns may help explain the etiology of these complications.

Aims

We aimed to review knowledge regarding esophageal motility and related complications in children with EA, evaluate patients' symptomatology and relate this to esophageal motility.

Methods

We performed a systematic review (PROSPERO: CRD42018092277), according to the PRISMA protocol. Two investigators independently conducted search strategies (OvidMEDLINE, PubMed, Cochrane Review, BMJ BestPractice), identifying complications in patients following EA repair. Rates of esophageal dysmotility, GERD, dysphagia, anastomotic leak, anastomotic stricture, recurrent fistula formation, and esophagitis were sought.

Results

A total of 65 publications met selection criteria (n = 4882). Rates of morbidity were high: esophageal dysmotility (78%), GERD (43%), dysphagia (44%), anastomotic leak (19%), anastomotic stricture (26%), recurrent fistula formation (7%), and esophagitis (47%). No correlation appeared to exist with severity of symptoms.

Conclusions

This systematic review identified high rates of complications in children with EA, with esophageal dysmotility present in the majority of patients. Increasing survival, with resultant longer timeframes to develop morbidities, makes standardized follow-up regimens crucial.

Type of study

Prognosis study.

Level of evidence

Level 3.

Section snippets

Protocol and registration

We performed a systematic review of the published literature, focusing upon the assessment of esophageal motility in patients with EA/TEF who had undergone a surgical repair. The review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2009 checklist [12]. The review protocol was registered in PROSPERO (registration number CRD42018092277).

Eligibility criteria

The inclusion criteria were:

•
Papers published between 2000 and 2020
•
Publication status of the selected

Study selection

Two hundred seventy-six papers were identified. (Fig. 1) A total of 65 studies satisfied the inclusion criteria and were suitable for analysis. (Fig. 1) Thirty-seven were prospective studies, 27 were retrospective and one was ambispective. Five of these were reviews, 46 were cross sectional studies, 10 were case controls and four were case studies. A total of 5399 patients (4882 EA/TEF, 517 controls) were included.

Study characteristics

Rates of primary and secondary outcomes were collected and analyzed for each

Discussion

Morbidity rates in children with EA/TEF are still high, with esophageal motility being one of the main pathophysiological causes. The current literature identifies different dysmotility patterns in children with EA/TEF. These patterns seem to have a causative effect to the development of further complications, such as GERD and aspiration. Etiology and causative effects of such dysmotility patterns and complications are not yet known. This systematic review identified the importance of further

Conclusion

In conclusion, this systematic review analyzed the most recent evidence regarding motility of the esophagus in patients post-EA/TEF repair. Dysmotility, gastro-esophageal reflux disease, dysphagia and respiratory pathology are among the most common sequelae of this condition and its surgical repair, which impacts on families' daily living. The etiology of these is not clear yet, but there is current need to develop standardized follow-up regimes to prevent the development of these complications

Acknowledgements

Associate Professor Sebastian King's and Associate Professor Warwick Teague's positions as Academic Pediatric Surgeons are generously supported by The Royal Children's Hospital Foundation.

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Anastomotic leakage (AL) and esophageal perforation are life-threatening complications following surgery or endoscopic dilations. “Replogle modified EVAC therapy” consists of placing a Replogle tube directly into the lumen or within an abscess cavity and remove by suction all intra-cavity fluids and secretion with a continuous low-pressure suction, promoting granulation tissue proliferation, thereby gradually decreasing the cavity size. The aim of our study was to evaluate the technical feasibility, safety, and efficacy of this technique in pediatric patients.
A retrospective review charts of consecutive pediatric patients that were treated with “Replogle modified EVAC therapy” at our pediatric referral center between 2013 and 2022 was conducted. The clinical, endoscopic, radiological, and surgical information and data of patients were collected and revised as well as their follow-up and outcomes.
Ten patients (6/10 male; mean age: 7.8 y.o., range: 1.1–18 y.o.) were treated using the “Replogle modified EVAC therapy”. Four out of ten patients developed esophageal perforations after endoscopic procedures. Six out of the ten enrolled patients had AL complications after surgical operations. All patients were successfully treated. There were no technical failures or complications with device placement. Mean treatment duration was 16 days (range 7–41 days). No additional treatment was needed for complete leak resolution.
“Replogle modified EVAC therapy” represents a promising and mini-invasive method to treat esophageal perforations and post-surgical leak in the paediatric age group. In our experience, the use of this technique was safe, effective, and particularly well suited also in complex paediatric patients.
IV.
Post-operative ventilation strategies after surgical repair in neonates with esophageal atresia: A retrospective cohort study
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Citation Excerpt :
We believe that our results are also related to the homogenous timing of post-operative extubation in our infants: if infants remained intubated and sedated for about 6–7 days, the anastomotic healing could be much more assured than after only 24–48 h from the surgical repair. Furthermore, by considering that functional gastrointestinal dysmotility is a common problem in preterm infants regardless of EA [20] and that the presence of an anomalous esophageal motility pattern has been hypothesized in EA/TEF infants postoperatively [1] we speculated that preterm infants with EA/TEF could have a more severe dysmotility pattern and this could play a key role in the pathogenesis of leak. Many of our infants received sedatives not only during mechanical ventilation but also during NIV, considering the biological plausibility that abrupt movements may hinder healing.
Infants affected by Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) may require non-invasive ventilation (NIV) in the post-operative period after elective extubation, especially if born preterm. The aim of the paper is to evaluate the role of different ventilation strategies on anastomotic complications, specifically on anastomotic leak (AL).
Retrospective single Institution study, including all consecutive neonates affected by EA with or without TEF in a 5-year period study (from 2014 to 2018). Only infants with a primary anastomosis were included in the study. All infants were mechanically ventilated after surgery and electively extubated after 6–7 days. The duration of invasive ventilation was decided on a case-by-case basis after surgery, based on the pre-operative esophageal gap and intraoperative findings. The need for non-invasive ventilation (NCPAP, NIPPV, and HHHFNC) after extubation and extubation failure with the need for mechanical ventilation in the post-operative period were assessed. The primary outcome evaluated was the rate of anastomotic leak.
102 EA/TEF infants were managed in the study period. Sixty-seven underwent primary anastomosis. Of these, 29 (43.3%) were born preterm. Patients who required ventilation (n = 32) had a significantly lower gestational age as well as birthweight (respectively p = 0.007 and p = 0.041). 4/67 patients had an AL after surgical repair, with no statistical differences among post-operative ventilation strategies.
We found no significant differences in the rate of anastomotic leak (AL) according to post-operative ventilation strategies in neonates operated on for EA/TEF.
Thoracoscopic repair for esophageal pulmonary fistula after esophageal atresia repair
2022, Journal of Pediatric Surgery
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Recurrent tracheoesophageal fistula (rTEF) is a rare complication after esophageal atresia/tracheoesophageal fistula (EA/TEF) repair, with an incidence of 2–24% [1,2].
Esophageal pulmonary fistula is a special type of acquired tracheoesophageal fistula that occurs after esophageal atresia/tracheoesophageal fistula repair. Thoracotomy is the surgical repair method currently in use, but postoperative outcomes are unclear. Therefore, we aimed to explore the preliminary safety, effectiveness, and feasibility of thoracoscopic surgical repair of esophageal pulmonary fistula.
We retrospectively collected data from all patients with esophageal atresia/tracheoesophageal fistula at Beijing Children's Hospital from January 2017 to October 2021, and the clinical characteristics of patients with esophageal pulmonary fistula were analyzed. Clinical information was recorded, and follow-up was performed.
Seven patients (five boys and two girls) were diagnosed as esophageal pulmonary fistula. All patients underwent multiple esophageal surgeries and had esophageal strictures before surgical repair. Clinical manifestations included cough, expectoration, and recurrent pneumonia. Esophagography indicated the location of the fistula with a 100% positive rate, while the positive rate of flexible bronchoscopy and chest computed tomography was 57% (4/7) and 43% (3/7), respectively. Surgical repair was achieved using thoracoscopy with an average operation time of 172 min. All patients developed esophageal strictures, four of which had refractory esophageal strictures and underwent esophageal dilations ranged from 5 to 56 times before this surgery, but anastomotic leakage or acquired esophageal pulmonary fistulas were absent post-surgery. After a median follow-up of 22 months, all patients survived, and the symptoms were well controlled.
Esophageal pulmonary fistula is a rare complication of atresia/tracheoesophageal fistula repair. Thoracoscopic surgery is still possible even after previous multiple surgeries in the chest with significant complications and satisfactory results can be achieved in the short term.
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Long term digestive outcome of œsophageal atresia
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Œsophageal motility dysfunction is more frequently distal, as this part of the œsophagus has been found to have low/absent œsophageal contraction. This distal dysfunction results in loss of continuity of the peristatic wave and slowing of œsophageal clearance [17]. Even if motility studies in these patients demonstrate significant altered œsophageal peristaltis and motility [18], several other causes can explain dysphagia (Fig. 2).
Œsophageal atresia is a rare neonatal malformation consisting in an interruption of the continuity of the œsophagus, with or without a tracheo-œsophageal fistula. Although mortality rate is now low and most cases can benefit from successful surgical repair soon after birth, morbidity –specially digestive and nutritional-remains high. Many of the adults born with œsophageal atresia will suffer from dysphagia, gastro-œsophageal reflux and/or œsophageal dysmotility, leading to nutritional consequences and quality of life impairment. Barrett's œsophagus, potential risk of œsophageal cancer as well as risk of anastomotic stenosis and eosinophilic œsophagitis justify transition to adulthood and a lifelong prolonged follow-up.
Minimally invasive surgical approach in children treated for oesophageal atresia is associated with attention problems at school age: a prospective cohort study
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Anastomotic leaks and the relationship with anastomotic strictures after esophageal atresia surgery; effects of patient characteristics
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View all citing articles on Scopus

^☆: Disclosures Funding: No funding was received to conduct this systematic review. Disclosures: The authors have no conflict of interests to declare. Authorship: All authors attest that they meet the current ICMJE criteria for Authorship.

View full text

Neonatal ConditionEsophageal morbidity in patients following repair of esophageal atresia: A systematic review☆

Abstract

Background

Aims

Methods

Results

Conclusions

Type of study

Level of evidence

Section snippets

Protocol and registration

Eligibility criteria

Study selection

Study characteristics

Discussion

Conclusion

Acknowledgements

J Pediatr Surg

Paediatr Respir Rev

J Pediatr Surg

Journal of Peditric Surgery

J Pediatr Surg

Semin Pediatr Surg

Journal of Peditric Surgery

Birth defects in Victoria 2005–2006. Victorian Government Department of Human Services

Midterm follow-up of esophageal anastomosis for esophageal atresia repair: long-gap versus non-long-gap

Dis Esophagus

Characterization of esophageal motility following esophageal atresia repair using high-resolution esophageal manometry

J Pediatr Gastroenterol Nutr

Long-term complications of congenital esophageal atresia, single institution experience

Indian Pediatr

Manometric evaluation of postoperative patients of esophageal atresia and tracheo-esophageal fistula

Evaluation of esophageal motility using multichannel intraluminal impedance in healthy children and children with gastroesophageal reflux

Primary repair in esophageal atresia. The results of long term follow-up

Minerva Pediatr

Radionuclide scintigraphy in the evaluation of gastro-oesophageal reflux in post-operative oesophageal atresia and tracheo-oesophageal fistula patients

Nucl Med Commun

Neonatal Condition
Esophageal morbidity in patients following repair of esophageal atresia: A systematic review☆