Long-term Complications of Congenital Esophageal Atresia and/or Tracheoesophageal Fistula

doi:10.1378/chest.126.3.915

Chest

Volume 126, Issue 3, September 2004, Pages 915-925

https://doi.org/10.1378/chest.126.3.915 Get rights and content

Congenital esophageal atresia (EA) and/or tracheoesophageal fistula (TEF) are common congenital anomalies. Respiratory and GI complications occur frequently, and may persist lifelong. Late complications of EA/TEF include tracheomalacia, a recurrence of the TEF, esophageal stricture, and gastroesophageal reflux. These complications may lead to a brassy or honking-type cough, dysphagia, recurrent pneumonia, obstructive and restrictive ventilatory defects, and airway hyperreactivity. Aspiration should be excluded in children and adults with a history of EA/TEF who present with respiratory symptoms and/or recurrent lower respiratory infections, to prevent chronic pulmonary disease.

Section snippets

Definition and Background

EA is defined as a complete interruption in the continuity of the esophageal lumen.² TEF may be defined as a congenital, fistulous connection between the proximal and/or distal esophagus, and the airway.³ While EA and TEF may exist as separate congenital anomalies, the great majority of patients with these congenital malformations have both EA and TEF. As EA, TEF, and EA/TEF generally have similar associations with other anomalies and complications, they will be considered together in this

Embryology

The median pharyngeal groove develops in the ventral aspect of foregut at day 22 of gestation. This tissue develops into the respiratory and digestive tubes. Normally, mesenchyme proliferating between the respiratory and digestive tubes separates the tubes. While several theories have attempted to explain the etiology of EA/TEF, it is currently believed that the development of an abnormal epithelial-lined connection between the two tubes results in the creation of a TEF.¹ The excess tissue

Classification

There are five types of congenital EA/TEF (Fig 1). Two similar classification systems, the Gross and the Vogt classification systems, are in use.¹⁷¹⁸ Gross type C (Vogt type 3B) EA/TEF, which consists of distal TEF with proximal EA, is the most common type, comprising approximately 88.5% of cases. Gross type A, or isolated EA, occurs in approximately 8% of cases. Gross type E EA/TEF, consisting of TEF without EA, or H-type TEF, occurs in approximately 4% of cases, with the remainder consisting

Surgical Management

Most infants with EA/TEF undergo repair in early infancy, with division of the TEF and primary esophageal anastomosis. Patients with EA and wide separation of the esophageal ends continue to represent a major surgical challenge. Lengthening procedures, with or without esophagomyotomy, may be used to allow esophageal anastomosis. Neonatal esophageal stretching has been reported to allow primary anastomosis of the esophagus even in infants with long-gap EA/TEF.¹⁹ In more severe cases, anastomosis

Growth

GI symptoms are common in children with a history of EA/TEF.²³ Weight and height percentiles of children with EA/TEF may be reduced compared to sibling pairs, although some studies²³²⁴ have reported normal growth.

Anastomotic Leak

Anastomotic leak is an uncommon early complication of surgical repair, occurring in up to 17% of patients. However, it has potentially significant long-term consequences.¹ While 95% resolve spontaneously or with pleural drainage, esophageal stricture follow in 50% of cases. They can be

Respiratory Complications of EA/TEF

Respiratory symptoms are common in patients with repaired EA/TEF. In one series,¹⁶ 46% of patients with EA/TEF (99% of whom had a type C EA/TEF) had respiratory complications, with 19% of patients having recurrent pneumonia, 10% having aspiration, and 13% had choking, gagging, or cyanosis, with feeds. Respiratory complications were due to GERD in 74% of cases, tracheomalacia in 13%, recurrent TEF in 13%, and esophageal stricture in 10%, although many patients had multiple causes.¹⁶ In another

Summary

Serious respiratory and GI complications, such as recurrent pneumonia, obstructive airway disease, airway hyperreactivity, GERD, and esophageal stenosis, are frequent in patients with a history of EA/TEF, although the frequency of such events appears to decrease quite significantly with age.²⁵²⁷²⁸³⁶³⁹⁶⁰ Respiratory and GI sequelae in patients with a history of EA/TEF result from a complex interplay of numerous potential complications, and some complications can exacerbate others. GERD, for

References (73)

SR Choudhury et al.
Survival of patients with esophageal atresia: influence of birth weight, cardiac anomaly, and late respiratory complications
J Pediatr Surg
(1999)
N Usui et al.
Anomalies of the tracheobronchial tree in patients with esophageal atresia
J Pediatr Surg
(1996)
Y Nakazato et al.
Abnormal Auerbach plexus in the esophagus and stomach of patients with esophageal atresia and tracheoesophageal fistula
J Pediatr Surg
(1986)
HK Dutta et al.
A histopathological study of esophageal atresia and tracheoesophageal fistula
J Pediatr Surg
(2000)
H Kawahara et al.
Clinical characteristics of congenital esophageal stenosis distal to associated esophageal atresia
Surgery
(2001)
FJ McLaughlin et al.
Tracheal bronchus: association with respiratory morbidity in childhood
J Pediatr
(1985)
PW Wales et al.
Horseshoe lung in association with other foregut anomalies: what is the significance?
J Pediatr Surg
(2002)
RY Huang et al.
Structural airway anomalies in patients with DiGeorge syndrome: a current review
Am J Otolaryngol
(2000)
I Karnak et al.
The diagnosis and treatment of H-type tracheoesophageal fistula
J Pediatr Surg
(1997)
D Azoulay et al.
Congenital respiratory-esophageal fistula in the adult
J Thorac Cardiovasc Surg
(1992)

MS Srikanth et al.

Communicating bronchopulmonary foregut malformations: classification and embryogenesis

J Pediatr Surg

(1992)

E Somppi et al.

Outcome of patients operated on for esophageal atresia: 30 years’ experience

J Pediatr Surg

(1998)

PN LeSouëf et al.

Etiologic factors in long-term respiratory function abnormalities following esophageal atresia repair

J Pediatr Surg

(1987)

C Romeo et al.

Gastric motility disorders in patients operated on for esophageal atresia and tracheoesophageal fistula: long-term evaluation

J Pediatr Surg

(2000)

DN Homnick

H-type tracheoesophageal fistula and congenital esophageal stenosis

Chest

(1993)

H Lindahl et al.

Colon interposition or gastric tube? Follow-up study of colon-esophagus and gastric tube-esophagus patients

J Pediatr Surg

(1983)

L Spitz et al.

Esophageal atresia: five year experience with 148 cases

J Pediatr Surg

(1987)

R Carachi et al.

Esophageal anastomosis: an experimental model to study the anastomotic lumen and the influence of transanastomotic tube

J Pediatr Surg

(1984)

S Chittmittrapap et al.

Anastomotic stricture following repair of esophageal atresia

J Pediatr Surg

(1990)

LM Hicks et al.

Esophageal atresia and tracheoesophageal fistula: review of thirteen years’ experience

J Thorac Cardiovasc Surg

(1981)

NA Myers et al.

Secondary esophageal surgery following repair of esophageal atresia with distal tracheoesophageal fistula

J Pediatr Surg

(1990)

SG Jolley et al.

Patterns of gastroesophageal reflux in children following repair of esophageal atresia and distal tracheoesophageal fistula

J Pediatr Surg

(1980)

DT Rideout et al.

The absence of clinically significant tracheomalacia in patients having esophageal atresia without tracheoesophageal fistula

J Pediatr Surg

(1991)

MA Schwartz et al.

Tracheal compression as a cause of apnea following repair of tracheoesophageal fistula: treatment by aortopexy

J Pediatr Surg

(1980)

RM Filler et al.

Severe tracheomalacia associated with esophageal atresia: results of surgical treatment

J Pediatr Surg

(1992)

RM Filler et al.

Tracheobronchial stenting for the treatment of airway obstruction

J Pediatr Surg

(1998)

SH Ein et al.

Recurrent Tracheoesophageal fistulas: seventeen-year review

J Pediatr Surg

(1983)

A Vos et al.

Congenital tracheoesophageal fistula: preventing recurrence

J Pediatr Surg

(1996)

DWA Milligan et al.

Lung function in children following repair of tracheoesophageal fistula

J Pediatr

(1979)

P Chetcuti et al.

Chest wall deformity in patients with repaired esophageal atresia

J Pediatr Surg

(1989)

K Kimura et al.

Aortosternopexy for tracheomalacia following repair of esophageal atresia: evaluation by cine-CT and technical refinement

J Pediatr Surg

(1990)

CM Harmon et al.

Congenital anomalies of the esophagus

G Fine et al.

Alimentary tract

EH Thilo et al.

The newborn infant

JL Emery et al.

Squamous epithelium in respiratory tract of children with tracheo-oesophageal fistula

Arch Dis Child

(1971)

B Benjamin

Endoscopy in esophageal atresia and tracheoesophageal fistula

Ann Otol Rhinol Laryngol

(1981)

Cited by (322)

Endoscopic Chemocauterization with Trichloroacetic Acid for Congenital or Recurrent Tracheoesophageal Fistula in Children with Esophageal Atresia: Experience from a Tertiary Center
2024, Journal of Pediatric Surgery
Recurrent tracheoesophageal fistula (rTEF) is a well-known complication after surgery of EA, occurring in roughly 3–10% of the patients. Recent studies have highlighted safety and efficacy of endoscopic management of recurrent TEF. The aim of this study was to evaluate the efficacy of chemocauterization with trichloroacetic acid (TCA) in rTEF and congenital tracheoesophageal fistula (cTEF).
Retrospective chart review of consecutive patients with recurrent or congenital TEF who underwent endoscopic chemo-cauterization between 2018 and 2022 at a tertiary center. Children diagnosed with TEF who underwent primary or secondary endoscopic treatment were included. Median follow up time was 19 months for rTEF and 33 months for cTEF.
During the study period, 18 patients were treated endoscopically by chemocatuerization with TCA at our institution. Treatment of recurrent TEF was successful in 13 of 14 patients (93%) Treatment of congenital TEF was successful in 2 of 4 patients (50%). In 14 patients, closure was seen after 1–2 treatments. There were no serious adverse reactions or complications to the endoscopic treatment of TEF.
Endoscopic chemocauterization is a minimal invasive technique with low morbidity and high success rate and may be considered as primary treatment for recurrent TEF.
III.
Long-term Airway Outcomes and Interventions in Children With Oesophageal Atresia With Tracheoesophageal Fistula: A 20-year Single Centre Observational Study
2024, Journal of Pediatric Surgery
Airway anomalies, symptoms and interventions are commonly reported in children with oesophageal atresia with tracheoesophageal fistula (OA/TOF). The purpose of this study was to assess the incidence of these airway pathologies and those requiring interventions in the long-term.
A retrospective case note review of all patients admitted to the Neonatal Unit at the Royal Hospital for Children, Glasgow between January 2000 and December 2015 diagnosed with OA/TOF. Included patients had a minimum of 5 years follow-up.
121 patients were identified. 118 proceeded to OA/TOF repair. 115 patients had long-term follow-up data. Ninety-five (83%) children had one or more airway symptom recorded. Thirty-six (31%) neonates underwent airway endoscopy at the time of their initial OA/TOF repair. Forty-six (40%) children underwent airway endoscopy at a later date due to airway symptoms. Airway pathologies identified included airway malacia, thirty-two (28%), subglottic stenosis, eleven (10%), tracheal pouch, twenty-five (22%), laryngeal cleft, seven (6%) and recurrent fistula, five (4%). Airway interventions included endoscopic division of tracheal pouch, ten (9%), tracheostomy, seven (6%), aortopexy, six (5%), repair of recurrent fistula, five (4%), endoscopic repair of laryngeal cleft, three (3%) and four (3%) required open airway reconstruction for subglottic stenosis. One child (1%) remains tracheostomy dependent.
Long-term airway pathologies are common in children with OA/TOF. Many of these are remediable with surgical intervention. Clinicians should be cognisant of this and refer to Airway Services appropriately.
Endoscopic treatment in pediatric patients with recurrent and H-type tracheoesophageal fistulas – A systematic review and meta-analysis
2023, International Journal of Pediatric Otorhinolaryngology
Endoscopic treatments for managing recurrent tracheoesophageal fistula (rTEF) and H-type TEF are being utilized lately; however, the preferred technique is yet to be determined. We aimed to systematically review existing publications on endoscopic treatment of rTEF and H-type TEF to analyze their success and complication rates.
PRISMA guidelines were followed. MEDLINE, EMBASE, CINAHL and the Cochrane Central Register of Controlled Trials were comprehensively searched in accordance to a priori developed protocol, from 1975 until 2020. English, Spanish and German papers were included. Studies were independently screened and analyzed by two reviewers.
84 full texts were assessed for eligibility out of 581 screened studies, of these, 39 studies with 127 patients were eligible for inclusion (115 rTEF and 12 H-type TEF). All included studies were cases reports or case series. Overall success rate was 45% with sealant injection, 87% with de-epithelialization and 80% with sealant injection and de-epithelialization combined. The mean number of required treatments for success was 1.9 (range 1–6). Mixed effect model meta-analysis of case series with n > 1 showed that sealant injection had a significantly lower success rate of 50% (95% CI 1–99%, I² 72%) compared to de-epithelialization 90% (95% CI 72–99%, I² 27%), p = 0.007 and the combination of both techniques 87% (95% CI 68–99%, I² 11%), p = 0.02. Nine patients (7%) had transient respiratory distress. No mortalities reported.
Endoscopic treatment for rTEF and H-type fistula is a minimally invasive technique with favorable outcome and considerably less morbidity compared to open surgery, suggesting it as a safe and effective first line treatment option. Repeated endoscopic treatment attempts can be expected to obtain complete closure. De-epithelization techniques with or without combined tissue adhesive injection had significantly better results than sealant injection techniques alone.
Management of Adults With Esophageal Atresia
2023, Clinical Gastroenterology and Hepatology
Esophageal atresia (EA) with or without trachea-esophageal fistula is relatively common congenital malformation with most patients living into adulthood. As a result, care of the adult patient with EA is becoming more common. Although surgical repair has changed EA from a fatal to a livable condition, the residual effects of the anomaly may lead to a lifetime of complications. These include effects related to the underlying deformity such as atonicity of the esophageal segment, fistula recurrence, and esophageal cancer to complications of the surgery including anastomotic stricture, gastroesophageal reflux, and coping with an organ transposition. This review discusses the occurrence and management of these conditions in adulthood and the role of an effective transition from pediatric to adult care to optimize adult care treatment.
Advances in Complex Congenital Tracheoesophageal Anomalies
2022, Clinics in Perinatology
The novel application of an emerging device for salvage of primary repair in high-risk complex esophageal atresia
2022, Journal of Pediatric Surgery
Preservation of native esophagus is a tenet of esophageal atresia (EA) repair. However, techniques for delayed primary anastomosis are severely limited for surgically and medically complex patients at high-risk for operative repair. We report our initial experience with the novel application of the Connect-EA, an esophageal magnetic compression anastomosis device, for salvage of primary repair in 2 high-risk complex EA patients. Compassionate use was approved by the FDA and treating institutions.
Two approaches using the Connect-EA are described – a totally endoscopic approach and a novel hybrid operative approach. To our knowledge, this is the first successful use of a hybrid operative approach with an esophageal magnetic compression device.
Salvage of delayed primary anastomosis was successful in both patients. The totally endoscopic approach significantly reduced operative time and avoided repeat high-risk operation. The hybrid operative approach salvaged delayed primary anastomosis and avoided cervical esophagostomy.
The Connect-EA is a novel intervention to achieve delayed primary esophageal repair in complex EA patients with high-risk tissue characteristics and multi-system comorbidities that limit operative repair. We propose a clinical algorithm for use of the totally endoscopic approach and hybrid operative approach for use of the Connect-EA in high-risk complex EA patients.

View all citing articles on Scopus

View full text

Chest

ReviewsLong-term Complications of Congenital Esophageal Atresia and/or Tracheoesophageal Fistula

Section snippets

Definition and Background

Embryology

Classification

Surgical Management

Growth

Anastomotic Leak

Respiratory Complications of EA/TEF

Summary

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

Surgery

J Pediatr

J Pediatr Surg

Am J Otolaryngol

J Pediatr Surg

J Thorac Cardiovasc Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

Chest

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Thorac Cardiovasc Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr

J Pediatr Surg

J Pediatr Surg

Congenital anomalies of the esophagus

Alimentary tract

The newborn infant

Squamous epithelium in respiratory tract of children with tracheo-oesophageal fistula

Arch Dis Child

Endoscopy in esophageal atresia and tracheoesophageal fistula

Ann Otol Rhinol Laryngol

Reviews
Long-term Complications of Congenital Esophageal Atresia and/or Tracheoesophageal Fistula