Esophageal atresia and tracheoesophageal fistula

Overview 
Theory 
Diagnosis 
Management 
Follow up 
Resources 

Last reviewed: 21 Nov 2024

Last updated: 22 Nov 2024

Esophageal atresia and tracheoesophageal fistula occurs in 1 of every 2500 to 4000 live births.

There are no known predisposing factors; however, the condition often presents with other congenital anomalies, including VACTERL complex (vertebral defects, anorectal anomalies, cardiac defects, tracheoesophageal abnormalities, radial and renal abnormalities, and limb anomalies).

Surgical intervention is required as a newborn. While repair is traditionally done via an open thoracotomy, thoracoscopic repair is safe and effective.

Patients may have long-term complications, such as GERD, tracheomalacia, and chronic respiratory infections.

Definition

Esophageal atresia (EA) and tracheoesophageal fistula (TEF) are congenital malformations that result from the defective separation of the common embryonic precursor to both the esophagus and trachea. The most common type is a blind-ending upper esophageal pouch with a fistulous connection between the distal esophageal segment and the trachea.

History and exam

Key diagnostic factors

maternal history of polyhydramniosis
inability to swallow secretions
inability to pass a nasogastric tube

Full details

Other diagnostic factors

labored respiration
coughing
choking
VACTERL association
cyanosis

Full details

Risk factors

trisomy 18 and 21
family history

Full details

Diagnostic tests

1st tests to order

prenatal ultrasound
prenatal MRI
x-ray chest and abdomen

Full details

Tests to consider

bronchoscopy
esophagoscopy
barium swallow
transthoracic echocardiogram

Full details

Treatment algorithm

ACUTE

type A

type B and D

type C

type E

Contributors

Authors

Alexander J. Bondoc, MD

Associate Professor

UC Department of Surgery

UC Department of Pediatrics

Cincinnati Children's Hospital

Cincinnati

Disclosures

AJB declares that he has no competing interests.

Aaron Garrison, MD

Associate Professor

UC Department of Surgery

Cincinnati Children's Hospital

Cincinnati

Disclosures

AG declares that he has no competing interests.

Daniel von Allmen, MD

Professor-Associate

University of Cincinnati

Department of Surgery

Cincinnati

Disclosures

DVA is an investor for GlobalCastMD.

Acknowledgements

Dr Alexander J. Bondoc would like to gratefully acknowledge Dr Steven S. Rothenberg, a previous contributor to this topic.

Disclosures

SSR declared that he had no competing interests.

Peer reviewers

Michael D. Klein, MD, FACS, FAAP

Surgeon in Chief

Professor of Surgery

Department of Pediatric Surgery

Children's Hospital of Michigan

Wayne State University School of Medicine

Detroit

Disclosures

MDK declares that he has no competing interests.

Timothy D. Kane, MD

Clinical Director

Division Pediatric General and Thoracic Surgery

Children's Hospital of Pittsburgh of UPMC

Pittsburgh

Disclosures

TDK declares that he has no competing interests.

Lewis Spitz, MBChB, PhD, FRCS, MD, FRCPCH, FAAP, FCS

Emeritus Nuffield Professor of Paediatric Surgery

Institute of Child Health

University College

London

Disclosures

LS declares that he has no competing interests.

Differentials
- Laryngeal cleft
More Differentials
Guidelines
- Oesophageal atresia/tracheoesophageal fistula
More Guidelines
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Esophageal atresia and tracheoesophageal fistula

Summary

Definition

History and exam

Key diagnostic factors

Other diagnostic factors

Risk factors

Diagnostic tests

1st tests to order

Tests to consider

Treatment algorithm

type A

type B and D

type C

type E

Contributors

Authors

Alexander J. Bondoc, MD

Disclosures

Aaron Garrison, MD

Disclosures

Daniel von Allmen, MD

Disclosures

Acknowledgements

Disclosures

Peer reviewers

Michael D. Klein, MD, FACS, FAAP

Disclosures

Timothy D. Kane, MD

Disclosures

Lewis Spitz, MBChB, PhD, FRCS, MD, FRCPCH, FAAP, FCS

Disclosures

Differentials

Guidelines

Log in or subscribe to access all of BMJ Best Practice

Log in or subscribe to access all of BMJ Best Practice

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