What Are the Types of Tracheoesophageal Fistulas?

Patient Presentation
A full-term male was born by vaginal delivery to a 24 year G1P1 female. In the delivery room, he had some difficulty breathing and was taken to the transition bed for monitoring. The nurse noted that he seemed to have more secretions and needed frequent suctioning. She tried to pass an NG tube to suction his stomach but was not able to pass it more than a few centimeters. He was taken to the neonatal intensive care unit, where his respiratory distress seemed to improve, but when he attempted to eat, he had a poor suck and had regurgitation. His tachypnea resumed. Again a nasogastric tube was tried, but could not be passed and on radiograph was found coiled in the proximal esophagus. The diagnosis of esophageal atresia and tracheoesophageal fistula was made. He was taken to the operating room and found to have proximal blind pouch esophageal atresia with a distal tracheoesophageal fistula. He was extubated on day 3 of life and was discharged on day 15 after surgical recovery and being able to take full oral feedings.

Discussion
Respiratory problems in infants can caused by many things including any type of obstruction from the nose to lung. Neonates and infants are particularly susceptible to changes in the size of the airway because of the physics of airflow resistance. Important reminders about infant airways:

• Resistance = 1/radius⁴ (Poiseuille’s equation) therefore even a 1 mm decrease in the airway circumferences increases the airflow resistance x16.
• Increased airflow causes less pressure along the walls, which can lead to collapse of the walls (Bernoulli’s and Venturi effects).
• Neonates have smaller lungs relative to the rest of their body therefore they have less pulmonary reserve.
• Neonates have higher oxygen consumption rates (about 50% more) than older children and adults.
• Neonates are preferential nose breathers.
• Neonates have immature regulation of vital functions and when stressed may do the opposite of what is expected. For example, they may become apneic with respiratory distress instead of increasing their respiratory rate.

Therefore anything that changes the airflow dynamics of the infant airway can cause dramatic effects for a neonate. Health care personnel need to be aware of subtle changes and may need to respond quickly to the infant’s respiratory status, even while trying to determine the cause of the problem.

Learning Point
Esophageal atresia (EA) with tracheoesophageal fistula are anomalies in the continuity of the esophagus with or without tracheal communication.
EA/TEF occurs in about 2500-3000 live births with most being a sporadic/non-syndromic. Familial or syndromic cases are about 1% with examples being CHARGE, VATER or VACTERL associations. To learn more about associations click here.

Presentations can vary depending on the particular type of EA/TEF. Prenatally, polyhydramnios, small or absent stomach bubble on prenatal ultrasound may indicate a EA/TEF. At birth, respiratory distress or cyanosis can occur. At birth or within a short time, increased salivation, regurgitation, coughing or choking on feeding may indicate a problem. Older children may present with recurrent coughing, wheezing, regurgitation or frequent pulmonary infections.

Chest/abdominal radiographs may show an air filled abdomen, or failed placement of NG tube (i.e. curled up in esophagus). H-type fistulas are difficult to detect by many means including upper gastrointestinal radiographic studies and even endoscopy.

The Gross Classification of EA/TEF includes:

• A – Isolated EA without TEF, 7-8.5%
• B – EA with proximal TEF, 1 to 2%
• C – “Blind pouch”, proximal EA with a distal TEF attached posteriorly to trachea, 85 – 86%
• D – “Double fistula”, EA with proximal and distal TEF, ~1%
• E – “H-type fistula”, TEF present between intact esophagus and trachea, 4%

The Vogt classification is similar but also includes absent esophagus. Concurrent anomalies occur in about 50% of patients including “…cardiovacular (29%), anorectal (14%), genitourinary (14%), gastrointestinal (13%), vertebral/skeletal (10%), and respiratory (6%) may be present.” Tracheomalacia is not considered a concurrent anomaly by most people, but part of the EA/TEF process. It occurs in about 75% of patients.

Surgery includes ligation of the TEF and anastomosis of the EA. This has been traditionally done by thoracotomy but is being done more often recently by thoracoscopy. Patients usually do well with only an ~2% mortality rate usually because of concurrent anomalies. Short term surgical complications include anastomatic leaks, esophageal stenosis or web, recurrent fistula, vocal fold paralysis, dysphagia, eating problems, gastroesophageal reflux disease and pulmonary infections. Longer term followup shows problems with GERD and pulmonary problems including wheezing or infections. Generally development is normal but children may have communication problems. There are few studies of adults who had EA/TEF as a child so data is sparse but shows GERD and pulmonary problems.

Questions for Further Discussion
1. What are the components of CHARGE association?
2. List other types of tracheal disorders?

Related Cases

Disease: Tracheoesophageal Fistula and Esophageal Atresia | Esophagus Disorders | Tracheal Disorders | Birth Defects

Symptom/Presentation: Respiratory Distress

Specialty: Allergy / Pulmonary Diseases | Neonatology | Surgery

Age: Newborn

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.
Information prescriptions for patients can be found at MedlinePlus for these topics: Esophagus Disorders and Tracheal Disorders.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Roberts K, Karpelowsky J, Fitzgerald DA, Soundappan SS. Outcomes of oesophageal atresia and tracheo-oesophageal fistula repair. J Paediatr Child Health. 2016 Jul;52(7):694-8.

Javia L, Harris MA, Fuller S. Rings, slings, and other tracheal disorders in the neonate. Semin Fetal Neonatal Med. 2016 Aug;21(4):277-84.

Vijayasekaran S, Lioy J, Maschhoff K. Airway disorders of the fetus and neonate: An overview. Semin Fetal Neonatal Med. 2016 Aug;21(4):220-9.

van der Zee DC, Tytgat SHA, van Herwaarden MYA. Esophageal atresia and tracheo-esophageal fistula. Semin Pediatr Surg. 2017 Apr;26(2):67-71.

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa