A tracheoesophageal fistula (TEF, or TOF; see

spelling differences Despite the various English dialects spoken from country to country and within different regions of the same country, there are only slight regional variations in English orthography, the two most notable variations being British and American ...

) is an abnormal connection (

fistula A fistula (plural: fistulas or fistulae ; from Latin ''fistula'', "tube, pipe") in anatomy is an abnormal connection between two hollow spaces (technically, two epithelialized surfaces), such as blood vessels, intestines, or other hollow or ...

) between the

esophagus The esophagus (American English) or oesophagus (British English; both ), non-technically known also as the food pipe or gullet, is an organ in vertebrates through which food passes, aided by peristaltic contractions, from the pharynx to the ...

and the

trachea The trachea, also known as the windpipe, is a Cartilage, cartilaginous tube that connects the larynx to the bronchi of the lungs, allowing the passage of air, and so is present in almost all air-breathing animals with lungs. The trachea extends ...

. TEF is a common

congenital A birth defect, also known as a congenital disorder, is an abnormal condition that is present at birth regardless of its cause. Birth defects may result in disabilities that may be physical, intellectual, or developmental. The disabilities can ...

abnormality, but when occurring late in life is usually the sequela of surgical procedures such as a

laryngectomy Laryngectomy is the removal of the larynx and separation of the airway from the mouth, nose and esophagus. In a total laryngectomy, the entire larynx is removed (including the vocal folds, hyoid bone, epiglottis, Thyroid cartilage, thyroid and cr ...

Presentation

Tracheoesophageal fistula is suggested in a newborn by copious

salivation Saliva (commonly referred to as spit) is an extracellular fluid produced and secreted by salivary glands in the mouth. In humans, saliva is around 99% water, plus electrolytes, mucus, white blood cells, epithelial cells (from which DNA can be e ...

associated with

choking Choking, also known as foreign body airway obstruction (FBAO), is a phenomenon that occurs when breathing is impeded by a blockage inside of the respiratory tract. An obstruction that prevents oxygen from entering the lungs results in oxygen de ...

, coughing, vomiting, and

cyanosis Cyanosis is the change of body tissue color to a bluish-purple hue as a result of having decreased amounts of oxygen bound to the hemoglobin in the red blood cells of the capillary bed. Body tissues that show cyanosis are usually in locations ...

coincident with the onset of feeding. Esophageal atresia and the subsequent inability to swallow typically cause polyhydramnios in utero. Rarely it may present in an

adult An adult is a human or other animal that has reached full growth. In human context, the term ''adult'' has meanings associated with social and legal concepts. In contrast to a " minor", a legal adult is a person who has attained the age of major ...

Complications

Surgical repair can sometimes result in complications, including: * Stricture, due to

gastric acid Gastric acid, gastric juice, or stomach acid is a digestive fluid formed within the stomach lining. With a pH between 1 and 3, gastric acid plays a key role in digestion of proteins by activating digestive enzymes, which together break down the ...

erosion of the shortened esophagus * Leak of contents at the point of anastomosis * Recurrence of fistula *

Gastro-esophageal reflux disease Gastroesophageal reflux disease (GERD) or gastro-oesophageal reflux disease (GORD) is one of the upper gastrointestinal chronic diseases where stomach content persistently and regularly flows up into the esophagus, resulting in symptoms and/ ...

Dysphagia Dysphagia is difficulty in swallowing. Although classified under "symptoms and signs" in ICD-10, in some contexts it is classified as a disease#Terminology, condition in its own right. It may be a sensation that suggests difficulty in the passag ...

Asthma Asthma is a long-term inflammatory disease of the airways of the lungs. It is characterized by variable and recurring symptoms, reversible airflow obstruction, and easily triggered bronchospasms. Symptoms include episodes of wheezing, cou ...

-like symptoms, such as persistent coughing/wheezing * Recurrent chest infections *

Tracheomalacia Tracheomalacia is a condition or incident where the cartilage that keeps the airway (trachea) open is soft such that the trachea partly collapses especially during increased airflow. This condition is most commonly seen in infants and young child ...

Associations

Neonates with TEF or esophageal atresia are unable to feed properly. Once diagnosed, prompt surgery is required to allow the food intake. Some children experience problems following TEF surgery; they can develop dysphagia and thoracic problems. Children with TEF can also be born with other abnormalities, most commonly those described in

VACTERL The VACTERL association (also VATER association, and less accurately VACTERL syndrome) refers to a recognized group of birth defects which tend to co-occur (see below). This pattern is a recognized association, as opposed to a syndrome, because t ...

association - a group of anomalies which often occur together, including heart, kidney and limb deformities. 6% of babies with TEF also have a

laryngeal cleft A laryngeal cleft or laryngotracheoesophageal cleft is a rare congenital abnormality in the posterior laryngo-tracheal wall. It occurs in approximately 1 in 10,000 to 20,000 births. It means there is a communication between the oesophagus and the ...

Cause

Congenital TEF can arise due to failed fusion of the

tracheoesophageal ridges The tracheoesophageal septum is an embryological structure. It is formed from the tracheoesophageal folds or ridges which fuse in the midline. It divides the oesophagus from the trachea during prenatal development. Developmental abnormalities ca ...

after the fourth week of

embryological development Prenatal development () includes the development of the embryo and of the fetus during a viviparous animal's gestation. Prenatal development starts with fertilization, in the germinal stage of embryonic development, and continues in fetal deve ...

. A

, from the Latin meaning 'a pipe', is an abnormal connection running either between two tubes or between a tube and a surface. In tracheo-esophageal fistula it runs between the trachea and the esophagus. This connection may or may not have a central cavity; if it does, then food within the esophagus may pass into the trachea (and on to the lungs) or alternatively, air in the trachea may cross into the esophagus. TEF can also occur due to pressure

necrosis Necrosis () is a form of cell injury which results in the premature death of cells in living tissue by autolysis. Necrosis is caused by factors external to the cell or tissue, such as infection, or trauma which result in the unregulated dige ...

by a

tracheostomy tube Tracheotomy (, ), or tracheostomy, is a surgical airway management procedure which consists of making an incision (cut) on the anterior aspect (front) of the neck and opening a direct airway through an incision in the trachea (windpipe). The re ...

in apposition to a

nasogastric tube Nasogastric intubation is a medical process involving the insertion of a plastic tube (nasogastric tube or NG tube) through the nose, down the oesophagus, and down into the stomach. Orogastric intubation is a similar process involving the inserti ...

(NGT).

Diagnosis

TEF should be suspected once the baby fails to swallow after their first feeding during the first day of life. Esophageal atresia can be diagnosed by Ryle nasogastric tube; if the Ryle fails to pass into the stomach, then this indicates esophageal atresia and loss of communication between stomach and esophagus. TEF may be diagnosed by MRI which clarifies the atretic esophagus (if presents) and TEF, as well as its location and anatomy. Gastrograffin contrast swallow should not be used if TEF is suspected, due to its high risk of allergy and severe intractable chest infection.

Classification

Fistulae between the trachea and esophagus in the newborn can be of diverse morphology and anatomical location. However, various pediatric surgical publications have attempted a classification system based on the below specified types. Not all types include both esophageal agenesis and tracheoesophageal fistula, but the most common types do. The letter codes are usually associated with the system used by Gross, while number codes are usually associated with Vogt.Vogt EC. Congenital esophageal atresia. Am J of Roentgenol. 1929;22:463–465. An additional type, "blind upper segment only" has been described, but this type is not usually included in most classifications. (For the purposes of this discussion, ''proximal esophagus'' indicates normal esophageal tissue arising normally from the pharynx, and ''distal esophagus'' indicates normal esophageal tissue emptying into the proximal stomach.)

Treatment

It is surgically corrected, with resection of any fistula and

anastomosis An anastomosis (, plural anastomoses) is a connection or opening between two things (especially cavities or passages) that are normally diverging or branching, such as between blood vessels, leaf#Veins, leaf veins, or streams. Such a connection m ...

of any discontinuous segments. Babies often need to spend time in a

neonatal intensive care unit A neonatal intensive care unit (NICU), also known as an intensive care nursery (ICN), is an intensive care unit (ICU) specializing in the care of ill or premature newborn infants. Neonatal refers to the first 28 days of life. Neonatal care, as kn ...

for feeding with a

stomach The stomach is a muscular, hollow organ in the gastrointestinal tract of humans and many other animals, including several invertebrates. The stomach has a dilated structure and functions as a vital organ in the digestive system. The stomach i ...

feeding tube Eating (also known as consuming) is the ingestion of food, typically to provide a heterotrophic organism with energy and to allow for growth. Animals and other heterotrophs must eat in order to survive — carnivores eat other animals, her ...

Antibiotic An antibiotic is a type of antimicrobial substance active against bacteria. It is the most important type of antibacterial agent for fighting bacterial infections, and antibiotic medications are widely used in the treatment and prevention of ...

pain relief Pain management is an aspect of medicine and health care involving relief of pain (pain relief, analgesia, pain control) in various dimensions, from acute and simple to chronic and challenging. Most physicians and other health professional ...

, a

chest drain A chest tube (also chest drain, thoracic catheter, tube thoracostomy or intercostal drain) is a surgical drain that is inserted through the chest wall and into the pleural space or the mediastinum in order to remove clinically undesired substan ...

oxygen Oxygen is the chemical element with the symbol O and atomic number 8. It is a member of the chalcogen group in the periodic table, a highly reactive nonmetal, and an oxidizing agent that readily forms oxides with most elements as wel ...

, and

ventilation Ventilation may refer to: * Ventilation (physiology), the movement of air between the environment and the lungs via inhalation and exhalation ** Mechanical ventilation, in medicine, using artificial methods to assist breathing *** Ventilator, a m ...

may all be needed.

References

External links

{{Congenital malformations and deformations of digestive system Congenital disorders of digestive system