| Tracheoesophageal fistula | |
|---|---|
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| Specialty | Medical genetics  |
A tracheoesophageal fistula (TEF, or TOF; see spelling differences) is an abnormal connection (fistula) between the esophagus and the trachea. TEF is a common congenital abnormality, but when occurring late in life is usually the sequela of surgical procedures such as a laryngectomy.
Tracheoesophageal fistula is suggested in a newborn by copious salivation associated with choking, coughing, vomiting, and cyanosis 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. [1]
Surgical repair can sometimes result in complications, including:[ citation needed ]
Neonates with TEF or esophageal atresia are unable to feed properly. Once diagnosed, prompt surgery is required to allow the food intake. [2] 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 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. [3]
Congenital TEF can arise due to failed fusion of the tracheoesophageal ridges after the fourth week of embryological development. [4]
A fistula, 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.[ citation needed ]
TEF can also occur due to pressure necrosis by a tracheostomy tube in apposition to a nasogastric tube (NGT). [5]
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.[ citation needed ]
Fistulae between the trachea and esophagus in the newborn can be of diverse morphology and anatomical location. [6] [7] However, various pediatric surgical publications have attempted a classification system based on the below specified types.[ citation needed ]
Not all types include both esophageal agenesis and tracheoesophageal fistula, but the most common types do.[ citation needed ]
| Gross | Vogt [8] | Description | EA? | TEF? |
|---|---|---|---|---|
| - | Type 1 | Esophageal agenesis. Very rare, and not included in the classification by Gross. [9] | Yes | No |
| Type A | Type 2 | Proximal and distal esophageal bud—a normal esophagus with a missing mid-segment. | Yes | No |
| Type B | Type 3A | Proximal esophageal termination on the lower trachea with distal esophageal bud. | Yes | Yes |
| Type C | Type 3B | Proximal esophageal atresia (esophagus continuous with the mouth ending in a blind loop superior to the sternal angle) with a distal esophagus arising from the lower trachea or carina. (Most common, up to 90% of cases.) | Yes | Yes |
| Type D | Type 3C | Proximal esophageal termination on the lower trachea or carina with distal esophagus arising from the carina. | Yes | Yes |
| Type E (or H-Type) | - | A variant of type D: if the two segments of esophagus communicate, this is sometimes termed an H-type fistula due to its resemblance to the letter H. TEF without EA. | No | Yes |
The letter codes are usually associated with the system used by Gross, [10] while number codes are usually associated with Vogt. [11]
An additional type, "blind upper segment only" has been described, [12] 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.)
It is surgically corrected, with resection of any fistula and anastomosis of any discontinuous segments. [2] Babies often need to spend time in a neonatal intensive care unit for feeding with a stomach feeding tube. [2] Antibiotics, pain relief, a chest drain, oxygen, and ventilation may all be needed. [2]
The trachea, also known as the windpipe, is a cartilaginous tube that connects the larynx to the bronchi of the lungs, allowing the passage of air, and so is present in almost all animals lungs. The trachea extends from the larynx and branches into the two primary bronchi. At the top of the trachea, the cricoid cartilage attaches it to the larynx. The trachea is formed by a number of horseshoe-shaped rings, joined together vertically by overlying ligaments, and by the trachealis muscle at their ends. The epiglottis closes the opening to the larynx during swallowing.
The esophagus, oesophagus, or œsophagus all ; pl.: ( e)(œ)sophagi or (œ)sophaguses), colloquially known also as the food pipe, food tube, or gullet, is an organ in vertebrates through which food passes, aided by peristaltic contractions, from the pharynx to the stomach. The esophagus is a fibromuscular tube, about 25 cm (10 in) long in adults, that travels behind the trachea and heart, passes through the diaphragm, and empties into the uppermost region of the stomach. During swallowing, the epiglottis tilts backwards to prevent food from going down the larynx and lungs. The word oesophagus is from Ancient Greek οἰσοφάγος (oisophágos), from οἴσω (oísō), future form of φέρω + ἔφαγον.
Esophageal atresia is a congenital medical condition that affects the alimentary tract. It causes the esophagus to end in a blind-ended pouch rather than connecting normally to the stomach. It comprises a variety of congenital anatomic defects that are caused by an abnormal embryological development of the esophagus. It is characterized anatomically by a congenital obstruction of the esophagus with interruption of the continuity of the esophageal wall.
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 children. The usual symptom is stridor when a person breathes out. This is usually known as a collapsed windpipe.
Esophagitis, also spelled oesophagitis, is a disease characterized by inflammation of the esophagus. The esophagus is a tube composed of a mucosal lining, and longitudinal and circular smooth muscle fibers. It connects the pharynx to the stomach; swallowed food and liquids normally pass through it.
In anatomy, a fistula is an abnormal connection joining two hollow spaces, such as blood vessels, intestines, or other hollow organs to each other, often resulting in an abnormal flow of fluid from one space to the other. An anal fistula connects the anal canal to the perianal skin. An anovaginal or rectovaginal fistula is a hole joining the anus or rectum to the vagina. A colovaginal fistula joins the space in the colon to that in the vagina. A urinary tract fistula is an abnormal opening in the urinary tract or an abnormal connection between the urinary tract and another organ. An abnormal communication between the bladder and the uterus is called a vesicouterine fistula, while if it is between the bladder and the vagina it is known as a vesicovaginal fistula, and if between the urethra and the vagina: a urethrovaginal fistula. When occurring between two parts of the intestine, it is known as an enteroenteral fistula, between the small intestine and the skin it's known as an enterocutaneous fistula, and between the colon and the skin as a colocutaneous fistula.
A feeding tube is a medical device used to provide nutrition to people who cannot obtain nutrition by mouth, are unable to swallow safely, or need nutritional supplementation. The state of being fed by a feeding tube is called gavage, enteral feeding or tube feeding. Placement may be temporary for the treatment of acute conditions or lifelong in the case of chronic disabilities. A variety of feeding tubes are used in medical practice. They are usually made of polyurethane or silicone. The outer diameter of a feeding tube is measured in French units. They are classified by the site of insertion and intended use.
Laryngectomy is the removal of the larynx. In a total laryngectomy, the entire larynx is removed with the separation of the airway from the mouth, nose and esophagus. In a partial laryngectomy, only a portion of the larynx is removed. Following the procedure, the person breathes through an opening in the neck known as a stoma. This procedure is usually performed by an ENT surgeon in cases of laryngeal cancer. Many cases of laryngeal cancer are treated with more conservative methods. A laryngectomy is performed when these treatments fail to conserve the larynx or when the cancer has progressed such that normal functioning would be prevented. Laryngectomies are also performed on individuals with other types of head and neck cancer. Less invasive partial laryngectomies, including tracheal shaves and feminization laryngoplasty may also be performed on transgender women and other female or non-binary identified individuals to feminize the larynx and/or voice. Post-laryngectomy rehabilitation includes voice restoration, oral feeding and more recently, smell and taste rehabilitation. An individual's quality of life can be affected post-surgery.
Pediatric surgery is a subspecialty of surgery involving the surgery of fetuses, infants, children, adolescents, and young adults.
Atresia is a condition in which an orifice or passage in the body is closed or absent.
The VACTERL association refers to a recognized group of birth defects which tend to co-occur. This pattern is a recognized association, as opposed to a syndrome, because there is no known pathogenetic cause to explain the grouped incidence.
A vascular ring is a congenital defect in which there is an abnormal formation of the aorta and/or its surrounding blood vessels. The trachea and esophagus are completely encircled and sometimes compressed by a "ring" formed by these vessels, which can lead to breathing and digestive difficulties.
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 can lead to a tracheoesophageal fistula.
A tracheo-esophageal puncture is a surgically created hole between the trachea (windpipe) and the esophagus in a person who has had a total laryngectomy, a surgery where the larynx is removed. The purpose of the puncture is to restore a person’s ability to speak after the vocal cords have been removed. This involves creation of a fistula between the trachea and the esophagus, puncturing the short segment of tissue or “common wall” that typically separates these two structures. A voice prosthesis is inserted into this puncture. The prosthesis keeps food out of the trachea but lets air into the esophagus for esophageal speech.
Bronchomalacia is a term for weak cartilage in the walls of the bronchial tubes, often occurring in children under a day. Bronchomalacia means 'floppiness' of some part of the bronchi. Patients present with noisy breathing and/or wheezing. There is collapse of a main stem bronchus on exhalation. If the trachea is also involved the term tracheobronchomalacia (TBM) is used. If only the upper airway the trachea is involved it is called tracheomalacia (TM). There are two types of bronchomalacia. Primary bronchomalacia is due to a deficiency in the cartilaginous rings. Secondary bronchomalacia may occur by extrinsic compression from an enlarged vessel, a vascular ring or a bronchogenic cyst. Though uncommon, idiopathic tracheobronchomalacia has been described in older adults.
Esophageal diseases can derive from congenital conditions, or they can be acquired later in life.
Double aortic arch is a relatively rare congenital cardiovascular malformation. DAA is an anomaly of the aortic arch in which two aortic arches form a complete vascular ring that can compress the trachea and/or esophagus. Most commonly there is a larger (dominant) right arch behind and a smaller (hypoplastic) left aortic arch in front of the trachea/esophagus. The two arches join to form the descending aorta which is usually on the left side. In some cases the end of the smaller left aortic arch closes and the vascular tissue becomes a fibrous cord. Although in these cases a complete ring of two patent aortic arches is not present, the term ‘vascular ring’ is the accepted generic term even in these anomalies.
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 trachea, which allows food or fluid to pass into the airway.
Tracheal agenesis is a rare birth defect with a prevalence of less than 1 in 50,000 in which the trachea fails to develop, resulting in an impaired communication between the larynx and the alveoli of the lungs. Although the defect is normally fatal, occasional cases have been reported of long-term survival following surgical intervention.
Mandibulofacial dysostosis with microcephaly syndrome, also known as growth delay-intellectual disability-mandibulofacial dysostosis-microcephaly-cleft palate syndrome, mandibulofacial dysostosis, guion-almeida type, or simply as MFDM syndrome is a rare genetic disorder which is characterized by developmental delays, intellectual disabilities, and craniofacial dysmorphisms.
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