Tracheoesophageal fistula and esophageal atresia
Tracheoesophageal fistula and esophageal atresia: Excerpt from Professional Guide to Diseases (Eighth Edition)
Tracheoesophageal fistula is a developmental anomaly characterized by an abnormal connection between the trachea and the esophagus. It usually accompanies esophageal atresia, in which the esophagus is closed off at some point. Although these malformations have numerous anatomic variations, the most common, by far, is esophageal atresia with fistula to the distal segment. (See Types of tracheoesophageal anomalies, pages 692 and 693.)
These disorders, two of the most serious surgical emergencies in neonates, require immediate diagnosis and correction. They may coexist with other serious anomalies, such as congenital heart disease, imperforate anus, genitourinary abnormalities, and intestinal atresia.
Causes and incidence
Tracheoesophageal fistula and esophageal atresia result from failure of the embryonic esophagus and trachea to develop and separate correctly. Respiratory system development begins at about day 26 of gestation. Abnormal development of the septum during this time can lead to tracheoesophageal fistula. The most common abnormality is type C tracheoesophageal fistula with esophageal atresia, in which the upper section of the esophagus terminates in a blind pouch, and the lower section ascends from the stomach and connects with the trachea by a short fistulous tract.
In type A atresia, both esophageal segments are blind pouches, and neither is connected to the airway. In type E (or H-type), tracheoesophageal fistula without atresia, the fistula may occur anywhere between the level of the cricoid cartilage and the midesophagus, but is usually higher in the trachea than in the esophagus. Such a fistula may be as small as a pinpoint. In types B and D, the upper portion of the esophagus opens into the trachea; neonates with this anomaly may experience life-threatening aspiration of saliva or food.
Esophageal atresia occurs in about 1 of every 1,500 to 3,000 live births; about one-third of these neonates are born prematurely.
Signs and symptoms
A neonate with type C tracheoesophageal fistula with esophageal atresia appears to swallow normally but soon after swallowing coughs, struggles, becomes cyanotic, and stops breathing as he aspirates fluids returning from the blind pouch of the esophagus through his nose and mouth. Stomach distention may cause respiratory distress; air and gastric contents (bile and gastric secretions) may reflux through the fistula into the trachea, resulting in chemical pneumonitis.
An infant with type A esophageal atresia appears normal at birth. The infant swallows normally, but as secretions fill the esophageal sac and overflow into the oropharynx, he develops mucus in the oropharynx and drools excessively. When the infant is fed, regurgitation and respiratory distress follow aspiration. Suctioning the mucus and secretions temporarily relieves these symptoms. Excessive secretions and drooling in the neonate strongly suggest esophageal atresia.
Repeated episodes of pneumonitis, pulmonary infection, and abdominal distention may signal type E (or H-type) tracheoesophageal fistula. When a child with this disorder drinks, he coughs, chokes, and becomes cyanotic. Excessive mucus builds up in the oropharynx. Crying forces air from the trachea into the esophagus, producing abdominal distention. Because such a child may appear normal at birth, this type of tracheoesophageal fistula may be overlooked, and diagnosis may be delayed as long as 1 year.
Type B (proximal fistula) and type D (fistula to both segments) cause immediate aspiration of saliva into the airway and bacterial pneumonitis.
Diagnosis
Respiratory distress and drooling in a neonate suggest tracheoesophageal fistula and esophageal atresia. The following procedures confirm the diagnosis:
❑ A size 10 or 12 French catheter passed through the nose meets an obstruction (esophageal atresia) approximately 4" to 5" (10 to 12.5 cm) distal from the nostrils. Aspirate of gastric contents is less acidic than normal.
❑ Chest X-ray demonstrates the position of the catheter and can also show a dilated, air-filled upper esophageal pouch, pneumonia in the right upper lobe, or bilateral pneumonitis. Both pneumonia and pneumonitis suggest aspiration.
❑ Abdominal X-ray shows gas in the bowel in a distal fistula (type C) but none in a proximal fistula (type B) or in atresia without fistula (type A).
❑ Cinefluorography allows visualization on a fluoroscopic screen. After a size 10 or 12 French catheter is passed through the patient’s nostril into the esophagus, a small amount of contrast medium is instilled to define the tip of the upper pouch and to differentiate between overflow aspiration from a blind end (atresia) and aspiration due to passage of liquid through a tracheoesophageal fistula.
Treatment
Tracheoesophageal fistula and esophageal atresia require surgical correction and are usually surgical emergencies. The type and timing of surgical procedure depend on the nature of the anomaly, the patient’s general condition, and the presence of coexisting congenital defects. In premature neonates (nearly 33% of neonates with this anomaly are born prematurely) who are poor surgical risks, correction of combined tracheoesophageal fistula and esophageal atresia is done in two stages: first, gastrostomy (for gastric decompression, prevention of reflux, and feeding) and closure of the fistula; then, 1 to 2 months later, anastomosis of the esophagus.
Before and after surgery, positioning varies with the physician’s philosophy and the infant’s anatomy: the infant may be placed supine, with his head low to facilitate drainage, or with his head elevated to prevent aspiration.
The infant should receive I.V. fluids, as necessary, and appropriate antibiotics for superimposed infection.
Postoperative complications after correction of tracheoesophageal fistula include recurrent fistulas, esophageal motility dysfunction, esophageal stricture, recurrent bronchitis, pneumothorax, and failure to thrive. Esophageal motility dysfunction or hiatal hernia may develop after surgery to correct esophageal atresia.
Correction of esophageal atresia alone requires anastomosis of the proximal and distal esophageal segments in one or two stages. End-to-end anastomosis commonly produces postoperative stricture; end-to-side anastomosis is less likely to do so. If the esophageal ends are widely separated, treatment may include a colonic interposition (grafting a piece of the colon) or elongation of the proximal segment of the esophagus by bougienage. About 10 days after surgery, and again 1 and 3 months later, X-rays are required to evaluate the effectiveness of surgical repair.
Postoperative treatment includes placement of a suction catheter in the upper esophageal pouch to control secretions and prevent aspiration, maintaining the infant in an upright position to avoid reflux of gastric juices into the trachea, I.V. fluids (nothing by mouth), gastrostomy to prevent reflux and allow feeding, and appropriate antibiotics for pneumonia.
Postoperative complications may include impaired esophageal motility (in one-third of patients), hiatal hernia, and reflux esophagitis.
Special considerations
Postoperative care should include the following:
❑ Monitor the infant’s respiratory status. Administer oxygen and perform pulmonary physiotherapy and suctioning, as needed. Provide a humid environment.
❑ Administer antibiotics and parenteral fluids, as ordered. Keep accurate intake and output records.
❑ If the infant has chest tubes postoperatively, check them frequently for patency. Maintain proper suction; measure and mark drainage periodically.
❑ Observe carefully for signs of complications, such as abnormal esophageal motility, recurrent fistulas, pneumothorax, and esophageal stricture.
❑ Maintain gastrostomy tube feedings, as ordered. Such feedings initially consist of dextrose and water (not more than 5% solution); later, add a proprietary formula (first diluted and then full strength). If the infant develops gastric atony, use an iso-osmolar formula. Oral feedings can usually resume 8 to 10 days postoperatively. If gastrostomy feedings and oral feedings are impossible because of intolerance to them or decreased intestinal motility, the infant requires total parenteral nutrition.
❑ If the infant can safely handle secretions, he may be given a pacifier to satisfy his sucking needs; however, this is done only when he can safely handle secretions because sucking stimulates saliva secretion.
❑ Offer the parents support and guidance in dealing with their infant’s acute illness. Encourage them to participate in the infant’s care and to hold and touch him as much as possible to facilitate bonding.
Pictures
Book Source Details
- Book Title: Professional Guide to Diseases (Eighth Edition)
- Author(s): Springhouse
- Year of Publication: 2005
- Copyright Details: Professional Guide to Diseases (Eighth Edition), Copyright © 2005 Lippincott Williams & Wilkins.
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