This is the most common congenital anomaly of the esophagus. Esophageal atresia may be suspected on antenatal ultrasound see below or in the neonate due to inability to swallow saliva or milk, aspiration during early feedings, or failure to pass a nasogastric tube into the stomach successfully. It results from a failure of the primitive foregut to divide into the trachea anteriorly and the esophagus posteriorly. General pathogenesis includes teratogenic effects caused by early pregnancy use of antithyroid drugs 7.
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Esophageal atresia is incomplete formation of the esophagus, frequently associated with tracheoesophageal fistula. Diagnosis is suspected by failure to pass a nasogastric or orogastric tube.
Treatment is surgical repair. See also Overview of Congenital Gastrointestinal Anomalies. Esophageal atresia is the most common gastrointestinal GI atresia. The estimated incidence is 1 in live births. Two syndromes in particular are associated with esophageal atresia:.
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There are 5 major types of esophageal atresia see Figure: Types and relative frequencies of esophageal atresia and tracheoesophageal fistula. Most of the types also involve a fistula between the trachea and esophagus. Most infants present during the neonatal period, but infants with the H type fistula may not present until later in life. Characteristic signs are excessive secretions, coughing and cyanosis after attempts at feeding, and aspiration pneumonia. Esophageal atresia with a distal fistula leads to abdominal distention because, as the infant cries, air from the trachea is forced through the fistula into the lower esophagus and stomach.
Routine prenatal ultrasonography may suggest esophageal atresia. Polyhydramnios may be present but is not diagnostic because it can occur with many other disorders. Less commonly, there is a dilated upper esophageal pouch, but this is typically looked for only in fetuses with polyhydramnios and no stomach bubble. After delivery, an NGT or an orogastric tube is inserted if esophageal atresia is suspected by prenatal ultrasonography or clinical findings; diagnosis of esophageal atresia is suggested by inability to pass the tube into the stomach.
A radiopaque catheter determines the location of the atresia on x-ray. In atypical cases, a small amount of water-soluble contrast material may be needed to define the anatomy under fluoroscopy. The contrast material should be quickly aspirated back because it can cause a chemical pneumonitis if it enters the lungs.
This procedure should be done only by an experienced radiologist at the center where neonatal surgery will be done. Preoperative management aims to get the infant into optimal condition for surgery and prevent aspiration pneumonia, which makes surgical correction more hazardous. Oral feedings are withheld. Continuous suction with an NGT in the upper esophageal pouch prevents aspiration of swallowed saliva.
If definitive repair must be deferred because of extreme prematurity, aspiration pneumonia, or other congenital malformations, a gastrostomy tube is placed to decompress the stomach. Suction through the gastrostomy tube then reduces the risk that gastric contents will reflux through the fistula into the tracheobronchial tree.
If a fistula is noted, it needs to be ligated. In the remaining cases, where an extremely long gap exists, options are to do a gastric transposition procedure or a colonic interposition procedure. Some pediatric surgeons do a Foker procedure. In this procedure, traction sutures are placed in the ends of the esophageal pouches, brought out through the skin, and fixed with silastic buttons.
Once the ends of the esophagus have come together, or are in close proximity, a primary anastomosis is done 1. The most common acute complications are leakage at the anastomosis site and stricture formation. This poor motility predisposes the infant to gastroesophageal reflux. If medical management for reflux fails, a Nissen fundoplication may be required. Bairdain S, Ricca R, Riehle K, et al : Early results of an objective feedback-directed system for the staged traction repair of long-gap esophageal atresia.
J Pediatr Surg 48 10 —, Clinical manifestations include excessive secretions, coughing and cyanosis after attempts at feeding, and aspiration pneumonia. From developing new therapies that treat and prevent disease to helping people in need, we are committed to improving health and well-being around the world.
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Test your knowledge. Which of the following imaging studies is most appropriate for initial diagnosis of hypertrophic pyloric stenosis? More Content. Esophageal Atresia By William J. Click here for Patient Education. Types and relative frequencies of esophageal atresia and tracheoesophageal fistula Relative frequencies are based on a compilation of various sources.
Prenatal: Ultrasonography. Postnatal: Nasogastric tube NGT or orogastric tube placement and x-ray. Surgical repair. There are 5 types of esophageal atresia; all but one also involve a tracheoesophageal fistula. Sometimes diagnosis is suspected based on prenatal ultrasonography. Was This Page Helpful? Yes No. Biliary Atresia. Anal Atresia. Overview of Esophageal and Swallowing Disorders.
Cystic Fibrosis: Defective Chloride Transport. Add to Any Platform. Relative frequencies are based on a compilation of various sources.
During early development, the esophagus and windpipe trachea begin as a single tube that normally divides into the two adjacent passages between four and eight weeks after conception. In esophageal atresia EA , the upper esophagus does not connect atresia to the lower esophagus and stomach. Almost 90 percent of babies born with esophageal atresia also have a tracheoesophageal fistula TEF , in which the esophagus and the trachea are abnormally connected, allowing fluids from the esophagus to get into the airways and interfere with breathing. A small number of infants have only one of these abnormalities. In more than 80 percent of cases, the lower section of the malformed esophagus is connected to the trachea EA with a distal TEF. Other possible configurations include having the upper section of the malformed esophagus connected to the trachea EA with a proximal TEF , connections to the trachea from both the upper and lower sections of the malformed esophagus EA with proximal and distal TEF , an esophagus that is malformed but does not connect to the trachea isolated EA , and a connection to the trachea from an otherwise normal esophagus H-type TEF with no EA. Saliva, liquids fed to the infant, or digestive fluids may enter the windpipe through the tracheoesophageal fistula, leading to coughing, respiratory distress, and a bluish appearance of the skin or lips cyanosis.
Esophageal atresia is a digestive disorder in which the esophagus does not develop properly. The esophagus is the tube that normally carries food from the mouth to the stomach. Esophageal atresia EA is a congenital defect. This means it occurs before birth.
Esophageal atresia is incomplete formation of the esophagus, frequently associated with tracheoesophageal fistula. Diagnosis is suspected by failure to pass a nasogastric or orogastric tube. Treatment is surgical repair. See also Overview of Congenital Gastrointestinal Anomalies. Esophageal atresia is the most common gastrointestinal GI atresia. The estimated incidence is 1 in live births. Two syndromes in particular are associated with esophageal atresia:.
Esophageal atresia/tracheoesophageal fistula
Click here to view a larger image. Esophageal atresia is a birth defect of the swallowing tube esophagus that connects the mouth to the stomach. In a baby with esophageal atresia, the esophagus has two separate sections—the upper and lower esophagus—that do not connect. A baby with this birth defect is unable to pass food from the mouth to the stomach, and sometimes difficulty breathing. Esophageal atresia often occurs with tracheoesophageal fistula, a birth defect in which part of the esophagus is connected to the trachea, or windpipe. Researchers estimate that about 1 in every 4, babies is born with esophageal atresia in the United States.