1. Esophageal atresia

2. >90% have an associated tracheoesophageal fistula (TEF)
Most frequent congenital anomaly of the esophagus, affecting 1 /4,000 neonates
an abnormality in which the middle portion of the esophagus is absent. The esophagus may end blindly into a pouch or may connect to the trachea
>90% have an associated tracheoesophageal fistula (TEF)
Tracheoesophageal fistula (TEF) represents an abnormal opening between the trachea and esophagus
Esophageal atresia is classified by the length of the esophagus and by the connection between the esophagus and the trachea

3. ‘”Long Gap", “Pure” or “Isolated” Esophageal Atresia
Type
Name
Description A
‘”Long Gap", “Pure” or “Isolated” Esophageal Atresia
Esophageal Atresia without fistula.Type A B
Esophageal Atresia with proximal TEF (tracheoesophageal fistula)
upper esophageal pouch connects abnormally to the trachea. C
Esophageal Atresia with distal TEF (tracheoesophageal fistula)
lower esophageal pouch makes an abnormal connection with the trachea.
The upper esophageal pouch ends blindly. D
Esophageal Atresia with both proximal and distal TEFs (two tracheoesophageal fistulas)
 upper and lower esophageal pouches make and abnormal connection with the trachea in two separate, isolated places. E
TEF (tracheoesophageal fistula) ONLY with no Esophageal Atresia
the esophagus fully intact and capable of its normal functions, however, there is an abnormal connection between the esophagus and the trachea F
Esophageal Stenosis
Also known as an Esophageal Stricture.
esophagus fully intact gradually narrows,

5. Presentation -Frothing or bubbling of the mouth and nose after birth - Drooling - Choking -Episodes of coughing, cyanosis and respiratory distress
-These problems are related to the saliva in the mouth having no way to reach the stomach. The saliva collects in the blind pouch of the esophagus. This may cause problems with airway obstruction and aspiration pneumonia. To protect the infant's airway, a nasogastric or orogastric tube will be placed. It is usually placed in the blind pouch and the salvia is then suctioned out.

6. Diagnosis Prenatal ultrasound- maternal polyhydramnios
inability to pass a nasogastric or orogastric tube in newborn
Plain radiography- shows radiolucent, blind-end dilated pouch of upper esophagus
On lateral view
Anterior displacement of trachea
Rarely, air-fluid level in pouch

7. Esophageal atresia with fistula (type D)
 Esophageal atresia with fistula (type D). (a) Frontal radiograph shows air within the distended upper esophageal pouch (arrows). The presence of gas in the bowel indicates fistulous communication between the lower esophageal segment and the trachea. (b) Lateral radiograph more clearly demonstrates the distended upper esophageal pouch with resulting pressure deformity of the trachea. An upper fistula was also found at surgery.

8. Lateral radiograph more clearly demonstrates the distended upper esophageal pouch with resulting pressure deformity of the trachea. An upper fistula was also found at surgery.

9. Preoperative Management -maintain a patent airway -prevent aspiration of stomach contents or saliva into the lungs -prone position to prevent stomach secretions from refluxing into lungs -Intravenous antibiotics to help prevent the development of pneumonia -The "VACTERL" workup is completed prior to surgical repair
-nasogastric or orogastric tube will be placed into the proximal esophageal (blind pouch) on continuous suction to prevent aspiration of secretions.

10. Features
V - Vertebral anomalies
A - Anal atresia
C - Cardiovascular anomalies
T - Tracheoesophageal fistula
E - Esophageal atresia
R - Renal (Kidney) and/or radial anomalies
L - Limb anomalies (in front of or above the central axis of the limb)

11. Surgical management -Primary anastamosis of proximal and distal esophagus usually when infant is a few months old - gastrostomy, gastric pull-up, colonic transposition and jejunum transposition -
Colonic interposition if primary anastamosis is impossible
Using the Foker technique, surgeons place traction sutures in the tiny esophageal ends and increase the tension on these sutures daily until the ends are close enough to be sewn together. The result is a normally functioning esophagus, virtually indistinguishable from one congenitally well formed

12. Surgery involves stretching the 2 ends of the esophagus and sewing them together
- If the child also has a tracheal connection this is divided and sewed also.
-The infant will have a chest tube after surgery to allow for drainage of secretions if a leak occurs at the surgical site.

15. -Newborns with prematurity, substantial respiratory difficulties, and/or associated anomalies and esophageal atresia and tracheoesophageal fistula complete surgical repair is not done with one operation. -While waiting for the infant to stabilize, gastrostomy tube may be placed to initiate feedings while the blind pouch continues to have suction via nasogastric tube. -When the child has grown and has been stable for a period of time the operation will be performed. -This usually involves closure of the TEF with reconstruction of the esophagus.
Infants with esophageal atresia and no tracheoesophageal fistula often have a very short esophagus. Thus surgical repair is done in stages over a series of months. Generally, a gastrostomy tube is placed to initiate feedings and to help increase the size of the stomach. Most children also have a nasogastric tube to suction in the blind pouch help the infant deal with oral secretions.
After the gastrostomy tube is place and the child is growing in first few months of life, the upper pouch is dilated daily. This is done in an attempt to stretch the esophagus.
At that point, a decision must be made whether to attempt to salvage the native esophagus. Options include: 1. After 8 to 12 weeks, growth of the proximal and distal esophageal segments will typically allow an esophagoesophagostomy to be performed; or 2) Creation of a cervical esophagostomy with gastrostomy tube placement in the newborn which will allow stable management until reconstruction of the esophagus with stomach, colon, or small bowel may be performed at one year of life.

16. Postoperative care
Postoperatively patients may be extubated as tolerated.
Any attempt at reintubation should be performed carefully in order to avoid accidental and catastrophic intubation of the esophagus.
The head is flexed slightly forward in order to decrease tension on the esophagoesophagostomy.
Oral suctioning performed only to the level of the pharynx.
Endotracheal tube suctioning must be performed carefully in order to avoid trauma or perforation at the site of the TEF closure.
A gastrostomy tube is placed at the time of esophageal repair only if it is anticipated that one will be needed for patient management in the future. Otherwise, a nasogastric tube passed through the anastomosis at the time of esophagoesophagostomy will suffice. A hypaque swallow is performed at seven days after operation. If the anastomosis appears intact, then feedings are initiated, antibiotics discontinued, and the retropleural chest tube removed.

17. Outcome Current overall survival rates= 85% - 90%
Mortality is usually secondary to associated anomalies.
Immediate postoperative complications include:
anastomotic leak in 15% of cases.
stricture formation in approximately 15% of cases
recurrent tracheoesophageal fistula in 5% of cases and requires reoperation with division and ligation of the fistula
The most common long term problems :
Gastroesophageal reflux in up to 70%
Tracheomalacia in approximately 25%
Almost all leaks will spontaneously resolve with continuation of IV antibiotics and chest tube drainage

18. extras

19. Radiologic diagnosis is based on findings at anteroposterior and lateral chest radiography, which reveals a blind pouch of the proximal esophagus that is distended with air. Radiographic evaluation should always include the abdomen to assess the presence of air in the gastrointestinal tract (distal fistula) (Fig 2). In types A and B, there is complete absence of gas in the stomach and intestinal tract, whereas in types C and D the gastrointestinal tract commonly appears distended with air. The type of esophageal atresia is confirmed by advancing a radiopaque feeding tube through the nose to the level of the atresia. The tube will curl when it approaches the blind end (Figs 3, 4). Atelectasis and pneumonia involving the upper right lobe are seen in up to 50% of cases. When type E (H-shaped tracheoesophageal fistula without atresia) is suspected, radiologic investigation is directed toward demonstration of the fistula, which typically courses forward and upward from the esophagus (Fig 5). Nonionic water-soluble contrast material is preferable in these patients, but a small amount of properly diluted barium can be used if necessary (2).

20. Red arrow points to end of orogastric tube which is blocked from entering the distal esophagus by the patient's esophageal atresia. Note the lack of gas in the abdomen indicating a fistulous tract does not connect the trachea to the distal esophagus