Esophageal Atresia and Tracheoesophageal Fistula

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Esophageal Atresia and Tracheoesophageal Fistula

• Pediatric Surgery Clinic

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Successive stages in the development of the
tracheoesophageal septum during embryologic
development.

• (A) The laryngotracheal diverticulum forms as a
  ventral outpouching from the caudal part of the
  primitive pharynx.
• (B) Longitudinal tracheoesophageal folds begin to
  fuse toward the midline to eventually form the
  tracheoesophageal septum.
• (C) The tracheoesophageal septum has completely
  formed.
• (D) If the tracheoesophageal septum deviates
  posteriorly, esophageal atresia with a
  tracheoesophageal fistula develops

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Esophageal atresia is a congenital abnormality in
which the midportion of the esophagus is absent.

• Incidence is between 1 in 3,570 and
• 1 in 4,500.

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Anatomic Variations

• 85
•  
• Most common
•  
• VOGTtype3(b)
•  
• GROSS type C
•  

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Anatomic Variations

• 6
• Atresia alone, 
• no fistula 
• Small stomach, 
• gasless abdomen 
• Usually has a long 
• gap between the 
• esophagealends 
• VOGT types 1 and 2 
• GROSS type A

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Anatomic Variations

• 2 
• Proximal tracheo- 
• esophageal fistula 
• No distal fistula 
• Small stomach, 
• gasless abdomen 
• Often has a long 
• gap between the 
• esophagealends 
• VOGT type 3(a) 
• GROSS type B 

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Anatomic Variations

• l
• Proximal and 
• distal fistulas 
• ("double fistula") 
• VOGT type 3(c) 
• GROSS type D 

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Anatomic Variations

• 6
• No atresia of
• the esophagus
•  Congenital
• tracheoesophageal 
• fistula
• "H" or "N" fistula 
• GROSS type E 

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Physiologic effects of distal tracheoesophageal
fistula

• 1. Hyaline membrane disease may necessitate
  higher ventilator pressures, which encourage air
  to pass through the distal fistula.
• 2. A distended abdomen elevates and "splints" the
  diaphragm.
• 3. Gastric distension may result in gastric
  rupture and pneumoperitoneum.
• 4. Passage of air through a distal
  tracheoesophageal fistula diminishes the
  effective tidal volume.
• . (B) 1. Aspiration of gastric juices leads to
  soiling of the lungs and pneumonia
• 2. Gastroesophageal reflux
• 3. Direction of gastric fluid proximally through
  distal fistula.
• 4. Overflow of secretions or inadvertent feeding
  may contribute to aspiration and contamination of
  the airway. .

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Associated Abnormalities

• Incidence of Associated Anomalies in Esophageal
  Atresia.
• Anomaly
  Frequency ()
• Congenital heart disease
  25
• Urinary tract
  22
• Orthopaedic (mostly vertebral and radial)
  15
• Gastrointestinal (e.g., duodenal
• atresia,imperforate anus)
  22
• Chromosomal (usually trisomy 18 or 21)
  7
• Total with one or more associated
  58
• anomalies

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Associated Congenital Anomalies Reported in
Patients with Esophageal Atresia
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DIAGNOSIS OF ESOPHAGEAL ATRESIA

• Antenatal Diagnosis (maternal polyhydramnios, a
  small stomach, a distended upper esophageal
  pouch, or abnormal swallowing)
• Diagnostic suspicion is increased when
  abnormalities known to be associated with
  esophageal atresia are identified.

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Fetal MRI

• This 32 week fetus had esophageal atresia and an
  absent stomach, resulting in marked
  polyhydramnios

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Clinical Diagnosis

• Prematurity
• Any excessively drooling (copious, fine, white,
  frothy bubbles of mucus in the mouth and,
  sometimes, the nose).

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Clinical Diagnosis

• . (A) Diagnosis of esophageal atresia is
  confirmed when a 10-gauge (French) catheter
  cannot be passed beyond 10 cm from the gums. (B)
  A smaller-caliber tube is not used because it may
  curl up in the upper esophageal segment, giving a
  false impression of esophageal continuity.

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The chest radiograph

• A plain radiograph will confirm the tube has not
  reached the stomach

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The Gasless Abdomen

• Absence of gas in the abdomen suggests that the
  patient has either atresia without a fistula or
  atresia with a proximal fistula only

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Contrast studies

• should be performed by an experienced pediatric
  radiologist, or after transfer to the tertiary
  institution, and with the use of a small amount
  (0.5 to 1 mL) of water-soluble contrast. Care
  must be taken to avoid aspiration.

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Management

• Measures should be taken to reduce the risk of
  aspiration(continuous suctioning of the upper
  pouch, the infant's head should be elevated).
• In infants with respiratory failure, endotracheal
  intubation should be performed.
• Transfer to a major tertiary pediatric
  institution is best not delayed .

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Summary of Preoperative Investigations

• A plain radiograph
• Renal ultrasonography and echocardiography are
  routine preoperative investigations
• Endoscopy or a careful midesophageal contrast
  study performed in a tertiary center. In some
  centers, bronchoscopy is performed routinely in
  all infants with esophageal atresia.

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Operative Repair of Esophageal Atresia

• Surgical repair is delayed (1-2days) in infants
  with low birth weight, pneumonia or other major
  anomalies.

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Operative Repair of Esophageal Atresia
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Operative Repair of Esophageal Atresia
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Tracheoesophageal fistula without atresia (type
E).

•  Respiratory difficulty after feedings in a
  3-day-old boy. Barium esophagogram clearly shows
  an H-shaped fistula between the trachea and the
  middle segment of the esophagus (arrowhead).
  Barium is filling the bronchi of the right lower
  lobe (arrows).

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Tracheoesophageal fistula without atresia (type
E).

• Esophagogram shows a fistula (arrow) arising from
  the anterior portion of the esophagus (e) and
  passing cephalad to the posterior portion of the
  trachea (t).

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Tracheoesophageal fistula without atresia (type
E).

• Endoscopic diagnosis

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Congenital tracheoesophageal  fistula
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The end