Tracheoesophageal Fistula

Tracheoesophageal Fistula
Michael A. Lopez
  • Esophageal atresia (EA) and tracheoesophageal fistula (TEF) are congenital malformations that occur approximately in 1:3500 live-born infants.1
  • It is defined as an interruption in the continuity of the esophagus.
  • TEFs may or may not be present in patients with EA.
  • This abnormality is usually due to improper separation of the trachea and esophagus during the 4th week of gestation when the primitive foregut is developed.1
  • The first successful primary repair of EA was accomplished in 1941 by Cameron Haight.
  • The high mortality rates associated with EA and TEFs have decreased, thanks to early diagnosis, preoperative management/preparation, and clever surgical techniques.
EPIDEMIOLOGY AND ETIOLOGY
  • Most cases are sporadic.
  • 35% to 60% of infants with EA will also have other congenital abnormalities.2
  • ˜25% of patients trisomy 18 will be born with EA/TEF.2
  • Once the diagnosis is made, a thorough physical examination must be performed to determine if any other abnormalities are present.
  • Approximately 10% to 20% occur with VACTERL or CHARGE (although not criteria for diagnosis).9
  • Associated abnormalities:2
    • Cardiovascular (ventricular septal defect, atrial septal defect, tetralogy of fallot) → 20% to 30%
    • Gastrointestinal (GI) (imperforate anus, duodenal atresia, Meckel diverticulum) → 15% to 25%
    • Genitourinary → 10% to 20%
    • Musculoskeletal → 10% to 15%
    • Craniofacial/Central nervous system → 5% to 10%
    • Chromosomal abnormalities → 3% to 5%
CLINICAL PRESENTATION
  • EA may be suspected when absence of gastric bubble, polyhydramnios (earliest symptom, found during second half of pregnancy), and distension of upper esophagus are seen on prenatal ultrasound.
  • Fetal MRI can confirm diagnosis of atresia or TEF when suspected.5
  • After birth, the child may present with excessive salivation with choking and cyanotic spells.
DIAGNOSIS
  • If clinical presentation suggests EA, the diagnosis can be made inserting 10- or 12-F Replogle into the esophagus.
  • Inability to advance tube further than 10 cm is highly suggestive of EA.
  • The tube can then be left in place to aspirate secretions, preventing aspiration.
  • Once the tube is in place, AP and lateral chest radiographs are performed while injecting a small amount of air into the tube.
  • This will provide the exact location of the atretic esophagus and demonstrate whether or not a TEF is present (Figure 32.1).
  • If abdominal gas is seen, it is very likely that a distal or H-type TEF is present.6
  • No air in the GI tract suggests pure atresia.
  • Injecting barium into the Replogle tube can be performed to confirm diagnosis but is rarely needed.8
  • The most commonly used systems to classify this disease process are described by Gross (Figure 32.2).
  • The most frequent type of EA is the blind-ending esophageal pouch with a fistula between the trachea and lower esophagus, classified as C (Gross).
  • Approximately 85% of patients diagnosed with EA will fall under this classification.7
  • Type A, pure atresia with TEF, is seen in approximately 7% of cases.
  • Type B, EA with a TEF between the trachea and upper esophageal pouch, is seen in approximately 2% of cases.
  • Type D, where both upper and lower esophageal segments communicate with the trachea, is seen in approximately 3% of cases.
  • Type E, also known as H-type TEF, is a different entity because no EA is present (Figure 32.3).
Figure 32.1 Esophageal atresia with distal tracheoesophageal fistula. A, Frontal radiograph of the chest and abdomen demonstrates a nasogastric tube within the upper esophageal pouch (arrow). The air in the bowel indicates a distal tracheoesophageal communication. B, Lateral view in another patient shows a dilated upper esophageal pouch (arrows) containing a nasogastric tube. (Reprinted with permission from Siegel MJ, Coley BD. Core Curriculum: Pediatric Imaging. Philadelphia, PA: Lippincott Williams & Wilkins; 2005.)
MEDICAL AND SURGICAL MANAGEMENT
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May 5, 2019 | Posted by in PEDIATRICS | Comments Off on Tracheoesophageal Fistula

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