Chapter 173. Tracheoesophageal Fistula (TEF)

See Figure 173-1.

• Approximately 1 in 3,000 babies is born with tracheoesophageal fistula (TEF)
• Thirty to 40% neonates are premature
• Associated anomalies such as cardiac, gastrointestinal, genitourinary, musculoskeletal, or craniofacial anomalies are present in 30–50% of newborns with esophageal atresia and TEFs
  • VATER (i.e., vertebral and vascular anomalies, imperforate anus, TEF, radial aplasia, and renal abnormalities)
  • VACTERL (i.e., vertebral anomalies, imperforate anus, cardiac anomalies, TEF, renal abnormalities, radial limb aplasia) association
• Most common abnormality is blind upper esophageal pouch with distal fistulous tract between the trachea and distal esophagus (Type IIIB), observed in 90% of cases

• Prenatal diagnosis by ultrasound; associated with polyhydramnios (decreased fluid swallowing)
• Confirmed postnatally by failure to pass orogastric tube into newborn’s stomach
• Neonatal symptoms: coughing and choking with first feeding:
  • Recurrent pneumonias associated with feeding
• Radiographic confirmation: tip of radio-opaque catheter in esophagus:
  • Air in stomach if fistula present
• Occasionally TEF diagnosis not made until later in the child’s life
• Morbidity and mortality associated with pulmonary and cardiac complications. Ascertain position of the aorta to decide on side of thoracotomy
• Minimize risk of aspiration pneumonitis by placing neonate in semirecumbent position and inserting oroesophageal catheter to decrease accumulation of secretions
• All feeds are held as these patients are at high risk of aspiration. Patients are often started on a dextrose intravenous solution, TPN or PPN
• Antibiotics may be necessary for treatment of pneumonia
• Emergency gastrostomy under local anesthesia may be necessary to relieve gastric distension and improve ventilation before the definitive surgery 48–72 hours later
• The Waterson classification was used extensively in the past. However, patients currently are individually categorized based on clinical status
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  Waterson Classification for Neonates with TEF

  A	Birthweight over 2.5 kg and healthy	95% survival
  B	Birthweight between 1.8 and 2.5 kg and healthy, or weight >2.5 kg with moderate pneumonia or other congenital anomalies	68% survival
  C	Birthweight under 1.8 kg or weight >1.8 kg with severe pneumonia or severe congenital anomalies	6% survival

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  Spitz Classification for Neonates with TEF

  I	Birthweight greater than 1.5 kg and no congenital heart defect	99% survival
  II	Birthweight less than 1.5 kg or congenital heart defect	82% survival
  III	Birthweight less than 1.5 kg and presence of congenital heart defect	50% survival
• With improved ICU care, respiratory status has become a smaller factor in survival
• In general, whatever classification system is used, infants who have stable cardiac and respiratory status undergo expedition thoracotomy and repair. High-risk infants, especially those who are premature (<1,000 g) and have severe respiratory ailments or congestive ...